Eosinophilic granulomatosis with polyangiitis

Churg-Strauss syndrome Microchapters
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Ali Poyan Mehr, M.D. [2]Associate Editor(s)-in-Chief: Chandrakala Yannam, MD [3]

Synonyms and keywords: Allergic granulomatosis; eosinophilic granulomatosis with polyangiitis, Churg-Strauss syndrome

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History and Symptoms | Physical Examination | Laboratory Findings | Chest X Ray | CT | MRI | Other Imaging Findings | Other Diagnostic Studies

Treatment

Medical Therapy | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case#1

Suggested Reading

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2. Jennette JC, Falk RJ, Andrassy K, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum. 1994;37:187-92
3. Leatherman J, Davies S, Hoidal J. Alveolar hemorrhage syndromes: diffuse microvascular lung hemorrhage in immune and idiopathic disorders. Medicine. 1984;63:65-81
4. Kallenberg CG. Antineutrophil cytoplasmic antibodies (ANCA) and vasculitis. Clin Rheumatol. 1990;9:S132-S35
5. Weller PF, Plaut M, Taggart V, Trontell A. The relationship of asthma therapy and Churg-Strauss syndrome: NIH workshop summary report. J Allergy Clin Immunol. 2001;108:175-83
6. Guillevin L, Cohen P, Gayraud M, Lhote F, Jarrousse B, Casassus P. Churg-Strauss syndrome. Clinical study and long-term follow-up of 96 patients. Medicine (Baltimore). 1999;78:26-37
7. Sehgal M, Swanson J, DeRemee R, Colby T. Neurologic manifestations of Churg-Strauss syndrome. Mayo Clin Proc. 1995;70:337-41
8. Lie J. Limited forms of Churg-Strauss syndrome. Pathol Annu. 1993;28:199-220
9. Watts RA, Carruthers DM, Scott DG. Epidemiology of systemic vasculitis: changing incidence or definition? Semin Arthritis Rheum. 1995;25:28-34
10. Lane SE, Watts RA, Bentham G, Innes NJ, Scott DGI. Are environmental factors important in the aetiology of primary systemic vasculitis (abstract) ? Arthritis Rheum. 2001;44(suppl9):S57
11. Watts RA, Lane SE, Scott DGI, Koldingsnes W, Nossent H, Gonzalez-Gay MA, Garcia-Porrua C, Bentham GA. Epidemiology of vasculitis in Europe. Ann Rheum Dis. 2001;60:1156-7
12. Fauci A, Haynes B, Katz P. The spectrum of vasculitis: clinical, pathologic, immunologic and therapeutic considerations. Ann Intern Med. 1978;89:660-76
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14. Guillevin L, Lê THD, Godeau P, Jaïs P, Wechsler B. Clinical findings and prognosis of polyarteritis nodosa and Churg-Strauss angiitis: a study in 165 patients. Br J Rheumatol. 1988;27:258-64
15. Guillevin L, Lhote F, Jarrousse B, Fain O. Treatment of polyarteritis nodosa and Churg-Strauss syndrome. A meta-analysis of 3 prospective controlled trials including 182 patients over 12 years. Ann Méd Interne (Paris). 1992;143:405-16
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18. Guillevin L, Fain O, Lhote F, et al. Lack of superiority of steroids plus plasma exchange to steroids alone in the treatment of polyarteritis nodosa and Churg-Strauss syndrome. A prospective, randomized trial in 78 patients. Arthritis Rheum. 1992;35:208-15
19. 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
20. Mahr A, Ayme S, Guillevin L. Prevalence and incidence of polyarteritis nodosa, microscopic polyangiitis, Wegener's granulomatosis and Churg-Strauss syndrome in a french suburban population in 2000: a capture-recapture analysis (abstract). Arthritis Rheum. 2001;44(suppl9):S332
21. Guillevin L, Guittard T, Bletry O, Godeau P, Rosenthal P. Systemic necrotizing angiitis with asthma: causes and precipitating factors in 43 cases. Lung. 1987;165:165-72
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23. Haas C, Géneau C, Odinot J, et al. L'angéïte allergique avec granulome: syndrome de Churg et Strauss. Etude rétrospective de 16 observations. Ann Méd Interne (Paris). 1991;142:135-42
24. Abu-Shakra M, Smythe H, Lewtas J, Badley E, Weber D, Keystone E. Outcome of polyarteritis nodosa and Churg-Strauss syndrome. Arthritis Rheum. 1994;37:1798-1803
