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Semin Respir Crit Care Med 2011; 32(3): 298-309
DOI: 10.1055/s-0031-1279826
© Thieme Medical Publishers

Churg-Strauss Syndrome: Clinical Symptoms, Complementary Investigations, Prognosis and Outcome, and Treatment

Bertrand Dunogué1 , 2 , Christian Pagnoux1 , 2 , Loïc Guillevin1 , 2
  • 1Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases (including Vasculitis, Scleroderma), Hôpital Cochin, Assistance Publique–Hôpitaux de Paris, Paris, France
  • 2Université Paris Descartes, Paris, France
Further Information

Publication History

Publication Date:
14 June 2011 (online)

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ABSTRACT

Churg-Strauss syndrome (CSS), first described in 1951, is a rare vasculitis of small- and medium-sized vessels. It is characterized by a constant association with asthma and eosinophilia, and by the presence of anti-myeloperoxidase (MPO) anti-neutrophil cytoplasmic antibodies (ANCA) in ~40% of the patients. Vasculitis typically develops in a previously asthmatic and eosinophilic middle-aged patient and most frequently involves the peripheral nerves and skin. Other organs, however, may be affected and must be screened for vasculitis, especially those associated with a poorer prognosis, such as the heart, kidney, and gastrointestinal tract, as assessed by the recently revised Five-Factor Score (FFS). Overall survival of CSS patients is excellent, but relapses are not uncommon and require maintenance or steroid-sparing therapies, depending on the original FFS-based prognosis at diagnosis. All patients require corticosteroids, often for prolonged periods, combined with immunosuppressants [e.g., induction (cyclophosphamide) and maintenance therapy (azathioprine)], for those with poorer prognoses. Recent insights, especially concerning clinical differences associated with ANCA status, showed that CSS patients might constitute a heterogeneous group, both clinically and pathogenically. Future therapies might reflect these differences more strongly.

KEYWORDS

Churg-Strauss syndrome - vasculitis - ANCA - steroids - immunosuppressants

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Loïc GuillevinM.D. 

Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases (including Vasculitis, Scleroderma), INSERM U1060, Hôpital Cochin, Assistance Publique–Hôpitaux de Paris, Université Paris Descartes

27, rue du faubourg Saint-Jacques, 75679 Paris Cedex 14, France

Email: loic.guillevin@cch.aphp.fr