Idiopathic Interstitial Pneumonias
Lung Diseases, Interstitial
Idiopathic Pulmonary Fibrosis
Cryptogenic Organizing Pneumonia
Pulmonary Fibrosis
Connective Tissue Diseases
Lung
Bronchoalveolar Lavage
Interleukin-13 Receptor alpha1 Subunit
Biopsy
Tomography, X-Ray Computed
Bronchoalveolar Lavage Fluid
Respiratory Function Tests
Pulmonary Surfactant-Associated Protein C
Prognosis
Retrospective Studies
Radiography, Thoracic
Pulmonary Surfactant-Associated Protein A
Collagen Diseases
Pneumonia, Pneumocystis
Dermatomyositis
MedlinePlus
Bleomycin
Idiopathic interstitial pneumonias: progress in classification, diagnosis, pathogenesis and management. (1/43)
The idiopathic interstitial pneumonias are a heterogeneous group of poorly understood diseases with often devastating consequences for those afflicted. Subclassification of the idiopathic interstitial pneumonia based on clinical-radiological-pathological criteria has highlighted important pathogenic, therapeutic and prognostic implications. The most critical distinction is the presence of usual interstitial pneumonia, the histopathological pattern seen in idiopathic pulmonary fibrosis. Idiopathic pulmonary fibrosis has a worse response to therapy and prognosis. New insight into the pathophysiology of usual interstitial pneumonia suggests a distinctly fibroproliferative process, and antifibrotic therapies show promise. While the clinical and radiographic diagnosis of idiopathic interstitial pneumonias can be made confidently in some cases, many patients require surgical lung biopsy to determine their underlying histopathology. A structured, clinical-radiological-pathological approach to the diagnosis of the idiopathic interstitial pneumonias, with particular attention to the identification of idiopathic pulmonary fibrosis, insures proper therapy, enhances prognostication, and allows for further investigation of therapies aimed at distinct pathophysiology. (+info)High serum levels of thrombospondin-1 in patients with idiopathic interstitial pneumonia. (2/43)
(+info)Clinical course and lung function change of idiopathic nonspecific interstitial pneumonia. (3/43)
(+info)Serum albumin concentration and waiting list mortality in idiopathic interstitial pneumonia. (4/43)
(+info)Exacerbation of idiopathic interstitial pneumonias associated with lung cancer therapy. (5/43)
OBJECTIVE AND METHODS: Idiopathic interstitial pneumonias (IIPs) frequently occur in association with lung cancer. However, there is no consensus on the best treatment of acute exacerbation of IIP in lung cancer patients (LC with IIP), including those with iatrogenic acute lung injury resulting from cancer treatments. We aimed to identify an appropriate strategy for treatment of this condition. We analyzed clinical features of 120 LC with IIP, retrospectively. RESULTS: The incidence of acute exacerbation related to anticancer treatment was 22.7%; when the incidence was examined separately for patients receiving chemotherapy or the best supportive care, the incidence was 20.0% and 31.3%, respectively. Additional investigations should be directed to finding suitable regimens for treatment of LC with IIP and the selection of appropriate patients with LC with IIP for chemotherapy. The incidence of acute exacerbation caused by combination regimens of carboplatin + paclitaxel or a platinum agent + etoposide was significantly lower than that of other regimens (0% vs. 18%, respectively; p=0.025, Fisher's Exact Test). Patients with high levels of C-reactive protein before chemotherapy had a significantly higher risk of developing acute exacerbation (odds ratio 5.60, p=0.028). CONCLUSION: There was no evidence that anticancer treatment, including chemotherapy, should be avoided in LC with IIP. To establish an appropriate cancer treatment for LC with IIP, a prospective clinical study should be performed to evaluate various treatment modalities in a larger patient population. (+info)Desquamative interstitial pneumonia (DIP) in a patient with rheumatoid arthritis: is DIP associated with autoimmune disorders? (6/43)
Desquamative interstitial pneumonia (DIP) is a rare pattern of diffuse parenchymal lung disease known as one of the idiopathic interstitial pneumonias and is considered to be a smoking- or dust inhalation-related interstitial pneumonia in the majority of cases. This report presents the first case of DIP in which the pulmonary manifestation preceded the onset of rheumatoid arthritis. This case and our review of twenty-four DIP cases (nineteen cases previously-reported from Japan, plus five cases in our departments) indicate the possibility that the DIP pattern is an additional form of diffuse interstitial pneumonia that may develop in association with autoimmune diseases. (+info)Recurrent lung cancer in the mediastinum noticed after a living-donor lobar lung transplantation. (7/43)
We describe a case of lung cancer in a living-donor lobar lung transplantation (LDLLT) recipient that was identified because of a recurrence in the mediastinum. The patient was a 55-year-old woman who had undergone bilateral LDLLT for nonspecific interstitial pneumonia. She developed dyspnea upon exertion at 15 months after transplantation and was diagnosed as suffering from chronic rejection. A computed tomography scan also revealed enlarged mediastinal lymph nodes (LNs) that were subsequently confirmed as poorly differentiated squamous cell carcinomas. Retrospectively, a small tumor was found in the explanted right lung tissue, the microscopic findings of which were similar to those of the mediastinal lesion. A whole body examination revealed no other lesions; thus we resected the LNs and subsequently irradiated the mediastinum. Recurrent disease appeared in her transplanted lungs 10 months after resection of the LNs, and she died of pneumonia with chronic rejection 2 years and 7 months after transplantation. (+info)Anti-synthetase syndrome in ANA and anti-Jo-1 negative patients presenting with idiopathic interstitial pneumonia. (8/43)
(+info)Idiopathic interstitial pneumonias (IIPs) are a group of rare lung diseases with no known cause, characterized by inflammation and scarring (fibrosis) of the lung tissue. The term "idiopathic" means that the cause is unknown, and "interstitial" refers to the spaces between the air sacs in the lungs where the inflammation and scarring occur.
IIPs are classified into several subtypes based on their clinical, radiological, and pathological features. These include:
1. Idiopathic Pulmonary Fibrosis (IPF): This is the most common and aggressive form of IIP, characterized by progressive scarring of the lung tissue, which leads to difficulty breathing and decreased lung function over time.
2. Nonspecific Interstitial Pneumonia (NSIP): This subtype is characterized by varying degrees of inflammation and fibrosis in the lung tissue. NSIP can be idiopathic or associated with connective tissue diseases.
3. Respiratory Bronchiolitis-Interstitial Lung Disease (RB-ILD): This subtype primarily affects smokers and is characterized by inflammation of the small airways and surrounding lung tissue.
4. Desquamative Interstitial Pneumonia (DIP): This subtype is also more common in smokers and is characterized by accumulation of pigmented macrophages in the lung tissue.
5. Cryptogenic Organizing Pneumonia (COP): This subtype is characterized by the formation of fibrous masses in the small airways and alveoli, leading to cough and shortness of breath.
6. Acute Interstitial Pneumonia (AIP)/Acute Respiratory Distress Syndrome (ARDS): This subtype is a severe form of IIP that can rapidly progress to respiratory failure and requires immediate medical attention.
The diagnosis of IIPs typically involves a combination of clinical evaluation, imaging studies, and lung biopsy. Treatment options may include corticosteroids, immunosuppressive medications, and oxygen therapy, depending on the severity and subtype of the disease.
Interstitial lung diseases (ILDs) are a group of disorders characterized by inflammation and scarring (fibrosis) in the interstitium, the tissue and space around the air sacs (alveoli) of the lungs. The interstitium is where the blood vessels that deliver oxygen to the lungs are located. ILDs can be caused by a variety of factors, including environmental exposures, medications, connective tissue diseases, and autoimmune disorders.
The scarring and inflammation in ILDs can make it difficult for the lungs to expand and contract normally, leading to symptoms such as shortness of breath, cough, and fatigue. The scarring can also make it harder for oxygen to move from the air sacs into the bloodstream.
