Churg–Strauss Syndrome

  • M. Habib Bouhaouala
  • Kamel Bouslama
  • Mehdi Charfi
  • Nadia Mhiri
  • M. Habib Ben Romdhane
  • Majed El Beji
  • Lotfi Hendaoui
Part of the Medical Radiology book series (MEDRAD)


Churg–Strauss syndrome is a rare diffuse vasculitis that is closely associated with a severe asthma. The clinical diagnosis may be difficult in the early stages. Nevertheless, the onset of severe asthma in an adult and the emergence of extrarespiratory manifestations should alert one to the probable diagnosis of Churg–Strauss syndrome. Imaging is useful to assess the cardiorespiratory system and the sinus cavities. Pathological confirmation can be challenging as the characteristic histological features are often incomplete. Prognosis is usually good with corticosteroid therapy alone or in combination with immunosuppressive drugs.


Allergic Rhinitis Constrictive Pericarditis Small Vessel Vasculitis Strauss Syndrome Eosinophilic Gastroenteritis 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


  1. Abril A, Calamia KT, Cohen MD (2003) The Churg Strauss syndrome (allergic granulomatous angiitis): review and update. Semin Arthritis Rheum 33:106–114PubMedCrossRefGoogle Scholar
  2. Abu-Shakra M, Smythe H, Lewtas J et al (1994) Outcome of polyarteritis nodosa and Churg–Strauss syndrome. An analysis of twenty-five patients. Arthritis Rheum 37:1798–1803PubMedCrossRefGoogle Scholar
  3. Alter P, Maisch B (2006) Endomyocardial fibrosis in Churg–Strauss syndrome assessed by cardiac magnetic resonance imaging. Int J Cardiol 108:112–113PubMedCrossRefGoogle Scholar
  4. Beduneau G, Berard H, Dominique S et al (2005) Rechute de syndrome de Churg et Strauss sous antileucotriène. Rev Med Interne 26:829–837CrossRefGoogle Scholar
  5. Bohl S, Schulz-Menger J (2010) Cardiovascular magnetic resonance imaging of non-ischaemic heart disease: established and emerging applications. Heart Lung Circ 19:117–132PubMedCrossRefGoogle Scholar
  6. Boyer D, Vargas SO, Slattery D et al (2006) Churg–Strauss syndrome in children: a clinical and pathologic review. Pediatrics 118:e914–e920PubMedCrossRefGoogle Scholar
  7. Castaner E, Alguersuari A, Gallardo X et al (2010) When to suspect pulmonary vasculitis: radiologic and clinical clues. Radiographics 30:33–53PubMedCrossRefGoogle Scholar
  8. Chakravarty K (1997) Vasculitis by organ systems. Baillieres Clin Rheum 2:357–393CrossRefGoogle Scholar
  9. Chumbley LC, Harrison EG Jr, DeRemee RA (1977) Allergic granulomatosis and angiitis (Churg–Strauss syndrome). Report and analysis of 30 cases. Mayo Clin Proc 52:477–484PubMedGoogle Scholar
  10. Chung MP, Yi CA, Lee HY, Han J et al (2010) Imaging of pulmonary vasculitis. Radiology 2:322–341CrossRefGoogle Scholar
  11. Churg J, Strauss L (1951) Allergic angiitis and periarteritis nodosa. Am J Pathol 27:277–301PubMedGoogle Scholar
  12. Churg A, Brallas M, Cronin SR et al (1995) Formes frustes of Churg–Strauss syndrome. Chest 108:320–323PubMedCrossRefGoogle Scholar
  13. Cottin V, Cordier JF (2003) Churg–Strauss syndrome. Rev Pneumol Clin 59:17–24PubMedGoogle Scholar
  14. Davies DJ (2005) Small vessel vasculitis. Cardiovasc Pathol 14:335–346PubMedCrossRefGoogle Scholar
  15. Della Rossa A, Baldini C, Tavoni A et al (2002) Churg–Strauss syndrome: clinical and serological features of 19 patients from a single Italian centre. Rheumatology 41:1286–1294PubMedCrossRefGoogle Scholar
  16. Dennert RM, Van Paassen P, Schalla S et al (2010) Cardiac Involvement in Churg–Strauss syndrome. Arthritis Rheum 62:627–634PubMedGoogle Scholar
  17. Dietz A, Hubner C, Andrassy K (1998) Macrolide antibiotic-induced vasculitis (Churg–Strauss syndrome). Laryngorhinootologie 77:111–114PubMedCrossRefGoogle Scholar
