Medical Care

Medical management of cardiovascular, cardiac, renal, and gastrointestinal complications of Churg-Strauss disease (CSD), now known as eosinophilic granulomatosis with polyangiitis (EGPA), falls under the purview of subspecialty consultation. Drugs used in the treatment of EGPA are described in Medication.

Surgical Care

Surgical procedures in patients with Churg-Strauss disease (CSD), now known as eosinophilic granulomatosis with polyangiitis (EGPA), most commonly entail biopsy of affected tissues.

In addition, surgical intervention may be indicated in patients who experience catastrophic complications, such as acute abdomen or intracranial hemorrhage.

Consultations

Consultations for patients with ofChurg-Strauss disease (CSD), now known as eosinophilic granulomatosis with polyangiitis (EGPA), depend on the manifestations of the disease. The cardiopulmonary manifestations typically are most important. Consultations with the following may be required:

Pulmonologist
Cardiologist
Rheumatologist
Gastroenterologist
Urologist

Diet

No dietary factors are clearly known to assist in the management of Churg-Strauss disease (CSD), now known as eosinophilic granulomatosis with polyangiitis (EGPA), except as are related to the management of congestive heart failure, renal failure, or gastrointestinal complications. The management of these factors falls under the purview of subspecialists involved in the care of these organ systems.

Activity

No clear evidence suggests that particular activities influence the course of Churg-Strauss disease (CSD), now known as eosinophilic granulomatosis with polyangiitis (EGPA), once it is established. Environmental exposures and medical treatment of asthma that may influence the chances for development of EGPA are discussed in Causes.

