Factors Associated With Disease Damage in Puerto Ricans With Primary Sjögren Syndrome.

Abstract

The aim of this study was to determine the association of demographic parameters, clinical manifestations, disease activity, and pharmacologic therapy with disease damage in a group of Puerto Ricans with primary Sjögren syndrome (pSS). A cross-sectional study was conducted in 100 Hispanics of Puerto Rico with pSS. Patients were 21 years or older and fulfilled the 2012 American College of Rheumatology classification criteria for pSS. Demographic factors, lifestyle behaviors, extraglandular manifestations, serologic tests, comorbidities, pharmacologic therapy, disease activity (per European League Against Rheumatism Sjögren Syndrome Disease Activity Index), and disease damage (per Sjögren Syndrome Disease Damage Index [SSDDI]) were assessed. Patients with disease damage (SSDDI ≥1) and without damage (SSDDI = 0) were compared using bivariate analysis and multivariate regression analysis adjusted for age, sex, and disease duration. The mean age of patients was 52.8 years; 94% were women. The mean disease duration was 5.9 years. Thirty-nine patients had disease damage. Disease damage was mainly attributed to pulmonary fibrosis and peripheral neuropathy. In the bivariate analysis, disease damage was associated with low C3 and C4, coronary artery disease, infections, and higher activity index and was more frequently treated corticosteroids and azathioprine. In the multivariate analysis, low C3, disease activity, and corticosteroid exposure retained significance. In this population of Puerto Ricans with pSS, C3 and C4 hypocomplementemia, coronary artery disease, infections, and exposure to corticosteroids and azathioprine were associated with damage accrual. Clinicians should be aware of these factors to identify those who may require close follow-up and early therapeutic intervention.