Churg-Strauss Syndrome
Vasculitis
Wegener Granulomatosis
Microscopic Polyangiitis
Polyarteritis Nodosa
Antibodies, Antineutrophil Cytoplasmic
Eosinophilic Granuloma
Systemic Vasculitis
Retinal Vasculitis
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
Inflammation
Vasculitis, Central Nervous System
MedlinePlus
Hypereosinophilic Syndrome
Eosinophils
Interleukin-5
Clinicopathological features of Churg-Strauss syndrome-associated neuropathy. (1/216)
We assessed the clinicopathological features of 28 patients with peripheral neuropathy associated with Churg-Strauss syndrome. Initial symptoms attributable to neuropathy were acute painful dysaesthesiae and oedema in the dysaesthetic portion of the distal limbs. Sensory and motor involvement mostly showed a pattern of mononeuritis multiplex in the initial phase, progressing into asymmetrical polyneuropathy, restricted to the limbs. Parallel loss of myelinated and unmyelinated fibres due to axonal degeneration was evident as decreased or absent amplitudes of sensory nerve action potentials and compound muscle action potentials, indicating acute massive axonal loss. Epineurial necrotizing vasculitis was seen in 54% of cases; infiltrates consisted mainly of CD8-positive suppressor/cytotoxic and CD4-positive helper T lymphocytes. Eosinophils were present in infiltrates, but in smaller numbers than lymphocytes. CD20-positive B lymphocytes were seen only occasionally. Deposits of IgG, C3d, IgE and major basic protein were scarce. The mean follow-up period was 4.2 years, with a range of 8 months to 10 years. Fatal outcome was seen only in a single patient, indicating a good survival rate. The patients who responded well to the initial corticosteroid therapy within 4 weeks regained self-controlled functional status in longterm follow-up (modified Rankin score was < or = 2), while those not responding well to the initial corticosteroid therapy led a dependent existence (P < 0.01). In addition the patients with poor functional outcomes had significantly more systemic organ damage caused by vasculitis (P < 0.05). Necrotizing vasculitis mediated by cytotoxic T cells, leading to ischaemic changes, appears to be a major cause of Churg-Strauss syndrome-associated neuropathy. The initial clinical course and the extent of systemic vasculitic lesions may influence the long-term functional prognosis. (+info)Pulmonary eosinophilia associated with montelukast. (2/216)
Antileukotriene drugs are new therapeutic agents that have recently been approved for the treatment of asthma. Several cases of eosinophilic conditions including Churg-Strauss syndrome have been reported to be associated with zafirlukast, a cysteinyl leukotriene type 1 receptor antagonist. So far no other leukotriene modifier has been associated with the syndrome. The case history is presented of a man with allergic rhinitis and asthma who had received intermittent pulse therapy with oral corticosteroids. Pulmonary eosinophilia developed while he was receiving treatment with montelukast, a chemically distinct cysteinyl leukotriene type 1 receptor antagonist. After discontinuation of montelukast therapy and administration of systemic corticosteroids the patient's symptoms reversed rapidly and there was prompt resolution of the pulmonary infiltrates. We believe that cysteinyl leukotriene type 1 receptor antagonists are safe and effective drugs for most patients with asthma but caution is needed for those with more severe disease who require systemic corticosteroids, especially if they show characteristics of the atypical allergic diathesis seen in the prodromal phase of Churg-Strauss syndrome. (+info)No association between neutrophil FcgammaRIIa allelic polymorphism and anti-neutrophil cytoplasmic antibody (ANCA)-positive systemic vasculitis. (3/216)
ANCA, implicated as having a pathogenic role in systemic vasculitis, can activate tumour necrosis factor-alpha (TNF-alpha)-primed neutrophils by cross-linking surface-expressed ANCA antigens with neutrophil FcgammaRIIa receptors to release reactive oxygen species. The FcgammaRIIa receptor exists as polymorphic variants, R131 and H131, which differ in their ability to ligate human IgG2 and IgG3. Neutrophils homozygous for the FcgammaRIIa-H131 allotype bind more efficiently to IgG3 than the FcgammaRIIa-R131 allotype and are the only human FcgammaR which bind IgG2. Our aim was to determine whether the homozygous FcgammaRIIa-H131 individuals are more susceptible to developing ANCA-associated systemic