Medication

Davies DJ, Moran JE. Segmental necrotizing glomerulonephritis with antineutrophil antibody: possible arbovirus aetiology?. BMJ. 1982. 285:606.
Churg J, Strauss L. Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol. 1951 Mar-Apr. 27(2):277-301. [QxMD MEDLINE Link].
Chumbley LC, Harrison EG Jr, DeRemee RA. Allergic granulomatosis and angiitis (Churg-Strauss syndrome). Report and analysis of 30 cases. Mayo Clin Proc. 1977 Aug. 52(8):477-84. [QxMD MEDLINE Link].
Lanham JG. Churg-Strauss syndrome. Br J Hosp Med. 1992 May 6-19. 47(9):667-73. [QxMD MEDLINE Link].
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 Aug. 33(8):1094-100. [QxMD MEDLINE Link].
Jennette JC, Falk RJ, Andrassy K, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum. 1994 Feb. 37(2):187-92. [QxMD MEDLINE Link].
Lie JT. Nomenclature and classification of vasculitis: plus ça change, plus c'est la même chose. Arthritis Rheum. 1994 Feb. 37(2):181-6. [QxMD MEDLINE Link].
Kallenberg CG, Brouwer E, Weening JJ, Tervaert JW. Anti-neutrophil cytoplasmic antibodies: current diagnostic and pathophysiological potential. Kidney Int. 1994 Jul. 46(1):1-15. [QxMD MEDLINE Link].
Cohen P, Guillevin L, Baril L, Lhote F, Noel LH, Lesavre P. 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. 1995 Mar-Apr. 13(2):193-8. [QxMD MEDLINE Link].
Tervaert JW, Goldschmeding R, Elema JD, von dem Borne AE, Kallenberg CG. Antimyeloperoxidase antibodies in the Churg-Strauss syndrome. Thorax. 1991 Jan. 46(1):70-1. [QxMD MEDLINE Link].
Tervaert JW, Goldschmeding R, Elema JD, et al. Association of autoantibodies to myeloperoxidase with different forms of vasculitis. Arthritis Rheum. 1990 Aug. 33(8):1264-72. [QxMD MEDLINE Link].
Watts RA, Scott DG. Classification and epidemiology of the vasculitides. Baillieres Clin Rheumatol. 1997 May. 11(2):191-217. [QxMD MEDLINE Link].
Capizzi SA, Specks U. Does infection play a role in the pathogenesis of pulmonary vasculitis?. Semin Respir Infect. 2003 Mar. 18(1):17-22. [QxMD MEDLINE Link].
Ramentol-Sintas M, Martínez-Valle F, Solans-Laqué R. Churg-Strauss Syndrome: An evolving paradigm. Autoimmun Rev. 2012 Dec. 12(2):235-40. [QxMD MEDLINE Link].
Wiik A. Autoantibodies in vasculitis. Arthritis Res Ther. 2003. 5(3):147-52. [QxMD MEDLINE Link].
Arm JP, Lee TH. Sulphidopeptide leukotrienes in asthma. Clin Sci (Lond). 1993 May. 84(5):501-10. [QxMD MEDLINE Link].
Drazen JM, Israel E. Should antileukotriene therapies be used instead of inhaled corticosteroids in asthma? Yes. Am J Respir Crit Care Med. 1998 Dec. 158(6):1697-8. [QxMD MEDLINE Link].
Drazen JM, Israel E, O'Byrne PM. Treatment of asthma with drugs modifying the leukotriene pathway. N Engl J Med. 1999 Jan 21. 340(3):197-206. [QxMD MEDLINE Link].
Wechsler ME, Garpestad E, Flier SR, et al. Pulmonary infiltrates, eosinophilia, and cardiomyopathy following corticosteroid withdrawal in patients with asthma receiving zafirlukast. JAMA. 1998 Feb 11. 279(6):455-7. [QxMD MEDLINE Link].
Churg A, Brallas M, Cronin SR, Churg J. Formes frustes of Churg-Strauss syndrome. Chest. 1995 Aug. 108(2):320-3. [QxMD MEDLINE Link].
D'Cruz DP, Barnes NC, Lockwood CM. Difficult asthma or Churg-Strauss syndrome?. BMJ. 1999 Feb 20. 318(7182):475-6. [QxMD MEDLINE Link].
Garcia-Marcos L, Schuster A, Perez-Yarza EG. Benefit-risk assessment of antileukotrienes in the management of asthma. Drug Saf. 2003. 26(7):483-518. [QxMD MEDLINE Link].
Kemp JP. Recent advances in the management of asthma using leukotriene modifiers. Am J Respir Med. 2003. 2(2):139-56. [QxMD MEDLINE Link].
Finkel TH, Hunter DJ, Paisley JE, Finkel RS, Larsen GL. Drug-induced lupus in a child after treatment with zafirlukast (Accolate). J Allergy Clin Immunol. 1999 Mar. 103(3 Pt 1):533-4. [QxMD MEDLINE Link].
Orriols R, Munoz X, Ferrer J, Huget P, Morell F. Cocaine-induced Churg-Strauss vasculitis. Eur Respir J. 1996 Jan. 9(1):175-7. [QxMD MEDLINE Link].
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 Jan. 78(1):26-37. [QxMD MEDLINE Link].
Shanks M, Ignaszewski AP, Chan SY, Allard MF. Churg-Strauss syndrome with myocarditis manifesting as acute myocardial infarction with cardiogenic shock: case report and review of the literature. Can J Cardiol. 2003 Sep. 19(10):1184-8. [QxMD MEDLINE Link].
