HSS Journal ®

, Volume 8, Issue 3, pp 313–319 | Cite as

Churg–Strauss Syndrome with Eosinophilic Myocarditis

A Clinical Pathology Conference Held by the Division of Rheumatology at Hospital for Special Surgery
  • Alana B. LevineEmail author
  • George Kalliolias
  • Mark Heaney
  • Yoshimi Endo
  • Adam Gersten
  • Jonathan W. Weinsaft
  • Robert F. Spiera
  • Anne Bass
  • Doruk Erkan
Clinical Pathology Conference

History and Physical Examination

An 85-year-old Chinese woman with a 4-year history of asthma presented with a severe headache for 1 week and substernal chest pain for 1 day.

Seven days prior to presentation, the patient was seen by her primary care physician for evaluation of a new-onset severe headache; she was prescribed an unknown dose of ibuprofen, which she took for 2 days before developing facial swelling and hives. The facial swelling and rash resolved shortly after discontinuing ibuprofen, but the headache continued. Two days prior to presentation, the patient went to the emergency room because of her headache; a non-contrast head computed tomography (CT) scan was negative and she was discharged without further workup. Two days later, the patient developed substernal chest pain. She returned to the emergency room and was admitted for further evaluation.

On admission, the patient reported frontal and temporal headache without nausea or photosensitivity. She also complained of...


Churg–Strauss Syndrome eosinophilic myocarditis hypereosinophila 



RS receives consulting and research funding from Roche-Genentech. All other author certifies that he or she has no commercial associations (e.g., consultancies, stock ownership, equity interest, patent/licensing arrangements, etc.) that might pose a conflict of interest in connection with the submitted article.

Each author certifies that his or her institution has approved the reporting of the case, that all investigations were conducted in conformity with the ethical principles of research, and that informed consent for participating in the study was obtained.


