Churg-Strauss vasculitis

Krause, T.H. Schumichen Check, A. Lang, B. Hohnloser, S. Moser, E. Scintigraphy using " in-labeled antimyosin in churg-strauss vasculitis with myocardial involvement. Nuklearmedizin 1990, 29, 177-179. 

Churg-Strauss vasculitis (allergic angiitis and granulomatosis) has been attributed to hepatitis B immunization... 

Vanoli M, Gambini D, Scorza R. A case of Churg-Strauss vasculitis after hepatitis B vaccination. Ann Rheum Dis 1998 57(4) 256-7. 

So far, we have only found MHC class Il-positive PMN in patients with active Wegener s granulomatosis, and in 3 patients with active Churg-Strauss vasculitis. Whether this indicates a certain disease specificity cannot be decided at this stage. A more reasonable explanation could be that all the patients with SLE or rheumatoid arthritis had received some type of immunosuppressive therapy, which would prevent upregulation of MHC class II. 

Churg-Strauss syndrome A systemic necrotizing vasculitis with prominent lung involvement including severe asthma and eosinophilia. 

Zafirlukast and montelukast are generally well tolerated. Rare elevations in serum aminotransferase concentrations and clinical hepatitis have been reported. An idiosyncratic syndrome similar to the Churg-Strauss syndrome, with marked circulating eosinophilia, heart failure, and associated eosinophilic vasculitis, has been reported in a small number of patients a direct causal association has not been established. 

Zafirlukast and montelukast are well tolerated. Zafirlukast increases plasma concentrations of warfarin and decreases the concentrations of theophylline and erythromycin. In rare cases, treatment of patients with CysLT receptor antagonists is associated with the development of Churg-Strauss syndrome, a condition marked by acute vasculitis, eosinophilia, and a worsening of pulmonary symptoms. Because these symptoms often appear when patients are given the leukotriene receptor antagonists when they are being weaned from oral corticosteroid therapy, it is not clear whether they are related to the action of the antagonists or are due to a sudden reduction in corticosteroid therapy. 

Of these agents, zileuton is the least prescribed because of reports of occasional liver toxicity. The receptor antagonists appear to have little toxicity. Reports of Churg-Strauss syndrome (a systemic vasculitis accompanied by worsening asthma, pulmonary infiltrates, and eosinophilia) appear to have been coincidental, with the syndrome unmasked by the reduction in prednisone dosage made possible by the addition of zafirlukast or montelukast. Of these two, montelukast is the most prescribed, probably because it can be taken without regard to meals and because of the convenience of once-daily treatment. 

Evans syndrome Churg-Strauss syndrome Necrotizing vasculitis Guillain-Barre syndrome... 

Table 1 Definitions of Wegener s Granulomatosis, Microscopic Polyangiitis, and Churg-Strauss Syndrome Adopted by the Chapel HiU Consensus Conference on the Nomenclature of Systemic Vasculitis...

Wegener s granulomatosis Microscopic polyangiitis Churg-Strauss syndrome Renal-limited vasculitis... 

Note that Wegener s granulomatosis limited to the upper respiratory tract has a lower frequency of ANCA (approximately 70%) compared to systemic disease, and Churg-Strauss syndrome with systemic vasculitis and glomerulonephritis has a higher frequency of ANCA than Churg-Strauss syndrome without glomerulonephritis. Abbreviations. ANCA, antineutrophil cytoplasmic autoantibodies MPO, myeloperoxidase PR3, proteinase 3. 

Lanham JG, Elkon KB, Pusey CD, et al. Systemic vasculitis with asthma and eosinophilia a clinical approach to the Churg-Strauss syndrome. Medicine (Baltimore) 1984 63(2) 65-81. 

Tai PC, Holt ME, Denny P, et al. Deposition of eosinophil cationic protein in granulomas in allergic granulomatosis and vasculitis the Churg-Strauss syndrome. Br Med J (Clin Res Ed) 1984 289(6442) 400-402. 

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