Lin YC, Oliveira GH, Villa-Forte A. Churg-Strauss syndrome and persistent heart failure: active disease or damage?. J Clin Rheumatol. 2013 Oct. 19(7):390-2; discussion 419. [QxMD MEDLINE Link].
Tatsukawa H, Nagano S, Umeno Y, Oribe M. Churg-strauss syndrome with cholecystitis and renal involvement. Intern Med. 2003 Sep. 42(9):893-6. [QxMD MEDLINE Link].
Lane SE, Watts R, Scott DG. Epidemiology of systemic vasculitis. Curr Rheumatol Rep. 2005 Aug. 7(4):270-5. [QxMD MEDLINE Link].
Booth AD, Almond MK, Burns A, et al. Outcome of ANCA-associated renal vasculitis: a 5-year retrospective study. Am J Kidney Dis. 2003 Apr. 41(4):776-84. [QxMD MEDLINE Link].
Kawakami T, Soma Y, Kawasaki K, Kawase A, Mizoguchi M. Initial cutaneous manifestations consistent with mononeuropathy multiplex in Churg-Strauss syndrome. Arch Dermatol. 2005 Jul. 141(7):873-8. [QxMD MEDLINE Link].
Eyler AE, Ahmad FA, Jahangir E. Magnetic resonance imaging of the cardiac manifestations of Churg-Strauss. JRSM Open. 2014 Apr. 5(4):2054270414525370. [QxMD MEDLINE Link]. [Full Text].
Abril A, Calamia KT, Cohen MD. The Churg Strauss syndrome (allergic granulomatous angiitis): review and update. Semin Arthritis Rheum. 2003 Oct. 33(2):106-14. [QxMD MEDLINE Link].
Azzopardi C, Montefort S, Mallia C. Cardiac involvement and left ventricular failure in a patient with the Churg-Strauss syndrome. Adv Exp Med Biol. 1999. 455:547-9. [QxMD MEDLINE Link].
Barry C, Davis S, Garrard P, Ferguson IT. Churg-Strauss disease: deterioration in a twin pregnancy. Successful outcome following treatment with corticosteroids and cyclophosphamide. Br J Obstet Gynaecol. 1997 Jun. 104(6):746-7. [QxMD MEDLINE Link].
Berger JR, Wei T, Wilson D. Idiopathic granulomatous angiitis of the CNS manifesting as diffuse white matter disease. Neurology. 1998 Dec. 51(6):1774-5. [QxMD MEDLINE Link].
Bili A, Condemi JJ, Bottone SM, Ryan CK. Seven cases of complete and incomplete forms of Churg-Strauss syndrome not related to leukotriene receptor antagonists. J Allergy Clin Immunol. 1999 Nov. 104(5):1060-5. [QxMD MEDLINE Link].
Boggi U, Mosca M, Giulianotti PC, Naccarato AG, Bombardieri S, Mosca F. Surviving catastrophic gastrointestinal involvement due to Churg-Strauss syndrome: report of a case. Hepatogastroenterology. 1997 Jul-Aug. 44(16):1169-71. [QxMD MEDLINE Link].
Brooklyn TN, Prouse P, Portmann B, Ramage JK. Churg-Strauss syndrome and granulomatous cholangiopathy. Eur J Gastroenterol Hepatol. 2000 Jul. 12(7):809-11. [QxMD MEDLINE Link].
Bruce IN, Bell AL. A comparison of two nomenclature systems for primary systemic vasculitis. Br J Rheumatol. 1997 Apr. 36(4):453-8. [QxMD MEDLINE Link].
Burke AP, Sobin LH, Virmani R. Localized vasculitis of the gastrointestinal tract. Am J Surg Pathol. 1995 Mar. 19(3):338-49. [QxMD MEDLINE Link].
Caballero J, Zayas R, Arana R, Cano L, Berruezo A, Pinero C. [Churg-Strauss syndrome with pericardial and myocardial involvement]. Rev Esp Cardiol. 1999 Sep. 52(9):745-7. [QxMD MEDLINE Link].
Chen KR, Su WP, Pittelkow MR, Leiferman KM. Eosinophilic vasculitis syndrome: recurrent cutaneous eosinophilic necrotizing vasculitis. Semin Dermatol. 1995 Jun. 14(2):106-10. [QxMD MEDLINE Link].
Cho KH, Kim YG, Yang SG, Lee DY, Chung JH. Inflammatory nodules of the lower legs: a clinical and histological analysis of 134 cases in Korea. J Dermatol. 1997 Aug. 24(8):522-9. [QxMD MEDLINE Link].
Davis MD, Daoud MS, McEvoy MT, Su WP. Cutaneous manifestations of Churg-Strauss syndrome: a clinicopathologic correlation. J Am Acad Dermatol. 1997 Aug. 37(2 Pt 1):199-203. [QxMD MEDLINE Link].
Dietz A, Hubner C, Andrassy K. [Macrolide antibiotic-induced vasculitis (Churg-Strauss syndrome)]. Laryngorhinootologie. 1998 Feb. 77(2):111-4. [QxMD MEDLINE Link].
Dillon MJ. Childhood vasculitis. Lupus. 1998. 7(4):259-65. [QxMD MEDLINE Link].
Dinç A, Soy M, Pay S, Simsek I, Erdem H, Sobaci G. A case of Churg-Strauss syndrome presenting with cortical blindness. Clin Rheumatol. 2000. 19(4):318-20. [QxMD MEDLINE Link].
Eustace JA, Nadasdy T, Choi M. Disease of the month. The Churg Strauss Syndrome. J Am Soc Nephrol. 1999 Sep. 10(9):2048-55. [QxMD MEDLINE Link].
Falk RJ, Nachman PH, Hogan SL, Jennette JC. ANCA glomerulonephritis and vasculitis: a Chapel Hill perspective. Semin Nephrol. 2000 May. 20(3):233-43. [QxMD MEDLINE Link].
Ferro JM. Vasculitis of the central nervous system. J Neurol. 1998 Dec. 245(12):766-76. [QxMD MEDLINE Link].
