A multisystemic disease of a complex genetic background. It is characterized by inflammation of the blood vessels (VASCULITIS) leading to damage in any number of organs. The common features include granulomatous inflammation of the RESPIRATORY TRACT and kidneys. Most patients have measurable autoantibodies (ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES) against neutrophil proteinase-3 (WEGENER AUTOANTIGEN).
A polymorphonuclear leukocyte-derived serine protease that degrades proteins such as ELASTIN; FIBRONECTIN; LAMININ; VITRONECTIN; and COLLAGEN. It is named for its ability to control myeloid cell growth and differentiation.
Autoantibodies directed against cytoplasmic constituents of POLYMORPHONUCLEAR LEUKOCYTES and/or MONOCYTES. They are used as specific markers for GRANULOMATOSIS WITH POLYANGIITIS and other diseases, though their pathophysiological role is not clear. ANCA are routinely detected by indirect immunofluorescence with three different patterns: c-ANCA (cytoplasmic), p-ANCA (perinuclear), and atypical ANCA.
An angiocentric and angiodestructive lymphoproliferative disorder primarily involving the lungs. It is caused by an Epstein-Barr virus-induced transformation of the B-cells, in a T-cell rich environment. Clinically and pathologically it resembles EXTRANODAL NK-T-CELL LYMPHOMA.
Inflammation of any one of the blood vessels, including the ARTERIES; VEINS; and rest of the vasculature system in the body.
The outermost of the three MENINGES, a fibrous membrane of connective tissue that covers the brain and the spinal cord.
A primary systemic vasculitis of small- and some medium-sized vessels. It is characterized by a tropism for kidneys and lungs, positive association with anti-neutrophil cytoplasmic antibodies (ANCA), and a paucity of immunoglobulin deposits in vessel walls.
A condition characterized by persistent or recurrent labial enlargement, ORAL ULCER, and other orofacial manifestations in the absence of identifiable CROHN DISEASE; or SARCOIDOSIS. Among experts there is disagreement on whether orofacial granulomatosis is a distinct clinical disorder or an initial presentation of Crohn disease.
Widespread necrotizing angiitis with granulomas. Pulmonary involvement is frequent. Asthma or other respiratory infection may precede evidence of vasculitis. Eosinophilia and lung involvement differentiate this disease from POLYARTERITIS NODOSA.
A heterogeneous group of diseases characterized by inflammation and necrosis of the blood vessel walls.
Inanimate objects that become enclosed in the body.
Inflammation of the retinal vasculature with various causes including infectious disease; LUPUS ERYTHEMATOSUS, SYSTEMIC; MULTIPLE SCLEROSIS; BEHCET SYNDROME; and CHORIORETINITIS.
Group of systemic vasculitis with a strong association with ANCA. The disorders are characterized by necrotizing inflammation of small and medium size vessels, with little or no immune-complex deposits in vessel walls.
A pathological process characterized by injury or destruction of tissues caused by a variety of cytologic and chemical reactions. It is usually manifested by typical signs of pain, heat, redness, swelling, and loss of function.
Inflammation of blood vessels within the central nervous system. Primary vasculitis is usually caused by autoimmune or idiopathic factors, while secondary vasculitis is caused by existing disease process. Clinical manifestations are highly variable but include HEADACHE; SEIZURES; behavioral alterations; INTRACRANIAL HEMORRHAGES; TRANSIENT ISCHEMIC ATTACK; and BRAIN INFARCTION. (From Adams et al., Principles of Neurology, 6th ed, pp856-61)
A surgical specialty which utilizes medical, surgical, and physical methods to treat and correct deformities, diseases, and injuries to the skeletal system, its articulations, and associated structures.

Alternating antineutrophil cytoplasmic antibody specificity: drug-induced vasculitis in a patient with Wegener's granulomatosis. (1/627)

We describe a patient who presented with Wegener's granulomatosis associated with antineutrophil cytoplasmic antibodies (ANCA) directed against proteinase 3 (PR3) with a cytoplasmic immunofluorescence pattern (cANCA), whose ANCA type changed to antimyeloperoxidase antibodies with a perinuclear immunofluorescence pattern (pANCA) when treated with propylthiouracil, and changed back to anti-PR3 antibodies with cANCA after the medication was discontinued. The patient developed flares of vasculitis symptoms associated with rises in either type of ANCA. Tests for antimyeloperoxidase ANCA were repeatedly negative before the drug was started, strongly implicating the drug as the cause of the episode. This case demonstrates that patients with idiopathic ANCA-positive vasculitis may quickly develop a superimposed drug-associated ANCA-positive vasculitis. Iatrogenic vasculitis should be suspected when a patient with idiopathic vasculitis with one type of ANCA develops the other type of ANCA.  (+info)

Interaction of purified human proteinase 3 (PR3) with reconstituted lipid bilayers. (2/627)

Proteinase 3 (PR3), the major target autoantigen in Wegener's granulomatosis is a serine proteinase that is normally stored intracellularly in the primary granules of quiescent neutrophils and monocytes. Upon cell activation, a significant portion of this antigen is detected on the cell surface membrane. The nature of the association of PR3 with the membrane and its functional significance are unknown. We investigated the interaction of purified human PR3 with mixtures of zwitterionic (dimyristoyl-L-alpha-phosphatidylcholine, DMPC) and anionic (dimyristoyl-L-alpha-phosphatidylglycerol, DMPG) phospholipids in reconstituted lipid bilayers using differential scanning calorimetry and lipid photolabeling, and measured the affinity of this interaction using spectrophotometry. Two other primary granule constituents, human neutrophil elastase (HNE) and myeloperoxidase (MPO) were investigated for comparison. In calorimetric assays, using lipid vesicles of mixed DMPC/DMPG, increasing PR3 concentrations (protein/lipid molar ratio from 0 to 1 : 110) induced a significant decrease of the main chain transition enthalpy and a shift in chain melting temperatures which is indicative of partial insertion of PR3 into the hydrophobic region of the lipid membranes. This was confirmed by hydrophobic photolabeling using liposomes containing trace amounts of the photoactivable [125I]-labeled phosphatidylcholine analog TID-PC/16. The molar affinity of PR3, HNE, and MPO to lipid vesicles of different DMPC/DMPG ratios was then determined by spectrophotometry. At a DMPC/DMPG ratio of 1 : 1, molar affinities of PR3, Kd = 4.5 +/- 0.3 microm; HNE, 14.5 +/- 1.2 microm; and MPO, 50 +/- 5 microm (n = 3) were estimated. The lipid-associated PR3 exhibited two-fold lower Vmax and Km values, and its enzyme activity was slightly more inhibited (Ki) by the natural alpha1-proteinase inhibitor (alpha1-PI) or an autoantibody to PR3.  (+info)

Cytokine profiles in Wegener's granulomatosis: predominance of type 1 (Th1) in the granulomatous inflammation. (3/627)

OBJECTIVE: To determine whether a specific cytokine pattern (type 1 [Th1] or type 2 [Th2]) predominates in Wegener's granulomatosis (WG), by evaluating interferon-gamma (IFNgamma) and interleukin-4 (IL-4) expression in different compartments of the body (i.e., biopsied nasal mucosal tissue [NBS], bronchoalveolar lavage [BAL] fluid, and peripheral blood [PB]) and comparing the findings with those in disease and healthy control subjects. METHODS: Competitive reverse transcriptase-polymerase chain reaction (RT-PCR) and enzyme-linked immunosorbent assay were used to assess IFNgamma and IL-4 expression in T cell clones (TCC), T cell lines (TCL), and polyclonal CD4+ and CD8+ cells derived from NBS, BAL, and PB. RESULTS: Patients with WG and chronic rhinitis were found to share in situ production of messenger RNA (mRNA) specific for IFNgamma (Th1). Only 2 patients with WG expressed IL-4, whereas IL-4 mRNA PCR products were found in inflamed tissues of the disease control patients. The granuloma-derived T cells of WG patients produced only IFNgamma, while TCC, TCL, and CD4+ and CD8+ T cells from BAL and PB produced mainly IFNgamma. CONCLUSION: Our data indicate that a Thl cytokine pattern predominates in the granulomatous inflammation in patients with WG.  (+info)

