Outcome and Prognostic Factors During the Course of Primary Small-Vessel Vasculitides
LAURA PAVONE, CHIARA GRASSELLI, ELISABETTA CHIERICI, UMBERTO MAGGIORE, GIANNI GARINI, NICOLETTA RONDA, PAOLO MANGANELLI, ALBERTO PESCI, WALTER TROISE RIODA, BRUNO TUMIATI, GIOVANNI PAVESI, AUGUSTO VAGLIO and CARLO BUZIO, on behalf of the Secondary and Primary Vasculitides (Se.Pri.Va.) Study Group
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 ≥ 100 mm/h, and serum creatinine level ≥ 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 ≥ 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.
Conclusion. 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. (First Release June 15 2006; J Rheumatol 2006;33:1299–306)
Key Indexing Terms:
PAUCI-IMMUNE SMALL-VESSEL VASCULITIS
From the Dipartimento di Clinica Medica, Nefrologia e Scienze della Prevenzione, and Dipartimento di Neuroscienze, Università degli Studi di Parma, Parma; Unità Operativa di Reumatologia e Medicina Interna, Azienda Ospedaliera di Parma, Parma; and II Divisione Medicina Interna, Azienda Ospedaliera Santa Maria Nuova, Reggio Emilia, Italy.
L. Pavone, MD; C. Grasselli, MD; U. Maggiore, MD; G. Garini, MD; N. Ronda, MD; A. Pesci, MD; A. Vaglio, MD; C. Buzio, MD, Dipartimento di Clinica Medica, Nefrologia e Scienze della Prevenzione, Università degli Studi di Parma; E. Chierici, MD; G. Pavesi, MD, Sezione di Neurologia, Dipartimento di Neuroscienze, Università degli Studi di Parma; P. Manganelli, MD; W. Troise Rioda, MD, Unità Operativa di Reumatologia e Medicina Interna, Azienda Ospedaliera di Parma; B. Tumiati, MD, II Divisione Medicina Interna, Azienda Ospedaliera Santa Maria Nuova.
Address reprint requests to Dr. L. Pavone, Dipartimento di Clinica Medica, Nefrologia e Scienze della Prevenzione, Ospedale Maggiore di Parma, Università degli Studi di Parma, Via Gramsci 14, 43100 Parma, Italy. E-mail: firstname.lastname@example.org
Accepted for publication February 13, 2006.