Gross WL, Csernok E. Immunodiagnostic and pathophysiologic aspects of antineutrophil cytoplasmic antibodies in vasculitis. Curr Opin Rheumatol. 1995 Jan. 7(1):11-9. [QxMD MEDLINE Link].
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 Feb. 35(2):208-15. [QxMD MEDLINE Link].
Hagen EC, Andrassy K, Csernok E, et al. Development and standardization of solid phase assays for the detection of anti-neutrophil cytoplasmic antibodies (ANCA). A report on the second phase of an international cooperative study on the standardization of ANCA assays. J Immunol Methods. 1996 Sep 13. 196(1):1-15. [QxMD MEDLINE Link].
Hauschild S, Csernok E, Schmitt WH, Gross WL. Antineutrophil cytoplasmic antibodies in systemic polyarteritis nodosa with and without hepatitis B virus infection and Churg-Strauss syndrome--62 patients. J Rheumatol. 1994 Jun. 21(6):1173-4. [QxMD MEDLINE Link].
Hellemans S, Dens J, Knockaert D. Coronary involvement in the Churg-Strauss syndrome. Heart. 1997 Jun. 77(6):576-8. [QxMD MEDLINE Link]. [Full Text].
Hellmich B, Csernok E, Gross WL. Proinflammatory cytokines and autoimmunity in Churg-Strauss syndrome. Ann N Y Acad Sci. 2005 Jun. 1051:121-31. [QxMD MEDLINE Link].
Hellmich B, Ehlers S, Csernok E, Gross WL. Update on the pathogenesis of Churg-Strauss syndrome. Clin Exp Rheumatol. 2003 Nov-Dec. 21(6 Suppl 32):S69-77. [QxMD MEDLINE Link].
Hellmich B, Gross WL. Recent progress in the pharmacotherapy of Churg-Strauss syndrome. Expert Opin Pharmacother. 2004 Jan. 5(1):25-35. [QxMD MEDLINE Link].
Hoffman PM, Godfrey T, Stawell RJ. A case of Churg-Strauss syndrome with visual loss following central retinal artery occlusion. Lupus. 2005. 14(2):174-5. [QxMD MEDLINE Link].
Jennette JC, Wilkman AS, Falk RJ. Anti-neutrophil cytoplasmic autoantibody-associated glomerulonephritis and vasculitis. Am J Pathol. 1989 Nov. 135(5):921-30. [QxMD MEDLINE Link].
Kim Y, Lee KS, Choi DC, Primack SL, Im JG. The spectrum of eosinophilic lung disease: radiologic findings. J Comput Assist Tomogr. 1997 Nov-Dec. 21(6):920-30. [QxMD MEDLINE Link].
Klemmer PJ, Chalermskulrat W, Reif MS, Hogan SL, Henke DC, Falk RJ. Plasmapheresis therapy for diffuse alveolar hemorrhage in patients with small-vessel vasculitis. Am J Kidney Dis. 2003 Dec. 42(6):1149-53. [QxMD MEDLINE Link].
Lamprecht P. Off balance: T-cells in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides. Clin Exp Immunol. 2005 Aug. 141(2):201-10. [QxMD MEDLINE Link].
Lamprecht P. TNF-alpha inhibitors in systemic vasculitides and connective tissue diseases. Autoimmun Rev. 2005 Jan. 4(1):28-34. [QxMD MEDLINE Link].
Langford CA, Sneller MC. New developments in the treatment of Wegener's granulomatosis, polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome. Curr Opin Rheumatol. 1997 Jan. 9(1):26-30. [QxMD MEDLINE Link].
Lhote F, Cohen P, Genereau T, Gayraud M, Guillevin L. Microscopic polyangiitis: clinical aspects and treatment. Ann Med Interne (Paris). 1996. 147(3):165-77. [QxMD MEDLINE Link].
Lie JT. Histopathologic specificity of systemic vasculitis. Rheum Dis Clin North Am. 1995 Nov. 21(4):883-909. [QxMD MEDLINE Link].
Marmursztejn J, Guillevin L, Trebossen R, Cohen P, Guilpain P, Pagnoux C, et al. Churg-Strauss syndrome cardiac involvement evaluated by cardiac magnetic resonance imaging and positron-emission tomography: a prospective study on 20 patients. Rheumatology (Oxford). 2012 Aug 29. [QxMD MEDLINE Link].
Martin-Suarez I, D'Cruz D, Mansoor M, Fernandes AP, Khamashta MA, Hughes GR. Immunosuppressive treatment in severe connective tissue diseases: effects of low dose intravenous cyclophosphamide. Ann Rheum Dis. 1997 Aug. 56(8):481-7. [QxMD MEDLINE Link].
Matsuo K, Tomioka T, Tajima Y, et al. Allergic granulomatous angitis (Churg-Strauss syndrome) with multiple intestinal fistulas. Am J Gastroenterol. 1997 Oct. 92(10):1937-8. [QxMD MEDLINE Link].
Metzler C, Lamprecht P, Hellmich B, Reuter M, Arlt AC, Gross WL. Leucoencephalopathy after treatment of Churg-Strauss syndrome with interferon {alpha}. Ann Rheum Dis. 2005 Aug. 64(8):1242-3. [QxMD MEDLINE Link].
Moosig F, Bremer JP, Hellmich B, Holle JU, Holl-Ulrich K, Laudien M, et al. A vasculitis centre based management strategy leads to improved outcome in eosinophilic granulomatosis and polyangiitis (Churg-Strauss, EGPA): monocentric experiences in 150 patients. Ann Rheum Dis. 2012 Aug 11. [QxMD MEDLINE Link].