Wegener's granulomatosis associated with renal cell carcinoma. (4/627)

OBJECTIVE: To determine the frequencies and types of malignant neoplasms occurring before or simultaneously with the diagnosis of Wegener's granulomatosis (WG), and to test for the presence of "Wegener's autoantigen," proteinase 3 (PR3), in malignant tissues from WG patients to ascertain whether an association exists between malignancy and WG. METHODS: A retrospective statistical analysis was performed on the medical records of 477 patients with WG as compared with a control group of 479 patients with rheumatoid arthritis (RA). A murine monoclonal antibody was used to test malignant tissues for the presence of PR3. RESULTS: A malignant neoplasm was found in 23 patients in the WG group and in 18 patients in the control group. The odds ratio for malignant neoplasm in the WG group was 1.79 (P = 0.0876, 95% confidence interval [95% CI] 0.92-3.48). Seven patients with renal cell carcinoma were found in the WG group compared with 1 patient in the control group, for an odds ratio of 8.73 (P = 0.0464, 95% CI 1.04-73.69). Simultaneous occurrence of cancer and WG was observed in 14 patients with WG compared with 1 control patient, for an odds ratio of 18.00 (P = 0.0059, 95% CI 230-140.67). Furthermore, the diseases occurred simultaneously in 5 of the 7 patients with both WG and renal cell carcinoma, but not in the single patient in the control group with RA and renal cell carcinoma. PR3 could not be detected in any of the 8 malignant tissue samples (4 renal cell carcinomas) investigated in the patients from the WG group. CONCLUSION: The close temporal association between renal cell carcinoma and WG suggests that malignancy is, in some cases, a trigger for the development of WG. However, since PR3 was not found in malignant tissues from the WG patients, the immunopathologic mechanisms leading to autoimmunity and vasculitis remain unclear.  (+info)

Pneumocystis carinii pneumonia in patients with connective tissue diseases: the role of hospital experience in diagnosis and mortality. (5/627)

OBJECTIVE: Pneumonia due to Pneumocystis carinii has been increasingly reported in patients with connective tissue diseases, but the frequency of this complication is not known. We sought to determine the frequency of P carinii pneumonia (PCP) in patients with connective tissue diseases, and to determine the role that a hospital's acquired immunodeficiency syndrome (AIDS)-related experience may have in the diagnosis of PCP in these patients. METHODS: We used a state hospitalization registry to identify all patients with PCP and either rheumatoid arthritis, systemic lupus erythematosus, Wegener's granulomatosis, polymyositis, dermatomyositis, polyarteritis nodosa, or scleroderma who had an emergent or urgent hospitalization in California from 1983 to 1994. We compared patient and hospital characteristics between these patients and patients with connective tissue diseases hospitalized with other types of pneumonia. RESULTS: Two hundred twenty-three patients with connective tissue diseases were diagnosed with PCP in the 12-year study period. The frequency of PCP ranged from 89 cases/10,000 hospitalizations/year in patients with Wegener's granulomatosis to 2 cases/10,000 hospitalizations/year in patients with rheumatoid arthritis. Compared with 5,457 patients with connective tissue diseases and pneumonia due to other organisms, patients with PCP were more likely to be younger, to be male, to have private medical insurance, and to have systemic lupus erythematosus, Wegener's granulomatosis, inflammatory myopathy, or polyarteritis nodosa rather than rheumatoid arthritis, and were less likely to be African American. Hospital size, teaching status, urban/rural location, proportion of admissions due to AIDS or PCP, and proportion of patients with pneumonia undergoing bronchoscopy were each associated with the likelihood of diagnosis of PCP in univariate analyses, but only the number of patients with PCP being treated at a hospital (odds ratio [OR] 1.03 for each additional 10 cases/year, 95% confidence interval [95% CI] 1.01-1.05) was associated with the likelihood of diagnosis of PCP in multivariate analyses. Patients were also somewhat more likely to be diagnosed with PCP if there had previously been a case of PCP in a patient with a connective tissue disease at the same hospital (OR 135, 95% CI 0.98-1.85). In-hospital mortality was 45.7%, and was unrelated to hospital characteristics. CONCLUSION: PCP is an uncommon, but often fatal, occurrence in patients with connective tissue disease. A hospital's prior experience with patients with PCP is associated with the likelihood that this condition is diagnosed in patients with connective tissue diseases who present with pneumonia, suggesting that diagnostic suspicion is an important factor in the correct identification of affected patients.  (+info)

Pituitary involvement by Wegener's granulomatosis: a report of two cases. (6/627)

We describe two cases of pituitary involvement by Wegener's granulomatosis. At initial presentation, or during subsequent disease "flares," a pattern of pituitary abnormality was suggested. During periods of remission, we found the pituitary returned to a nearly normal appearance. Loss of the normal posterior pituitary T1 hyper-intensity matched a clinical persistence of diabetes insipidus, suggesting there is permanent damage to this structure by the initial disease process.  (+info)

Expression of major histocompatibility class II antigens on polymorphonuclear neutrophils in patients with Wegener's granulomatosis. (7/627)

BACKGROUND: Wegener's granulomatosis is a systemic inflammatory disease of unknown etiology. Many studies suggest that autoimmune reactions are involved, and there is good evidence for the participation of immunocompetent cells. In that context, we examined the activation of polymorphonuclear neutrophils (PMNs) of patients with Wegener's granulomatosis. METHODS: In a prospective study, the expression on the surface of PMNs of CD64 and of the major histocompatibility class II (MHC II) antigen was measured by cytofluorometry in whole blood. The expression of those antigens was correlated to disease activity. RESULTS: Up to 15% of the peripheral PMNs of patients with active disease expressed MHC II. Follow-up studies showed that expression correlated closely with disease activity and that it decreased rapidly under immunosuppressive therapy. Expression of CD64 was seen in approximately 50% of the patients, regardless of disease activity. CONCLUSION: MHC II expression on PMNs might serve as a novel diagnostic marker for active disease and appears to be suitable for monitoring immunotherapy. Moreover, our data provide evidence that PMNs, which are normally MHC II negative, acquire MHC II antigens in the course of disease and may be an unrecognized function within the afferent limb of the immune response.  (+info)

C3 and C4 allotypes in anti-neutrophil cytoplasmic autoantibody (ANCA)-positive vasculitis. (8/627)

In ANCA-associated small vessel vasculitis few genetic factors have proven to be of importance for disease susceptibility, an exception being deficiency of alpha1-anti-trypsin, the main inhibitor of proteinase 3 (PR3). Alerted by our finding that myeloperoxidase has affinity for C3, and the finding of an increased frequency of the C3F allele in systemic vasculitis in a British cohort, we examined polymorphism of C3 and C4 in patients with ANCA+ small vessel vasculitis. After identification of all patients at our department with a positive ANCA test during the period 1991-95 and a diagnosis of small vessel vasculitis, blood samples were collected after informed consent. The 67 included patients were grouped according to ANCA serology and disease phenotype using the Chapel Hill nomenclature. The gene frequency of C3F was found to be increased (0. 32) compared with controls (0.20; P < 0.05) in the PR3-ANCA+ subgroup. The frequency of C4A3 was increased in the group as a whole, but no increase of C4 null alleles was seen. The findings imply a role for the complement system in the pathogenesis of ANCA-associated small vessel vasculitis.  (+info)

Wegener Granulomatosis is a rare, chronic granulomatous vasculitis that affects small and medium-sized blood vessels. It is also known as granulomatosis with polyangiitis (GPA). The disease primarily involves the respiratory tract (nose, sinuses, trachea, and lungs) and kidneys but can affect other organs as well.

The characteristic features of Wegener Granulomatosis include necrotizing granulomas, vasculitis, and inflammation of the blood vessel walls. These abnormalities can lead to various symptoms such as cough, shortness of breath, nosebleeds, sinus congestion, skin lesions, joint pain, and kidney problems.

