Abstract

Churg-Strauss syndrome is an uncommon multisystem disease involving small and medium-sized arteries, capillaries, veins, and venules. It is characterised by the presence of asthma, hypereosinophilia, and evidence of vasculitis affecting a number of organs. It is important for acute physicians to recognise this condition as delayed diagnosis results in a higher morbidity and mortality. Here we describe a case of Churg-Strauss syndrome in a 41 year old woman presenting with a recurrent painful rash. The clinical manifestations and management of this condition are discussed.

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References

1. Churg J, Strauss L. Allergic granulomatosis, allergic angiitis and periarteritis nodosa. Am J Pathol 1951; 27: 277-301.
2. Davies DJ. Small vessel vasculitis. Cardiovascular Pathology 2005; 14: 335-346.
3. Eustace JA, Nadasdy T, Choi M. Disease of the month. The Churg Strauss Syndrome. J Am Soc Nephrol. 1999; 10: 2048-55.
4. Lanham JG, Elkon KB, Pusey CD, et al. Systematic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome. Medicine (Baltimore) 1984; 63: 65-81.
5. Lhote F, Guillevin L. Polyarteritis nodosa, microscopic angiitis, and Churg-Strauss syndrome. Sem Respir Crit Care Med 1998; 19: 27-45.
6. Davis MD, Daoud MS, McEvoy MT, et al. Cutaneous manifestations of Churg-Strauss syndrome: a clinicopathologic correlation. J Am Acad Dermatol 1997; 37: 199-203.
7. Schwartz RA, Churg J. Churg-Strauss syndrome. Br J Dermatol 1992; 127: 199-204.
8. Gibson LE. Granulomatous vasculitides and the skin. Dermatol Clin 1990; 8: 335-345.
9. Sehgal M, Swanson JW, DeRemee RA, et al. Neurologic manifestations of Churg-Strauss syndrome. Mayo Clin Proc 1995;70: 337-341.
10. Moore PM, Calabrese LH. Neurologic manifestations of systemic vasculitides. Semin Neurol 1994; 14: 300-306.
11. Liou HH, Liu HM, Chiang IP, et al. Churg-Strauss syndrome presented as multiple cerebral haemorrhage. Lupus 1997; 6: 279-282.
12. Guillevin L, Guittard T, Bletry O, et al. Systemic necrotizing angiitis with asthma: causes and precipitating factors in 43 cases. Lung 1987; 165: 165-172.
13. Hellemans S, Dens J, Knockaert D. Coronary involvement in the Churg-Strauss syndrome. Heart 1997; 77: 576-578.
14. 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 1990; 33: 1094-1100.
15. Hunt SA, Abraham WT, Chin MH, et al. 2009 focused update incorporated into the ACC/AHA 2005 guidelines for the diagnosis and management of heart failure in adults: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines: developed in collaboration with the International Society for Heart and Lung Transplantation. Circulation 2009; 119: e391-e479.
16. Dickstein K, Cohen-Solal A, Filippatos G, et al. ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure 2008: the Task Force for the Diagnosis and Treatment of Acute and Chronic Heart Failure 2008 of the European Society of Cardiology. Developed in collaboration with the Heart Failure
    Association of the ESC (HFA) and endorsed by the European Society of Intensive Care Medicine (ESICM). Eur Heart J 2008; 29: 2388-2442.
17. Faris R, Flather MD, Purcell H, et al. Diuretics for heart failure. Cochrane Database Syst Rev 2006; CD003838.
18. Hagen EC, Daha MR, Hermans J, et al. Diagnostic value of standardized assays for antineutrophil cytoplasmic antibodies in idiopathic systemic vasculitis, Kidney Int 1998; 53: 743-753.
19. Sable-Fourtassou R, Cohen P, Mahr A, et al. Antineutrophil cytoplasmic antibodies and the Churg-Strauss syndrome. Ann Intern Med 2005; 143: 632-638.
20. Grau RG. Churg-Strauss syndrome:2005-2008 update. Curr Rheumatol Rep. 2008; 10: 453-458.
21. Mukhtyar C, Flossmann O, Hellmich B, et al. Outcomes from studies of antineutrophil cytoplasm antibody associated vasculitis: a systematic review by the European League Against Rheumatism systemic vasculitis task force. Ann Rheum Dis. 2008; 67: 1004- 1010.
22. Sinico RA, Bottero P. Churg-Strauss angiitis. Best Pract Res Clin Rheumatol 2009; 23: 355-366.
23. Guillevin L, Jarrousse B, Lok C, et al. Long term follow up after treatment of polyarteritis nodosa and Churg-Strauss angiitis with comparison of steroids, plasma exchange and cyclophosphamide to steroids and plasma exchange. A prospective randomised trial of 71 patients. The Cooperative Study Group for Polyarteritis Nodosa. J Rheumatol 1991; 18: 567-574.
24. 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) 1996; 75: 17-28.
25. Bourgarit A, Le Toumelin P, Pagnoux C, et al. Deaths occurring during the first year after treatment onset for polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome: a retrospective analysis of causes and factors predictive of mortality based on 595 patients. Medicine (Baltimore) 2005; 84: 323-330.
26. Gayraud M, Guillevin L, Le Toumelin P, et al. Long term follow up of polyarteritis nodosa, microscopic polyangiitis, and ChurgStrauss syndrome: analysis of four prospective trials including 278 patients. Arthritis Rheum 2001; 44: 666-675.