Muschen M, Warskulat U, Perniok A, et al. Involvement of soluble CD95 in Churg-Strauss syndrome. Am J Pathol. 1999 Sep. 155(3):915-25. [QxMD MEDLINE Link].
Niles JL. Antineutrophil cytoplasmic antibodies in the classification of vasculitis. Annu Rev Med. 1996. 47:303-13. [QxMD MEDLINE Link].
Pellissier JF, Figarella-Branger D, Serratrice G. [Neuromuscular diseases with eosinophilia]. Med Trop (Mars). 1998. 58(4 Suppl):471-6. [QxMD MEDLINE Link].
Primack SL, Müller NL. Radiologic manifestations of the systemic autoimmune diseases. Clin Chest Med. 1998 Dec. 19(4):573-86, vii. [QxMD MEDLINE Link].
Reid AJC, Harrison RA, Watkin SW, McCann BG, and Scott DGI. Churg-Strauss syndrome in a district hospital. Q J Med. 1998. 91:219-229.
Rutgers A, Heeringa P, Tervaert JW. The role of myeloperoxidase in the pathogenesis of systemic vasculitis. Clin Exp Rheumatol. 2003 Nov-Dec. 21(6 Suppl 32):S55-63.
Sable-Fourtassou R, Cohen P, Mahr A, et al. Antineutrophil cytoplasmic antibodies and the Churg-Strauss syndrome. Ann Intern Med. 2005 Nov 1. 143(9):632-8. [QxMD MEDLINE Link].
Sakamoto S, Ohba S, Eguchi K, et al. Churg-Strauss syndrome presenting with subarachnoid hemorrhage from ruptured dissecting aneurysm of the intracranial vertebral artery. Clin Neurol Neurosurg. 2005 Aug. 107(5):428-31. [QxMD MEDLINE Link].
Sanders JS, Stegeman CA, Kallenberg CG. The Th1 and Th2 paradigm in ANCA-associated vasculitis. Kidney Blood Press Res. 2003. 26(4):215-20. [QxMD MEDLINE Link].
Sharma BK, Daga MK, Sharma M. A limited form of Churg-Strauss syndrome presenting without asthma and eosinophilia. Med J Aust. 2004 Nov 1. 181(9):498-9. [QxMD MEDLINE Link].
Somogyi A, Muzes G, Molnar J, Tulassay Z. Drug-related Churg-Strauss syndrome?. Adverse Drug React Toxicol Rev. 1998 Jun-Sep. 17(2-3):63-74. [QxMD MEDLINE Link].
Sonneville R, Lagrange M, Guidoux C, et al. [The association of cardiac involvement and ischemic stroke in Churg Strauss syndrome]. Rev Neurol (Paris). 2006 Feb. 162(2):229-32. [QxMD MEDLINE Link].
Steinfeld S, Golstein M, De Vuyst P. Chronic eosinophilic pneumonia (CEP) as a presenting feature of Churg-Strauss syndrome (CSS). Eur Respir J. 1994 Nov. 7(11):2098. [QxMD MEDLINE Link].
Sudo K, Tashiro K. Idiopathic granulomatous angiitis of the CNS manifesting as diffuse white matter disease. Neurology. 1998 Dec. 51(6):1774; author reply 1775. [QxMD MEDLINE Link].
Sullivan EJ, Hoffman GS. Pulmonary vasculitis. Clin Chest Med. 1998 Dec. 19(4):759-76, ix. [QxMD MEDLINE Link].
Tatsis E, Schnabel A, Gross WL. Interferon-alpha treatment of four patients with the Churg-Strauss syndrome. Ann Intern Med. 1998 Sep 1. 129(5):370-4. [QxMD MEDLINE Link].
Terasaki F, Hayashi T, Hirota Y, et al. Evolution to dilated cardiomyopathy from acute eosinophilic pancarditis in Churg-Strauss syndrome. Heart Vessels. 1997. 12(1):43-8. [QxMD MEDLINE Link].
Thuy GN, Cuguilliere A, Arteaga C, Miltgen J, Bonnet D. [Idiopathic chronic eosinophilic pneumonia]. Med Trop (Mars). 1998. 58(4 Suppl):455-8. [QxMD MEDLINE Link].
Vital C, Vital A, Canron MH, et al. Combined nerve and muscle biopsy in the diagnosis of vasculitic neuropathy. A 16-year retrospective study of 202 cases. J Peripher Nerv Syst. 2006 Mar. 11(1):20-9. [QxMD MEDLINE Link].
Watts RA, Lane SE, Bentham G, Scott DG. Epidemiology of systemic vasculitis: a ten-year study in the United Kingdom. Arthritis Rheum. 2000 Feb. 43(2):414-9. [QxMD MEDLINE Link].
Weissler JC. Syndromes of severe asthma. Am J Med Sci. 2000 Mar. 319(3):166-76. [QxMD MEDLINE Link].
Wolf M, Rose H, Smith RN. Case records of the Massachusetts General Hospital. Case 28-2005. A 42-year-old man with weight loss, weakness, and a rash. N Engl J Med. 2005 Sep 15. 353(11):1148-57. [QxMD MEDLINE Link].
Yoshihara K, Arimura Y, Kobayashi O, et al. [Clinical study on five myeloperoxidase specific anti-neutrophil cytoplasmic antibody (MPO-ANCA) positive Churg-Strauss syndrome cases]. Ryumachi. 1998 Oct. 38(5):696-704. [QxMD MEDLINE Link].
Zhao MH, Short AK, Lockwood CM. Antineutrophil cytoplasm autoantibodies and vasculitis. Curr Opin Hematol. 1995 Jan. 2(1):96-102. [QxMD MEDLINE Link].
Lanham JG, Elkon KB, Pusey CD, Hughes GR. Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome. Medicine (Baltimore). 1984 Mar. 63 (2):65-81. [QxMD MEDLINE Link].
