Churg-Strauss syndrome

CSS Churg-Strauss syndrome CT Computed tomography CTAP-III Connective tissueactivating peptide CTD Connective tissue diseases C terminus Carboxy terminus of peptide... 

If it is necessary to use systemic corticosteroids for long-term control therapy, once-daily or every-other-day therapy should be used and repeated attempts should be made to decrease the dose or discontinue the drug. Withdrawal of chronic therapy may precipitate adrenal failure or unmask underlying inflammatory disorders such as Churg-Strauss syndrome. 

Both of these agents have been associated with rare reports of Churg-Strauss syndrome. This syndrome may result from the corticosteroid dose reduction, as it has also been reported when systemic corticosteroids have been reduced or withdrawn in conjunction with the initiation of high-potency inhaled corticosteroids.35... 

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

Hattori, N., Ichimura, M., Nagamatsu, M. et al. Clinico-pathological features of Churg-Strauss syndrome-associated neuropathy. Brain 122 427-439,1999,... 

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. 

Le Gall C, Pham S, Vignes S, Garcia G, Nunes H, Fichet D, Simonneau G, Duroux P, Humbert M. Inhaled corticosteroids and Churg-Strauss syndrome a report of five cases. Eur Respir J 2000 15(5) 978-81. 

A 37-year-old woman who was pregnant developed Churg-Strauss syndrome after withdrawal of her usual high-dose inhaled glucocorticoid therapy (drug not stated) that she had used for 3 years for bronchial asthma (148). 

The authors of the second report commented that activated eosinophils and their cytotoxic products, such as eosinophil catatonic protein, may play a part in the pathogenesis of Churg-Strauss syndrome. Measuring serum concentrations of eosinophil catatonic protein may be useful in monitoring disease activity, since concentrations were increased before treatment and normalized afterwards. 

Priori R, Tomassini M, Magrini L, Conti F, Valesini G. Churg-Strauss syndrome during pregnancy after steroid withdrawal. Lancet 1998 352(9140) 1599-600. 

Chumbley, L.C., Harrison, E.G., DeRemee, R.A. Allergic granulomatosis and angiitis (Churg-Strauss syndrome). Report and analysis of 30 cases. Mayo Clin. Proc. 1977 52 477 - 484... 

Hubner C, Dietz A, Stremmel W, Stiehl A, Andrassy H. Macrolide-induced Churg-Strauss syndrome in a patient with atopy. Lancet 1997 350(9077) 563. 

Leukotriene receptor antagonists and Churg-Strauss syndrome... 

Eight patients with steroid-dependent asthma, who had been able to either discontinue or reduce their oral glucocorticoid requirement subsequent to starting treatment with zafirlukast, developed Churg-Strauss syndrome (7). 

A 50-year-old man with a history of severe asthma and tapering of prednisone took montelukast and developed an erythematous rash and mononeuritis multiplex skin biopsy confirmed the diagnosis of Churg-Strauss syndrome (10). 

A 54-year-old man with no history of glucocorticoid therapy presented with systemic symptoms and a purpuric rash after taking zafirlukast the diagnosis was Churg-Strauss syndrome (11). 

An 18-year-old woman with childhood asthma, using inhaled glucocorticoids and zafirlukast, developed Churg-Strauss syndrome 10 days after starting to use rokitamycin (12). 

A 52-year-old woman developed Churg-Strauss syndrome some 12 weeks after beginning treatment with pranlukast and 8 weeks after discontinuing low-dose oral prednisolone (5 mg on alternate days) (17). 

Churg-Strauss syndrome has been reported with montelukast in a patient who had not taken oral glucocorticoids. Symptoms developed within 2 days of beginning treatment with montelukast (18). 

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

Lilly CM, Churg A, Lazarovich M, Pauwels R, Hendeles L, Rosenwasser LJ, Ledford D, Wechsler ME. Asthma therapies and Churg-Strauss syndrome. J Allergy Chn Immunol 2002 109(1) S1-19. 

Churg A, Brallas M, Cronin SR, Churg J. Formes frustes of Churg-Strauss syndrome. Chest 1995 108(2) 320-3. 

Jamaleddine G, Diab K, Tabbarah Z, Tawil A, Arayssi T. Leukotriene antagonists and the Churg-Strauss syndrome. Semin Arthritis Rheum 2002 31(4) 218-27. 

Solans R, Bosch JA, Selva A, Orriols R, Vilardell M. Montelukast and Churg-Strauss syndrome. Thorax 2002 57(2) 183-5. 

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