25. Solans R, Bosch JA, Pérez-Bocanegra C, Selva A, Huguet P, Alijotas J, Orriols R, Armadans L, Vilardell M. Churg-Strauss syndome: outcome and long-term follow-up of 32 patients. Rheumatology. 2001;40:763-71
26. Lai R, Lin S, Lee P. Churg-Strauss syndrome presenting with capillaritis and diffuse alveolar hemorrhage. Scand J Rheumatol. 1998;27:230-2
27. Lhote F, Cohen P, Chemlal K, Jarrousse B, Guillevin L. Les manifestations pleurales au cours de la périartérite noueuse, de la maladie de Wegener et du lupus érythémateux systémique. Ann Méd Interne (Paris). 1992;143:228-32
28. Herreman G, Ferme I, Puech H, Caubarrère I. Angéite granulomateuse de Churg et Strauss avec paralysie phrénique. Deux observations. Presse Méd. 1980;9:3631
29. Cohen-Tervaert J, Kallenberg C. Neurologic manifestations of systemic vasculitides. Rheum Dis Clin North Am. 1993;19:913-40
30. Ng K, Yeung H, Loo K, Chan H, Wong C, Li P. Acute fulminant neuropathy in a patient with Churg Strauss syndrome. Postgrad Med J. 1997;73:236-8
31. Chang Y, Kargas S, Goates J, Horouplan D. Intraventricular and sub-arachnoid hemorrhage resulting from necrotizing vasculitis of the choroid plexus in a patient with Churg Strauss syndrome. Clin Neuropathol. 1993;12:84-7
32. Davis M, Daoud M, McEvoy M, Su D. Cutaneous manifestations of Churg Strauss syndrome: a clinicopathologic correlation. J Acad Dermatol. 1997;37:199-203
33. Finan M, Winkelmann R. The cutaneous extravascular necrotizing granuloma (Churg Strauss granuloma) and systemic disease: a review of 27 cases. Medicine (Baltimore). 1983;62:148-58
34. Suen K, Burton J. The spectrum of eosinophilic infiltration of the gastrointestinal tract and its relationship to other disorders of angiitis and granulomatosis. Hum Pathol. 1979;10:31-43
35. Guillevin L, Lhote F, Gallais V, et al. Gastrointestinal tract involvement in polyarteritis nodosa and Churg-Strauss syndrome. Ann Med Interne (Paris). 1995;146:260-7
36. Sharma A, de Varennes B, Sniderman A. Churg-Strauss syndrome presenting with marked eosinophilia and pericardial effusion. Can J Cardiol. 1993;9:329-30
37. Kozak M, Gill E, Green L. The Churg Strauss syndrome. A case report with angiographically documented coronary involvement and a review of the literature. Chest. 1995;107:578-80
38. Isaka N, Araki S, Shibata M, al. e. Reversal of coronary artery occlusions in allergic granulomatosis and angiitis (Churg-Strauss syndrome). Am Heart J. 1994;128:609-13
39. Hasley P, Follansbee N, Couleman J. Cardiac manifestations of Churg Strauss syndrome: report of a case and review of the literature. Am Heart J. 1990;120:996-9
40. De Vlam K, De Keyser F, Goemaere S, Praet M, Veys E. Churg-Strauss syndrome presenting as polymyositis. Clin Exp Rheumatol. 1995;13:505-7
41. Gaskin G, Clutterbuck E, Pusey C. Renal disease in the Churg Strauss syndrome. Diagnosis, management and outcome. Contrib Nephrol. 1991;94:58-65
42. Guillevin L, Lhote F, Brauner M, Casassus P. Antineutrophil cytoplasmic antibodies (ANCA) and abnormal angiograms in polyarteritis nodosa and Churg-Strauss syndrome: indications for the diagnosis of microscopic polyangiitis. Ann Méd Interne (Paris). 1995;146:548-50
43. Azar N, Guillevin L, Huong DL, Herreman G, Meyrier A, Godeau P. Symptomatic urogenital manifestations of polyarteritis nodosa and Churg-Strauss angiitis: analysis of 8 of 165 patients. J Urol. 1989;142:136-8
44. Nissim F, Von der Valde J, Czernobilsky B. A limited form of Churg-Strauss syndrome. Ocular and cutaneous manifestations. Arch Pathol Lab Med. 1982;106:305-7
45. Takanashi T, Uchida S, Arita M, Okada M, Kashii S. Orbital inflammatory pseudotumor and ischemic vasculitis in Churg-Strauss syndrome: report of two cases and review of the literature. Ophtalmology. 2001;108:1129-33
46. Généreau T, Lortholary O, Pottier MA, Michon-Pasturel U, Ponge T, de Wazieres B, Liozon E, Pinede L, Hachulla E, Roblot P, Barrier JH, Herson S, Guillevin L. Temporal artery biopsy : a diagnostic tool for systemic necrotizing vasculitis. Arthritis Rheum. 1999;42:2674-81
47. Churg A, Brallas M, Cronin S, Churg J. Formes frustes of Churg Strauss syndrome. Chest. 1995;108:320-3
