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Rheumatology > Vasculitis
Churg-Strauss Syndrome
Article Last Updated: Jan 5, 2007
AUTHOR AND EDITOR INFORMATION
Section 1 of 11  
Author: Mehran Farid-Moayer, MD, Adjunct Clinical Faculty, Department of Psychiatry, Sleep Disorders Clinic, Stanford Medical Center
Mehran Farid-Moayer is a member of the following medical societies: American College of Chest Physicians, American College of Physicians-American Society of Internal Medicine, American Medical Association, and American Thoracic Society
Coauthor(s):
Sandra L Sessoms, MD, Clinical Assistant Professor, Department of Medicine, Division of Immunology, Allergy and Rheumatology, Baylor College of Medicine
Editors: Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; Lawrence H Brent, MD, Associate Professor of Medicine, Thomas Jefferson University; Chair, Program Director, Department of Medicine, Division of Rheumatology, Albert Einstein Medical Center; Alex J Mechaber, MD, FACP, Assistant Dean for Medical Curriculum, Associate Professor of Medicine, Division of General Internal Medicine, University of Miami Miller School of Medicine; Herbert S Diamond, MD, Professor of Medicine, Temple University School of Medicine; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital
Author and Editor Disclosure
Synonyms and related keywords:
Churg-Strauss syndrome, CSS, allergic granulomatosis angiitis, granulomatous small-vessel vasculitis, syndrome of small- to medium-sized arteries and veins, antibodies to neutrophil cytoplasmic antigens, ANCA, asthma, wheezing, expiratory rhonchi, eosinophilia, paranasal sinusitis, pulmonary infiltrates, vasculitis with extravascular eosinophils, mononeuritis multiplex or polyneuropathy, Churg-Strauss–like syndrome, CSS-like syndrome, allergic rhinitis, eosinophilic pneumonia, gastroenteritis
Background
Churg-Strauss syndrome (CSS), or allergic granulomatous angiitis, is a rare syndrome that affects small- to medium-sized arteries and veins. Wegener granulomatosis (WG), Churg-Strauss syndrome, and the microscopic form of periarteritis (ie, microscopic polyangiitis) are 3 closely related vasculitic syndromes that affect medium- and small-sized vessels and are associated with antibodies to neutrophil cytoplasmic antigens (ANCAs).
In 1951, Churg and Strauss first described the syndrome in 13 patients who had asthma, eosinophilia, granulomatous inflammation, necrotizing systemic vasculitis, and necrotizing glomerulonephritis.
The American College of Rheumatology (ACR) has proposed 6 criteria for the diagnosis of Churg-Strauss syndrome. The presence of 4 or more criteria yields a sensitivity of 85% and a specificity of 99.7%. These criteria are (1) asthma (wheezing, expiratory rhonchi), (2) eosinophilia of more than 10% in peripheral blood, (3) paranasal sinusitis, (4) pulmonary infiltrates (may be transient), (5) histological proof of vasculitis with extravascular eosinophils, and (6) mononeuritis multiplex or polyneuropathy.
The recent Chapel Hill consensus conference on the classification of vasculitides did not modify the ACR criteria.
Pathophysiology
Churg-Strauss syndrome is a granulomatous small-vessel vasculitis. The cause of this allergic angiitis and granulomatosis is not known. No data have been reported regarding the role of immune complexes or cell-mediated mechanisms in this disease, although autoimmunity is evident with the presence of hypergammaglobulinemia, increased levels of immunoglobulin E (IgE), rheumatoid factor, and ANCA.
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) with reduction in their oral steroid dose. The Churg-Strauss syndrome–like complication is reported in people whose withdrawal of oral steroids is also facilitated by inhaled steroids. This complication is probably related to steroid withdrawal, which unmasks underlying Churg-Strauss syndrome, rather than to the drugs themselves. However, in rare cases, this syndrome has developed when a leukotriene receptor antagonist has been substituted for inhaled steroids without a history of oral steroid withdrawal.
Frequency
United States
Incidence is 1-3 cases per 100,000 adults per year.
