title: Churg-strauss syndrome: clinical symptoms, complementary investigations, prognosis and outcome, and treatment.
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.
authors.affiliation: Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases (including Vasculitis, Scleroderma), Hôpital Cochin, Assistance Publique-Hôpitaux de Paris, France.
authors.forename: Bertrand
authors.initials: B
authors.lastname: Dunogué
authors.forename: Christian
authors.initials: C
authors.lastname: Pagnoux
authors.forename: Loïc
authors.initials: L
authors.lastname: Guillevin
mesh_terms: D000305:Adrenal Cortex Hormones / Q000008:administration & dosage* / Q000627:therapeutic use; D001249:Asthma / Q000150:complications; D001379:Azathioprine / Q000008:administration & dosage / Q000627:therapeutic use; D015267:Churg-Strauss Syndrome / Q000175:diagnosis / Q000453:epidemiology / Q000503:physiopathology / Q000628:therapy; D003520:Cyclophosphamide / Q000008:administration & dosage / Q000627:therapeutic use; D003937:Diagnosis, Differential; D004802:Eosinophilia / Q000209:etiology; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage* / Q000627:therapeutic use; D015994:Incidence; D026741:Physical Therapy Modalities; D010951:Plasma Exchange; D015995:Prevalence; D011379:Prognosis; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D001379:Azathioprine