Chakraborty RK, Aeddula NR. Churg Strauss Syndrome (Allergic Granulomatosis). 2019 Jan. [QxMD MEDLINE Link]. [Full Text].
Mahr A, Moosig F, Neumann T, Szczeklik W, Taillé C, Vaglio A, et al. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): evolutions in classification, etiopathogenesis, assessment and management. Curr Opin Rheumatol. 2014 Jan. 26 (1):16-23. [QxMD MEDLINE Link].
Cottin V, Bel E, Bottero P, Dalhoff K, Humbert M, et al. Revisiting the systemic vasculitis in eosinophilic granulomatosis with polyangiitis (Churg-Strauss): A study of 157 patients by the Groupe d'Etudes et de Recherche sur les Maladies Orphelines Pulmonaires and the European Respiratory Society Taskforce on eosinophilic granulomatosis with polyangiitis (Churg-Strauss). Autoimmun Rev. 2017 Jan. 16 (1):1-9. [QxMD MEDLINE Link].
Wieczorek S, Hellmich B, Gross WL, Epplen JT. Associations of Churg-Strauss syndrome with the HLA-DRB1 locus, and relationship to the genetics of antineutrophil cytoplasmic antibody-associated vasculitides: comment on the article by Vaglio et al. Arthritis Rheum. 2008 Jan. 58 (1):329-30. [QxMD MEDLINE Link].
Vaglio A, Martorana D, Maggiore U, Grasselli C, Zanetti A, Pesci A, et al. HLA-DRB4 as a genetic risk factor for Churg-Strauss syndrome. Arthritis Rheum. 2007 Sep. 56 (9):3159-66. [QxMD MEDLINE Link].
Bibby S, Healy B, Steele R, Kumareswaran K, Nelson H, Beasley R. Association between leukotriene receptor antagonist therapy and Churg-Strauss syndrome: an analysis of the FDA AERS database. Thorax. 2010 Feb. 65 (2):132-8. [QxMD MEDLINE Link].
Kiene M, Csernok E, Müller A, Metzler C, Trabandt A, Gross WL. Elevated interleukin-4 and interleukin-13 production by T cell lines from patients with Churg-Strauss syndrome. Arthritis Rheum. 2001 Feb. 44 (2):469-73. [QxMD MEDLINE Link].
Rolla G, Guida G, Heffler E. Churg-Strauss syndrome: still a clinical challenge. Expert Rev Clin Immunol. 2007 Nov. 3 (6):833-7. [QxMD MEDLINE Link].
Shiota Y, Matsumoto H, Kanehisa Y, Tanaka M, Kanazawa K, Hiyama J, et al. Serum interleukin-5 levels in a case with allergic granulomatous angiitis. Intern Med. 1997 Oct. 36 (10):709-11. [QxMD MEDLINE Link].
Thiel J, Troilo A, Salzer U, Schleyer T, Halmschlag K, Rizzi M, et al. Rituximab as Induction Therapy in Eosinophilic Granulomatosis with Polyangiitis Refractory to Conventional Immunosuppressive Treatment: A 36-Month Follow-Up Analysis. J Allergy Clin Immunol Pract. 2017 Nov - Dec. 5 (6):1556-1563. [QxMD MEDLINE Link].
Jakiela B, Sanak M, Szczeklik W, Sokolowska B, Plutecka H, Mastalerz L, et al. Both Th2 and Th17 responses are involved in the pathogenesis of Churg-Strauss syndrome. Clin Exp Rheumatol. 2011 Jan-Feb. 29 (1 Suppl 64):S23-34. [QxMD MEDLINE Link].
Polzer K, Karonitsch T, Neumann T, Eger G, Haberler C, Soleiman A, et al. Eotaxin-3 is involved in Churg-Strauss syndrome--a serum marker closely correlating with disease activity. Rheumatology (Oxford). 2008 Jun. 47 (6):804-8. [QxMD MEDLINE Link].
Dallos T, Heiland GR, Strehl J, Karonitsch T, Gross WL, Moosig F, et al. CCL17/thymus and activation-related chemokine in Churg-Strauss syndrome. Arthritis Rheum. 2010 Nov. 62 (11):3496-503. [QxMD MEDLINE Link].
Zwerina J, Bach C, Martorana D, Jatzwauk M, Hegasy G, Moosig F, et al. Eotaxin-3 in Churg-Strauss syndrome: a clinical and immunogenetic study. Rheumatology (Oxford). 2011 Oct. 50 (10):1823-7. [QxMD MEDLINE Link].
Porges AJ, Redecha PB, Kimberly WT, Csernok E, Gross WL, Kimberly RP. Anti-neutrophil cytoplasmic antibodies engage and activate human neutrophils via Fc gamma RIIa. J Immunol. 1994 Aug 1. 153 (3):1271-80. [QxMD MEDLINE Link].
Xiao H, Heeringa P, Hu P, Liu Z, Zhao M, Aratani Y, et al. Antineutrophil cytoplasmic autoantibodies specific for myeloperoxidase cause glomerulonephritis and vasculitis in mice. J Clin Invest. 2002 Oct. 110 (7):955-63. [QxMD MEDLINE Link].
Ewert BH, Jennette JC, Falk RJ. Anti-myeloperoxidase antibodies stimulate neutrophils to damage human endothelial cells. Kidney Int. 1992 Feb. 41 (2):375-83. [QxMD MEDLINE Link].
Terrier B, Geri G, Chaara W, Allenbach Y, Rosenzwajg M, Costedoat-Chalumeau N, et al. Interleukin-21 modulates Th1 and Th17 responses in giant cell arteritis. Arthritis Rheum. 2012 Jun. 64 (6):2001-11. [QxMD MEDLINE Link].