International
Incidence is approximately 2.5 cases per 100,000 adults per year.
Mortality/Morbidity
- The principal causes of morbidity and mortality are myocarditis and myocardial infarction secondary to coronary arteritis.
- With treatment, the 1-year survival rate is 90% and the 5-year survival rate is 62%.
Sex
Males are affected slightly more frequently than females.
Age
The age at onset varies from 15-70 years, with a mean of approximately 38 years. Churg-Strauss syndrome in pediatric patients is well described, but mostly as case reports. The mean age at diagnosis is around 50 years.
History
Churg-Strauss syndrome has 3 phases—allergic rhinitis and asthma; eosinophilic infiltrative disease, such as eosinophilic pneumonia or gastroenteritis; and systemic medium- and small-vessel vasculitis with granulomatous inflammation. The vasculitic phase usually develops within 3 years of the onset of asthma, although it may be delayed for several decades. The most prominent symptoms and signs are those related to pulmonary, cardiac, dermatologic, renal, and peripheral nerve involvement. Mononeuritis multiplex is a major clinical finding. The following list includes the symptoms and signs of the disease as reported by Guillevin et al (1999) in his case series:
- Constitutional symptoms - Malaise, fatigue, flulike symptoms, weight loss (70%), fever (57%), myalgias (52%)
- Asthma symptoms (97%; Asthma may precede vasculitis by up to 10 years or, less frequently, coincide with the appearance of vasculitis. Asthma symptoms are usually persistent; therefore, patients are usually treated with steroids. This, in turn, might mask other features of the syndrome.)
- Paranasal sinusitis (61%) - Usually responds to oral steroids
- Allergic rhinitis (This is a common symptom. Additionally, recurrent sinusitis and polyposis are seen. But, unlike in Wegener granulomatosis, necrotizing lesions of the upper airway are unusual.)
- Pulmonary symptoms (37%), including cough and hemoptysis
- Arthralgias (40%)
- Skin manifestations (49%)
- Purpura
- Skin nodules
- Urticarial rash
- Necrotic bullae
- Digital ischemia
- Cardiac manifestations - Symptoms related to heart failure, myocarditis, pericarditis, constrictive pericarditis, and myocardial infarction
- Gastrointestinal symptoms (31%) - Symptoms related to GI vasculitis, eosinophilic gastritis, colitis (This includes abdominal pain [59%], diarrhea [33%], and GI bleeding [18%].)
- Peripheral neuropathy - Mononeuritis multiplex (most frequent form, occurring in as many as 77% of patients)
- Less frequent symptoms - Symptoms related to stroke, ophthalmologic involvement, and other rare symptoms
Physical
The physical findings are specific to organ-system involvement. Pulmonary involvement is the most prominent. In fact, a pneumonitis plus eosinophilia warrants consideration of this syndrome and a search for evidence of systemic vasculitis elsewhere. In addition to asthma and eosinophilia, a dermato-pulmonary-renal syndrome is the feature of this disease. Mononeuritis multiplex is common.
- Fever
- Skin involvement (60%)
- Leukocytoclastic angiitis with palpable purpura
- Livedo reticularis, skin necrosis and gangrene, digital ischemia, urticaria, and subcutaneous nodules
- Upper respiratory involvement
- Allergic rhinitis
- Paranasal sinusitis
- Nasal polyposis
- Lower respiratory system physical findings related to the following:
- Asthma (ie, wheeze), expiratory rhonchi
- Pneumonitis
- Hemoptysis secondary to pulmonary alveolar hemorrhage (alveolar capillaritis)
- Cardiovascular system
- Myocarditis and signs related to heart failure
- Myocardial infarction secondary to coronary vasculitis
- Renal system
- Hypertension
- Signs of uremia and advanced renal failure
- Gastrointestinal system
- GI bleeding
- Bowel ischemia and perforation
- Gastroenteritis
- Appendicitis
- Pancreatitis
- Nervous system
- Peripheral neuropathy (includes mononeuritis multiplex [77%])
- Central nervous system (includes stroke [5%])
Causes
Causes of Churg-Strauss syndrome are unknown. Churg-Strauss syndrome is possibly an allergic or autoimmune reaction to an environmental agent or drug.