vasculitis and nephritis due to differential IgG binding and activation. FcgammaRIIa allotype was determined by both allele-specific polymerase chain reaction (PCR) and Southern blotting with allele-specific oligonucleotide probes end-labelled with 32P-gammaATP, after PCR amplification of genomic FcgammaRIIa DNA in 107 Caucasian patients with ANCA+ vasculitis (of whom 89 had renal disease) and 100 ethnically matched controls. Phenotyping of neutrophil FcgammaRIIa alleles was confirmed in some patients by quantitative flow cytometry using murine MoAbs 41H16 and IV.3. Of the patients with ANCA+ systemic vasculitis, 75 had ANCA with specificity for proteinase 3 and 32 with specificity for myeloperoxidase. Overall, no skewing in FcgammaRIIa allotypes was seen in patients compared with controls. No significant increase of the FcgammaRIIa-H131 allotype was found amongst patients irrespective of ANCA specificity, and no association between the FcgammaRIIa allotype and nephritis was found. Our data suggest that the FcgammaRIIa receptor allotype is not a major factor predisposing to the development of ANCA+ systemic vasculitis, or to nephritis. (+info)Inflammatory cells and cellular activation in the lower respiratory tract in Churg-Strauss syndrome. (4/216)
BACKGROUND: To obtain insight into the mechanisms of tissue injury in lung disease due to Churg-Strauss syndrome (CSS), the bronchoalveolar lavage (BAL) cell profile and the levels in the BAL fluid of cell products released by activated eosinophils and neutrophils were assessed. METHODS: Thirteen patients with active progressive CSS (n = 7) or CSS in partial remission (n = 6) underwent clinical staging and bronchoalveolar lavage. The levels of eosinophil cationic protein (ECP), myeloperoxidase (MPO), and peroxidase activity in the BAL fluid were determined and the results were compared with those of 19 patients with pulmonary active Wegener's granulomatosis (WG) and nine control subjects. RESULTS: In patients with progressive CSS the BAL cell profile was dominated by eosinophils, neutrophil elevation being the exception. The eosinophilia was associated with high ECP levels (4.39 ng/ml and 0. 40 ng/ml in the two CSS groups compared with unmeasurable values in the controls). Individual patients with highly active CSS also had raised MPO levels, comparable to the levels in the most active WG patients. Peroxidase activity in the BAL fluid was 1.26 U/ml and 0. 10 U/ml in the two groups of patients with CSS and 0.20 U/ml in the controls. Pulmonary disease in patients with WG was characterised by an extensive increase in MPO (0.30 ng/ml versus 0.13 ng/ml in the controls) together with high peroxidase activity in the BAL fluid (4. 37 U/ml), but only a small increase in ECP levels was seen. No correlation was found between the ECP and MPO levels in patients with CSS which suggests that eosinophil and neutrophil activation vary independently of each other. CONCLUSIONS: These findings suggest that, in addition to eosinophil activation, neutrophil activation is an important feature in some patients with highly active CSS. The balance of neutrophil and eosinophil involvement appears to be variable and this may be one explanation for the individually variable treatment requirements of patients with CSS. (+info)Disease of the month. The Churg Strauss Syndrome. (5/216)
The Churg Strauss Syndrome is an eosinophil-associated small vessel vasculitis. Although its pathogenesis may be distinctive and the association with severe late-onset asthma typical, the clinical features during the vasculitic phase widely overlap with those of the other forms of necrotizing vasculitis, and no single clinical or histologic feature is pathognomic of the condition. Renal involvement is common, although usually mild, and even when severe it tends to respond well to treatment. The prognosis for both patient and renal survival with adequate treatment is in general good. The optimal treatment strategy, however, is uncertain, and may differ from that for the other vasculitides. In particular, in contrast to Wegener's granulomatosis, the need for routine cyclophosphamide treatment is unconfirmed and requires further study. (+info)Involvement of soluble CD95 in Churg-Strauss syndrome. (6/216)