There are many different types of ILDs, including:
* Idiopathic pulmonary fibrosis (IPF): a type of ILD that is caused by unknown factors and tends to progress rapidly
* Hypersensitivity pneumonitis: an ILD that is caused by an allergic reaction to inhaled substances, such as mold or bird droppings
* Connective tissue diseases: ILDs can be a complication of conditions such as rheumatoid arthritis and scleroderma
* Sarcoidosis: an inflammatory disorder that can affect multiple organs, including the lungs
* Asbestosis: an ILD caused by exposure to asbestos fibers
Treatment for ILDs depends on the specific type of disease and its underlying cause. Some treatments may include corticosteroids, immunosuppressive medications, and oxygen therapy. In some cases, a lung transplant may be necessary.
Idiopathic Pulmonary Fibrosis (IPF) is a specific type of chronic, progressive, and irreversible fibrotic lung disease of unknown cause, characterized by scarring (fibrosis) in the lungs that thickens and stiffens the lining of the air sacs (alveoli). This makes it increasingly difficult for the lungs to transfer oxygen into the bloodstream, leading to shortness of breath, cough, decreased exercise tolerance, and, eventually, respiratory failure.
The term "idiopathic" means that the cause of the disease is unknown. The diagnosis of IPF requires a combination of clinical, radiological, and pathological findings, excluding other known causes of pulmonary fibrosis. It primarily affects middle-aged to older adults, with a higher prevalence in men than women.
The progression of IPF varies from person to person, but the prognosis is generally poor, with a median survival time of 3-5 years after diagnosis. Currently, there are two FDA-approved medications for the treatment of IPF (nintedanib and pirfenidone), which can help slow down disease progression but do not cure the condition. Lung transplantation remains an option for select patients with advanced IPF.
Cryptogenic organizing pneumonia (COP) is a type of lung disorder that is characterized by the presence of inflammation and scarring in the lungs. The term "cryptogenic" means that the cause of the condition is unknown or unclear.
Organizing pneumonia is a specific pattern of injury to the lungs that can be caused by various factors, including infections, medications, and autoimmune disorders. However, in cases of COP, there is no clear underlying cause that can be identified.
The main symptoms of COP include cough, shortness of breath, fever, and fatigue. The condition can also cause crackles or wheezing sounds when listening to the lungs with a stethoscope. Diagnosis of COP typically involves a combination of imaging studies, such as chest X-rays or CT scans, and lung biopsy.
Treatment for COP usually involves the use of corticosteroids, which can help to reduce inflammation and improve symptoms. In some cases, other medications may also be used to manage the condition. The prognosis for people with COP is generally good, with most individuals responding well to treatment and experiencing improvement in their symptoms over time. However, recurrence of the condition is possible, and long-term monitoring may be necessary.
Pulmonary fibrosis is a specific type of lung disease that results from the thickening and scarring of the lung tissues, particularly those in the alveoli (air sacs) and interstitium (the space around the air sacs). This scarring makes it harder for the lungs to properly expand and transfer oxygen into the bloodstream, leading to symptoms such as shortness of breath, coughing, fatigue, and eventually respiratory failure. The exact cause of pulmonary fibrosis can vary, with some cases being idiopathic (without a known cause) or related to environmental factors, medications, medical conditions, or genetic predisposition.
Connective tissue diseases (CTDs) are a group of disorders that involve the abnormal production and accumulation of abnormal connective tissues in various parts of the body. Connective tissues are the structural materials that support and bind other tissues and organs together. They include tendons, ligaments, cartilage, fat, and the material that fills the spaces between cells, called the extracellular matrix.
Connective tissue diseases can affect many different systems in the body, including the skin, joints, muscles, lungs, kidneys, gastrointestinal tract, and blood vessels. Some CTDs are autoimmune disorders, meaning that the immune system mistakenly attacks healthy connective tissues. Others may be caused by genetic mutations or environmental factors.
Some examples of connective tissue diseases include:
* Systemic lupus erythematosus (SLE)
* Rheumatoid arthritis (RA)
* Scleroderma
* Dermatomyositis/Polymyositis
* Mixed Connective Tissue Disease (MCTD)
* Sjogren's syndrome
* Ehlers-Danlos syndrome
* Marfan syndrome
* Osteogenesis imperfecta
The specific symptoms and treatment of connective tissue diseases vary depending on the type and severity of the condition. Treatment may include medications to reduce inflammation, suppress the immune system, or manage pain. In some cases, surgery may be necessary to repair or replace damaged tissues or organs.
A lung is a pair of spongy, elastic organs in the chest that work together to enable breathing. They are responsible for taking in oxygen and expelling carbon dioxide through the process of respiration. The left lung has two lobes, while the right lung has three lobes. The lungs are protected by the ribcage and are covered by a double-layered membrane called the pleura. The trachea divides into two bronchi, which further divide into smaller bronchioles, leading to millions of tiny air sacs called alveoli, where the exchange of gases occurs.
Bronchoalveolar lavage (BAL) is a medical procedure in which a small amount of fluid is introduced into a segment of the lung and then gently suctioned back out. The fluid contains cells and other materials that can be analyzed to help diagnose various lung conditions, such as inflammation, infection, or cancer.
The procedure is typically performed during bronchoscopy, which involves inserting a thin, flexible tube with a light and camera on the end through the nose or mouth and into the lungs. Once the bronchoscope is in place, a small catheter is passed through the bronchoscope and into the desired lung segment. The fluid is then introduced and suctioned back out, and the sample is sent to a laboratory for analysis.
BAL can be helpful in diagnosing various conditions such as pneumonia, interstitial lung diseases, alveolar proteinosis, and some types of cancer. It can also be used to monitor the effectiveness of treatment for certain lung conditions. However, like any medical procedure, it carries some risks, including bleeding, infection, and respiratory distress. Therefore, it is important that the procedure is performed by a qualified healthcare professional in a controlled setting.
Interleukin-13 receptor alpha1 subunit (IL-13Rα1) is a protein that forms part of a type II cytokine receptor complex. This receptor complex binds the cytokine IL-13, which is involved in the regulation of immune and inflammatory responses. The IL-13Rα1 subunit combines with the IL-4 receptor alpha chain (IL-4Rα) to form the type II IL-13 receptor, which is expressed on a variety of cell types including epithelial cells, endothelial cells, and immune cells. The binding of IL-13 to this receptor complex triggers intracellular signaling pathways that lead to various biological responses, such as the regulation of inflammation, immunity, and tissue remodeling.
Defects in the gene encoding IL-13Rα1 have been associated with some immune-related diseases, including asthma and allergies. Additionally, IL-13Rα1 has been identified as a potential therapeutic target for the treatment of these conditions, due to its role in mediating the effects of IL-13 in the body.
A biopsy is a medical procedure in which a small sample of tissue is taken from the body to be examined under a microscope for the presence of disease. This can help doctors diagnose and monitor various medical conditions, such as cancer, infections, or autoimmune disorders. The type of biopsy performed will depend on the location and nature of the suspected condition. Some common types of biopsies include:
1. Incisional biopsy: In this procedure, a surgeon removes a piece of tissue from an abnormal area using a scalpel or other surgical instrument. This type of biopsy is often used when the lesion is too large to be removed entirely during the initial biopsy.
2. Excisional biopsy: An excisional biopsy involves removing the entire abnormal area, along with a margin of healthy tissue surrounding it. This technique is typically employed for smaller lesions or when cancer is suspected.
3. Needle biopsy: A needle biopsy uses a thin, hollow needle to extract cells or fluid from the body. There are two main types of needle biopsies: fine-needle aspiration (FNA) and core needle biopsy. FNA extracts loose cells, while a core needle biopsy removes a small piece of tissue.
4. Punch biopsy: In a punch biopsy, a round, sharp tool is used to remove a small cylindrical sample of skin tissue. This type of biopsy is often used for evaluating rashes or other skin abnormalities.
5. Shave biopsy: During a shave biopsy, a thin slice of tissue is removed from the surface of the skin using a sharp razor-like instrument. This technique is typically used for superficial lesions or growths on the skin.
After the biopsy sample has been collected, it is sent to a laboratory where a pathologist will examine the tissue under a microscope and provide a diagnosis based on their findings. The results of the biopsy can help guide further treatment decisions and determine the best course of action for managing the patient's condition.
X-ray computed tomography (CT or CAT scan) is a medical imaging method that uses computer-processed combinations of many X-ray images taken from different angles to produce cross-sectional (tomographic) images (virtual "slices") of the body. These cross-sectional images can then be used to display detailed internal views of organs, bones, and soft tissues in the body.
