PT  - JOURNAL ARTICLE
AU  - Esha Abrol
AU  - Cristina González-Pulido
AU  - Juan M Praena-Fernández
AU  - David A Isenberg
TI  - A retrospective study of long-term outcomes in 152 patients with primary Sjögren's syndrome: 25-year experience
AID  - 10.7861/clinmedicine.14-2-157
DP  - 2014 Apr 01
TA  - Clinical Medicine
PG  - 157--164
VI  - 14
IP  - 2
4099  - http://www.rcpjournals.org/content/14/2/157.short
4100  - http://www.rcpjournals.org/content/14/2/157.full
SO  - Clin Med2014 Apr 01; 14
AB  - The objective of this study was to evaluate the 25-year outcome of patients with primary Sjögren's syndrome (pSS). One hundred and fifty-two patients diagnosed with pSS (American–European classification criteria) were retrospectively and descriptively analysed (1986–2011). Of all 152 patients, 55.9% were alive, 18.4% had died and 25.7% discontinued follow-up (mostly due to old age). Malignancy affected 28.3% and non-Hodgkin's lymphoma (NHL) affected 10.5%. The adjusted risk for development of NHL was an odds ratio (OR) of 10.5 (95% confidence interval [CI]: 3.05–36.42) in patients with vasculitis (p<0.001), and OR 3.4 (95% CI 1.05–11.2) in the presence of glandular complications (parotid swelling, lymphadenopathy) (p < 0.041). Seventy-five patients (49.3%) developed other autoimmune diseases (autoimmune thyroid disease [15.8%], pulmonary fibrosis [7.2%] and vasculitis [10.5%]). Although the course of pSS is relatively benign, over 25 years patients experience more clinical complications than previously described. In addition, vasculitis and glandular manifestations were significant predictors for NHL.