The exact cause of Wegener Granulomatosis is unknown, but it is believed to be an autoimmune disorder where the body's immune system mistakenly attacks its own tissues and organs. The diagnosis of Wegener Granulomatosis typically involves a combination of clinical symptoms, laboratory tests, imaging studies, and biopsy findings. Treatment usually includes immunosuppressive therapy to control the inflammation and prevent further damage to the affected organs.

Myeloblastin is not typically used as a medical term in current literature. However, in the field of hematology, "myeloblast" refers to an immature cell that develops into a white blood cell called a granulocyte. These myeloblasts are normally found in the bone marrow and are part of the body's immune system.

If you meant 'Myeloperoxidase,' I can provide a definition for it:

Myeloperoxidase (MPO) is a peroxidase enzyme that is abundant in neutrophil granulocytes, a type of white blood cell involved in the immune response. MPO plays an essential role in the microbicidal activity of these cells by generating hypochlorous acid and other reactive oxygen species to kill invading pathogens.

Antineutrophil cytoplasmic antibodies (ANCAs) are a type of autoantibody that specifically target certain proteins in the cytoplasm of neutrophils, which are a type of white blood cell. These antibodies are associated with several types of vasculitis, which is inflammation of the blood vessels.

There are two main types of ANCAs: perinuclear ANCAs (p-ANCAs) and cytoplasmic ANCAs (c-ANCAs). p-ANCAs are directed against myeloperoxidase, a protein found in neutrophil granules, while c-ANCAs target proteinase 3, another protein found in neutrophil granules.

The presence of ANCAs in the blood can indicate an increased risk for developing certain types of vasculitis, such as granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA), and microscopic polyangiitis (MPA). ANCA testing is often used in conjunction with other clinical findings to help diagnose and manage these conditions.

It's important to note that while the presence of ANCAs can indicate an increased risk for vasculitis, not everyone with ANCAs will develop the condition. Additionally, ANCAs can also be found in some individuals without any associated disease, so their presence should be interpreted in the context of other clinical findings.

Lymphomatoid Granulomatosis is a rare, progressive and potentially fatal inflammatory disorder characterized by the proliferation of atypical B-lymphocytes infiltrating the lungs, skin, and less frequently other organs. It is considered an angiocentric and angiodestructive lymphoproliferative disease with varying degrees of malignancy. The condition is often associated with Epstein-Barr virus (EBV) infection and is more prevalent in middle-aged men.

The disorder presents with a wide range of symptoms, depending on the affected organs. Lung involvement can cause cough, shortness of breath, chest pain, or hemoptysis, while skin manifestations may include papules, nodules, or ulcers. Other possible sites of involvement are the central nervous system, kidneys, and liver.

Lymphomatoid Granulomatosis is classified into three grades based on the number of atypical lymphocytes and degree of necrosis: grade I (few atypical cells, minimal necrosis), grade II (more atypical cells, mild to moderate necrosis), and grade III (large numbers of atypical cells, extensive necrosis). Grades II and III are considered high-grade lymphomas.

The diagnosis is established through a combination of clinical presentation, radiological findings, and histopathological examination of biopsy specimens. Treatment options include corticosteroids, chemotherapy, immunomodulatory agents, and radiation therapy. The prognosis varies depending on the grade, extent of disease, and response to treatment.

Vasculitis is a group of disorders characterized by inflammation of the blood vessels, which can cause changes in the vessel walls including thickening, narrowing, or weakening. These changes can restrict blood flow, leading to organ and tissue damage. The specific symptoms and severity of vasculitis depend on the size and location of the affected blood vessels and the extent of inflammation. Vasculitis can affect any organ system in the body, and its causes can vary, including infections, autoimmune disorders, or exposure to certain medications or chemicals.

Dura Mater is the thickest and outermost of the three membranes (meninges) that cover the brain and spinal cord. It provides protection and support to these delicate structures. The other two layers are called the Arachnoid Mater and the Pia Mater, which are thinner and more delicate than the Dura Mater. Together, these three layers form a protective barrier around the central nervous system.

Microscopic Polyangiitis (MPA) is a rare type of vasculitis, which is a group of disorders that cause inflammation in the blood vessels. In MPA, the small blood vessels in various organs become inflamed and damaged, leading to symptoms that can affect multiple organ systems.

The term "microscopic" refers to the fact that the diagnosis of this condition typically requires examination of tissue samples under a microscope to see the characteristic patterns of inflammation and damage in the small blood vessels.

MPA is an autoimmune disorder, which means that the body's immune system mistakenly attacks its own tissues and organs. In MPA, the immune system produces abnormal antibodies called ANCA (antineutrophil cytoplasmic antibodies) that target certain proteins in the white blood cells, leading to their activation and subsequent damage to the blood vessels.

The symptoms of MPA can vary widely depending on which organs are affected, but they may include fever, fatigue, weight loss, joint pain, skin rashes, cough, shortness of breath, and kidney problems such as proteinuria and hematuria. Treatment typically involves the use of immunosuppressive medications to suppress the overactive immune system and reduce inflammation in the blood vessels.

Orofacial granulomatosis is not a specific medical diagnosis but rather a term used to describe a group of conditions that are characterized by the presence of granulomas in the tissues of the oral and facial regions. Granulomas are collections of immune cells, specifically macrophages, that form in response to chronic inflammation or foreign substances in the body.

The term "orofacial granulomatosis" is often used interchangeably with "Granulomatous cheilitis," which specifically refers to the inflammation and swelling of the lips due to granulomas. However, orofacial granulomatosis can also affect other areas of the face, such as the cheeks, nose, and eyes.

The exact cause of orofacial granulomatosis is not fully understood, but it has been associated with several conditions, including:

* Crohn's disease, a chronic inflammatory bowel disease that can affect any part of the gastrointestinal tract
* Sarcoidosis, a multisystem disorder characterized by the formation of granulomas in various organs
* Allergic reactions to certain foods or dental materials
* Infections with mycobacteria or other bacteria

The symptoms of orofacial granulomatosis can vary depending on the severity and location of the granulomas. They may include:

* Swelling, redness, or pain in the lips, gums, or other areas of the face
* Cracks or fissures in the corners of the mouth (angular cheilitis)
* Difficulty swallowing or speaking due to swelling in the throat or tongue
* Recurrent mouth ulcers or sores
* Nasal congestion or discharge

The diagnosis of orofacial granulomatosis is typically made based on a combination of clinical examination, medical history, and imaging studies such as MRI or CT scans. A biopsy may also be performed to confirm the presence of granulomas in the tissue. Treatment for orofacial granulomatosis depends on the underlying cause and may include medications to reduce inflammation, antibiotics to treat infections, or dietary modifications to avoid allergic triggers.

Churg-Strauss syndrome (CSS), also known as eosinophilic granulomatosis with polyangiitis (EGPA), is a rare autoimmune disorder characterized by inflammation of small- to medium-sized blood vessels (vasculitis) and the presence of eosinophils, a type of white blood cell. The syndrome typically affects multiple organ systems, including the respiratory tract, peripheral nerves, skin, heart, and kidneys.

The classic triad of symptoms includes asthma, allergies, and peripheral blood eosinophilia (high levels of eosinophils in the blood). Other common features include sinusitis, rhinitis, cough, shortness of breath, skin rashes, neuropathy (nerve damage), and cardiac involvement.

The exact cause of Churg-Strauss syndrome is not well understood, but it is believed to involve an abnormal immune response in genetically susceptible individuals. Treatment typically involves the use of immunosuppressive medications to control inflammation and prevent organ damage. Corticosteroids are often used as a first-line therapy, while other agents such as cyclophosphamide or rituximab may be added for more severe cases.

Systemic vasculitis is a group of disorders characterized by inflammation of the blood vessels (vasculitis) that can affect various organs and systems throughout the body. This condition can cause damage to the walls of the blood vessels, leading to narrowing, blockage, or weakening of the vessel walls, which can further result in reduced blood flow, tissue damage, and organ dysfunction.

The symptoms of systemic vasculitis depend on the severity and location of the affected blood vessels. They may include fever, fatigue, weight loss, joint pain, skin rashes or lesions, muscle weakness, nerve damage, and organ dysfunction such as kidney failure, lung disease, or gastrointestinal bleeding.