Deficiency of CD95 (Apo-1/Fas)-mediated apoptosis has recently been found in some autoimmune lymphoproliferative disorders due to inherited mutations of the CD95 gene. In this study, impairment of CD95 ligand-mediated killing of lymphocytes and eosinophils in Churg-Strauss Syndrome (CSS), which was a result of variation of CD95 receptor isoform expression, is demonstrated. Compared to those from healthy individuals, peripheral blood lymphocytes from eight CSS patients exhibit a switch from the membrane-bound CD95 receptor expression to its soluble splice variant, which protects from CD95L-mediated apoptosis. In five out of seven CSS patients recurrent oligoclonal T cell expansions were found, all using a Vbeta-gene from the Vbeta21 family associated with similar CDR3 motifs, indicating the predominance of T cell clones of a similar specificity in the CSS patients. In two of them, the effect of immunosuppressive therapy was studied. In both cases aberrant overexpression of the soluble CD95 receptor isoform and deviations from normal TCR Vbeta-gene usage normalized in parallel with the clinical improvement. Furthermore, soluble CD95 was identified as a survival factor for eosinophils rescuing eosinophils from apoptosis in the absence of growth factors in vitro. Given the role of eosinophils as effector cells in CSS, these findings suggest that soluble CD95 may be mechanistically involved in the disease. (+info)Subclinical alveolar bleeding in pulmonary vasculitides: correlation with indices of disease activity. (7/216)
Haemosiderin-laden alveolar macrophages are a common finding in patients with alveolar bleeding. Iron-positive macrophages, suggestive of subclinical alveolar bleeding, were found to be fairly common in bronchoalveolar lavage (BAL) fluid in primary systemic vasculitis but uncommon in collagen vascular diseases (CVDs) and rheumatoid arthritis (RA). To substantiate the impression that subclinical alveolar bleeding may be a feature distinguishing between these disorders, fibreoptic bronchoscopy and BAL were performed in 49 patients with active Wegener's granulomatosis or Churg-Strauss syndrome and 44 patients with CVDs or RA, all of them without clinically manifest alveolar bleeding. The percentage of iron-positive cells was compared with clinical and radiological findings. Only a minority of the CVD and RA patients had iron-positive alveolar macrophages; the 95th percentile of the median number of such cells was 5%. Fifty-three per cent of the patients in the vasculitis group had >5% iron-positive cells, with individual counts ranging up to 95%. Patients with iron-positive macrophages had more extensive disease, more frequent microhaematuria, a higher antineutrophil cytoplasmic antibody titre, a higher myeloperoxidase concentration in the BAL fluid and somewhat more frequent low-attenuation opacities in pulmonary high-resolution computed tomography than the patients with a low iron-positive cell count. In conclusion, subclinical alveolar bleeding was, indeed, a common finding in antineutrophil cytoplasmic antibody-associated vasculitis, which distinguished these disorders from lung disease due to collagen vascular diseases or rheumatoid arthritis. Its association with indices of disease activity, although weak in this cross-sectional study, merits a longitudinal study of its value for the long-term monitoring of vasculitis patients. (+info)An approach to diagnosis and initial management of systemic vasculitis. (8/216)
Systemic vasculitis occurs in a heterogeneous group of primary disorders or can be a manifestation of infection, an adverse drug reaction, malignancy or a connective tissue disease. A vasculitic process should be suspected in patients with unexplained ischemia or multiple organ involvement, especially when such features as polymyalgia rheumatica, inflammatory arthritis, palpable purpura, glomerulonephritis or multiple mononeuropathy are also present. The clinical features of systemic vasculitis depend on the organs involved and, in turn, organ involvement is largely influenced by the size of the affected blood vessels. The diagnostic work-up should be tailored to the clinical situation and geared toward a tissue or angiographic diagnosis, bearing in mind that the findings from these studies are not always pathognomonic. Emphasis should also be placed on exclusion of a secondary process. The diagnosis of the specific type of vasculitis may be made on the basis of the clinical features and the histopathologic or angiographic findings. Initial therapy for most types of systemic vasculitis consists of high-dose corticosteroids, with the addition of immunosuppressive therapy in certain patients. (+info)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.
