title: Clinical features and outcomes in 348 patients with polyarteritis nodosa: a systematic retrospective study of patients diagnosed between 1963 and 2005 and entered into the French Vasculitis Study Group Database.
abstract: OBJECTIVE
  Previous studies of polyarteritis nodosa (PAN) included patients with microscopic polyangiitis, because these entities were not distinguished prior to the Chapel Hill Consensus Conference (CHCC). This study was undertaken to describe the main characteristics of and long-term outcomes in patients with well-characterized PAN diagnoses.
  
  
  METHODS
  We conducted a systematic retrospective study of 348 patients who were diagnosed as having PAN between March 1963 and October 2005, were registered in the French Vasculitis Study Group database, and satisfied the American College of Rheumatology and CHCC criteria. Patient characteristics and outcomes were analyzed and compared according to hepatitis B virus (HBV) status.
  
  
  RESULTS
  At diagnosis, the mean +/- SD age was 51.2 +/- 17.3 years. The most frequent findings were general symptoms (93.1%), neurologic manifestations (79%), skin involvement (49.7%), abdominal pain (35.6%), and hypertension (34.8%); 66.2% had renal artery microaneurysms; 70.1% had histologically proven PAN. Patients with HBV-related PAN (n = 123) had more frequent peripheral neuropathy, abdominal pain, cardiomyopathy, orchitis, and hypertension compared with patients with non-HBV-related PAN (n = 225). During a mean +/- SD followup of 68.3 +/- 63.5 months, 76 patients (21.8%) relapsed (63 with non-HBV-related PAN [28%] versus 13 with HBV-related PAN [10.6%]; P < 0.001); 86 patients (24.7%) died (44 with non-HBV-related PAN [19.6%] versus 42 with HBV-related PAN [34.1%]; P = 0.003). Five-year relapse-free survival rates were 59.4% (95% confidence interval [95% CI] 52.6-67.0) versus 67.0% (95% CI 58.5-76.8) for non-HBV-related PAN and HBV-related PAN, respectively. Multivariate analysis retained age >65 years, hypertension, and gastrointestinal manifestations requiring surgery or at least consultation with a surgeon as independent predictors of death, whereas patients with cutaneous manifestations or non-HBV-related PAN had a higher risk of relapse.
  
  
  CONCLUSIONS
  Our findings indicate that the rate of mortality from PAN remains high, especially for the elderly, and relapses do occur, particularly in patients with non-HBV-related PAN with cutaneous manifestations.
authors.affiliation: Université Paris Descartes, Hôpital Cochin, Assistance Publique Hôpitaux de Paris, Paris, France. christian.pagnoux@cch.aphp.fr
authors.forename: Christian
authors.initials: C
authors.lastname: Pagnoux
authors.forename: Raphaèle
authors.initials: R
authors.lastname: Seror
authors.forename: Corneliu
authors.initials: C
authors.lastname: Henegar
authors.forename: Alfred
authors.initials: A
authors.lastname: Mahr
authors.forename: Pascal
authors.initials: P
authors.lastname: Cohen
authors.forename: Véronique
authors.initials: V
authors.lastname: Le Guern
authors.forename: Boris
authors.initials: B
authors.lastname: Bienvenu
authors.forename: Luc
authors.initials: L
authors.lastname: Mouthon
authors.forename: Loïc
authors.initials: L
authors.lastname: Guillevin
mesh_terms: D015746:Abdominal Pain / Q000401:mortality / Q000473:pathology; D000328:Adult; D000368:Aged; D000783:Aneurysm / Q000401:mortality / Q000473:pathology; D001706:Biopsy; D015897:Comorbidity; D016208:Databases, Factual; D005260:Female; D005500:Follow-Up Studies; D005602:France / Q000453:epidemiology; D006801:Humans; D006973:Hypertension / Q000401:mortality / Q000473:pathology; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D010523:Peripheral Nervous System Diseases / Q000401:mortality / Q000473:pathology; D010488:Polyarteritis Nodosa / Q000401:mortality* / Q000473:pathology*; D011237:Predictive Value of Tests; D012008:Recurrence; D012189:Retrospective Studies; D012871:Skin Diseases / Q000401:mortality / Q000473:pathology