Several case reports have described drug-induced forms of Churg-Strauss syndrome. Mesalazine-induced Churg-Strauss syndrome has been reported in a patient with Crohn disease and sclerosing cholangitis (Sinico, 2006); 4 publications have addressed the association between propylthiouracil, methimazole, and vasculitides, including Churg-Strauss syndrome. One report is available on the association of freebase cocaine and Churg-Strauss syndrome (Orriols, 1996).
Acute Mesenteric Ischemia
Antiglomerular Basement Membrane Disease
Asthma
Cryoglobulinemia
Eosinophilia
Eosinophilic Gastroenteritis
Eosinophilic Pneumonia
Glomerulonephritis, Crescentic
Goodpasture Syndrome
Hypereosinophilic Syndrome
Infective Endocarditis
Leukocytoclastic Vasculitis
Microscopic Polyangiitis
Pneumonia, Bacterial
Polyarteritis Nodosa
Wegener Granulomatosis
Other Problems to be Considered
Segmental glomerulonephritis
Acute nephritis
Allergic bronchopulmonary aspergillosis
Asthma
Other vasculitis syndromes (including Wegener granulomatosis)
Medication-induced eosinophilia
Other causes of pulmonary infiltration with eosinophilia (PIE)
Lab Studies
- Hematology
- Eosinophilia, usually at least 10% eosinophils (or 5000-9000 eosinophils/µL)
- Anemia
- Elevated erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels
- Renal tests
- Elevated serum BUN and creatinine levels in cases of renal involvement
- Abnormal urine sediment, proteinuria, microscopic hematuria, and RBC casts
- Antineutrophil cytoplasmic antibodies: 70% of patients are perinuclear-ANCA (p-ANCA)–positive (antimyeloperoxidase antibodies)
- Increased serum IgE levels
- Hypergammaglobulinemia
- Positive results for rheumatoid factor at low titer
- Other immunologic tests include the following:
- Levels of eosinophil cationic protein (ECP), soluble interleukin-2 receptor (sIL-2R), and soluble thrombomodulin (sTM), which is a marker of endothelial cell damage, are elevated.
- Elevated sIL-2R and ECP levels in Churg-Strauss syndrome indicate an immunoregulatory defect associated with vasculitis and eosinophilia.
- Eosinophilia in bronchioalveolar lavage (BAL), evident in 33% of cases
Imaging Studies
- Chest radiograph
- Pulmonary opacities can be found in 26-77% of cases, and films demonstrate no abnormalities in approximately 25% of patients.
- Localized parenchymal opacities usually are bilateral, peripheral, and patchy.
- Cavitation is rare.
- Pulmonary infiltrates may be transient. Occasionally, infiltrates are similar to those observed in patients with chronic eosinophilic pneumonia, or they may be nodular.
- Extensive air-space opacities in the setting of a drop in hemoglobin levels suggest massive intra-alveolar hemorrhage as a result of pulmonary alveolar capillaritis. Hemoptysis is present in only 45-66% of cases.
- Pleural effusions are observed in 5-30% of cases and can be eosinophilic.
- Hilar nodal enlargement has occasionally been reported.
- Computed tomography scan
- In the limited number of patients studied with CT scanning, findings include peripheral areas of parenchymal consolidation with ground-glass attenuation similar to that of chronic eosinophilic pneumonia.
- Much less commonly, parenchymal nodules (from 5 mm to 3.5 cm), with cavitation or air bronchograms, can be observed.
- Bronchial dilatation and bronchial wall thickening also may be visible.
- High-resolution CT scanning of chest produces findings that include significant enlargement of peripheral pulmonary arteries with stellate and irregular configuration—a vasculitis pattern.
- Other imaging studies are indicated for the complications of the disease and specific organ-system involvement, including abdominal CT scan for pancreatitis, coronary angiography for myocardial ischemia and infarction, and echocardiography for congestive heart failure (CHF).