  18. Esposito A, De Cobelli F, Belloni E et al (2010) Magnetic resonance imaging of a hypereosinophilic endocarditis with apical thrombotic obliteration in Churg–Strauss syndrome complicated with acute abdominal aortic embolic occlusion. Int J Cardiol 143:e48–e50PubMedCrossRefGoogle Scholar
  19. Fries JF, Hunder GG, Bloch DA et al (1990) The American College of Rheumatology 1990 criteria for the classification of vasculitis. Summary. Arthritis Rheum 33:1135–1136CrossRefGoogle Scholar
  20. Gaskin G, Clutterbuck EJ, Pusey CD (1991) Renal disease in the Churg–Strauss syndrome. Diagnosis, management and outcome. Contrib Nephrol 94:58–65PubMedGoogle Scholar
  21. Gayraud M, Guillevin L, le Toumelin P et al (2001) Longterm followup of polyarteritis nodosa, microscopic polyangiitis, and Churg–Strauss syndrome: analysis of four prospective trials including 278 patients. Arthritis Rheum 44:666–675PubMedCrossRefGoogle Scholar
  22. Guillevin L, Pagnoux C (2006) Principales vascularites nécrosantes systémiques. EMC (Elsevier SAS, Paris), Appareil locomoteur, 14-245-F-10Google Scholar
  23. Guillevin L, Le Thi Huong D, Godeau P et al (1988) Clinical findings and prognosis of polyarteritis nodosa and Churg–Strauss angiitis: a study in 165 patients. Br J Rheum 27:258–264CrossRefGoogle Scholar
  24. Guillevin L, Lhote F, Gayraud M et al (1996) Prognostic factors in polyarteritis nodosa and Churg–Strauss syndrome: a prospective study in 342 patients. Medicine 75:17–28PubMedCrossRefGoogle Scholar
  25. Guillevin L, Cohen P, Gayraud M et al (1999) Churg–Strauss syndrome. Clinical study and long-term follow-up of 96 patients. Medicine 78:26–37PubMedCrossRefGoogle Scholar
  26. Guillevin L, Pagnoux C, Cohen P (2004) Syndrome de Churg–Strauss. Rev Fr Allergol Ammunol Clin 44:96–102Google Scholar
  27. Guilpain P, Viallard JF, Lagarde P et al (2002) Churg–Strauss syndrome in two patients receiving montelukast. Rheumatology 41:535–539PubMedCrossRefGoogle Scholar
  28. Haas C, Geneau C, Odinot JM et al (1991) Allergic angiitis with granulomatosis: Churg–Strauss syndrome. Retrospective study of 16 cases. Ann Med Interne (Paris) 142:335–342Google Scholar
  29. Haas C, Le Jeunne C, Choubrac P et al (2001) Churg–Strauss syndrome. Retrospective study of 20 cases. Bull Acad Nat Med 185:1113–1130PubMedGoogle Scholar
  30. Hoffman G, Langford C (2005) Are they different forms of life in the antineutrophil cytoplasmic antibody universe (editorial)? Ann Intern Med 143:683–685PubMedGoogle Scholar
  31. Hunsaker JC, O’Connor WN, Lie JT (1992) Spontaneous coronary arterial dissection and isolated eosinophilic coronary arteritis: sudden cardiac death in a patient with a limited variant of Churg–Strauss syndrome. Mayo Clin Proc 67:761–766PubMedGoogle Scholar
  32. Huong DLT, De Gennesl C, Papal T et al (1996) Manifestations pleuropulmonaires des vascularites systémiques. Rev Med Interne 17:640–652PubMedCrossRefGoogle Scholar
  33. Imai H, Nakamoto Y, Hirokawa M et al (1989) Carbamazepine-induced granulomatous necrotizing angiitis with acute renal failure. Nephron 51:405–408PubMedCrossRefGoogle Scholar
  34. Jennette JC, Falk RJ, Andrassy K et al (1994) Nomenclature of systemic vasculitides: proposal of an international consensus conference. Arthritis Rheum 37:187–192PubMedCrossRefGoogle Scholar
  35. Kallenberg CG (2005) Churg–Strauss syndrome: just one disease entity (editorial)? Arthritis Rheum 52:2589–2593PubMedCrossRefGoogle Scholar
  36. Keogh KA, Specks U (2006) Churg–Strauss syndrome. Semin Respir Crit Care Med 27:148–157PubMedCrossRefGoogle Scholar
  37. Khasnis A, Langford CA (2009) Update on vasculitis. J Allerg Clin Immunol 123:1226–1236CrossRefGoogle Scholar
  38. Kim YK, Lee KS, Chung MP et al (2007) Pulmonary involvement in Churg–Stauss syndrome: an analysis of CT, clinical, and pathologic findings. Eur Radiol 17:3157–3165PubMedCrossRefGoogle Scholar