  1. 1.
    Bhagirath KM, Paulson K, Ahmadie R, Bhalla RS, Robinson D, Jassal DS. Clinical utility of cardiac magnetic resonance imaging in Churg–Strauss syndrome: case report and review of the literature. Rheumatol Int 2009;29:445–9.PubMedCrossRefGoogle Scholar
  2. 2.
    Blanchard C, Rothenberg ME. Biology of the eosinophil. Adv Immunol 2009;101:81–121.PubMedCrossRefGoogle Scholar
  3. 3.
    Cartin-Ceba R, Keogh KA, Specks U, Sethi S, Fervenza FC. Rituximab for the treatment of Churg–Strauss syndrome with renal involvement. Nephrol Dial Transplant 2011; 26:2865–71.PubMedCrossRefGoogle Scholar
  4. 4.
    Churg J, Strauss L. Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol 1951;27:277–301.PubMedGoogle Scholar
  5. 5.
    Cools J, DeAngelo DJ, Gotlib J, et al. A tyrosine kinase created by fusion of the PDGFRA and FIP1L1 genes as a therapeutic target of imatinib in idiopathic hypereosinophilic syndrome. N Engl J Med 2003;348:1201–14.PubMedCrossRefGoogle Scholar
  6. 6.
    Donvik KK, Omdal R. Churg–Strauss syndrome successfully treated with rituximab. Rheumatol Int 2009;31:89–91.PubMedCrossRefGoogle Scholar
  7. 7.
    Gotlib J. Eosinophilic myeloid disorders: new classification and novel therapeutic strategies. Curr Opin Hematol 2010;17:117–24.PubMedCrossRefGoogle Scholar
  8. 8.
    Greenan TJ, Grossman RI, Goldberg HI. Cerebral vasculitis: MR imaging and angiographic correlation. Radiology 1992;182:65–72.PubMedGoogle Scholar
  9. 9.
    Guillevin L, Cohen P, Mahr A, et al. Treatment of polyarteritis nodosa and microscopic polyangiitis with poor prognosis factors: a prospective trial comparing glucocorticoids and six or twelve cyclophosphamide pulses in sixty-five patients. Arthritis Rheum 2003;49:93–100.PubMedCrossRefGoogle Scholar
  10. 10.
    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.CrossRefGoogle Scholar
  11. 11.
    Heiserman JE, Drayer BP, Keller PJ, Fram EK. Intracranial vascular stenosis and occlusion: evaluation with three-dimensional time-of-flight MR 12. angiography. Radiology 1992;185:667–73.PubMedGoogle Scholar
  12. 12.
    Hogan SP, Rosenberg HF, Moqbel R, et al. Eosinophils: biological properties and role in health and disease. Clin Exp Allergy 2008;38:709–50.PubMedCrossRefGoogle Scholar
  13. 13.
    Jennette JC, Falk RJ, Andrassy K, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum 1994;37:187–92.PubMedCrossRefGoogle Scholar
  14. 14.
    Kahn JE, Grandpeix-Guyodo C, Marroun I, et al. Sustained response to mepolizumab in refractory Churg–Strauss syndrome. J Allergy Clin Immunol 2010;125:267–70.PubMedCrossRefGoogle Scholar
  15. 15.
    Kim S, Marigowda G, Oren E, Israel E, Wechsler ME. Mepolizumab as a steroid-sparing treatment option in patients with Churg–Strauss syndrome. J Allergy Clin Immunol 2010;125:1336–43.PubMedCrossRefGoogle Scholar
  16. 16.
    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;63:65–81.Google Scholar
  17. 17.
    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;33:1094–100.PubMedCrossRefGoogle Scholar
  18. 18.
    Metzgeroth G, Walz C, Erben P, et al. Safety and efficacy of imatinib in chronic eosinophilic leukaemia and hypereosinophilic syndrome: a phase-II study. Br J Haematol 2008;143:707–15.PubMedCrossRefGoogle Scholar
  19. 19.
    Nair P, Pizzichini MM, Kjarsgaard M, et al. Mepolizumab for prednisone-dependent asthma with sputum eosinophilia. N Engl J Med 2009;360:985–93.PubMedCrossRefGoogle Scholar
  20. 20.
    Pagnoux C, Guilpain P, Guillevin L. Churg–Strauss syndrome. Curr Opin Rheumatol 2007;19:25–32.PubMedCrossRefGoogle Scholar
  21. 21.
    Ribi C, Cohen P, Pagnoux C, 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;58:586–94.PubMedCrossRefGoogle Scholar
  22. 22.
    Rothenberg ME, Klion AD, Roufosse FE, et al. Treatment of patients with the hypereosinophilic syndrome with mepolizumab. N Engl J Med 2008;358:1215–28.PubMedCrossRefGoogle Scholar
  23. 23.
    Sable-Fourtassou R, Cohen P, Mahr A, et al. Antineutrophil cytoplasmic antibodies and the Churg–Strauss syndrome. Ann Intern Med 2005;143:632–8.PubMedGoogle Scholar
  24. 24.
    Saech J, Owczarczyk K, Rosgen S, Petereit H, Hallek M, Rubbert-Roth A. Successful use of rituximab in a patient with Churg–Strauss syndrome and refractory central nervous system involvement. Ann Rheum Dis 2010;69:1254–5.PubMedCrossRefGoogle Scholar
  25. 25.
    Sehgal M, Swanson JW, DeRemee RA, Colby TV. Neurologic manifestations of Churg–Strauss syndrome. Mayo Clin Proc 1995;70:337–41.PubMedCrossRefGoogle Scholar
  26. 26.
    Sinico RA, Di Toma L, Maggiore U, et al. Prevalence and clinical significance of antineutrophil cytoplasmic antibodies in Churg–Strauss syndrome. Arthritis Rheum 2005;52:2926–35.PubMedCrossRefGoogle Scholar
  27. 27.
    Stock KW, Radue EW, Jacob AL, Bao XS, Steinbrich W. Intracranial arteries: prospective blinded comparative study of MR angiography and DSA in 50 patients. Radiology 1995;195:451–6.PubMedGoogle Scholar
  28. 28.
    Wadleigh M, Tefferi A. Classification and diagnosis of myeloproliferative neoplasms according to the 2008 World Health Organization criteria. Int J Hematol 2010;91:174–9.PubMedCrossRefGoogle Scholar
  29. 29.
    Zwerina J, Axmann R, Jatzwauk M, Sahinbegovic E, Polzer K, Schett G. Pathogenesis of Churg–Strauss syndrome: recent insights. Autoimmunity 2009;42:376–9.PubMedCrossRefGoogle Scholar

Copyright information

© Hospital for Special Surgery 2012

Authors and Affiliations

  • Alana B. Levine
    • 1
    Email author
  • George Kalliolias
    • 1
  • Mark Heaney
    • 2
  • Yoshimi Endo
    • 3
  • Adam Gersten
    • 4
  • Jonathan W. Weinsaft
    • 5
  • Robert F. Spiera
    • 1
  • Anne Bass
    • 1
  • Doruk Erkan
    • 1
  1. 1.Division of RheumatologyHospital for Special SurgeryNew YorkUSA
  2. 2.Department of MedicineMemorial Sloan-Kettering Cancer CenterNew YorkUSA
  3. 3.Department of RadiologyHospital for Special SurgeryNew YorkUSA
  4. 4.Department of PathologyWeill-Cornell Medical College–New York Presbyterian HospitalNew YorkUSA
  5. 5.Division of CardiologyWeill-Cornell Medical College–New York Presbyterian HospitalNew YorkUSA

Personalised recommendations