The term "computed tomography" is used instead of "CT scan" or "CAT scan" because the machines take a series of X-ray measurements from different angles around the body and then use a computer to process these data to create detailed images of internal structures within the body.
CT scanning is a noninvasive, painless medical test that helps physicians diagnose and treat medical conditions. CT imaging provides detailed information about many types of tissue including lung, bone, soft tissue and blood vessels. CT examinations can be performed on every part of the body for a variety of reasons including diagnosis, surgical planning, and monitoring of therapeutic responses.
In computed tomography (CT), an X-ray source and detector rotate around the patient, measuring the X-ray attenuation at many different angles. A computer uses this data to construct a cross-sectional image by the process of reconstruction. This technique is called "tomography". The term "computed" refers to the use of a computer to reconstruct the images.
CT has become an important tool in medical imaging and diagnosis, allowing radiologists and other physicians to view detailed internal images of the body. It can help identify many different medical conditions including cancer, heart disease, lung nodules, liver tumors, and internal injuries from trauma. CT is also commonly used for guiding biopsies and other minimally invasive procedures.
In summary, X-ray computed tomography (CT or CAT scan) is a medical imaging technique that uses computer-processed combinations of many X-ray images taken from different angles to produce cross-sectional images of the body. It provides detailed internal views of organs, bones, and soft tissues in the body, allowing physicians to diagnose and treat medical conditions.
Pneumonia is an infection or inflammation of the alveoli (tiny air sacs) in one or both lungs. It's often caused by bacteria, viruses, or fungi. Accumulated pus and fluid in these air sacs make it difficult to breathe, which can lead to coughing, chest pain, fever, and difficulty breathing. The severity of symptoms can vary from mild to life-threatening, depending on the underlying cause, the patient's overall health, and age. Pneumonia is typically diagnosed through a combination of physical examination, medical history, and diagnostic tests such as chest X-rays or blood tests. Treatment usually involves antibiotics for bacterial pneumonia, antivirals for viral pneumonia, and supportive care like oxygen therapy, hydration, and rest.
Bronchoalveolar lavage (BAL) fluid is a type of clinical specimen obtained through a procedure called bronchoalveolar lavage. This procedure involves inserting a bronchoscope into the lungs and instilling a small amount of saline solution into a specific area of the lung, then gently aspirating the fluid back out. The fluid that is recovered is called bronchoalveolar lavage fluid.
BAL fluid contains cells and other substances that are present in the lower respiratory tract, including the alveoli (the tiny air sacs where gas exchange occurs). By analyzing BAL fluid, doctors can diagnose various lung conditions, such as pneumonia, interstitial lung disease, and lung cancer. They can also monitor the effectiveness of treatments for these conditions by comparing the composition of BAL fluid before and after treatment.
BAL fluid is typically analyzed for its cellular content, including the number and type of white blood cells present, as well as for the presence of bacteria, viruses, or other microorganisms. The fluid may also be tested for various proteins, enzymes, and other biomarkers that can provide additional information about lung health and disease.
Respiratory Function Tests (RFTs) are a group of medical tests that measure how well your lungs take in and exhale air, and how well they transfer oxygen and carbon dioxide into and out of your blood. They can help diagnose certain lung disorders, measure the severity of lung disease, and monitor response to treatment.
RFTs include several types of tests, such as:
1. Spirometry: This test measures how much air you can exhale and how quickly you can do it. It's often used to diagnose and monitor conditions like asthma, chronic obstructive pulmonary disease (COPD), and other lung diseases.
2. Lung volume testing: This test measures the total amount of air in your lungs. It can help diagnose restrictive lung diseases, such as pulmonary fibrosis or sarcoidosis.
3. Diffusion capacity testing: This test measures how well oxygen moves from your lungs into your bloodstream. It's often used to diagnose and monitor conditions like pulmonary fibrosis, interstitial lung disease, and other lung diseases that affect the ability of the lungs to transfer oxygen to the blood.
4. Bronchoprovocation testing: This test involves inhaling a substance that can cause your airways to narrow, such as methacholine or histamine. It's often used to diagnose and monitor asthma.
5. Exercise stress testing: This test measures how well your lungs and heart work together during exercise. It's often used to diagnose lung or heart disease.
Overall, Respiratory Function Tests are an important tool for diagnosing and managing a wide range of lung conditions.
Pulmonary surfactant-associated protein C (SP-C) is a small hydrophobic protein that is a component of pulmonary surfactant. Surfactant is a complex mixture of lipids and proteins that reduces surface tension in the alveoli of the lungs, preventing collapse during expiration and facilitating lung expansion during inspiration. SP-C plays a crucial role in maintaining the structural integrity and stability of the surfactant film at the air-liquid interface of the alveoli.
Deficiency or dysfunction of SP-C has been associated with several pulmonary diseases, including respiratory distress syndrome (RDS) in premature infants, interstitial lung diseases (ILDs), and pulmonary fibrosis. Mutations in the gene encoding SP-C (SFTPC) can lead to abnormal protein processing and accumulation, resulting in lung injury and inflammation, ultimately contributing to the development of these conditions.
Prognosis is a medical term that refers to the prediction of the likely outcome or course of a disease, including the chances of recovery or recurrence, based on the patient's symptoms, medical history, physical examination, and diagnostic tests. It is an important aspect of clinical decision-making and patient communication, as it helps doctors and patients make informed decisions about treatment options, set realistic expectations, and plan for future care.
Prognosis can be expressed in various ways, such as percentages, categories (e.g., good, fair, poor), or survival rates, depending on the nature of the disease and the available evidence. However, it is important to note that prognosis is not an exact science and may vary depending on individual factors, such as age, overall health status, and response to treatment. Therefore, it should be used as a guide rather than a definitive forecast.
Retrospective studies, also known as retrospective research or looking back studies, are a type of observational study that examines data from the past to draw conclusions about possible causal relationships between risk factors and outcomes. In these studies, researchers analyze existing records, medical charts, or previously collected data to test a hypothesis or answer a specific research question.
Retrospective studies can be useful for generating hypotheses and identifying trends, but they have limitations compared to prospective studies, which follow participants forward in time from exposure to outcome. Retrospective studies are subject to biases such as recall bias, selection bias, and information bias, which can affect the validity of the results. Therefore, retrospective studies should be interpreted with caution and used primarily to generate hypotheses for further testing in prospective studies.
Thoracic radiography is a type of diagnostic imaging that involves using X-rays to produce images of the chest, including the lungs, heart, bronchi, great vessels, and the bones of the spine and chest wall. It is a commonly used tool in the diagnosis and management of various respiratory, cardiovascular, and thoracic disorders such as pneumonia, lung cancer, heart failure, and rib fractures.
During the procedure, the patient is positioned between an X-ray machine and a cassette containing a film or digital detector. The X-ray beam is directed at the chest, and the resulting image is captured on the film or detector. The images produced can help identify any abnormalities in the structure or function of the organs within the chest.
Thoracic radiography may be performed as a routine screening test for certain conditions, such as lung cancer, or it may be ordered when a patient presents with symptoms suggestive of a respiratory or cardiovascular disorder. It is a safe and non-invasive procedure that can provide valuable information to help guide clinical decision making and improve patient outcomes.
Bacterial pneumonia is a type of lung infection that's caused by bacteria. It can affect people of any age, but it's more common in older adults, young children, and people with certain health conditions or weakened immune systems. The symptoms of bacterial pneumonia can vary, but they often include cough, chest pain, fever, chills, and difficulty breathing.
The most common type of bacteria that causes pneumonia is Streptococcus pneumoniae (pneumococcus). Other types of bacteria that can cause pneumonia include Haemophilus influenzae, Staphylococcus aureus, and Mycoplasma pneumoniae.
Bacterial pneumonia is usually treated with antibiotics, which are medications that kill bacteria. The specific type of antibiotic used will depend on the type of bacteria causing the infection. It's important to take all of the prescribed medication as directed, even if you start feeling better, to ensure that the infection is completely cleared and to prevent the development of antibiotic resistance.
In severe cases of bacterial pneumonia, hospitalization may be necessary for close monitoring and treatment with intravenous antibiotics and other supportive care.