Systemic vasculitis can be caused by various factors, including infections, autoimmune diseases, medications, and underlying medical conditions. The diagnosis of systemic vasculitis typically involves a combination of physical examination, laboratory tests, imaging studies, and sometimes biopsy of the affected tissue. Treatment may include corticosteroids, immunosuppressive drugs, and other medications to control inflammation and prevent organ damage.

"Foreign bodies" refer to any object or substance that is not normally present in a particular location within the body. These can range from relatively harmless items such as splinters or pieces of food in the skin or gastrointestinal tract, to more serious objects like bullets or sharp instruments that can cause significant damage and infection.

Foreign bodies can enter the body through various routes, including ingestion, inhalation, injection, or penetrating trauma. The location of the foreign body will determine the potential for harm and the necessary treatment. Some foreign bodies may pass through the body without causing harm, while others may require medical intervention such as removal or surgical extraction.

It is important to seek medical attention if a foreign body is suspected, as untreated foreign bodies can lead to complications such as infection, inflammation, and tissue damage.

Retinal vasculitis is a medical condition characterized by inflammation of the blood vessels in the retina, which is the light-sensitive tissue located at the back of the eye. This condition can cause damage to the retina and may lead to vision loss if not treated promptly. The inflammation can affect both the small and large blood vessels in the retina and can occur as a result of various systemic diseases or infections, including autoimmune disorders, tuberculosis, syphilis, and toxoplasmosis. In some cases, retinal vasculitis may also be associated with uveitis, which is inflammation of the middle layer of the eye. Treatment typically involves addressing the underlying cause of the inflammation and may include corticosteroids or other immunosuppressive therapies to reduce inflammation and prevent further damage to the retina.

Anti-Neutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitis (AAV) is a group of autoimmune diseases characterized by inflammation and damage to small blood vessels, particularly capillaries, venules, and arterioles. The condition is named after the presence of ANCAs in the patient's serum, which are autoantibodies that target specific proteins in the neutrophil cytoplasm.

AAV includes several subtypes, including:

1. Granulomatosis with Polyangiitis (GPA, formerly known as Wegener's granulomatosis) - a form of AAV that typically affects the respiratory tract and kidneys, characterized by the presence of granulomas (clusters of inflammatory cells).
2. Microscopic Polyangiitis (MPA) - a form of AAV that primarily affects small vessels in various organs, such as the kidneys, lungs, and skin.
3. Eosinophilic Granulomatosis with Polyangiitis (EGPA, formerly known as Churg-Strauss syndrome) - a form of AAV that involves asthma, allergies, and eosinophilia (an increased number of eosinophils in the blood), along with vasculitis affecting various organs.

The exact cause of ANCA-Associated Vasculitis is not fully understood, but it is believed to involve an interplay between genetic factors, environmental triggers, and dysregulation of the immune system. The condition can lead to a wide range of symptoms depending on which organs are affected, including fever, fatigue, weight loss, joint pain, skin rashes, cough, shortness of breath, nosebleeds, and kidney problems. Treatment typically involves immunosuppressive medications to control inflammation and prevent further damage to the affected organs.

Inflammation is a complex biological response of tissues to harmful stimuli, such as pathogens, damaged cells, or irritants. It is characterized by the following signs: rubor (redness), tumor (swelling), calor (heat), dolor (pain), and functio laesa (loss of function). The process involves the activation of the immune system, recruitment of white blood cells, and release of inflammatory mediators, which contribute to the elimination of the injurious stimuli and initiation of the healing process. However, uncontrolled or chronic inflammation can also lead to tissue damage and diseases.

Vasculitis, Central Nervous System (CNS), refers to a group of disorders characterized by inflammation of blood vessels within the brain and/or spinal cord. This inflammation can cause damage to the blood vessel walls, leading to narrowing, blocking or weakening of the vessels, and in some cases, formation of aneurysms or rupture of the vessels.

The causes of CNS vasculitis are varied and can include infections, autoimmune diseases, medications, and unknown factors. The symptoms of CNS vasculitis depend on the severity and location of the inflammation, and may include headache, seizures, stroke-like symptoms (such as weakness or numbness in the face, arms, or legs), cognitive changes, and in severe cases, coma.

Diagnosis of CNS vasculitis typically involves a combination of clinical evaluation, imaging studies (such as MRI or angiography), and laboratory tests (including blood tests and analysis of cerebrospinal fluid). Treatment may involve corticosteroids, immunosuppressive medications, and/or other therapies aimed at reducing inflammation and preventing further damage to the blood vessels.

Orthopedics is a branch of medicine that deals with the prevention, diagnosis, and treatment of disorders of the musculoskeletal system, which includes the bones, joints, muscles, ligaments, tendons, and nerves. The goal of orthopedic care is to help patients maintain or restore their mobility, function, and quality of life through a variety of treatments, including medication, physical therapy, bracing, and surgery. Orthopedic surgeons are medical doctors who have completed additional training in the diagnosis and treatment of musculoskeletal conditions, and they may specialize in specific areas such as sports medicine, spine care, joint replacement, or pediatric orthopedics.