  39. Lanham JG, Churg J (1991) Churg–Strauss syndrome. In: Churg A, Churg J (eds) Systemic vasculitides. Igaku-Shoin, New York, pp 101–120Google Scholar
  40. Lanham JG, Elkon KB, Pusey CD et al (1984) Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg–Strauss syndrome. Medicine 63:65–81PubMedCrossRefGoogle Scholar
  41. Langford CA (2010) Vasculitis. J Allerg Clin Immunol S216–S225Google Scholar
  42. Lesens O, Hansmann Y, Nerson J et al (2002) Severe Churg-Strauss syndrome with mediastinal lymphadenopathy treated with interferon therapy. Eur J Intern Med 13:458–462PubMedCrossRefGoogle Scholar
  43. Levin DL, Edinburgh KJ, Hatabu H (1999) Churg–Strauss syndrome. Eur J Radiol 29:149–151PubMedCrossRefGoogle Scholar
  44. Lhote F (2007) Syndrome de Churg et Strauss. Presse Med 36:875–889PubMedCrossRefGoogle Scholar
  45. Lhote F, Cohen P, Guilpain P et al (2008) Syndrome de Churg et Strauss. Rev Prat 58:1165–1174PubMedGoogle Scholar
  46. Lie JT (1995) Histopathologic specificity of systemic vasculitis. Rheum Dis Clin North Am 21:883–910PubMedGoogle Scholar
  47. Lin TL, Wang CR, Liu MF (2001) Multiple colonic ulcers caused by Churg–Strauss syndrome in a 15-year-old girl. Clin Rheum 20:362–364CrossRefGoogle Scholar
  48. Luqmani R, Exley A, Kitas G et al (1997) Disease assessment and management of the vasculitides. Clin Rheumatol 11:424–446Google Scholar
  49. Mahr A, Guillevin L, Poissonnet M et al (2004) Prevalences of polyarteritis nodosa, microscopic polyangiitis, Wegener’s granulomatosis, and Churg–Strauss syndrome in a French urban multiethnic population in 2000: a capture–recapture estimate. Arthritis Rheum 51:92–99PubMedCrossRefGoogle Scholar
  50. Masi AT, Hunder GG, Lie JT et al (1990) The American College of Rheumatology 1990 criteria for the classification of Churg–Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum 33:1094–1100PubMedCrossRefGoogle Scholar
  51. Mathur S, Dooley J, Scheuer PJ (1990) Quinine induced granulomatous hepatitis and vasculitis. BMJ 300:613PubMedCrossRefGoogle Scholar
  52. Mavrogeni S, Tsirogianni AK, Gialafos EJ et al (2009) Detection of myocardial inflammation by contrast-enhanced MRI in a patient with Churg–Strauss syndrome. Int J Cardiol 131:e54–e55PubMedCrossRefGoogle Scholar
  53. Neumann T, Manger B, Schmid M et al (2009) Cardiac involvement in Churg–Strauss syndrome. Impact of endomyocarditis. Medicine 88:236–243PubMedCrossRefGoogle Scholar
  54. Noth I, Strek ME, Leff AR (2003) Churg–Strauss syndrome. Lancet 361:587–594PubMedCrossRefGoogle Scholar
  55. Ouertani D, Smiti Khanfir M, Larbi T et al (2009) Entéropathie exsudative au cours d’un syndrome de Churg et Strauss. Rev Med Interne 30:S77–S151Google Scholar
  56. Ozen S, Fuhlbrigge RC (2009) Update in paediatric vasculitis. Best Pract Res Clin Rheum 23:679–688CrossRefGoogle Scholar
  57. Pela G, Tirabassi G, Pattoneri P et al (2006) Cardiac involvement in the Churg–Strauss syndrome. Am J Cardiol 97:1519–1524PubMedCrossRefGoogle Scholar
  58. Pipitone N, Salvarani C (2008) Role of imaging in vasculitis and connective tissue diseases. Best Pract Res Clin Rheum 6:1075–1091CrossRefGoogle Scholar
  59. Puéchal X (2007) Antineutrophil cytoplasmic antibody-associated vasculitides. Rev Rhum 74:824–832CrossRefGoogle Scholar
  60. Reid AJ, Harrison BD, Watts RA et al (1998) Churg–Strauss in a district hospital. QJM 91:219–229PubMedCrossRefGoogle Scholar
  61. Ribi C, Cohen P, Pagnoux C et al (2008) Treatment of Churg–Strauss syndrome without poor-prognosis factors: a multicenter, prospective, randomized open-label study of seventy-two patients. Arthritis Rheum 58:586–594PubMedCrossRefGoogle Scholar
  62. Rosato E, Vitarelli A, Pisarri S et al (2009) An unusual manifestation in a patient with Churg–Strauss syndrome: isolated cardiac septum involvement. Joint Bone Spine 76:718–725CrossRefGoogle Scholar