Viral pneumonia is a type of pneumonia caused by viral infection. It primarily affects the upper and lower respiratory tract, leading to inflammation of the alveoli (air sacs) in the lungs. This results in symptoms such as cough, difficulty breathing, fever, fatigue, and chest pain. Common viruses that can cause pneumonia include influenza virus, respiratory syncytial virus (RSV), and adenovirus. Viral pneumonia is often milder than bacterial pneumonia but can still be serious, especially in young children, older adults, and people with weakened immune systems. Treatment typically involves supportive care, such as rest, hydration, and fever reduction, while the body fights off the virus. In some cases, antiviral medications may be used to help manage symptoms and prevent complications.
Pulmonary Surfactant-Associated Protein A (SP-A) is a protein that is a major component of pulmonary surfactant, which is a complex mixture of lipids and proteins found in the alveoli of the lungs. SP-A is produced by specialized cells called type II alveolar epithelial cells and has several important functions in the lung.
SP-A plays a role in innate immunity by binding to pathogens, such as bacteria and viruses, and facilitating their clearance from the lungs. It also helps to regulate surfactant homeostasis by participating in the reuptake and recycling of surfactant components. Additionally, SP-A has been shown to have anti-inflammatory effects and may help to modulate the immune response in the lung.
Deficiencies or mutations in SP-A have been associated with various respiratory diseases, including acute respiratory distress syndrome (ARDS), pulmonary fibrosis, and chronic obstructive pulmonary disease (COPD).
Collagen diseases, also known as collagen disorders or connective tissue diseases, refer to a group of medical conditions that affect the body's connective tissues. These tissues provide support and structure for various organs and systems in the body, including the skin, joints, muscles, and blood vessels.
Collagen is a major component of connective tissues, and it plays a crucial role in maintaining their strength and elasticity. In collagen diseases, the body's immune system mistakenly attacks healthy collagen, leading to inflammation, pain, and damage to the affected tissues.
There are several types of collagen diseases, including:
1. Systemic Lupus Erythematosus (SLE): This is a chronic autoimmune disease that can affect various organs and systems in the body, including the skin, joints, kidneys, heart, and lungs.
2. Rheumatoid Arthritis (RA): This is a chronic inflammatory disease that primarily affects the joints, causing pain, swelling, and stiffness.
3. Scleroderma: This is a rare autoimmune disorder that causes thickening and hardening of the skin and connective tissues, leading to restricted movement and organ damage.
4. Dermatomyositis: This is an inflammatory muscle disease that can also affect the skin, causing rashes and weakness.
5. Mixed Connective Tissue Disease (MCTD): This is a rare autoimmune disorder that combines symptoms of several collagen diseases, including SLE, RA, scleroderma, and dermatomyositis.
The exact cause of collagen diseases is not fully understood, but they are believed to be related to genetic, environmental, and hormonal factors. Treatment typically involves a combination of medications, lifestyle changes, and physical therapy to manage symptoms and prevent complications.
Pneumonia, pneumococcal is a type of pneumonia caused by the bacterium Streptococcus pneumoniae (also known as pneumococcus). This bacteria can colonize the upper respiratory tract and occasionally invade the lower respiratory tract, causing infection.
Pneumococcal pneumonia can affect people of any age but is most common in young children, older adults, and those with weakened immune systems. The symptoms of pneumococcal pneumonia include fever, chills, cough, chest pain, shortness of breath, and rapid breathing. In severe cases, it can lead to complications such as bacteremia (bacterial infection in the blood), meningitis (inflammation of the membranes surrounding the brain and spinal cord), and respiratory failure.
Pneumococcal pneumonia can be prevented through vaccination with the pneumococcal conjugate vaccine (PCV) or the pneumococcal polysaccharide vaccine (PPSV). These vaccines protect against the most common strains of Streptococcus pneumoniae that cause invasive disease. It is also important to practice good hygiene, such as covering the mouth and nose when coughing or sneezing, and washing hands frequently, to prevent the spread of pneumococcal bacteria.
"Pneumonia, Pneumocystis" is more commonly referred to as "Pneumocystis pneumonia (PCP)." It is a type of pneumonia caused by the microorganism Pneumocystis jirovecii. This organism was previously classified as a protozoan but is now considered a fungus.
PCP is an opportunistic infection, which means that it mainly affects people with weakened immune systems, such as those with HIV/AIDS, cancer, transplant recipients, or people taking immunosuppressive medications. The symptoms of PCP can include cough, shortness of breath, fever, and difficulty exercising. It is a serious infection that requires prompt medical treatment, typically with antibiotics.
It's important to note that PCP is not the same as pneumococcal pneumonia, which is caused by the bacterium Streptococcus pneumoniae. While both conditions are types of pneumonia, they are caused by different organisms and require different treatments.
Dermatomyositis is a medical condition characterized by inflammation and weakness in the muscles and skin. It is a type of inflammatory myopathy, which means that it causes muscle inflammation and damage. Dermatomyositis is often associated with a distinctive rash that affects the skin around the eyes, nose, mouth, fingers, and toes.
The symptoms of dermatomyositis can include:
* Progressive muscle weakness, particularly in the hips, thighs, shoulders, and neck
* Fatigue
* Difficulty swallowing or speaking
* Skin rash, which may be pink or purple and is often accompanied by itching
* Muscle pain and tenderness
* Joint pain and swelling
* Raynaud's phenomenon, a condition that affects blood flow to the fingers and toes
The exact cause of dermatomyositis is not known, but it is believed to be related to an autoimmune response in which the body's immune system mistakenly attacks healthy tissue. Treatment for dermatomyositis typically involves medications to reduce inflammation and suppress the immune system, as well as physical therapy to help maintain muscle strength and function.
MedlinePlus is not a medical term, but rather a consumer health website that provides high-quality, accurate, and reliable health information, written in easy-to-understand language. It is produced by the U.S. National Library of Medicine, the world's largest medical library, and is widely recognized as a trusted source of health information.
MedlinePlus offers information on various health topics, including conditions, diseases, tests, treatments, and wellness. It also provides access to drug information, medical dictionary, and encyclopedia, as well as links to clinical trials, medical news, and patient organizations. The website is available in both English and Spanish and can be accessed for free.
Bleomycin is a type of chemotherapeutic agent used to treat various types of cancer, including squamous cell carcinoma, testicular cancer, and lymphomas. It works by causing DNA damage in rapidly dividing cells, which can inhibit the growth and proliferation of cancer cells.
Bleomycin is an antibiotic derived from Streptomyces verticillus and is often administered intravenously or intramuscularly. While it can be effective in treating certain types of cancer, it can also have serious side effects, including lung toxicity, which can lead to pulmonary fibrosis and respiratory failure. Therefore, bleomycin should only be used under the close supervision of a healthcare professional who is experienced in administering chemotherapy drugs.
Fibrosis is a pathological process characterized by the excessive accumulation and/or altered deposition of extracellular matrix components, particularly collagen, in various tissues and organs. This results in the formation of fibrous scar tissue that can impair organ function and structure. Fibrosis can occur as a result of chronic inflammation, tissue injury, or abnormal repair mechanisms, and it is a common feature of many diseases, including liver cirrhosis, lung fibrosis, heart failure, and kidney disease.
In medical terms, fibrosis is defined as:
"The process of producing scar tissue (consisting of collagen) in response to injury or chronic inflammation in normal connective tissue. This can lead to the thickening and stiffening of affected tissues and organs, impairing their function."
Idiopathic interstitial pneumonia
Non-specific interstitial pneumonia
Usual interstitial pneumonia
Desquamative interstitial pneumonia
Cryptogenic organizing pneumonia
Averill A. Liebow
Idiopathic pulmonary fibrosis
MMP2
Pneumonia
Interstitial lung disease
Riociguat
Respiratory bronchiolitis
Ground-glass opacity
Lymphoproliferative disorders
Paul Eston Lacy
High-resolution computed tomography
Richard B. Lanman
Alveolar lung disease
Fever of unknown origin
Dip
Acute interstitial pneumonitis
Restrictive lung disease
Diffuse alveolar damage
List of diseases (I)
IIP
Crazy paving (medicine)
Corticosteroid
LRBA deficiency
List of syndromes
Respiratory disease
Idiopathic interstitial pneumonia - Wikipedia
Classification and natural history of the idiopathic interstitial pneumonias
Idiopathic Interstitial Pneumonia
Idiopathic nonspecific interstitial pneumonia: lung manifestation of undifferentiated connective tissue disease?