Other Tests
- The following tests are for specific organ-system involvement:
- ECG for cardiac manifestations
- Gastrointestinal endoscopy for GI bleeding
- Electromyelogram (EMG) and nerve conduction studies for peripheral neuropathies
Procedures
- Biopsy: If local organ involvement exists, obtaining a biopsy of that organ is most helpful in confirming the diagnosis. However, if no localizing finding exists, obtaining nerve or muscle biopsy may be considered. Sural nerve biopsy is the most feasible procedure. In the case of renal involvement, kidney biopsy results may show focal or crescentic glomerulonephritis; however, this pathological change is consistent with, but not diagnostic of, Churg-Strauss syndrome.
- Skin
- Lung - Open or video-assisted thoracoscopic biopsy preferred over transbronchial
- Renal
- Nerve
- Muscle
Histologic Findings
The characteristic pathologic changes, found especially in the lung, include small necrotizing granulomas, as well as necrotizing vasculitis involving small arteries and venules. The granulomas are composed of a central eosinophilic core surrounded radially by macrophages and epithelioid giant cells. Churg-Strauss syndrome affects medium- and small-sized vessels.
Glomerulonephritis is not as common or severe as in Wegener granulomatosis, but, when present, it is usually focal and segmental and indistinguishable from other forms of so-called pauci-immune (without significant tissue deposition of immune complexes) glomerulonephritis.
Medical Care
Churg-Strauss syndrome is a systemic vasculitis that involves multiple organ systems; the type of support and care depends on the type of organ system involvement.
Consultations
- Consultation with a rheumatologist
- Specific organ-system involvement may require the following:
- Consultation with a pulmonologist for the management of hemoptysis and respiratory failure secondary to pulmonary alveolar capillaritis
- Consultation with a cardiologist for management of myocardial infarction, myocarditis, and heart failure
- Consultation with a nephrologist for the management of renal failure
- Consultations with a gastroenterologist and neurologist
Diet
Adjust nutritional requirements according to the particular clinical situation (eg, renal failure, heart failure).
Glucocorticoids alone are usually adequate for the treatment of Churg-Strauss syndrome. Cytotoxic drugs are necessary in fewer than 20% of patients. Case reports have described infliximab use in patients with steroid-dependent Churg-Strauss syndrome. Major life-threatening organ involvement may require treatment with pulse doses of intravenous corticosteroids and other cytotoxic agents. Other treatments include intravenous immune globulin, interferon alpha, rituximab, and plasma exchange. Plasma exchange, which has not improved the course of the disease, has been used but has not added benefit to the treatment of patients who were treated with prednisone or cyclophosphamide. This is based on a meta-analysis of 140 patients with glomerulonephritis and Churg-Strauss syndrome and microscopic polyangiitis. One case report is available on intravenous immune globulin for treatment of Churg-Strauss syndrome in pregnancy (Bonaci-Nikolic, 2005).
The laboratory tests that might correlate with disease activity include ESR and peripheral blood eosinophilia. ANCA levels do not correlate with disease activity.
Drug Category: Corticosteroids
These agents have anti-inflammatory properties and cause profound and varied metabolic effects. Corticosteroids modify the immune response to diverse stimuli.
| Drug Name | Prednisone (Deltasone, Orasone, Sterapred) |
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| Description | Immunosuppressant for treatment of autoimmune disorders. May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Oral prednisone is usually sufficient to control disease activity. |
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| Adult Dose | 0.5-1.5 mg/kg/d or 40-60 mg/d PO, taper over 6-12 wk as symptoms resolve; long-term low doses may be required indefinitely |
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| Pediatric Dose | 0.5-1 mg/kg/d PO initially and taper as in adults |
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| Contraindications | Documented hypersensitivity; severe bacterial, viral, or fungal infection; peptic ulcer disease; diabetes mellitus |
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| Interactions | Coadministration with estrogens or ketoconazole may decrease prednisone clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, aminoglutethimide, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics; increases toxicity of anticoagulants and theophylline; cholestyramine decreases effects; decreases effect of salicylates |
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| Pregnancy | B - Usually safe but benefits must outweigh the risks.
