title: Outcome and prognostic factors during the course of primary small-vessel vasculitides.
abstract: OBJECTIVE
  To identify the prognostic factors of relapse and/or death during the course of primary small-vessel vasculitides (PSVV), and to differentiate their prognostic relevance by the type of vasculitis.
  
  
  METHODS
  Seventy-five patients were retrospectively followed up after diagnosis: 36 with Wegener's granulomatosis (WG), 23 with Churg-Strauss syndrome (CSS), and 16 with microscopic polyangiitis. Cox regression analysis was used to identify the significant predictors of relapse and death.
  
  
  RESULTS
  Gastrointestinal (GI) involvement was associated with an increased risk of relapse, mainly in the patients with CSS, whereas renal disease and perinuclear antineutrophil cytoplasmic antibody positivity were correlated with a lower risk of relapse. Presence of nasal Staphylococcus aureus tended to increase the risk of relapse in CSS [hazard ratio (HR) 4.45, p = 0.087], but to decrease it in WG (HR 0.12, p = 0.066). Older age, renal and hepatic involvement, erythrocyte sedimentation rate >or= 100 mm/h, and serum creatinine level >or= 1.5 mg/dl were all related to higher risk of death in univariate analysis; however, only cerebral (HR 8.52, p = 0.021) and hepatic involvement (HR 4.40, p = 0.028) and serum creatinine level >or= 1.5 mg/dl (HR 5.72, p = 0.044) were independently correlated with an unfavorable prognosis for survival. The risk of death associated with each of these indicators did not depend on the form of PSVV.
  
  
  CONCLUSIONS
  GI involvement increases the risk of relapse in CSS, whereas the prognostic significance of nasal S. aureus in terms of relapse seems to be opposite in patients with CSS and those with WG. Patients with cerebral, hepatic, and renal involvement have the poorest prognosis for survival. Our data do not show that the prognostic relevance of these factors depends on the form of PSVV.
authors.affiliation: Dipartimento di Clinica Medica, Nefrologia e Scienze della Prevenzione, and Dipartimento di Neuroscienze, Università degli Studi di Parma, Parma, Italy. laurapavone@hotmail.com
authors.forename: Laura
authors.initials: L
authors.lastname: Pavone
authors.forename: Chiara
authors.initials: C
authors.lastname: Grasselli
authors.forename: Elisabetta
authors.initials: E
authors.lastname: Chierici
authors.forename: Umberto
authors.initials: U
authors.lastname: Maggiore
authors.forename: Gianni
authors.initials: G
authors.lastname: Garini
authors.forename: Nicoletta
authors.initials: N
authors.lastname: Ronda
authors.forename: Paolo
authors.initials: P
authors.lastname: Manganelli
authors.forename: Alberto
authors.initials: A
authors.lastname: Pesci
authors.forename: Walter Troise
authors.initials: WT
authors.lastname: Rioda
authors.forename: Bruno
authors.initials: B
authors.lastname: Tumiati
authors.forename: Giovanni
authors.initials: G
authors.lastname: Pavesi
authors.forename: Augusto
authors.initials: A
authors.lastname: Vaglio
authors.forename: Carlo
authors.initials: C
authors.lastname: Buzio
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015267:Churg-Strauss Syndrome / Q000401:mortality / Q000473:pathology; D015897:Comorbidity; D018572:Disease-Free Survival; D005260:Female; D005767:Gastrointestinal Diseases / Q000401:mortality / Q000473:pathology; D014890:Granulomatosis with Polyangiitis / Q000401:mortality / Q000473:pathology; D006801:Humans; D007558:Italy / Q000453:epidemiology; D008297:Male; D008833:Microcirculation / Q000473:pathology*; D008875:Middle Aged; D012008:Recurrence; D012189:Retrospective Studies; D012307:Risk Factors; D013203:Staphylococcal Infections / Q000401:mortality / Q000473:pathology; D013211:Staphylococcus aureus / Q000302:isolation & purification; D015996:Survival Rate; D014657:Vasculitis / Q000145:classification / Q000401:mortality* / Q000473:pathology*