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.
A syndrome, in medical terms, is a set of symptoms that collectively indicate or characterize a disease, disorder, or underlying pathological process. It's essentially a collection of signs and/or symptoms that frequently occur together and can suggest a particular cause or condition, even though the exact physiological mechanisms might not be fully understood.
For example, Down syndrome is characterized by specific physical features, cognitive delays, and other developmental issues resulting from an extra copy of chromosome 21. Similarly, metabolic syndromes like diabetes mellitus type 2 involve a group of risk factors such as obesity, high blood pressure, high blood sugar, and abnormal cholesterol or triglyceride levels that collectively increase the risk of heart disease, stroke, and diabetes.
It's important to note that a syndrome is not a specific diagnosis; rather, it's a pattern of symptoms that can help guide further diagnostic evaluation and management.
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.
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.
Polyarteritis nodosa (PAN) is a rare, systemic necrotizing vasculitis that affects medium-sized and small muscular arteries. It is characterized by inflammation and damage to the walls of the arteries, leading to the formation of microaneurysms (small bulges in the artery wall) and subsequent narrowing or complete occlusion of the affected vessels. This can result in tissue ischemia (reduced blood flow) and infarction (tissue death), causing a wide range of clinical manifestations that vary depending on the organs involved.
The exact cause of PAN remains unclear, but it is believed to involve an autoimmune response triggered by various factors such as infections or exposure to certain drugs. The diagnosis of PAN typically requires a combination of clinical findings, laboratory tests, and imaging studies, often supported by histopathological examination of affected tissues. Treatment usually involves the use of immunosuppressive medications to control inflammation and prevent further damage to the arteries and organs.
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.
Eosinophilic granuloma is a term used in pathology to describe a specific type of inflammatory lesion that is characterized by the accumulation of eosinophils, a type of white blood cell, and the formation of granulomas. A granuloma is a small nodular structure formed by the accumulation of immune cells, typically including macrophages, lymphocytes, and other inflammatory cells.
Eosinophilic granulomas can occur in various organs of the body, but they are most commonly found in the lungs, skin, and bones. In the lungs, eosinophilic granulomas are often associated with hypersensitivity reactions to inhaled antigens, such as dust mites or fungal spores. They can also be seen in association with certain diseases, such as Langerhans cell histiocytosis, an uncommon disorder characterized by the abnormal proliferation of a type of immune cell called Langerhans cells.
The symptoms of eosinophilic granuloma depend on the location and extent of the lesion. In the lungs, eosinophilic granulomas may cause cough, chest pain, or shortness of breath. In the skin, they may present as nodules, plaques, or ulcers. In the bones, they can cause pain, swelling, and fractures.
The diagnosis of eosinophilic granuloma is typically made based on a combination of clinical, radiological, and pathological findings. Treatment may include avoidance of known antigens, corticosteroids, or other immunosuppressive medications, depending on the severity and location of the lesion.
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.
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Hypereosinophilic Syndrome (HES) is a group of disorders characterized by persistent eosinophilia (an abnormal increase in the number of eosinophils, a type of white blood cell) leading to organ damage. The eosinophil count in the peripheral blood is typically greater than 1500 cells/μL. HES can affect various organs, including the heart, skin, nervous system, and digestive tract, causing symptoms such as shortness of breath, cough, fatigue, skin rashes, muscle weakness, and abdominal pain. The exact cause of HES is not fully understood, but it is thought to be related to abnormal production or activation of eosinophils. Treatment may include corticosteroids, immunosuppressive drugs, and targeted therapies that reduce eosinophil levels.