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| Precautions | Observe patients for weight increase, edema, hypertension, and excessive potassium excretion; abrupt discontinuation of glucocorticoids may cause adrenal crisis; monitor for negative nitrogen balance resulting from protein catabolism; other symptoms may include euphoria, insomnia, mood swings, personality changes, severe depression, and psychosis |
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Drug Category: Cytotoxic agents
These agents inhibit cell growth and proliferation. They are reserved for cases resistant to corticosteroids.
| Drug Name | Cyclophosphamide (Cytoxan, Neosar) |
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| Description | Chemically related to nitrogen mustards. As an alkylating agent, the mechanism of action of the active metabolites may involve cross-linking of DNA, which interferes with growth of rapidly proliferating cells. |
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| Adult Dose | 1-2 mg/kg/d PO
May also be administered in pulsed doses of 500-1000 mg/m2 IV qmo
Dose is adjusted according to clinical response, hematologic response, and toxicity
Single IV dose of 500 mg/m2 in patients with normal renal function and 350 mg/m2 in patients with CrCl of <10 is an alternative option for the short-term management of life-threatening events, including alveolar hemorrhage or gastrointestinal bleeding |
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| Pediatric Dose | Administer as in adults |
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| Contraindications | Documented hypersensitivity; active infection; severely depressed bone marrow function; severe cytopenias |
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| Interactions | Allopurinol may increase risk of bleeding or infection and enhance myelosuppressive effects of cyclophosphamide; may potentiate doxorubicin-induced cardiotoxicity; may reduce digoxin serum levels and antimicrobial effects of quinolones; chloramphenicol may increase half-life of cyclophosphamide while decreasing metabolite concentrations; may increase effect of anticoagulants; coadministration with high doses of phenobarbital may increase rate of metabolism and leukopenic activity of cyclophosphamide; thiazide diuretics may prolong cyclophosphamide-induced leukopenia, and neuromuscular effects of succinylcholine may be potentiated by IV cyclophosphamide |
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| Pregnancy | D - Unsafe in pregnancy
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| Precautions | Caution in leukopenia, thrombocytopenia, tumor cell infiltration of bone marrow, previous radiation therapy, and impaired renal function; alert anesthesiologist if treatment with cyclophosphamide is within 10 d of surgery; adjust dose prn in adrenalectomy (agent reported to be more toxic in adrenalectomized dogs); regularly examine hematologic profile (particularly neutrophils and platelets) to monitor for hematopoietic suppression; regularly examine urine for RBCs, which may precede hemorrhagic cystitis; increased risk of bladder cancer; slows wound healing |
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Further Inpatient Care
- Patients' cases must be followed up very closely at a rheumatology clinic or another specialty clinic. Patients usually need long-term immunosuppressive medications.
- The patient's clinical status, ESR, and eosinophilia should be monitored. The level of ANCA does not have a good correlation with disease activity.
In/Out Patient Meds
- Inpatient medications include the following:
- Prednisone: Start at 0.5-1 mg/kg/d PO.
- Methylprednisolone is administered in intravenous form at higher doses if major organ involvement, including cardiac, pulmonary (hemorrhage), renal, or neuropathy, is present.
- Cyclophosphamide is administered in patients with severe or life-threatening complications. The single intravenous dose is 500 mg per square meter of body-surface area. The dose should be reduced in patients with renal failure.
- Other medications include azathioprine, mycophenolate mofetil, and intravenous immune globulin administered in similar fashion as it is used in microscopic polyangiitis or Wegener granulomatosis.
- Plasmapheresis is probably not helpful.
- Interferon alpha might be helpful in treatment. Very little data are available on this subject.
Complications
- Complications of vasculitis depend on the specific organ system involvement.
Prognosis
- Overall, without treatment, the 5-year survival rate is about 25%.