  63. Roux M, Fabre M (2004) Macrolide-induced Churg–Strauss syndrome. Eur J Intern Med 15:474PubMedCrossRefGoogle Scholar
  64. Sablé-Fourtassou R, Cohen P, Mahr A et al (2005) Antineutrophil cytoplasmic antibodies and the Churg–Strauss syndrome. Ann Intern Med 143:632–638PubMedGoogle Scholar
  65. Saka N, Seo T, Shimano K et al (2009) A case of Churg–Strauss syndrome with refractory otitis media. Auris Nasus Larynx 36:79–81PubMedCrossRefGoogle Scholar
  66. Sakamoto S, Ohba S, Eguchi K et al (2005) Churg–Strauss syndrome presenting with subarachnoid hemorrhage from ruptured dissecting aneurysm of the intracranial vertebral artery. Clin Neurol Neurosurg 107:428–431PubMedCrossRefGoogle Scholar
  67. Sauvetre G, Faresb J, Caudron J et al (2010) Usefulness of magnetic resonance imaging in Churg–Strauss syndrome related cardiac involvement. A case series of three patients and literature review. Rev Med Interne 31:600–605PubMedCrossRefGoogle Scholar
  68. Sinico RA, Bottero P (2009) Churg–Strauss angiitis. Res Clin Rheum 23:355–366CrossRefGoogle Scholar
  69. Solans R, Bosh JA, Pérez-Bocanegra C et al (2001) Churg–Strauss syndrome: outcome and long term follow-up of 32 patients. Rheumatology 40:763–771PubMedCrossRefGoogle Scholar
  70. Szwarc D, Veillon F, Moser T et al (2009) Syndrome de Churg–Strauss sous omalizumab: une manifestation viscérale exceptionnelle. J Radiol 90:1737–1739PubMedCrossRefGoogle Scholar
  71. Takanashi T, Uchida S, Arita M et al (2001) Orbital inflammatory pseudotumor and ischemic vasculitis in Churg–Strauss syndrome: report of two cases and review of the literature. Ophthalmology 108:1129–1133PubMedCrossRefGoogle Scholar
  72. Val-Bernal JF, Mayorga M, Garcı-Alberdi E et al (2003) Churg–Strauss syndrome and sudden cardiac death. Cardiovasc Pathol 12:94–97PubMedCrossRefGoogle Scholar
  73. Vallejo E, Mendoza-Gonzalez C, Aranda A (2004) Churg–Strauss syndrome and myocardial perfusion SPECT imaging. J Nucl Cardiol 3:358–360CrossRefGoogle Scholar
  74. Vignaux O, Marmursztejn J, Cohen P et al (2007) Imagerie cardiaque dans les vascularites associAces aux ANCA. Presse Med 36:902–906PubMedCrossRefGoogle Scholar
  75. Wassmuth R, Göbel U, Natusch A et al (2008) Cardiovascular magnetic resonance imaging detects cardiac involvement in Churg–Strauss syndrome. J Card Fail 10:856–860CrossRefGoogle Scholar
  76. Watts RA, Scott DGI, Lane SE (2002) Epidemiology of Wegener’s granulomatosis, microscopic polyangiitis and Churg–Strauss syndrome. Cleve Clin J Med 69(Suppl 2):84–89Google Scholar
  77. Wechsler ME, Finn D, Gunawardena D et al (2000) Churg–Strauss syndrome in patients receiving montelukast as treatment for asthma. Chest 117:708–713PubMedCrossRefGoogle Scholar
  78. Zwerina J, Eger G, Englbrecht M, Manger B, Schett G (2008) Churg–Strauss syndrome in childhood: a systematic literature review and clinical comparison with adult patients. Semin Arthritis Rheum 39:108–115PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg  2011

Authors and Affiliations

  • M. Habib Bouhaouala
    • 1
  • Kamel Bouslama
    • 2
  • Mehdi Charfi
    • 1
  • Nadia Mhiri
    • 3
  • M. Habib Ben Romdhane
    • 4
  • Majed El Beji
    • 3
  • Lotfi Hendaoui
    • 5
  1. 1.Department of Medical Imaging, Medical School of TunisInterior Security Forces Teaching HospitalLa MarsaTunisia
  2. 2.Department of Internal Medicine, Medical School of TunisMongi Slim University HospitalLa Marsa, Sidi DaoudTunisia
  3. 3.Department of Pneumology, Medical School of TunisLa Rabta University HospitalTunis JabbariTunisia
  4. 4.Department of ImagingCentre Hospitalier Intercommunal des Portes de l’OiseCreteilFrance
  5. 5.Department of Medical Imaging and Imaging Guided InterventionsMongi Slim University HospitalLa Marsa, Sidi DaoudTunisia

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