"Granulomatosis with polyangiitis (Wegener's): an alternative name for Wegener's granulomatosis". Annals of the Rheumatic ... leading to the eponymous name Wegener's granulomatosis or Wegener granulomatosis (English: /ˈvɛɡənər/). In 2011, the American ... Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis (WG), after the Nazi German physician ... "Vasculitis Foundation " Granulomatosis with Polyangiitis (GPA/Wegener's)". www.vasculitisfoundation.org. Archived from the ...
Sproul, E.E. (1963) Wegener's and Lethal Midline Granulomatosis. JAMA: The Journal of the American Medical Association, 177(13 ...
Carrington, C. B., & Liebow, A. A. (1966). Limited forms of angiitis and granulomatosis of Wegener's type. The American journal ... Liebow, A. A. (1973). The J. Burns Amberson lecture-pulmonary angiitis and granulomatosis. American Review of Respiratory ... Katzenstein, A. L. A., Carrington, C. B., & Liebow, A. A. (1979). Lymphomatoid granulomatosis. A clinicopathologic study of 152 ... Liebow, A. A. (1972). Lymphomatoid granulomatosis. California medicine, 116(5), 48. Bahadori, M., & Liebow, A. A. (1973). ...
Wegener's Granulomatosis is named for Friedrich Wegener, a Nazi physician). It was renamed to granulomatosis with polyangiitis ... Henry Asbury Christian Wegener's granulomatosis - Friedrich Wegener (This usage is now formally discouraged by professional ... Wegener's Nazi ties were discovered. Its referent varies by country (e.g., sideropenic dysphagia is Plummer-Vinson syndrome in ... The disease is now known as granulomatosis with polyangiitis.) Weil's disease - Adolf Weil Welander distal myopathy - Lisa ...
Woywodt, A.; E. L. Matteson (2006-08-03). "Wegener's Granulomatosis-Probing the Untold Past of the Man Behind the Eponym". ...
Wikidata () Fauci, Anthony S.; Haynes, Barton F.; Katz, Paul; Wolff, Sheldon M. (January 1983). "Wegener's Granulomatosis: ... He developed therapies for formerly fatal diseases such as polyarteritis nodosa, granulomatosis with polyangiitis, and ... members of the American Rheumatism Association ranked Fauci's work on the treatment of polyarteritis nodosa and granulomatosis ... lymphomatoid granulomatosis. In a 1985 Stanford University Arthritis Center Survey, ...
Bachmeyer, C; Halioua, B (May 2013). ""Granulomatosis with polyangiitis (Wegener's)" for "Wegener granulomatosis": dermatology ... "Granulomatosis with polyangiitis (Wegener's): an alternative name for Wegener's granulomatosis". Arthritis Rheum. 63 (4): 863-4 ... from the 1950s onwards it was generally referred to as Wegener's granulomatosis. Friedrich Wegener was born on 7 April 1907 in ... "Wegener's granulomatosis". Lancet. 22, 367(9519): 1362-1366. Lubitz, MG (February 2018). "Granulomatosis With Polyangiitis-A ...
Association of a syndrome resembling Wegener's granulomatosis with low surface expression of HLA class-I molecules. Lancet 354: ...
... frequencies of wegener's granulomatosis, polymorphic reticulosis, malignant lymphoma and other related conditions". ... LMG was thought to be a manifestation of three or four different diseases: the well-characterized disease of granulomatosis ... Since cases of LMG that were manifestations of granulomatosis with polyangiitis, a vascular inflammatory but not malignant ... cells infiltrating the midline tissues in cases of lethal midline granuloma that were not clearly diagnosed as granulomatosis ...
... (EC 3.4.21.76, leukocyte proteinase 3, leukocyte proteinase 4, Wegener's granulomatosis autoantigen, proteinase PR ...
British physician and researcher Friedrich Wegener (1907-1990), German pathologist (see granulomatosis with polyangiitis). ...
Wegener's granulomatosis (now known as granulomatosis with polyangiitis) Embolic (pulmonary, septic) Infection (anaerobes, ...
... an area in North London Wegener's granulomatosis, now known as granulomatosis with polyangiitis, is an inflammatory disease ...
... weber Friedrich Wegener, German pathologist - Wegener's granulomatosis (not discovered by him, now known as granulomatosis with ...
Weber-Parkes syndrome Weber-Sturge-Dimitri syndrome Weber-Christian disease Webster-Deming syndrome Wegener's granulomatosis ( ... now known as granulomatosis with polyangiitis) Wegmann-Jones-Smith syndrome Weil syndrome Weinstein-Kliman-Scully syndrome ...
... a Dutch media conglomerate Alfred Wegener Institute for Polar and Marine Research Wegener's granulomatosis, now known as ... Wegener may refer to: 29227 Wegener, a main-belt asteroid Wegener (lunar crater) Wegener (Martian crater) Wegener Range, an ... Wegener (born 1952), American psychologist Ulrich Wegener (1929-2017), German police officer Wilhelm Wegener (1895-1944), ... German singer Doris Inge Wegener Einar Mogens Wegener (1882-1931), birth name of transsexual pioneer Lili Elbe Emmy Wegener ( ...
... formerly known as Wegener granulomatosis, is a rare multisystem autoimmune disease of unknown etiology. Its hallmark features ... 2C formerly Wegener Granulomatosis)) and Granulomatosis with Polyangiitis (GPA, formerly Wegener Granulomatosis) What to Read ... Granulomatosis with Polyangiitis (GPA, formerly Wegener Granulomatosis) * Sections Granulomatosis with Polyangiitis (GPA, ... Granulomatosis with polyangiitis (Wegeners): an alternative name for Wegeners granulomatosis. Ann Rheum Dis. 2011. 70:704. [ ...
Limited prognostic value of changes in antineutrophil cytoplasmic antibody titer in patients with Wegeners granulomatosis ... titers with disease activity in patients with Wegeners granulomatosis (WG). Methods: One hundred six patients with WG had ...
Wegeners Granulomatosis Etanercept Trial Research Group: John H Stone, Gary S Hoffman, Janet T Holbrook, Curtis L Meinert, ... Methods: This study used the BVAS/WG data from the Wegeners Granulomatosis Etanercept Trial. The scoring frequencies of the 34 ... Assessment of the item selection and weighting in the Birmingham vasculitis activity score for Wegeners granulomatosis ... Objective: To assess the Birmingham Vasculitis Activity Score for Wegeners Granulomatosis (BVAS/WG) with respect to its ...
Two cases of Wegeners granulomatosis are described in which the early and predominating lesions were found in the breast. The ...
Understanding Wegeners Granulomatosis and Granulomatosis with Polyangiitis. Wegeners Granulomatosis and Granulomatosis with ... Differentiating between Wegeners Granulomatosis and Granulomatosis with Polyangiitis. Wegeners Granulomatosis and ... Wegeners Granulomatosis, Granulomatosis with Polyangiitis: Symptoms, Causes, Treatment, and More. FacebookTwitterLinkedIn ... Causes of Wegeners Granulomatosis and Granulomatosis with Polyangiitis. The exact cause of GPA is unknown, but it is believed ...
Re: GRANULOMATOSIS DE WEGENER Anonimo 30/05/13 06:27 AM Re: mi hermano tiene GRANULOMATOSIS DE WEGENER Anonimo 03/09/12 03:31 ... Autoinmune = GRANULOMATOSIS DE WEGENER Anonimo 26/12/16 09:59 PM Re: mi madre tiene GRANULOMATOSIS DE WEGENER Anonimo 28/02/08 ... Re: mi madre tiene GRANULOMATOSIS DE WEGENER [Re: Anonimo] An nimo No registrado a mi hija con 14 a os la diagnosticaron ... Probable Granulomatosis de Wegener bajo que ex men Dr. Tox 30/10/14 02:44 AM ...
Background: Upper airway compromise due to tracheobronchial stenosis commonly occurs in patients with Wegeners granulomatosis ... Although our paper argues against the use of any stents in Wegeners, we have discussed a proviso that if stenting does need to ... of endoscopic surgery and intralesional steroid therapy for airway compromise due to tracheobronchial Wegeners granulomatosis ... of endoscopic surgery and intralesional steroid therapy for airway compromise due to tracheobronchial Wegeners granulomatosis ...
... formerly known as Wegener granulomatosis, is a rare multisystem autoimmune disease of unknown etiology. Its hallmark features ... 2C formerly Wegener Granulomatosis)) and Granulomatosis with Polyangiitis (GPA, formerly Wegener Granulomatosis) What to Read ... Granulomatosis with Polyangiitis (GPA, formerly Wegener Granulomatosis) * Sections Granulomatosis with Polyangiitis (GPA, ... Granulomatosis with polyangiitis (Wegeners): an alternative name for Wegeners granulomatosis. Ann Rheum Dis. 2011. 70:704. [ ...
Friedrich Wegener, who described the disease in 1936. Wegeners granulomatosis is part of a larger group of vasculitic ... Wegeners granulomatosis is a form of vasculitis that affects the lungs, kidneys and other organs. Due to its end-organ damage ... Although Wegeners granulomatosis affects small and medium-sized vessels, it is formally classified as one of the small vessel ... Wegeners granulomatosis is usually only suspected when a patient has had unexplained symptoms for a long period of time. ...
The aim of our poster is to present several cases of Wegeners granulomatosis (WG) patients with head and neck manifestations ... lymphomatoid granulomatosis (LYG), and excessive use of intranasal corticosteroids. ...