- With treatment, the 1-year survival rate is 90% and the 5-year survival rate is 62%.
- Causes of death include the following:
- Cardiac failure, myocardial infarction, or both (most common cause)
- Renal failure
- Cerebral hemorrhage
- Gastrointestinal bleeding
- Status asthmaticus
Medical/Legal Pitfalls
- In all syndromes of vasculitis that involve medium- and small-sized vessels, including Churg-Strauss syndrome, recognizing the severity of the disease and avoiding overtreatment or undertreatment is prudent.
- Treatment with cyclophosphamide is associated with major adverse effects, including transitional cell carcinoma of the bladder, which should be considered in choosing the treatment plan.
| Media file 1:
Transient pulmonary infiltrates in a patient with Churg-Strauss syndrome (CSS). |
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| Media file 2:
Eosinophilic granuloma in a patient with Churg-Strauss syndrome (CSS). |
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Media type: Photo
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| Media file 3:
The skin rashes of Churg-Strauss syndrome. The biopsy of this rash showed eosinophilic leukocytoclastic angiitis with poorly formed granulomas. |
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Media type: Photo
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- Bonaci-Nikolic B, Nikolic MM, Andrejevic S, et al. Antineutrophil cytoplasmic antibody (ANCA)-associated autoimmune diseases induced by antithyroid drugs: comparison with idiopathic ANCA vasculitides. Arthritis Res Ther. 2005;7(5):R1072-81.
- Boyer D, Vargas SO, Slattery D, et al. Churg-Strauss syndrome in children: a clinical and pathologic review. Pediatrics. Sep 2006;118(3):e914-20.
- Chumbley LC, Harrison EG, DeRemee RA. Allergic granulomatosis and angiitis (Churg-Strauss syndrome). Report and analysis of 30 cases. Mayo Clin Proc. Aug 1977;52(8):477-84. [Medline].
- Churg J, Strauss L. Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol. Mar-Apr 1951;27(2):277-301. [Medline].
- Cohen P, Guillevin L, Baril L, et al. Persistence of antineutrophil cytoplasmic antibodies (ANCA) in asymptomatic patients with systemic polyarteritis nodosa or Churg-Strauss syndrome: follow-up of 53 patients. Clin Exp Rheumatol. Mar-Apr 1995;13(2):193-8. [Medline].
- Eustace JA, Nadasdy T, Choi M. Disease of the month. The Churg Strauss Syndrome. J Am Soc Nephrol. Sep 1999;10(9):2048-55. [Medline].
- Franco J, Artes MJ. Pulmonary eosinophilia associated with montelukast. Thorax. Jun 1999;54(6):558-60. [Medline].
- Green RL, Vayonis AG. Churg-Strauss syndrome after zafirlukast in two patients not receiving systemic steroid treatment. Lancet. Feb 27 1999;353(9154):725-6. [Medline].
- Guillevin L, Cohen P, Gayraud M, et al. Churg-Strauss syndrome. Clinical study and long-term follow-up of 96 patients. Medicine (Baltimore). Jan 1999;78(1):26-37. [Medline].
- Guillevin L, Lhote F, Gayraud M, et al. Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients. Medicine (Baltimore). Jan 1996;75(1):17-28. [Medline].
- Guillevin L, Cevallos R, Durand-Gasselin B, et al. Treatment of glomerulonephritis in microscopic polyangiitis and Churg-Strauss syndrome. Indications of plasma exchanges, Meta-analysis of 2 randomized studies on 140 patients, 32 with glomerulonephritis. Ann Med Interne (Paris). 1997;148(3):198-204. [Medline].
- Hellmich B, Ehlers S, Csernok E, Gross WL. Update on the pathogenesis of Churg-Strauss syndrome. Clin Exp Rheumatol. Nov-Dec 2003;21(6 Suppl 32):S69-77. [Medline].
- Jennette JC, Falk RJ, Andrassy K, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum. Feb 1994;37(2):187-92. [Medline].