Overview of Idiopathic Interstitial Pneumonias - Pulmonary Disorders - Merck Manuals Professional Edition
SSc-related Interstitial Lung Disease Not Linked to Idiopathic Interstitial Pneumonia
2012 ICD-9-CM Diagnosis Code 516.30 : Idiopathic interstitial pneumonia, not otherwise specified
Overview of Idiopathic Interstitial Pneumonias - Pulmonary Disorders - MSD Manual Professional Edition
Definition of Idiopathic Interstitial Pneumonia in English - Acronym Monster
Idiopathic Pulmonary Fibrosis, IPF, Nonspecific Interstitial Pneumonia, NSIP | Bioqrâ„¢ Testimonial
AMO-19-006 Molecular diagnosis of idiopathic interstitial pneumonias: a prospective study Marco Mura - IFPOC
Acute and subacute idiopathic interstitial pneumonias. | Hospital Medicine Virtual Journal Club | Washington University in St....
Idiopathic Pulmonary Fibrosis (IPF) Clinical Presentation: History, Physical Examination, Complications
Pathology of Nonspecific Interstitial Pneumonia: Definition, Epidemiology, Etiology
An autopsy case of idiopathic interstitial pneumonia with diffuse alveolar hemorrhage due to acute exacerbation<...
Chronic Hypersensitivity Pneumonitis: Differentiation from Idiopathic Pulmonary Fibrosis and Nonspecific Interstitial Pneumonia...
Interstitial lung disease - adults - discharge: MedlinePlus Medical Encyclopedia
Connective Tissue Disease-Associated Interstitial Lung Disease (CTD-ILD) Medication: Corticosteroids, Pulmonary, Tyrosine...
Hypersensitivity Pneumonitis: Spectrum of High-Resolution CT and Pathologic Findings | AJR
An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune...
Desquamative interstitial pneumonia: a systematic review of its features and outcomes | European Respiratory Society
Interstitial Lung Disease (ILD) Program
Relafen (Nabumetone): Uses, Dosage, Side Effects, Interactions, Warning
NIOSHTIC-2 Search Results - Full View
Pirfenidone in idiopathic pulmonary fibrosis | European Respiratory Society
Riociguat Uses, Side Effects & Warnings
Australasian interstitial lung disease registry (AILDR): objectives, design and rationale of a bi-national prospective database...
Prognostic Value of Dual-Time-Point 18F-FDG PET for Idiopathic Pulmonary Fibrosis | Journal of Nuclear Medicine
Hypersensitivity Pneumonitis - Pulmonary Disorders - Merck Manuals Professional Edition
Nonspecific14
- Nonspecific interstitial pneumonia is characterized by a distinct histopathologic appearance and a better prognosis than idiopathic pulmonary fibrosis. (nih.gov)
- However, there is still confusion and controversy over the relationship between idiopathic pulmonary fibrosis and fibrotic nonspecific interstitial pneumonia. (nih.gov)
- Idiopathic nonspecific interstitial pneumonia: lung manifestation of undifferentiated connective tissue disease? (nih.gov)
- The American Thoracic Society/European Respiratory Society International Consensus Classification panel identified the clinical entity idiopathic nonspecific interstitial pneumonia (NSIP) as a provisional diagnosis and recommended further study. (nih.gov)
- Acute or subacute IIPs include acute interstitial pneumonia (AIP), cryptogenic organizing pneumonia (COP), nonspecific interstitial pneumonia (NSIP), acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) and AE-NSIP. (wustl.edu)
- The clinical symptoms of idiopathic pulmonary fibrosis (IPF) are nonspecific. (medscape.com)
- Nonspecific interstitial pneumonia (NSIP) is a form of interstitial lung disease. (medscape.com)
- To retrospectively assess the accuracy of thin-section computed tomography (CT) in distinguishing chronic hypersensitivity pneumonitis (HP) from idiopathic pulmonary fibrosis (IPF) and nonspecific interstitial pneumonia (NSIP), with histologic results as the reference standard. (rsna.org)
- Unclassifiable ILDs, autoimmune ILDs, chronic hypersensitivity pneumonitis, sarcoidosis, myositis, Sjögren syndrome, coal worker pneumoconiosis, and idiopathic forms of interstitial pneumonias (eg, idiopathic nonspecific interstitial pneumonia [NSIP]) are among the diseases that may develop a progressive form of chronic fibrosing ILD. (medscape.com)
- The high-resolution CT and pathologic features of chronic HP frequently overlap with those of nonspecific interstitial pneumonia and usual interstitial pneumonia. (ajronline.org)
- HP is often difficult to diagnose because the clinical manifestations are nonspecific and the radiologic and histologic patterns can mimic those of other interstitial and small airway diseases [ 2 ]. (ajronline.org)
- A pattern of diffuse alveolar damage and temporally uniform, nonspecific, chronic interstitial pneumonitis may also be seen [ 1 , 3 ]. (ajronline.org)
- Furthermore, in some patients the predominant histologic pattern is nonspecific interstitial pneumonia (NSIP) or usual interstitial pneumonia (UIP). (ajronline.org)
- Idiopathic nonspecific interstitial pneumonia: an interstitial lung disease associated with autoimmune disorders? (google.it)
NSIP11
- We hypothesized that idiopathic NSIP is an autoimmune disease and the lung manifestation of undifferentiated connective tissue disease (UCTD), a recently described, distinct entity. (nih.gov)
- Compared with the control group, patients with UCTD-ILD were significantly more likely to have ground-glass opacity on high-resolution computed tomography (HRCT) and NSIP pattern on biopsy, and less likely to have honeycombing on HRCT or usual interstitial pneumonia on biopsy. (nih.gov)
- At our center, the majority of patients classified as idiopathic NSIP (88%) met the criteria for UCTD. (nih.gov)
- Most patients diagnosed with idiopathic NSIP meet the case definition of UCTD. (nih.gov)
- Furthermore, these results show that the clinical entity idiopathic NSIP is different from idiopathic pulmonary fibrosis and appears to be an autoimmune disease. (nih.gov)
- I have suffered from NSIP for four years, with IPF (idiopathic pulmonary fibrosis). (biotherapy.asia)
- Most cases of NSIP are idiopathic. (medscape.com)
- [ 1 ] Therefore, the diagnosis of NSIP should lead to a careful clinical workup for connective-tissue disease and possible adverse drug reaction before idiopathic NSIP is diagnosed. (medscape.com)
- Furthermore, the NSIP pattern can be seen in patients with hypersensitivity pneumonia (cellular interstitial pneumonia without the other classic findings of chronic bronchiolitis and poorly formed granulomas) or who have had recent acute lung injury (ie, histologic overlap between organizing diffuse alveolar damage [DAD] and NSIP). (medscape.com)
- Therefore, clinical history, time course of disease, exposures, and radiographic findings should all be correlated before idiopathic NSIP is diagnosed. (medscape.com)
- The radiographic features of NSIP significantly overlap with those of other diagnoses, including UIP, hypersensitivity pneumonia, OP, and DIP. (medscape.com)
Fibrosis62
- HMF=heavy metal fibrosis, no longer grouped with diffuse lung disease Lymphoid interstitial pneumonia was originally included in this category, then excluded, then included again. (wikipedia.org)
- The prognosis of idiopathic pulmonary fibrosis is very poor, with median survival of 2-4 yr after the diagnosis, yet the course of individual patients is highly variable. (nih.gov)
- According to abbreviationfinder , idiopathic interstitial pneumonia is an umbrella term for a large group of diseases of the lungs characterized by an inflammatory response and pulmonary scarring (fibrosis) of the connective tissue, capillaries and/or alveoli. (acronymmonster.com)
- Patients with idiopathic pulmonary fibrosis are mostly older than 60 years. (acronymmonster.com)
- Keow J, Cecchini M, Jayawardena N, Zompatori M, Joseph M, Mura M. Digital quantification of p16-positive foci in fibrotic interstitial lung disease identifies a senescent phenotype of idiopathic pulmonary fibrosis with reduced survival. (ifpoc.org)
- Dyspnea, which is the most prominent symptom in idiopathic pulmonary fibrosis, usually begins insidiously and is often progressive. (medscape.com)
- The reported median duration of symptoms before the diagnosis of idiopathic pulmonary fibrosis is established is one to two years. (medscape.com)
- Approximately 5% of patients have no presenting symptoms when idiopathic pulmonary fibrosis is diagnosed. (medscape.com)