Eosinophilia is a medical condition characterized by an abnormally high concentration of eosinophils in the circulating blood. Eosinophils are a type of white blood cell that play an important role in the immune system, particularly in fighting off parasitic infections and regulating allergic reactions. However, when their numbers become excessively high, they can contribute to tissue damage and inflammation.
Eosinophilia is typically defined as a count of more than 500 eosinophils per microliter of blood. Mild eosinophilia (up to 1,500 cells/μL) may not cause any symptoms and may be discovered during routine blood tests. However, higher levels of eosinophilia can lead to various symptoms such as coughing, wheezing, skin rashes, and organ damage, depending on the underlying cause.
The causes of eosinophilia are varied and can include allergic reactions, parasitic infections, autoimmune disorders, certain medications, and some types of cancer. Accurate diagnosis and treatment of eosinophilia require identification and management of the underlying cause.
Eosinophils are a type of white blood cell that play an important role in the body's immune response. They are produced in the bone marrow and released into the bloodstream, where they can travel to different tissues and organs throughout the body. Eosinophils are characterized by their granules, which contain various proteins and enzymes that are toxic to parasites and can contribute to inflammation.
Eosinophils are typically associated with allergic reactions, asthma, and other inflammatory conditions. They can also be involved in the body's response to certain infections, particularly those caused by parasites such as worms. In some cases, elevated levels of eosinophils in the blood or tissues (a condition called eosinophilia) can indicate an underlying medical condition, such as a parasitic infection, autoimmune disorder, or cancer.
Eosinophils are named for their staining properties - they readily take up eosin dye, which is why they appear pink or red under the microscope. They make up only about 1-6% of circulating white blood cells in healthy individuals, but their numbers can increase significantly in response to certain triggers.
Interleukin-5 (IL-5) is a type of cytokine, which is a small signaling protein that mediates and regulates immunity, inflammation, and hematopoiesis. IL-5 is primarily produced by activated T cells, especially Th2 cells, as well as mast cells, eosinophils, and innate lymphoid cells (ILCs).
The primary function of IL-5 is to regulate the growth, differentiation, activation, and survival of eosinophils, a type of white blood cell that plays a crucial role in the immune response against parasitic infections. IL-5 also enhances the ability of eosinophils to migrate from the bone marrow into the bloodstream and then into tissues, where they can participate in immune responses.
In addition to its effects on eosinophils, IL-5 has been shown to have a role in the regulation of B cell function, including promoting the survival and differentiation of B cells into antibody-secreting plasma cells. Dysregulation of IL-5 production and activity has been implicated in several diseases, including asthma, allergies, and certain parasitic infections.
I'm sorry for any confusion, but "Gross Domestic Product" (GDP) is an economic term, not a medical one. GDP is the total monetary or market value of all the finished goods and services produced within a country's borders in a specific time period. It serves as a comprehensive measure of a nation’s overall economic activity.
If you have any medical questions or terms you would like defined, I would be happy to help!
Pulmonary eosinophilia is a condition characterized by an increased number of eosinophils, a type of white blood cell, in the lungs or pulmonary tissues. Eosinophils play a role in the body's immune response to parasites and allergens, but an overabundance can contribute to inflammation and damage in the lungs.
The condition may be associated with various underlying causes, such as:
1. Asthma or allergic bronchopulmonary aspergillosis (ABPA)
2. Eosinophilic lung diseases, like eosinophilic pneumonia or idiopathic hypereosinophilic syndrome
3. Parasitic infections, such as ascariasis or strongyloidiasis
4. Drug reactions, including certain antibiotics and anti-inflammatory drugs
5. Connective tissue disorders, like rheumatoid arthritis or Churg-Strauss syndrome
6. Malignancies, such as lymphoma or leukemia
7. Other less common conditions, like tropical pulmonary eosinophilia or cryptogenic organizing pneumonia
Symptoms of pulmonary eosinophilia can vary but often include cough, shortness of breath, wheezing, and chest discomfort. Diagnosis typically involves a combination of clinical evaluation, imaging studies, and laboratory tests, such as complete blood count (CBC) with differential, bronchoalveolar lavage (BAL), or lung biopsy. Treatment depends on the underlying cause and may include corticosteroids, antibiotics, or antiparasitic medications.