Orbital socket contracture: a complication of inflammatory orbital disease in patients with Wegeners granulomatosis ... Orbital socket contracture: a complication of inflammatory orbital disease in patients with Wegeners granulomatosis ... Orbital socket contracture: a complication of inflammatory orbital disease in patients with Wegeners granulomatosis ...
... G. Di Comite, E.P. Bozzolo, L. Praderio ... ABSTRACT: Meningeal involvement is a rare occurrence in Wegener`s Granulomatosis (WG). A Medline search uncovered only 48 ... Histology typically shows necrotizing granulomatosis. Meningeal involvement is by farmore frequent in the setting of localized ... be recognized by a constellation of clinical and radiological findings and by histological signs of necrotizing granulomatosis ...
The ERS-education website provides centralised access to all educational material produced by the European Respiratory Society. It is the worlds largest CME collection for lung diseases and treatment offering high quality e-learning and teaching resources for respiratory specialists. This distance learning portal contains up-to-date study material for the state-of-the-art in Pulmonology.
A serum proteomic approach to gauging the state of remission in Wegeners granulomatosis.. Publication , Journal Article ... "A serum proteomic approach to gauging the state of remission in Wegeners granulomatosis." Arthritis Rheum, vol. 52, no. 3, Mar ... "A serum proteomic approach to gauging the state of remission in Wegeners granulomatosis." Arthritis Rheum 52, no. 3 (March ... Stone, J. H., Rajapakse, V. N., Hoffman, G. S., Specks, U., Merkel, P. A., Spiera, R. F., … Wegeners Granulomatosis Etanercept ...
Wegener`s granulomatosis is a vasculitis that usually begins as a localized granulomatous inflammation of the upper and/or ... Primary systemic vasculitis - Wegener`s granulomatosis: a case report and short overview. Authors: ... On the basis of repeated histological investigations, Wegener`s granulomatosis was suspected. The diagnosis was confirmed at ...
Dive into the research topics of Autoantibody diagnosis in Wegeners granulomatosis. Together they form a unique fingerprint ... against myelomonocytic cells were demonstrable in 54 of 72 patients with Wegeners granulomatosis (WG). Other autoantibodies ( ... against myelomonocytic cells were demonstrable in 54 of 72 patients with Wegeners granulomatosis (WG). Other autoantibodies ( ... against myelomonocytic cells were demonstrable in 54 of 72 patients with Wegeners granulomatosis (WG). Other autoantibodies ( ...
Wegeners granulomatosis. In this granulomatous vasculitis of unknown cause, fever, weight loss and fatigue accompany ...
Wegeners granulomatosis. Share on Pinterest. One of the symptoms of Wegeners granulomatosis is eye pain.. ... Wegeners granulomatosis causes inflammation and injury to blood vessels and affects several organs including the lungs, ...
By using our site, you agree to our Terms and Conditions and Privacy Policy. Medsurgeindia does not provide medical advice, diagnosis, or treatment. The information provided on this site is designed to support, not replace, the relationship that exists between a patient/site visitor and his/her existing physician. ...
Granulomatosis with Polyangiitis Wegeners Granulomoatsis Support Group Help Information ... Welcome to the Wegeners Granulomatosis/GPA Disease Support Forums. We hope you find lots of valuable information here. There is ...
Granulomatosis with Polyangiitis Wegeners Granulomoatsis Support Group Help Information ... Welcome to the Wegeners Granulomatosis/GPA Disease Support Forums. We hope you find lots of valuable information here. There is ...
... and treatment options for Wegeners Granulomatosis, a form of granulomatosis with polyangiitis (GPA). ... "Diagnosis Process for Wegeners Granulomatosis". Diagnosing Wegeners granulomatosis, also known as polyangiitis, can be a ... FAQ 1: What are some common symptoms of Wegeners Granulomatosis?. Wegeners Granulomatosis, as detailed by et al in the ... "Symptoms and Causes of Wegeners Granulomatosis". Common Symptoms Associated with GPA. Wegeners Granulomatosis, also known as ...
Memingeal involvement in Wegener granulomatosis. / Specks, Ulrich; Moder, Kevin G.; McDonald, Thomas J. In: Mayo Clinic ... Specks, U., Moder, K. G., & McDonald, T. J. (2000). Memingeal involvement in Wegener granulomatosis. Mayo Clinic proceedings, ... Specks, U, Moder, KG & McDonald, TJ 2000, Memingeal involvement in Wegener granulomatosis, Mayo Clinic proceedings, vol. 75, ... Memingeal involvement in Wegener granulomatosis. Mayo Clinic proceedings. 2000;75(8):856-859. doi: 10.4065/75.8.856 ...
title = "A rare case of postmenopausal bleeding due to Wegeners granulomatosis",. keywords = "Wegeners granulomatosis, ... Ahson, GZ, Parkin, D, Gulliford, C & Ashok, PW 2002, A rare case of postmenopausal bleeding due to Wegeners granulomatosis, ... A rare case of postmenopausal bleeding due to Wegeners granulomatosis. G. Z. Ahson, David Parkin, Catherine Gulliford, Premila ... A rare case of postmenopausal bleeding due to Wegeners granulomatosis. / Ahson, G. Z.; Parkin, David; Gulliford, Catherine et ...
Such as symptoms, causes, diagnosis and treatment of wegeners granulomatosis. ... Read everything you need to know about granulomatosis with polyangiitis here. ... Symptoms of wegeners granulomatosis. Symptoms of wegeners granulomatosis. wegeners granulomatosis symptoms - Granulomatosis ... Wegeners granulomatosis is not a contagious disease.. How rare is wegeners granulomatosis?. Wegeners granulomatosis is a ...
... formerly known as Wegener granulomatosis, is a rare multisystem autoimmune disease of unknown etiology. Its hallmark features ... encoded search term (Granulomatosis with Polyangiitis (Wegener Granulomatosis)) and Granulomatosis with Polyangiitis (Wegener ... Granulomatosis with polyangiitis (Wegeners): an alternative name for Wegeners granulomatosis. Ann Rheum Dis. 2011. 70:704. [ ... Limited versus severe Wegeners granulomatosis: baseline patient data on patients in the Wegeners granulomatosis etanercept ...
... formerly known as Wegener granulomatosis, is a rare multisystem autoimmune disease of unknown etiology. Its hallmark features ... encoded search term (Granulomatosis with Polyangiitis (Wegener Granulomatosis)) and Granulomatosis with Polyangiitis (Wegener ... Granulomatosis with polyangiitis (Wegeners): an alternative name for Wegeners granulomatosis. Ann Rheum Dis. 2011. 70:704. [ ... Limited versus severe Wegeners granulomatosis: baseline patient data on patients in the Wegeners granulomatosis etanercept ...
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Wegeners granulomatosis) is a type of vasculitis which can be categorized as a sub-branch of anti-neutrophil cytoplasmic ... 1990 classification criteria for Wegeners granulomatosis. A total of 59 GPA-diagnosed patients (55.9% male, 44.1% female) were ... The medical records of patients admitted to the Department and Clinic of Rheumatology who were diagnosed with granulomatosis ... This study examined the demographic, clinical, radiographic and laboratory characteristics of patients with granulomatosis with ...
  • Granulomatosis with polyangiitis (GPA), formerly known as Wegener granulomatosis, is a rare multisystem autoimmune disease of unknown etiology. (medscape.com)
  • Necrotic, purpuric, and blistering plaque on the wrist in a patient with granulomatosis with polyangiitis. (medscape.com)
  • If you or a loved one has been diagnosed with Wegener's Granulomatosis or Granulomatosis with Polyangiitis (GPA), it's important to learn as much as possible about this chronic autoimmune disease in order to better manage your symptoms and treatment plan. (articleinsider.com)
  • Wegener's Granulomatosis and Granulomatosis with Polyangiitis are two different names for the same disease, which is an autoimmune disorder that primarily affects the blood vessels in the lungs, kidneys, and upper respiratory tract. (articleinsider.com)
  • Wegener's Granulomatosis and Granulomatosis with Polyangiitis are essentially the same disease, with the latter being the more modern name for the condition. (articleinsider.com)
  • Many trials include data on pooled groups of people with wegener's granulomatosis and microscopic polyangiitis. (checkorphan.org)
  • Wegener's Granulomatosis, also known as Granulomatosis with Polyangiitis (GPA), is an autoimmune disease that comes along with a range of symptoms, including glomerulonephritis and associated vasculitis. (drmaggieyu.com)
  • When your immune system goes rogue and starts attacking your own body in granulomatosis with polyangiitis (GPA), it results in inflammation similar to arthritis rheum, manifesting as a disease. (drmaggieyu.com)