- Among asymptomatic patients with idiopathic pulmonary fibrosis (diagnosed by radiographic abnormalities found on routine chest radiograph screening and lung biopsy showing usual interstitial pneumonia), symptoms developed approximately 1000 days after the recognition of the radiographic abnormality. (medscape.com)
- Oxidant stress from smoking may damage alveolar epithelial cells and contribute to the pathogenesis of idiopathic pulmonary fibrosis. (medscape.com)
- [ 36 ] Any patient with idiopathic pulmonary fibrosis who is a current smoker should be encouraged to quit. (medscape.com)
- Physicians should pay attention to historical clues that may suggest the presence of obstructive sleep apnea (OSA) because a 2009 study demonstrated the high prevalence of OSA in patients with idiopathic pulmonary fibrosis. (medscape.com)
- Fifty outpatients with stable idiopathic pulmonary fibrosis were prospectively evaluated for the presence of OSA. (medscape.com)
- [ 37 ] Therefore, the prevalence of OSA in this sample was 88%, suggesting that OSA in patients with idiopathic pulmonary fibrosis may have been previously underrecognized. (medscape.com)
- In most patients with idiopathic pulmonary fibrosis (IPF), the physical examination reveals fine bibasilar inspiratory crackles (Velcro crackles). (medscape.com)
- Additionally, digital clubbing is seen in 25-50% of patients with idiopathic pulmonary fibrosis. (medscape.com)
- [ 12 ] Extrapulmonary involvement does not occur with idiopathic pulmonary fibrosis, and, therefore, physical examination findings do not help to confirm the diagnosis. (medscape.com)
- Pulmonary hypertension is a common comorbidity in patients with idiopathic pulmonary fibrosis, and an estimated 20-40% of patients with idiopathic pulmonary fibrosis who are evaluated or listed for lung transplantation have pulmonary hypertension at rest. (medscape.com)
- On histology, it shows a temporally uniform interstitial process with varying proportions of interstitial inflammation and fibrosis. (medscape.com)
- The autopsy findings demonstrated diffuse alveolar hemorrhage, diffuse alveolar damage, interstitial pneumonia, and pulmonary fibrosis. (elsevierpure.com)
- Can CT distinguish hypersensitivity pneumonitis from idiopathic pulmonary fibrosis? (rsna.org)
- Interstitial lung disease (ILD) is a broad category of lung diseases that includes more than 130 disorders characterized by scarring (i.e. 'fibrosis') and / or inflammation of the lungs. (nationaljewish.org)
- Purpose of review: Recent epidemiologic investigations suggest that occupational and environmental exposures contribute to the overall burden of idiopathic pulmonary fibrosis (IPF). (cdc.gov)
- Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease without proven effective therapy. (ersjournals.com)
- Idiopathic pulmonary fibrosis (IPF) is a devastating, progressive fibrotic lung disease with a median survival of 3-5 yrs without proven effective therapy 1 , 2 . (ersjournals.com)
- Idiopathic pulmonary fibrosis (31%) and connective-tissue disease related ILD (21.7%) were the two most common subtypes. (springer.com)
- Interstitial lung disease (ILD) encompasses a heterogeneous group of respiratory disorders characterised by inflammation and/or fibrosis of the lung interstitium. (springer.com)
- Firstly, there are the Idiopathic Interstitial Pneumonias (IIPs) including Idiopathic Pulmonary Fibrosis (IPF), the most common IIP, along with idiopathic non-specific idiopathic pneumonia (iNSIP), acute interstitial pneumonia (AIP) and respiratory bronchiolitis-associated ILD (RB-ILD), to name a few. (springer.com)
- The aim of this prospective study was to clarify whether dual-time-point 18 F-FDG PET imaging results are useful to predict long-term survival of idiopathic pulmonary fibrosis (IPF) patients. (snmjournals.org)
- Patients with idiopathic pulmonary fibrosis (IPF) experience progressive respiratory failure and have a median survival of less than 3 y after diagnosis ( 1 ). (snmjournals.org)
- all are characterized by acute interstitial inflammation and development of granulomas and fibrosis with long-term exposure. (merckmanuals.com)
- Background Bronchiolisation of distal airspaces is an unexplained feature of idiopathic pulmonary fibrosis (IPF). (bmj.com)
- Abnormal differentiation of the respiratory epithelium is a feature of idiopathic pulmonary fibrosis (IPF). (bmj.com)
- To compare survival of patients with connective tissue disease-associated interstitial lung disease (CTD-ILD) versus idiopathic pulmonary fibrosis (IPF) and patients with systemic sclerosis-associated ILD (SSc-ILD) versus other CTD-ILD followed at our center. (jrheum.org)
- Interstitial lung disease (ILD) represents a heterogeneous group of diseases that involves inflammation and interstitial fibrosis of the lung parenchyma. (jrheum.org)
- The most common types of ILD include idiopathic pulmonary fibrosis (IPF) and connective tissue disease (CTD)-associated ILD (CTD-ILD). (jrheum.org)
- For example, idiopathic pulmonary fibrosis (IPF) is a clinical term describing a slowly progressive, chronic interstitial pneumonia. (medicalmarijuana.com)
- The term "usual" refers to the fact that UIP is the most common form of interstitial fibrosis. (medicalmarijuana.com)
- Pneumonia" indicates "lung abnormality", which includes fibrosis and inflammation. (medicalmarijuana.com)
- Since the medical term for conditions of unknown cause is "idiopathic" the clinical term for UIP of unknown cause is idiopathic pulmonary fibrosis (IPF). (medicalmarijuana.com)
- Rheumatoid arthritis-associated interstitial lung disease (RA-ILD) is a non-neoplastic disorder resulting from damage to the lung parenchyma by varying patterns of inflammation and fibrosis. (bmj.com)
- Dr. Kaminski's main research interests involve applying genomic approaches to elucidate basic mechanisms and improve diagnosis and treatment of Idiopathic Pulmonary Fibrosis (IPF), a chronic mostly lethal and currently untreatable scarring lung disease and other chronic lung diseases such Chronic Obstructive Pulmonary Disease (COPD), severe asthma and sarcoidosis. (yale.edu)
- In 65% of ILD cases the cause is still unknown (known as idiopathic interstitial pneumonias, of which idiopathic pulmonary fibrosis is the most frequent). (european-lung-foundation.org)
- Researchers claim a common SNP in the putative promoter of the MUC5B gene appears to be strongly associated with the development of both familial interstitial pneumonia (FIP) and idiopathic pulmonary fibrosis (IPF). (genengnews.com)
- On the basis of our findings, the population attributable risk of familial interstitial pneumonia or idiopathic pulmonary fibrosis for this promoter polymorphism is likely to be substantial," the authors state. (genengnews.com)
- Idiopathic pulmonary fibrosis (IPF) is a fatal disorder without an effective therapy to date. (atsjournals.org)
- Idiopathic pulmonary fibrosis (IPF) is a relentlessly progressive and fatal disorder characterized by high-resolution computed tomography (HRCT) and histologic features of usual interstitial pneumonia (UIP) in adults over 50 years of age with exertional dyspnea, abnormal pulmonary function tests (PFTs), and ineffective therapy ( 1 , 2 ). (atsjournals.org)
- Scarring in the lungs is referred to as pulmonary fibrosis (PF) or Interstitial Pneumonia(IP). (henryford.com)
- Idiopathic pulmonary fibrosis (IPF) is a type of scarring lung disease without a known cause. (henryford.com)
- That means we're up to date on the latest treatments, including studies on new medication therapies for idiopathic pulmonary fibrosis. (henryford.com)
- ILD includes lung diseases such as pulmonary fibrosis, non-infectious pneumonia and vasculitis (blood vessel inflammation). (henryford.com)
- Pulmonary fibrosis share the pathogenesis process of interstitial lung disease which involve the pulmonary parenchyma . (wikidoc.org)
- Idiopathic pulmonary fibrosis (IPF) is a rare form of chronic, progressive fibrosing interstitial pneumonia of unknown etiology. (medscape.com)
- From "traction bronchiectasis" to honeycombing in idiopathic pulmonary fibrosis: a spectrum of bronchiolar remodeling also in radiology? (google.it)
- Features and outcome of familial idiopathic pulmonary fibrosis. (google.it)
