title: Long-Term Followup of a Multicenter Cohort of 101 Patients With Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss).
abstract: OBJECTIVE
  To assess the long-term outcome in eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA).
  
  
  METHODS
  A total of 101 patients fulfilling the American College of Rheumatology criteria for EGPA were included between 1990 and 2011. Clinical features, antineutrophil cytoplasm autoantibodies (ANCAs), and Five-Factors Score (FFS) were assessed at diagnosis. Overall and cumulative survival rates, relapse-free survival, and sequelae were studied based on ANCA status and FFS.
  
  
  RESULTS
  The rate of cardiomyopathy did not differ according to ANCA status. A total of 79.6% of patients achieved first remission, but 81.1% relapsed. ANCA-positive patients did not relapse more frequently but exhibited more severe disease with mononeuritis (P = 0.0004) and renal involvement (P = 0.02). Being Italian was the only prognostic factor associated with a higher relapse-free survival (P = 0.01), thanks to a longer maintenance of immunosuppressive drugs, suggesting the need for prolonged low-dose corticosteroids. Overall, survival reached 93.1% after a median followup of 6 years. No factor was associated with mortality, but patients over age 65 years with cardiomyopathy or ANCA positivity had more serious outcomes. Sequelae affected 83.2% of patients. Ear, nose, and throat (ENT) involvement was a protective factor for renal (P = 0.04) and cardiac (P = 0.03) morbidity. ANCA positivity was correlated with chronic kidney disease (P = 0.03) and chronic neurologic disability (P = 0.02).
  
  
  CONCLUSIONS
  The actual challenges of EGPA management concern morbidity prevention and quality of life improvement. Long-term corticosteroid treatment appears to reduce relapse risk. ENT involvement is associated with less renal and cardiac morbidity. ANCA positivity predicts renal and neurologic damage.
authors: Hôpital Edouard Herriot, Lyon, France.
authors: Cécile-Audrey
authors: CA
authors: Durel
authors: Université de Lyon and Epidémiologie, Pharmacologie, Investigation Clinique, Information Médicale, Hôpital Femme-Mère-Enfant, Lyon, France.
authors: Julien
authors: J
authors: Berthiller
authors: Specialista Ambulatoriale Allergologia e Immunologia Clinica, Casa di Cura Polispecialistica Sant'Elena, Cagliari, Italy.
authors: Silvia
authors: S
authors: Caboni
authors: Addenbrooke's Hospital, Cambridge, UK.
authors: David
authors: D
authors: Jayne
authors: Hôpital Edouard Herriot, Lyon, France.
authors: Jacques
authors: J
authors: Ninet
authors: Hôpital Edouard Herriot, Lyon, France.
authors: Arnaud
authors: A
authors: Hot
mesh_terms: D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000009:adverse effects / Q000627:therapeutic use*; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood; D015415:Biomarkers / Q000097:blood; D015267:Churg-Strauss Syndrome / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy* / Q000453:epidemiology; D018450:Disease Progression; D018572:Disease-Free Survival; D005060:Europe / Q000453:epidemiology; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000009:adverse effects / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D012008:Recurrence; D012074:Remission Induction; D012189:Retrospective Studies; D012720:Severity of Illness Index; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000305:Adrenal Cortex Hormones; D019268:Antibodies, Antineutrophil Cytoplasmic; D015415:Biomarkers; D007166:Immunosuppressive Agents