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Polyangiitis9
- Churg-Strauss syndrome is also called eosinophilic granulomatosis with polyangiitis, or EGPA. (webmd.com)
- Eosinophilic granulomatosis with polyangiitis (EGPA)-or, as it was traditionally termed, Churg-Strauss syndrome-is a rare systemic necrotizing vasculitis that affects small-to-medium-sized vessels and is associated with severe asthma and blood and tissue eosinophilia. (medscape.com)
- To determine the long-term outcome of patients with polyarteritis nodosa (PAN), microscopic polyangiitis (MPA), and Churg-Strauss syndrome (CSS), to compare the long-term outcome with the overall French population, to evaluate the impact on outcome of the type of vasculitis, prognostic factors, and treatments administered at diagnosis, and to analyze treatment side effects and sequelae. (nih.gov)
- The spectrum of AAV comprises granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA), the later formerly known as Churg-Strauss syndrome (CSS). (biomedcentral.com)
- Churg-Strauss syndrome, granulomatosis with polyangiitis (Wegener granulomatosis), and the microscopic form of periarteritis (ie, microscopic polyangiitis) are three closely related vasculitic syndromes that affect medium- and small-sized vessels and are associated with antibodies to neutrophil cytoplasmic antigens (ANCAs). (medscape.com)
- The authors examined a 28-year-old man with a previous diagnosis of eosinophilic granulomatosis with polyangiitis , formerly known as Churg-Strauss disease , who presented with bilateral orbital inflammation . (bvsalud.org)
- 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)
- Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): evolutions in classification, etiopathogenesis, assessment and management. (medscape.com)
- Given the patient's history of recurrent sinusitis, asthma, eosinophilia, and coronary aneurysms, we diagnosed eosinophilic granulomatosis with polyangiitis ( Churg-Strauss syndrome , CSS) and cardiac involvement, and recommended high-dose corticosteroid treatment. (medscape.com)
Granulomatosis2
- Churg A. Pulmonary angiitis and granulomatosis revisited. (medscape.com)
- Churg-Strauss syndrome (allergic angiitis or granulomatosis) is rare disorder marked by blood vessel inflammation. (drugs.com)
EGPA1
- An EGPA-like syndrome is a rare complication that develops in steroid-dependent patients with asthma who have their oral steroid dose reduced after they start treatment with a leukotriene receptor antagonist (eg, montelukast, zafirlukast). (medscape.com)
Eosinophilia7
- Leukotriene receptor antagonist-associated Churg-Strauss syndrome may occur in asthma patients being treated with leukotriene receptor antagonists, occurring 2 days to 10 months after the antagonist has been started, with features of the syndrome including peripheral eosinophilia, pulmonary infiltrates, and less commonly neuropathy, sinusitis, and cardiomyopathy. (wikipedia.org)
- In 1951, Churg and Strauss first described the syndrome in 13 patients who had asthma , eosinophilia , granulomatous inflammation, necrotizing systemic vasculitis, and necrotizing glomerulonephritis. (medscape.com)
- Churg-Strauss syndrome is a vasculitis accompanied by asthma and eosinophilia. (qxmd.com)
- [ 47 ] Also consider terbinafine-induced DRESS (drug reaction with eosinophilia and systemic symptoms) syndrome. (medscape.com)
- a syndrome of fleeting pulmonary findings and peripheral blood eosinophilia, is another eosinophilic pulmonary disease. (merckmanuals.com)
- Pulmonary opacities on chest imaging associated with blood eosinophilia are sometimes called PIE (pulmonary infiltrates with eosinophilia) syndrome. (merckmanuals.com)
- Systemic eosinophilia, sometimes presenting with clinical features of vasculitis consistent with Churg-Strauss syndrome, has been reported. (nih.gov)
Vasculitis5