- RESULTS: Exploratory and validation cohorts consisting of 460 and 414 patients with IIPs, respectively, were included (159 and 159 patients had idiopathic pulmonary fibrosis [IPF], and 301 and 255 had non-IPF, respectively). (bvsalud.org)
- A novel gene signature based on the hub genes of COVID-19 predicts the prognosis of idiopathic pulmonary fibrosis. (cdc.gov)
- Non-coding RNA in idiopathic interstitial pneumonia and Covid-19 pulmonary fibrosis. (cdc.gov)
- Genetic overlap between idiopathic pulmonary fibrosis and COVID-19. (cdc.gov)
- Research on the mechanism of berberine in the treatment of COVID-19 pneumonia pulmonary fibrosis using network pharmacology and molecular docking. (cdc.gov)
- Idiopathic pulmonary fibrosis (IPF) is a fatal disease that exhibits patterns of usual interstitial pneumonia with honeycombing. (lu.se)
- Idiopathic Pulmonary Fibrosis IPF is a chronic, progressive fibrosing interstitial pneumonia of unknown cause that primarily occurs in older adults. (medscape.com)
IIPs4
- General reference Idiopathic interstitial pneumonias (IIPs) are interstitial lung diseases of unknown etiology that share similar clinical and radiologic features and are distinguished primarily by the histopathologic. (merckmanuals.com)
- Idiopathic interstitial pneumonias (IIPs) may have an acute or subacute presentation, or acute exacerbation may occur in a previously subclinical or unrecognized chronic IIP. (wustl.edu)
- Interstitial lung diseases (ILDs) including connective tissue disease (CTD) associated ILD, hypersensitivity pneumonitis, acute eosinophilic pneumonia, drug-induced lung disease and diffuse alveolar haemorrhage need to be differentiated from acute and subacute IIPs. (wustl.edu)
- However, the utility of LIPI in patients with idiopathic interstitial pneumonias (IIPs) is unknown. (bvsalud.org)
Histologic1
- Idiopathic interstitial pneumonia can be subclassified based on histologic appearance into the following patterns: Usual interstitial pneumonia is the most common type. (wikipedia.org)
Pneumonitis5
- Histologically, subacute HP is characterized by the presence of cellular bronchiolitis, noncaseating granulomas, and bronchiolocentric lymphocytic interstitial pneumonitis. (ajronline.org)
- H ypersensitivity pneumonitis (HP) is a diffuse granulomatous interstitial lung disease caused by inhalation of various antigenic organic particles [ 1 ]. (ajronline.org)
- Anticancer drug gefitinib causes inflammation-based side effects, such as interstitial pneumonitis. (nature.com)
- Together our results reveal the potential ability of gefitinib to initiate sterile inflammation via two distinct mechanisms, and identified IL-1β and HMGB1 as key determinants of gefitinib-induced inflammation that may provide insights into gefitinib-induced interstitial pneumonitis. (nature.com)
- Giant cell interstitial pneumonitis (GIP) is now considered a hard metal pneumoconiosis. (medicalmarijuana.com)
Lungs8
- Fibrosing ILDs are heterogeneous group of scarring disorders of lungs (mainly including idiopathic interstitial pneumonias) causing progressive loss of lung function, shortness of breath on minimal exertion, respiratory failure and in the absence of lung transplantation, premature death. (ifpoc.org)
- interstitial pneumonia: chronic lung disease affecting the interstitial tissue of the lungs. (medicalmarijuana.com)
- Interstitial lung disease is a term used to refer to a particular type of inflammation of the interstitium of the lungs. (medicalmarijuana.com)
- Interstitial pneumonia (medical condition): A category of chronic lung diseases characterized by scarring and/or inflammation of the lungs. (medicalmarijuana.com)
- Usual interstitial pneumonia (UIP) is a form of lung disease characterized by progressive scarring of both lungs. (medicalmarijuana.com)
- The interstitial space around the alveoli can become inflamed, thickened or scarred because of a variety of insults or reactions in the lungs. (stanford.edu)
- In 2000, IPF was defined as a specific form of chronic, progressive, fibrosing interstitial pneumonia of unknown cause, occurring primarily in older adults and limited to the lungs. (medicalcriteria.com)
- Fungal pneumonia is an infectious process in the lungs caused by one or more endemic or opportunistic fungi. (medscape.com)
Desquamative interstitial1
- Background Desquamative Interstitial Pneumonia (DIP) is a rare form of idiopathic interstitial pneumonia (IIP). (ersjournals.com)
Unexplained interstitial lung di1
- Idiopathic interstitial pneumonia should be suspected in any patient with unexplained interstitial lung disease. (merckmanuals.com)
Pattern of usual interstit2
- Recent findings: IPF patients demonstrate a histopathologic pattern of usual interstitial pneumonia. (cdc.gov)
- two out of six expert radiologists independently evaluated the HRCT images to agree and determine whether the pattern of usual interstitial pneumonia (UIP) was present or not in accordance with the predetermined protocol (see online supplementary material). (ersjournals.com)
Alveolar2
- Overview of Interstitial Lung Disease Interstitial lung diseases are a heterogeneous group of disorders characterized by alveolar septal thickening, fibroblast proliferation, collagen deposition, and, if the process remains unchecked. (merckmanuals.com)
- There has hitherto been no report describing idiopathic interstitial pneumonia associated with diffuse alveolar hemorrhage, but we herein report one such rare case. (elsevierpure.com)
Clinical4
- We studied 28 consecutive patients with idiopathic interstitial pneumonia (IIP) enrolled in the University of California, San Francisco Interstitial Lung Disease Center who met prespecified criteria for UCTD, as follows: at least one clinical manifestation of connective tissue disease, serologic evidence of systemic inflammation in the absence of clinical infection, and absence of sufficient American College of Rheumatology criteria for another connective tissue disease. (nih.gov)
- Many patients with an idiopathic interstitial pneumonia (IIP) have clinical features that suggest an underlying autoimmune process but do not meet established criteria for a connective tissue disease (CTD). (ersjournals.com)
- The task force proposes the term "interstitial pneumonia with autoimmune features" (IPAF) and offers classification criteria organised around the presence of a combination of features from three domains: a clinical domain consisting of specific extra-thoracic features, a serologic domain consisting of specific autoantibodies, and a morphologic domain consisting of specific chest imaging, histopathologic or pulmonary physiologic features. (ersjournals.com)
- The diagnosis of IPF was in accordance with the American Thoracic Society (ATS)/European Respiratory Society (ERS) Consensus statement 16 and the fourth version of the clinical diagnostic criteria guidelines for idiopathic interstitial pneumonia in Japan 17 . (ersjournals.com)
Form of interstitial lung di1
- UIP is thus classified as a form of interstitial lung disease. (medicalmarijuana.com)
Shortness of bre2
- The main symptoms of idiopathic interstitial pneumonia are shortness of breath and a dry cough. (acronymmonster.com)
- The symptoms vary for each of the pneumonias but most are characterized by a slowly progressive shortness of breath. (medicalmarijuana.com)
Associated with interstitial lung di2
- Drugs used to manage connective tissue disease (CTD) associated with interstitial lung disease (ILD) (CTD-ILD) include nintedanib, corticosteroids, and antineoplastic agents. (medscape.com)
- Objective A post-hoc analysis of the INCREASE trial and its open-label extension (OLE) was performed to evaluate whether inhaled treprostinil has a long-term survival benefit in patients with pulmonary hypertension associated with interstitial lung disease (PH-ILD). (bmj.com)
Bronchiolitis obliterans2
- Areas of organizing pneumonia (bronchiolitis obliterans with organizing pneumonia) may be identified [ 6 ]. (ajronline.org)
- and organizing pneumonia (bronchiolitis obliterans with organizing pneumonia reaction) in two patients. (ajronline.org)
Patients13
- The study, "Genetic susceptibility loci of idiopathic interstitial pneumonia do not represent risk for systemic sclerosis: a case control study in Caucasian patients," was published in the journal Arthritis Research and Therapy . (rtmagazine.com)