- Kaushik VV, Reddy HV, Bucknall RC. Successful use of rituximab in a patient with recalcitrant Churg-Strauss syndrome. Ann Rheum Dis. Aug 2006;65(8):1116-7.
- Keogh KA, Specks U. Churg-Strauss syndrome: clinical presentation, antineutrophil cytoplasmic antibodies, and leukotriene receptor antagonists. Am J Med. Sep 2003;115(4):284-90. [Medline].
- Kim Y, Lee KS, Choi DC, et al. The spectrum of eosinophilic lung disease: radiologic findings. J Comput Assist Tomogr. Nov-Dec 1997;21(6):920-30. [Medline].
- Lanham JG, Elkon KB, Pusey CD, Hughes GR. Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome. Medicine (Baltimore). Mar 1984;63(2):65-81. [Medline].
- Lhote F, Guillevin L. Polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome. Clinical aspects and treatment. Rheum Dis Clin North Am. Nov 1995;21(4):911-47. [Medline].
- Lie JT. Histopathologic specificity of systemic vasculitis. Rheum Dis Clin North Am. Nov 1995;21(4):883-909. [Medline].
- Masi AT, Hunder GG, Lie JT, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum. Aug 1990;33(8):1094-100. [Medline].
- Orriols R, Munoz X, Ferrer J. Cocaine-induced Churg-Strauss vasculitis. Eur Respir J. Jan 1996;9(1):175-7.
- Schmitt WH, Csernok E, Kobayashi S, et al. Churg-Strauss syndrome: serum markers of lymphocyte activation and endothelial damage. Arthritis Rheum. Mar 1998;41(3):445-52. [Medline].
- Sinico RA, Sabadini E, Maresca AM. Mesalazine-induced Churg-Strauss syndrome in a patient with Crohn''s disease and sclerosing cholangitis. Clin Exp Rheumatol. Mar-Apr 2006;24(2 Suppl 41):S104.
- Specks U, DeRemee RA. Granulomatous vasculitis. Wegener''s granulomatosis and Churg-Strauss syndrome. Rheum Dis Clin North Am. May 1990;16(2):377-97. [Medline].
- Tatsis E, Schnabel A, Gross WL. Interferon-alpha treatment of four patients with the Churg-Strauss syndrome. Ann Intern Med. Sep 1 1998;129(5):370-4. [Medline].
- Tidman M, Olander R, Svalander C, Danielsson D. Patients hospitalized because of small vessel vasculitides with renal involvement in the period 1975-95: organ involvement, anti-neutrophil cytoplasmic antibodies patterns, seasonal attack rates and fluctuation of annual frequencies. J Intern Med. Aug 1998;244(2):133-41. [Medline].
- Tiliakos A, Shaia S, Hostoffer R. The use of infliximab in a patient with steroid-dependent churg-strauss syndrome. J Clin Rheumatol. Apr 2004;10(2):96-7.
- Todd DC, Cockcroft DW. Prolonged survival in Churg-Strauss syndrome. Ann Allergy Asthma Immunol. Jan 2004;92(1):92-3. [Medline].
- Tuggey JM, Hosker HS. Churg-Strauss syndrome associated with montelukast therapy. Thorax. Sep 2000;55(9):805-6. [Medline].
- Wechsler ME, Garpestad E, Flier SR, et al. Pulmonary infiltrates, eosinophilia, and cardiomyopathy following corticosteroid withdrawal in patients with asthma receiving zafirlukast. JAMA. Feb 11 1998;279(6):455-7. [Medline].
- Wechsler ME, Finn D, Gunawardena D, et al. Churg-Strauss syndrome in patients receiving montelukast as treatment for asthma. Chest. Mar 2000;117(3):708-13. [Medline].
- Worthy SA, Muller NL, Hansell DM, Flower CD. Churg-Strauss syndrome: the spectrum of pulmonary CT findings in 17 patients. AJR Am J Roentgenol. Feb 1998;170(2):297-300. [Medline].
Churg-Strauss Syndrome excerpt Article Last Updated: Jan 5, 2007
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