Rheumatology Advance Access published online on June 14, 2007
Rheumatology, doi:10.1093/rheumatology/kem079
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Predicting adverse outcomes in primary Sjögren's syndrome: identification of prognostic factors
Department of Autoimmune Diseases and 1Statistic Unit, Department of Public Health, Department of Medicine, School of Medicine, University of Barcelona, Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Hospital Clínic, Barcelona, Spain
Correspondence to:
M. Ramos-Casals, Servei de Malalties Autoimmunes, Hospital Clínic, C/Villarroel, 170, 08036-Barcelona, Spain. Phone: 34-93-2275774, Fax: 34-93-2275774. E-mail: mramos{at}clinic.ub.es
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Abstract |
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Objective. To identify features present at diagnosis that were prospectively associated with adverse outcomes in a large cohort of patients with primary Sjögren's syndrome (SS).
Methods. Two hundred and sixty-six patients diagnosed with primary SS in our department between 1984 and 2002 were consecutively included and followed up. Outcomes measured were vasculitis, B-cell lymphoma and death. Cox regression analysis was used to evaluate the effect of variables at diagnosis on outcomes.
Results. Twenty-five (9%) patients developed vasculitis. Multivariate analysis identified parotid scintigraphy grades III or IV (HR 3.55, P = 0.05) and C4 levels <0.11 g/l (HR 8.26, P < 0.001) as variables predicting the development of vasculitis. Nine (3%) patients developed B-cell lymphoma. Multivariate analysis identified C3 levels <0.82 g/l (HR 7.54, P = 0.016) as a predictive factor of lymphoma development. Twenty-five (9%) patients died during follow-up. Systemic involvement (HR 4.51, P = 0.022), vasculitis (HR 4.58, P = 0.042), C4 levels <0.11 g/l (HR 5.47, P = 0.027) and cryoglobulins (HR 4.58, P = 0.013) were independently associated with death. The presence of at least two of the above-mentioned predictive factors (parotid scintigraphy, vasculitis, hypocomplementaemia and cryoglobulinaemia) was associated with a lower survival in comparison with patients with no factor (log rank and Breslow tests <0.001).
Conclusion. The main prognostic factors for an adverse outcome identified in our cohort of patients with primary SS were vasculitis, severe involvement in parotid scintigraphy, hypocomplementaemia and/or cryoglobulins at diagnosis. Patients with at least two of these factors need a closer follow-up.
KEY WORDS: Sjögren syndrome, Mortality, Vasculitis, Hypocomplementaemia, Cryoglobulins
In memoriam (1953–2006).
Submitted 7 December 2006;
revised version accepted 7 December 2006.
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