- The aim of study is matching patients with fibrosing interstitial lung disease (ILD) with most effective treatment right from start, improving their survival and quality of life. (ifpoc.org)
- Nintedanib is indicated to slow the rate of decline in pulmonary function in patients who have interstitial lung disease (ILD) associated with scleroderma. (medscape.com)
- The "European Respiratory Society/American Thoracic Society Task Force on Undifferentiated Forms of Connective Tissue Disease-associated Interstitial Lung Disease" was formed to create consensus regarding the nomenclature and classification criteria for patients with IIP and features of autoimmunity. (ersjournals.com)
- The primary objective of this multidisciplinary task force was to develop consensus surrounding the nomenclature and classification of patients with suggestive forms of connective tissue disease-associated interstitial lung disease (CTD-ILD). (ersjournals.com)
- 18 F-FDG PET has recently been applied for assessing disease activity in interstitial pneumonia patients ( 11 - 14 ). (snmjournals.org)
- Immunostaining for components of those pathways and mucins were performed on lung tissue obtained from patients with IPF (n=20), chronic obstructive pulmonary disease (n=13), idiopathic pulmonary artery hypertension (n=5) and from organ donors (n=6). (bmj.com)
- NRG1α and NRG1β were quantified in bronchoalveolar lavage fluid (BALF) of patients with early IPF (n=20), controls (n=9), and patients with other interstitial pneumonias (n=13). (bmj.com)
- Interstitial lung disease (ILD) is an extra-articular manifestation of RA, which occurs frequently in up to 80% of patients with RA. (hindawi.com)
- 7 Following the introduction of anti-tumour necrosis factor (anti-TNF) therapies for patients with RA, there have been a number of case reports and case series documenting either a rapid progression of pre-existing RA-ILD or the development of new-onset interstitial disease. (bmj.com)
- The Committee recommended that Adempas (riociguat) should not be used in patients with symptomatic pulmonary hypertension associated with idiopathic interstitial pneumonia or PH-IIP (high blood pressure in lung arteries caused by a lung disease called idiopathic interstitial pneumonia). (europa.eu)
- Patients evaluated in the Henry Ford Interstitial Lung Disease Clinic undergo a thorough history and physical exam. (henryford.com)
- Opportunistic fungal organisms (eg, Candida species, Aspergillus species, Mucor species) tend to cause pneumonia in patients with congenital or acquired defects in the host immune defenses. (medscape.com)
Classification3
- American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. (wikipedia.org)
- In the American Thoracic Society/European Respiratory Society consensus classification, idiopathic interstitial pneumonias are classified into seven clinicopathologic entities. (nih.gov)
- In 2002, the American Thoracic Society and the European Respiratory Society published a joint classification of idiopathic interstitial pneumonia. (acronymmonster.com)
Causes of interstitial1
- It is critical to obtain a complete history, including medication history, drug use, social history, occupational, recreational, and environmental respiratory exposure history, risk factors for human immunodeficiency virus infection, and review of systems, to ensure other causes of interstitial lung disease are excluded. (medscape.com)
Usual4
- Asbestosis and environmental causes of usual interstitial pneumonia. (cdc.gov)
- In the absence of a known cause or association, a usual interstitial pneumonia pattern leads to an IPF diagnosis, which is a progressive and often terminal fibrotic lung disease. (cdc.gov)
- Die Diagnosesicherung erfordert den Nachweis des Befundmusters einer usual interstitial pneumonia (UIP) im hochaufl senden D nnschicht-Computertomogramm (HR-CT) oder histologisch durch eine Lungenbiopsie sowie den Ausschluss anderer Ursachen, wie Asbestose oder Kollagenosen. (aerzteblatt.de)
- Usual interstitial pneumonia (UIP) is the histopathological pattern of IPF. (medicalcriteria.com)
Alveolitis1
- in which repeated exposure to antigen in genetically susceptible people leads to acute neutrophilic and mononuclear alveolitis, followed by interstitial lymphocytic infiltration and granulomatous reaction. (merckmanuals.com)
Lymphocytic1
- Lymphocytic interstitial pneumonia (LIP) is now considered a lymphoproliferative disease. (medicalmarijuana.com)
Diseases5
- Idiopathic interstitial pneumonia (IIP), or noninfectious pneumonia are a class of diffuse lung diseases. (wikipedia.org)
- The study confirmed that SSc-ILD and idiopathic interstitial pneumonia are different diseases, not sharing a genetic basis. (rtmagazine.com)
- Idiopathic interstitial pneumonia combines a large group of lung diseases that share the components of inflammation and lung scarring to varying degrees. (acronymmonster.com)
- Interstitial pneumonias are a confusing and frustrating set of diseases both for the treating physician and for the diagnostic pathologist. (medicalmarijuana.com)
- A handout on this topic is available at http://familydoctor.org/familydoctor/en/diseases-conditions/rheumatoid-arthritis.html . (aafp.org)
Rheumatoid4
- In recent years, the number of studies on rheumatoid arthritis-related interstitial lung disease (RA-ILD) has been increasing, which has led to many publications on this topic. (hindawi.com)
- The retrieval steps and strategies were as follows: Title = rheumatoid arthritis AND Title = (interstitial lung disease OR interstitial pneumonia) AND Language = English AND Document type = (review OR article) AND Time span =1980 to 2021. (hindawi.com)
- Background Anti-tumour necrosis factor (anti-TNF) therapy has been associated with reports of rapid severe progression of rheumatoid arthritis-associated interstitial lung disease (RA-ILD). (bmj.com)
- Nordimet (methotrexate) was recommended for the treatment of active rheumatoid arthritis, juvenile idiopathic arthritis and severe psoriatic arthritis. (europa.eu)
HRCT1
- HRCT, which distinguishes airspace from interstitial disease, is the most useful test and is always done. (merckmanuals.com)
Disease13
- Researchers showed that systemic sclerosis-related interstitial lung disease is not genetically linked to idiopathic interstitial pneumonia in a recent study. (rtmagazine.com)
- You were in the hospital to treat your breathing problems that are caused by interstitial lung disease . (medlineplus.gov)
- The National Jewish Health Interstitial Lung Disease Center for Patient Care, Education, Discovery and Innovation is one of the largest interstitial lung disease (ILD) centers in the country. (nationaljewish.org)
- Interstitial Lung Disease can take on many forms. (nationaljewish.org)
- Interstitial Lung Disease (ILD) is a group of respiratory conditions affecting the lung interstitium often associated with progressive respiratory failure. (springer.com)
- Interstitial pneumonia is a disease in which the mesh-like walls of the alveoli become inflamed. (medicalmarijuana.com)
- Interstitial pneumonia: Another name for Interstitial lung disease (or close medical condition association). (medicalmarijuana.com)
- Interstitial Lung Disease refers to a large group of lung disorders that cause inflammation or scarring in the lung tissue. (stanford.edu)
- Dr. Kaminski was the director of the Dorothy P. and Richard P. Simmons Center for Interstitial Lung Disease and the Lung, Blood and Vascular Center for Genomic Medicine at the division of Pulmonary, Allergy and Critical Care Medicine in University of Pittsburgh. (yale.edu)
- Interstitial lung disease (ILD) is a large group of disorders that can affect the small air sacs (alveoli) or surrounding structures (interstitium) of the lung. (henryford.com)
- Interstitial lung disease is a group of disorders that involve pulmonary parenchyma . (wikidoc.org)
- How Can Interstitial Lung Disease Phenotypes Guide Care? (medscape.com)
- Pulmonary involvement in pSS includes interstitial lung disease (ILD) and airway disease, together with lymphoproliferative disorders. (ersjournals.com)
Biopsy1
- Surgical biopsy, however, is often needed for the definitive diagnosis of both subacute and chronic HP and for reliable differentiation of chronic HP from idiopathic interstitial pneumonia [ 3 , 4 ]. (ajronline.org)