  • This inflammation, often seen in granulomatosis with polyangiitis and arthritis, leads to granulomas (or small nodules) forming in various organs which can cause all those different symptoms we talked about earlier related to these diseases. (drmaggieyu.com)
  • Essentially, it's not just about the strauss syndrome, but how it triggers an inflammatory response causing multiple side effects, including arthritis, polyangiitis, granulomatosis, and associated vasculitis. (drmaggieyu.com)
  • Diagnosing Wegener's granulomatosis, also known as polyangiitis, can be a tricky business, given the wide range of associated vasculitis symptoms including disease manifestations like arthritis, and their variability. (drmaggieyu.com)
  • They'll examine for signs of systemic vasculitis, polyangiitis, arthritis and other symptoms linked to Wegener's granulomatosis, a blood disease. (drmaggieyu.com)
  • Wegener's granulomatosis also called granulomatosis with polyangiitis is a chronic, inflammatory disease of the blood vessels with small, knot-like thickening of the skin (granulomas). (healthtwentyfour.com)
  • Read everything you need to know about granulomatosis with polyangiitis here. (healthtwentyfour.com)
  • Granulomatosis with polyangiitis is a systemic disease. (healthtwentyfour.com)
  • Until 2011, granulomatosis with polyangiitis was known as Morbus Wegener (also known as Wegener's granulomatosis or Wegener's granulomatosis). (healthtwentyfour.com)
  • wegener's granulomatosis symptoms - Granulomatosis with polyangiitis can affect different organ systems. (healthtwentyfour.com)
  • Starting from the nose, granulomatosis with polyangiitis (Wegener's disease) can spread further into the paranasal sinuses and cause inflammation there (inflammation of the paranasal sinuses, sinusitis). (healthtwentyfour.com)
  • In extreme cases, granulomatosis with polyangiitis can even lead to deafness. (healthtwentyfour.com)
  • Throat: Hoarseness, dysphagia or a dry cough appear when the granulomatosis with polyangiitis spreads in the throat and pharynx. (healthtwentyfour.com)
  • Eyes: Around 50 percent of those affected suffer from eye pain, burning eyes, eye inflammation or visual disturbances (loss of vision) during the course of granulomatosis with polyangiitis (Wegener's disease). (healthtwentyfour.com)
  • Granulomatosis with polyangiitis (Wegener's granulomatosis) is a type of vasculitis which can be categorized as a sub-branch of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides. (rheumres.org)
  • This study examined the demographic, clinical, radiographic and laboratory characteristics of patients with granulomatosis with polyangiitis (GPA) in Imam Reza Hospital, Mahshad, Iran. (rheumres.org)
  • The medical records of patients admitted to the Department and Clinic of Rheumatology who were diagnosed with granulomatosis with polyangiitis between January 1, 2006 and December 31, 2016 were retrospectively studied. (rheumres.org)
  • Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis, is a type of vasculitis which can be categorized as a sub-branch of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides [1]. (rheumres.org)
  • In January 2011, Wegener granulomatosis was renamed granulomatosis with polyangiitis (GPA) because of Dr. Wegener's connection with the Nazi regime [3, 4]. (rheumres.org)
  • Granulomatosis with polyangiitis is the third most common type of vasculitis in Iran after Behcet's disease and cutaneous leukocytoclastic angiitis [6]. (rheumres.org)
  • Granulomatosis with Polyangiitis (GPA) (Wegener's Granulomatosis) and Microscopic Polyangiitis (MPA) in adult patients in combination with glucocorticoids ( 1.4 ). (drugs.com)
  • Polyarteritis nodosa, granulomatosis with polyangiitis, Henoch-Schönlein purpura, scleroderma, and otherwise nonspecified vasculitides also were reported to have caused ESRD during this period. (medscape.com)
  • Churg-Strauss Syndrome (CSS), now also referred to by its medically more accurate term eosinophilic granulomatosis with polyangiitis (EGPA), is a rare systemic vasculitis (inflammation in the wall of blood vessels of the body), predominantly affecting small-sized vessels. (vasculitisfoundation.org)
  • Because antineutrophil cytoplasmic auto-antibodies (ANCA) can be detected in the serum (liquid and a cellular part of the blood) in up to 40% of the patients, it is also considered one of the three ANCA-associated vasculitides, along with granulomatosis with polyangiitis (Wegener's) and microscopic polyangiitis. (vasculitisfoundation.org)
  • Granulomatosis with polyangiitis (GPA) (formerly known as Wegener's granulomatosis) is an uncommon disease affecting the sinuses, nose and lungs and frequently involves the kidneys, lungs, eyes, ears, throat, skin and other body organs. (vasculitisfoundation.org)
  • Granulomatosis with polyangiitis (Wegener's): an alternative name for Wegener's granulomatosis. (ox.ac.uk)
  • Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis (WG), after the Nazi German physician Friedrich Wegener, is a rare long-term systemic disorder that involves the formation of granulomas and inflammation of blood vessels (vasculitis). (wikipedia.org)
  • Granulomatosis with polyangiitis is usually suspected only when a person has had unexplained symptoms for a long period of time. (wikipedia.org)
  • Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis, is a rare disease. (medlineplus.gov)
  • Granulomatosis with polyangiitis is characterized by necrotizing granulomatous inflammation, small- and medium-sized vessel vasculitis, and focal necrotizing glomerulonephritis, often with crescent formation. (merckmanuals.com)
  • A model to predict cardiovascular events in patients with newly diagnosed wegener's granulomatosis and microscopic polyangiitis. (ox.ac.uk)
  • To create a prognostic tool to quantify the 5 year cardiovascular (CV) risk in patients with newly diagnosed Wegener's granulomatosis (WG) and microscopic polyangiitis (MPA) without pre-morbid CV disease. (ox.ac.uk)
  • To assess the Birmingham Vasculitis Activity Score for Wegener's Granulomatosis (BVAS/WG) with respect to its selection and weighting of items. (nih.gov)
  • Wegener's granulomatosis is a form of vasculitis that affects the lungs, kidneys and other organs. (checkorphan.org)
  • It may be recognized by a constellation of clinical and radiological findings and by histological signs of necrotizing granulomatosis, with little or no vasculitis. (clinexprheumatol.org)
  • Wegener`s granulomatosis is a vasculitis that usually begins as a localized granulomatous inflammation of the upper and/or lower respiratory tract mucosa. (eestiarst.ee)
  • Wegener's Granulomatosis (GPA ), a form of systemic vasculitis , is not a battle we fight alone, but one that requires awareness and understanding. (drmaggieyu.com)
  • By delving deeper into Wegener's Granulomatosis, an associated vasculitis and autoimmune disease often studied in rheumatology, we can arm ourselves with knowledge about this form of glomerulonephritis and contribute to a future where this illness is less daunting. (drmaggieyu.com)
  • Wegener's granulomatosis is a rare inflammation of the blood vessels ( vasculitis ) accompanied by the formation of small tissue nodules in the affected areas. (healthtwentyfour.com)
  • Histopathologic examination of incisional biopsies supported a diagnosis of Wegener's granulomatosis, an autoimmune vasculitis previously unreported in the veterinary literature. (ncsu.edu)
  • It is named after Dr. Friedrich Wegener, who described the disease in 1936. (checkorphan.org)
  • The name change recommended by American and European rheumatism associations is based on the controversial role of the former namesake Friedrich Wegener during the National Socialist era. (healthtwentyfour.com)
  • Originally, the disease was named for Friedrich Wegener who discussed the disorder in 1936 [2]. (rheumres.org)
  • This study used the BVAS/WG data from the Wegener's Granulomatosis Etanercept Trial. (nih.gov)
  • The model was tested using the Wegener's Granulomatosis Etanercept Trial (WGET) cohort. (ox.ac.uk)
  • The differential diagnosis of septal perforations associated with WG includes sarcoidosis, cocaine use, SLE, extranodal nasal lymphoma, lymphomatoid granulomatosis (LYG), and excessive use of intranasal corticosteroids. (egms.de)
  • Such as symptoms, causes, diagnosis and treatment of wegener's granulomatosis. (healthtwentyfour.com)
  • Diagnosis and management of Wegener's granulomatosis in a dog. (ncsu.edu)
  • Ludemann, G & Gross, WL 1987, ' Autoantibodies against cytoplasmic structures of neutrophil granulocytes in Wegener's granulomatosis ', Clinical and Experimental Immunology , vol. 69, no. 2, pp. 350-357. (uni-luebeck.de)
  • ABSTRACT: Meningeal involvement is a rare occurrence in Wegener`s Granulomatosis (WG). (clinexprheumatol.org)
