Churg-strauss syndrome: clinical symptoms, complementary investigations, prognosis and outcome, and treatment

Semin Respir Crit Care Med. 2011 Jun;32(3):298-309. doi: 10.1055/s-0031-1279826. Epub 2011 Jun 14.

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.

MeSH terms

  • Adrenal Cortex Hormones / administration & dosage*
  • Adrenal Cortex Hormones / therapeutic use
  • Asthma / complications
  • Azathioprine / administration & dosage
  • Azathioprine / therapeutic use
  • Churg-Strauss Syndrome* / diagnosis
  • Churg-Strauss Syndrome* / epidemiology
  • Churg-Strauss Syndrome* / physiopathology
  • Churg-Strauss Syndrome* / therapy
  • Cyclophosphamide / administration & dosage
  • Cyclophosphamide / therapeutic use
  • Diagnosis, Differential
  • Eosinophilia / etiology
  • Humans
  • Immunosuppressive Agents / administration & dosage*
  • Immunosuppressive Agents / therapeutic use
  • Incidence
  • Physical Therapy Modalities
  • Plasma Exchange
  • Prevalence
  • Prognosis
  • Treatment Outcome

Substances

  • Adrenal Cortex Hormones
  • Immunosuppressive Agents
  • Cyclophosphamide
  • Azathioprine