Jen HY, Chuang YH, Lin SC, Chiang BL, Yang YH. Increased serum interleukin-17 and peripheral Th17 cells in children with acute Henoch-Schönlein purpura. Pediatr Allergy Immunol. 2011 Dec. 22 (8):862-8. [QxMD MEDLINE Link].
Nogueira E, Hamour S, Sawant D, Henderson S, Mansfield N, Chavele KM, et al. Serum IL-17 and IL-23 levels and autoantigen-specific Th17 cells are elevated in patients with ANCA-associated vasculitis. Nephrol Dial Transplant. 2010 Jul. 25 (7):2209-17. [QxMD MEDLINE Link].
Abdulahad WH, Stegeman CA, Limburg PC, Kallenberg CG. Skewed distribution of Th17 lymphocytes in patients with Wegener's granulomatosis in remission. Arthritis Rheum. 2008 Jul. 58 (7):2196-205. [QxMD MEDLINE Link].
Saito H, Tsurikisawa N, Tsuburai T, Oshikata C, Akiyama K. Cytokine production profile of CD4+ T cells from patients with active Churg-Strauss syndrome tends toward Th17. Int Arch Allergy Immunol. 2009. 149 Suppl 1:61-5. [QxMD MEDLINE Link].
Tsurikisawa N, Oshikata C, Tsuburai T, Sugano S, Nakamura Y, Shimoda T, et al. Th17 cells reflect colon submucosal pathologic changes in active eosinophilic granulomatosis with polyangiitis. BMC Immunol. 2015 Dec 29. 16:75. [QxMD MEDLINE Link].
Harrold LR, Andrade SE, Go AS, Buist AS, Eisner M, Vollmer WM, et al. Incidence of Churg-Strauss syndrome in asthma drug users: a population-based perspective. J Rheumatol. 2005 Jun. 32 (6):1076-80. [QxMD MEDLINE Link].
De Salvo G, Li Calzi C, Anastasi M, Lodato G. Branch retinal vein occlusion followed by central retinal artery occlusion in Churg-Strauss syndrome: unusual ocular manifestations in allergic granulomatous angiitis. Eur J Ophthalmol. 2009 Mar-Apr. 19 (2):314-7. [QxMD MEDLINE Link].
Knockaert DC. Cardiac involvement in systemic inflammatory diseases. Eur Heart J. 2007 Aug. 28 (15):1797-804. [QxMD MEDLINE Link].
Boggi U, Mosca M, Giulianotti PC, Naccarato AG, Bombardieri S, Mosca F. Surviving catastrophic gastrointestinal involvement due to Churg-Strauss syndrome: report of a case. Hepatogastroenterology. 1997 Jul-Aug. 44 (16):1169-71. [QxMD MEDLINE Link].
Zhang W, Zhou G, Shi Q, Zhang X, Zeng XF, Zhang FC. Clinical analysis of nervous system involvement in ANCA-associated systemic vasculitides. Clin Exp Rheumatol. 2009 Jan-Feb. 27 (1 Suppl 52):S65-9. [QxMD MEDLINE Link].
Ewert BH, Jennette JC, Falk RJ. Anti-myeloperoxidase antibodies stimulate neutrophils to damage human endothelial cells. Kidney Int. 1992 Feb. 41 (2):375-83. [QxMD MEDLINE Link].
Keogh KA, Specks U. Churg-Strauss syndrome: clinical presentation, antineutrophil cytoplasmic antibodies, and leukotriene receptor antagonists. Am J Med. 2003 Sep. 115 (4):284-90. [QxMD MEDLINE Link].
Yılmaz İ, Tutar N, Şimşek ZÖ, Oymak FS, Gülmez İ. Clinical and Serological Features of Eosinophilic and Vasculitic Phases of Eosinophilic Granulomatosis with Poliangiitis: a Case Series of 15 Patients. Turk Thorac J. 2017 Jul. 18 (3):72-77. [QxMD MEDLINE Link].
Vaglio A, Strehl JD, Manger B, Maritati F, Alberici F, Beyer C, et al. IgG4 immune response in Churg-Strauss syndrome. Ann Rheum Dis. 2012 Mar. 71 (3):390-3. [QxMD MEDLINE Link].
Hellmich B, Gross WL. Recent progress in the pharmacotherapy of Churg-Strauss syndrome. Expert Opin Pharmacother. 2004 Jan. 5 (1):25-35. [QxMD MEDLINE Link].
Ribi C, Cohen P, Pagnoux C, Mahr A, Arène JP, Lauque D, et al. Treatment of Churg-Strauss syndrome without poor-prognosis factors: a multicenter, prospective, randomized, open-label study of seventy-two patients. Arthritis Rheum. 2008 Feb. 58 (2):586-94. [QxMD MEDLINE Link].
Roccatello D, Baldovino S, Alpa M, Rossi D, Napoli F, Naretto C, et al. Effects of anti-CD20 monoclonal antibody as a rescue treatment for ANCA-associated idiopathic systemic vasculitis with or without overt renal involvement. Clin Exp Rheumatol. 2008 May-Jun. 26 (3 Suppl 49):S67-71. [QxMD MEDLINE Link].
Guillevin L, Pagnoux C, Karras A, Khouatra C, Aumaître O, et al. Rituximab versus azathioprine for maintenance in ANCA-associated vasculitis. N Engl J Med. 2014 Nov 6. 371 (19):1771-80. [QxMD MEDLINE Link].
Sinico RA, Di Toma L, Maggiore U, Bottero P, Radice A, Tosoni C, et al. Prevalence and clinical significance of antineutrophil cytoplasmic antibodies in Churg-Strauss syndrome. Arthritis Rheum. 2005 Sep. 52 (9):2926-35. [QxMD MEDLINE Link].