- Necrotizing vasculitis mediated by cytotoxic T cells, leading to ischaemic changes, appears to be a major cause of Churg-Strauss syndrome-associated neuropathy. (lookformedical.com)
- Churg-Strauss syndrome is a granulomatous small-vessel vasculitis. (medscape.com)
- Churg-Strauss syndrome (CSS) is a granulomatous necrotizing vasculitis of unknown etiology associated with bronchial asthma. (elsevierpure.com)
- Churg-Strauss syndrome (CSS) is a rare form of systemic vasculitis occurring in patients with asthma. (uni-luebeck.de)
- Lung biopsies revealed elements characteristic of Churg-Strauss syndrome, including eosinophilic microabscesses and vasculitis. (qxmd.com)
Allergic2
- We believe that cysteinyl leukotriene type 1 receptor antagonists are safe and effective drugs for most patients with asthma but caution is needed for those with more severe disease who require systemic corticosteroids, especially if they show characteristics of the atypical allergic diathesis seen in the prodromal phase of Churg-Strauss syndrome. (lookformedical.com)
- Churg-Strauss syndrome (CSS), or allergic granulomatous angiitis, is a rare syndrome that affects small- to medium-sized arteries and veins. (medscape.com)
Asthma4
- It's also approved to treat eosinophilic asthma, a severe form of asthma that's linked to Churg-Strauss. (webmd.com)
- A Churg-Strauss syndrome-like syndrome develops as a rare complication in people with asthma who are steroid-dependent and who are treated with leukotriene receptor antagonists (eg, montelukast, zafirlukast) upon reduction in their oral steroid dose. (medscape.com)
- Asthma is a clinical syndrome characterized by episodic reversible airway obstruction, increased bronchial reactivity, and airway inflammation. (medscape.com)
- Worldwide, economic costs for asthma are more than those for tuberculosis and acquired immunodeficiency syndrome (AIDS) combined. (medscape.com)
Infiltrates1
- Transient pulmonary infiltrates in a patient with Churg-Strauss syndrome (CSS). (medscape.com)
Clinical4
- Keogh KA, Specks U. Churg-Strauss syndrome: clinical presentation, antineutrophil cytoplasmic antibodies, and leukotriene receptor antagonists. (medscape.com)
- Churg-Strauss syndrome in children: a clinical and pathologic review. (qxmd.com)
- Espana A, Sanz ML, Sola J, Gil P. Wells' syndrome (eosinophilic cellulitis): correlation between clinical activity, eosinophil levels, eosinophil cation protein and interleukin-5. (medscape.com)
- Clinical presentation, which may resemble Löffler syndrome, includes chronic eosinophilic pneumonia and eosinophilic gastroenteritis. (msdmanuals.com)
19511
- La granulomatose éosinophilique avec polyangéite (GEPA) ou syndrome de Churg-Strauss, est une vascularite rare décrite pour la première fois par Jacob Churg et Lotte Strauss en 1951. (bvsalud.org)
Pediatric4
- Churg-Strauss syndrome in pediatric patients is well described, but mostly as case reports. (medscape.com)
- Here we report 2 pediatric patients with Churg-Strauss syndrome manifesting with prominent pulmonary involvement. (qxmd.com)
- We report these cases to expand the scant knowledge about Churg-Strauss syndrome in pediatric patients and to heighten awareness that this serious disease may affect the pediatric population. (qxmd.com)
- Wells syndrome (eosinophilic cellulitis) following vaccination: Two pediatric cases with positive patch test to aluminium salts. (medscape.com)
Arteritis1
- The principal causes of morbidity and mortality in Churg-Strauss syndrome are myocarditis and myocardial infarction secondary to coronary arteritis. (medscape.com)
Peripheral2
- We assessed the clinicopathological features of 28 patients with peripheral neuropathy associated with Churg-Strauss syndrome. (lookformedical.com)
- Koga C, Sugita K, Kabashima K, Matsuoka H, Nakamura M, Tokura Y. High responses of peripheral lymphocytes to mosquito salivary gland extracts in patients with Wells syndrome. (medscape.com)
Eosinophils1