48. Phanupak P, Kohler P. Onset of polyarteritis nodosa during allergic hyposensitization treatment. Am J Med. 1980;68:1866-72
49. Drazen J, Israel E, O'Byrne P. Treatment of asthma with drugs modifying the leukotriene pathway. N Engl J Med. 1999;340:197-206
50. Katz R, Papernik M. Zafirlukast and Churg-Strauss syndrome. JAMA. 1998;279:1949
51. Knoell D, Lucas J, Allen J. Churg Strauss syndrome associated with zafirlukast. Chest. 1998;114:332-4
52. Wechsler M, Garpestad E, Flier S, et al. Pulmonary infiltrates, eosinophilia and cardiomyopathy following corticosteroid withdrawal in patients with asthma receiving zafirlukast. JAMA. 1998;279:455-7
53. Guillevin L, Visser H, Noël LH, et al. Antineutrophil cytoplasm antibodies in systemic polyarteritis nodosa with and without hepatitis B virus infection and Churg-Strauss syndrome: 62 patients. J Rheumatol. 1993;20:1345-9
54. Cohen-Tervaert J, Goldschmeding R, Elema JD, von den Borne A, Kallenberg CG. Antimyeloperoxidase antibodies in the Churg Strauss syndrome. Thorax. 1991;46:70-1
55. Cogan E, Schandené L, Crusiaux A, Cochaux P, Velu T, Goldman M. Clonal proliferation of type 2 helper T cells in a man with the hypereosinophilic syndrome. N Engl J Med. 1994;330:535-8
56. Stillwell T, Benson R. Cyclophosphamide-induced hemorrhagic cystitis: a review of 100 patients. Cancer. 1988;61:451-7
57. Baker G, Kahl L, Zee B, Stolzer B, Agarwal A, Medsger TJ. Malignancy following treatment of rheumatoid arthritis with cyclophosphamide. Long-term case-control follow-up study. Am J Med. 1987;83:1-9
58. Jarrousse B, Guillevin L, Bindi P, et al. Increased risk of Pneumocystis carinii pneumonia in patients with Wegener's granulomatosis. Clin Exp Rheumatol. 1993;11:615-21
59. Guillevin L, Lhote F, Cohen P, et al. Corticosteroids plus pulse cyclophosphamide and plasma exchanges versus corticosteroids plus pulse cyclophosphamide alone in the treatment of polyarteritis nodosa and Churg-Strauss syndrome patients with factors predicting poor prognosis. A prospective, randomized trial in sixty-two patients. Arthritis Rheum. 1995;38:1638-45
60. Gayraud M, Guillevin L, Cohen P, et al. Treatment of good-prognosis polyarteritis nodosa and Churg-Strauss syndrome: comparison of steroids and oral or pulse cyclophosphamide in 25 patients. French Cooperative Study Group for Vasculitides. Br J Rheumatol. 1997;36:1290-7
61. Luqmani R, Exley A, Kitas G, Bacon P. Disease assessment and management of the vasculitides. Baillere's Clin Rheumatol. 1997;11:423-46
62. Hamilos D, Christensen J. Treatment of Churg Strauss syndrome with high-dose intravenous immunoglobulins. J Allergy Clin Immunol. 1991;88:823-4
63. Jayne DR, Lockwood CM. Intravenous immunoglobulins as sole therapy for systemic vasculitis. Br J Rheumatol. 1996;35:1150-3
64. Henderson R, Hasleton P, Hamid B. Recurrence of Churg-Strauss vasculitis in a transplanted heart. Br Heart J. 1993;70:553
65. McDermott E, Powell R. Cyclosporin in the treatment of Churg-Strauss syndrome. Ann Rheum Dis. 1998;57:256-7
66. Tatsis E, Schnabel A, Gross W. Interferon alpha treatment in four patients with the Churg Strauss syndrome. Ann Intern Med. 1998;129:370-4
67. Koutantji M, Pearce S, Harrold E. Psychological aspects of vasculitis. Rheumatol. 2000;39:1173-9
68. Guillevin L, Cohen P, Larroche C, Queyrel V, Loustaud-Ratti V, Imbert B, Hausfater P, Ramanoelina J, Lacombe F, Roudier J, Bielefeld P, Petitjean P, Smadja D. The French Vasculitis Study Group. Treatment of HBV-related polyarteritis nodosa with lamivudine and plasma exchanges: a prospective, multicenter, pilot trial in 10 patients (abstract). Arthritis Rheum. 2001;44(suppl9):S271

External links

• FAQ
• Churg-Strauss syndrome - MedLink Neurology Clinical Summary
• Template:GPnotebook
• One Person's Experience With The Disease
• Natural History of Churg-Strauss Syndrome
• (http://www.mlcss.com/ My Domestic Life A Blog That Focuses On My Everyday Life With Churg Strauss Syndrome)

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