  • We describe 2 cases and review the literature on the spectrum of clinical manifestations associated with meningeal involvement in patients with Wegener granulomatosis (WG). (elsevierpure.com)
  • In this case study, we represent a known case of Wegener's granulomatosis (WG), with concomitant sinusal mucormycosis mimicking vasculitic disease relapse, which was successfully treated with surgical debridement, amphotericine, and intravenous immunoglobuline. (ac.ir)
  • Mucormycosis may mimic disease relapse in Wegener's granulomatosis. (ac.ir)
  • On the basis of repeated histological investigations, Wegener`s granulomatosis was suspected. (eestiarst.ee)
  • Kidneys: The kidney vessels also become inflamed in around 50 percent of those affected by granulomatosis (glomerulonephritis). (healthtwentyfour.com)
  • Autoantibodies against cytoplasmic components of neutrophil granulocytes (ACPA) were detected in 18 of 32 patients with Wegener's granulomatosis (WG), but in none of the controls (n = 900), including patients with glomerulonephritis, sarcoidosis, tuberculosis, polyarteritis nodosa, and connective tissue diseases, and healthy blood donors. (uni-luebeck.de)
  • To assess the correlation and prognostic value of antineutrophil cytoplasmic antibody (cANCA) titers with disease activity in patients with Wegener's granulomatosis (WG). (nih.gov)
  • Upper airway compromise due to tracheobronchial stenosis commonly occurs in patients with Wegener's granulomatosis (WG). (bmj.com)
  • The aim of our poster is to present several cases of Wegener's granulomatosis (WG) patients with head and neck manifestations including epistaxis, nasal crusting, prolonged sinus infections with atypical microbiological causes and nasal polyposis. (egms.de)
  • OBJECTIVE: To identify serum ion patterns that distinguish remission from active disease in patients with Wegener's granulomatosis (WG). (duke.edu)
  • Anticytoplasmic antibodies (ACPA) against myelomonocytic cells were demonstrable in 54 of 72 patients with Wegener's granulomatosis (WG). (uni-luebeck.de)
  • Lungs, pleura, heart : In about 60 percent of Morbus Wegener patients, the disease affects the lungs after some time. (healthtwentyfour.com)
  • Patients were classified according to the American College of Rheumatology (ACR) 1990 classification criteria for Wegener's granulomatosis. (rheumres.org)
  • Patients were classified according to the American College of Rheumatology (ACR) 1990 classification criteria for Wegener's granulomatosis [8] and 2012 Revised International Chapel Hill Consensus Conference Nomenclature [1]. (rheumres.org)
  • The standard treatment for Wegener's granulomatosis is cyclophosphamide and high dose corticosteroids for remission induction and less toxic immunosuppressants like azathioprine, leflunomide, methotrexate or mycophenolate mofetil. (checkorphan.org)
  • Scholars@Duke publication: A serum proteomic approach to gauging the state of remission in Wegener's granulomatosis. (duke.edu)
  • Jr The American College of Rheumatology 1990 criteria for the classification of Wegener's granulomatosis. (ac.ir)
  • Two cases of Wegener's granulomatosis are described in which the early and predominating lesions were found in the breast. (bmj.com)
  • Expansion of circulating NKG2D+ effector memory T-cells and expression of NKG2D-ligand MIC in granulomaous lesions in Wegener's granulomatosis. (ox.ac.uk)
  • Wegener's granulomatosis is part of a larger group of vasculitic syndromes, all of which feature the presence of an abnormal type of circulating antibody termed ANCAs (antineutrophil cytoplasmic antibodies) and affect small and medium-size blood vessels. (checkorphan.org)
  • Although Wegener's granulomatosis affects small and medium-sized vessels, it is formally classified as one of the small vessel vasculitides in the Chapel Hill system. (checkorphan.org)
  • Welcome to the Wegeners Granulomatosis/GPA Disease Support Forums. (wegeners-granulomatosis.com)
  • Wegener's granulomatosis (WG) is an idiopathic systemic disease that usually onsets in adolescence and is rare in young children. (unipr.it)
  • If left untreated, granulomatosis later spreads to other organs, some of which are vital. (healthtwentyfour.com)
  • A 26 year old man with subacute hoarseness and stridor was shown to have Wegener's granulomatosis isolated to the trachea and larynx. (johnshopkins.edu)
  • These granulomas are the main reason for the appellation of "Wegener's granulomatosis," although it is not an essential feature. (checkorphan.org)
  • The term "granulomatosis" refers to the formed tissue nodules (= granulomas). (healthtwentyfour.com)
  • [ 1 ] A retrospective review of 34 cases of DAH revealed nearly one-third of the cases were caused by Wegener granulomatosis. (medscape.com)
  • Wegener`s granulomatosis and mucoromycosis: A case study and review of literature', Advanced Biomedical Research , 2012(July), pp. 1-4. (ac.ir)
  • It was formerly known as Wegener's granulomatosis. (medlineplus.gov)
  • This disorder is formerly known as Wegener granulomatosis. (nih.gov)
  • Das Ungewöhnliche bei unserem Patienten war die auf das Ohr beschränkte klinische Symptomatik mit Fazialisparese als Erstsymptom einer Wegener-Granulomatose ohne Mitbeteiligung der Luftwege und Nieren. (thieme-connect.com)
  • Isolierte einseitige Otitis mit Fazialisparese als Erstsymptom bei Wegener-Granulomatose. (thieme-connect.com)
  • Wegener's Granulomatosis (WG) is an autoimmune disease with manifestations in different organ systems. (nih.gov)
  • The purpose of this review is to give a systematic overview on Wegener's granulomatosis manifestations of the PNS and CNS and to highlight new findings regarding manifestations, diagnosis and therapy. (medscape.com)
  • In this review, we will give an overview of the clinical manifestations of the PNS and CNS in Wegener's granulomatosis and highlight new findings regarding neurological manifestations and current therapy strategies. (medscape.com)
  • Common and uncommon manifestations of Wegener granulomatosis at chest CT: radiologic-pathologic correlation. (medscape.com)
  • The lack of symptoms in the upper respiratory tract in our patient was unusual, indicating that in patients with recurrent otitis media, facial palsy, mastoiditis, or external otitis Wegener's granulomatosis should be ruled out as differential diagnosis. (thieme-connect.com)
  • Wegener's granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. (medscape.com)
  • 17. Detection and clinical implication of anti-neutrophil cytoplasm antibodies in Wegener's granulomatosis and rapidly progressive glomerulonephritis. (nih.gov)
  • The diagnosis of Wegener's granulomatosis is made on the basis of the clinical picture, serum cANCA, and histologic examination of biopsies. (thieme-connect.com)
  • Common symptoms of Wegener's granulomatosis in the nasal region include frequent nose bleeds and sinusitis. (jamespbradleymd.com)
  • Is PR3-ANCA formation initiated in Wegener's granulomatosis lesions? (nih.gov)
  • Pathogenesis of Wegener's granulomatosis. (nih.gov)
  • 10. Wegener's granulomatosis: Possible role of environmental agents in its pathogenesis. (nih.gov)
  • Wegener's granulomatosis correction in New York City can address the nasal manifestation of this rare autoimmune disorder. (jamespbradleymd.com)
  • Cite this: Neurological Involvement in Wegener's Granulomatosis - Medscape - Jan 01, 2011. (medscape.com)
  • Cindy has been fighting a rare disease for many years, Wegener's Granulomatosis. (iditarod.com)
  • Patient Talks About Nasal Reconstructive Surgery, Wegener's Granulomatosis Correction. (jamespbradleymd.com)
  • To correct this, Dr. Bradley will perform Wegener's granulomatosis correction, which can reconstruct the nasal bridge and repair any damaged nasal tissue. (jamespbradleymd.com)
  • You may be an ideal candidate for Wegener's granulomatosis correction if your nose has a pug-like appearance due to the collapse of the nasal bridge. (jamespbradleymd.com)
  • 11. Long-term follow-up of repair of external nasal deformities in patients with Wegener's granulomatosis. (nih.gov)
  • Acute Wegener's granulomatosis. (nih.gov)
  • 5. Increasing incidence of Wegener's granulomatosis in Sweden, 1975-2001. (nih.gov)
  • The main risk with Wegener's granulomatosis is kidney failure so it is important that the person be monitored on a regular basis. (medic8.com)
  • Coronary arteritis in Wegener's granulomatosis causing fatal myocardial infarction. (ox.ac.uk)
  • You have to be properly diagnosed with Wegener's granulomatosis before you can undergo treatment. (jamespbradleymd.com)
  • Dr. Lally has experience treating conditions like Temporal Arteritis, Wegener's Granulomatosis and Granulomatosis among other conditions at varying frequencies. (sharecare.com)