Media Gallery

of 0

Author

Nir Shimony, MD Assistant Professor of Neurological Surgery, Division of Pediatric Neurosurgery, Department of Surgery, St Jude Children’s Research Hospital, Le Bonheur Neuroscience Institute, Le Bonheur Children’s Hospital, Baptist Children’s Hospital, and Semmes Murphey Clinic, University of Tennessee Health Science Center College of Medicine; Adjunct Faculty, Department of Neurosurgery, Johns Hopkins University School of Medicine

Nir Shimony, MD is a member of the following medical societies: American Association of Neurological Surgeons, American Epilepsy Society, Congress of Neurological Surgeons, International Society of Pediatric Neurosurgery, Society for Neuro-Oncology

Disclosure: Nothing to disclose.

Coauthor(s)

George I Jallo, MD Professor of Neurosurgery, Pediatrics, and Oncology, Director, Clinical Pediatric Neurosurgery, Department of Neurosurgery, Johns Hopkins University School of Medicine

George I Jallo, MD is a member of the following medical societies: American Association of Neurological Surgeons, American Medical Association, American Society of Pediatric Neurosurgeons

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Stephen L Nelson, Jr, MD, PhD, FAACPDM, FAAN, FAAP, FANA Professor of Pediatrics, Neurology, Neurosurgery, and Psychiatry, Medical Director, Tulane Center for Autism and Related Disorders, Tulane University School of Medicine; Pediatric Neurologist and Epileptologist, Ochsner Hospital for Children; Professor of Neurology, Louisiana State University School of Medicine

Stephen L Nelson, Jr, MD, PhD, FAACPDM, FAAN, FAAP, FANA is a member of the following medical societies: American Academy for Cerebral Palsy and Developmental Medicine, American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, American Medical Association, American Neurological Association, Association of Military Surgeons of the US, Child Neurology Society, Southern Pediatric Neurology Society

Disclosure: Nothing to disclose.

Additional Contributors

Robert J Baumann, MD Professor of Neurology and Pediatrics, Department of Neurology, University of Kentucky College of Medicine

Robert J Baumann, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, Child Neurology Society

Disclosure: Nothing to disclose.

Robert Stanley Rust, Jr, MD, MA Former Thomas E Worrell Jr Professor of Epileptology and Neurology, Co-Director of FE Dreifuss Child Neurology and Epilepsy Clinics, Director, Child Neurology, University of Virginia School of Medicine; Chair-Elect, Child Neurology Section, American Academy of Neurology

Robert Stanley Rust, Jr, MD, MA is a member of the following medical societies: American Academy of Neurology, American Epilepsy Society, American Headache Society, American Neurological Association, Child Neurology Society, International Child Neurology Association, Society for Pediatric Research

Disclosure: Nothing to disclose.