- People with Churg-Strauss syndrome make too many eosinophils, which cause inflammation and damage. (webmd.com)
Symptoms3
- This medicine can put your disease into remission, which is when you no longer have any symptoms or signs of Churg-Strauss syndrome. (webmd.com)
- There are three stages of Churg-Strauss syndrome, each with its own signs and symptoms, depending on which organs are affected. (arthritis-unplugged.com)
- There's no cure for Churg-Strauss syndrome, but certain medications may help even people with serious symptoms achieve remission. (drugs.com)
Steroid1
- However, in rare cases, this syndrome has developed when a leukotriene receptor antagonist has been substituted for inhaled steroids ini patients without a history of oral steroid withdrawal. (medscape.com)
Inflammation2
- Churg-Strauss syndrome causes inflammation all over the body. (webmd.com)
- Churg-Strauss Syndrome is a disorder that causes inflammation in blood vessels, which restricts blood flow to various organs. (arthritis-unplugged.com)
Likelihood1
- HLA-DRB4 positivity may be a genetic risk factor for the development of Churg-Strauss syndrome and may increase the likelihood of vasculitic manifestations of the disease. (medscape.com)
Infiltration1
- Massive Orbital Infiltration and Trigeminal Enlargement in Churg-Strauss Syndrome Associated With IgG4 Plasma Cell Positivity. (bvsalud.org)
Incidence2
- The incidence of Churg-Strauss syndrome in the United States is 1-3 cases per 100,000 adults per year. (medscape.com)
- The international incidence of Churg-Strauss syndrome is approximately 2.5 cases per 100,000 adults per year. (medscape.com)
Complication1
- [ 8 ] The Churg-Strauss syndrome-like complication is reported in people whose withdrawal of oral steroids is also facilitated by inhaled steroids. (medscape.com)
Organs1
- In Churg-Strauss syndrome, your immune system makes chemicals that damage your organs and tissues. (webmd.com)
Immune system1
- Churg-Strauss syndrome is likely due to an overzealous immune system response. (arthritis-unplugged.com)
Remission2
- The goal in treating Churg-Strauss syndrome is to put your disease into remission. (webmd.com)
- There's no cure for Churg-Strauss syndrome, but some medications may help you achieve remission. (arthritis-unplugged.com)
Cutaneous1
- [ 34 ] Cutaneous dirofilariasis may also clinically resemble Wells syndrome. (medscape.com)
Patients2
Affects1
- Churg-Strauss syndrome is an uncommon disorder, and it affects males and females equally. (arthritis-unplugged.com)
Rare1
- They'd been through turbulent years, with Watt suffering and finally recovering from a rare auto-immune condition, Churg-Strauss syndrome, that nearly ended his life. (djmag.com)
Cases5
- Several cases of eosinophilic conditions including Churg-Strauss syndrome have been reported to be associated with zafirlukast, a cysteinyl leukotriene type 1 receptor antagonist. (lookformedical.com)
- Two cases of formes frustes variants of Churg-Strauss syndrome are reported, who were treated with an antibody against immunoglobulin E as an addition on rescue therapy. (qxmd.com)
- The relevant literature on Churg-Strauss syndrome, with specific reference to childhood cases, is reviewed. (qxmd.com)
- Brehmer-Andersson E, Kaaman T, Skog E, Frithz A. The histopathogenesis of the flame figure in Wells' syndrome based on five cases. (medscape.com)
- Wells Syndrome in children and atopy: Retrospective study of 11 cases and review of the literature]. (medscape.com)
Zafirlukast2
- Discontinuation of zafirlukast could decrease dihydrocodeine plasma concentrations, decrease opioid efficacy, and potentially lead to a withdrawal syndrome in those with physical dependence to dihydrocodeine. (pdr.net)
- If zafirlukast is discontinued, hydrocodone plasma concentrations will decrease resulting in reduced efficacy of the opioid and potential withdrawal syndrome in a patient who has developed physical dependence to hydrocodone. (pdr.net)