A retrospective study of long-term outcomes in 152 patients with primary Sjogren's syndrome: 25-year experience.
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.
University College London Medical School, London UK.
Esha
E
Abrol
Cristina
C
González-Pulido
Juan M
JM
Praena-Fernández
David A
DA
Isenberg
D000328:Adult; D000368:Aged; D001327:Autoimmune Diseases / Q000453:epidemiology*; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008206:Lymphatic Diseases / Q000209:etiology; D008228:Lymphoma, Non-Hodgkin / Q000453:epidemiology*; D008297:Male; D008875:Middle Aged; D009369:Neoplasms / Q000453:epidemiology; D016017:Odds Ratio; D011379:Prognosis; D011658:Pulmonary Fibrosis / Q000453:epidemiology; D012189:Retrospective Studies; D012307:Risk Factors; D012859:Sjogren's Syndrome / Q000150:complications / Q000453:epidemiology* / Q000401:mortality; D013967:Thyroiditis, Autoimmune / Q000453:epidemiology; D014657:Vasculitis / Q000453:epidemiology
Sjögren’s syndrome; autoimmune diseases; long-term follow-up; neoplasia; non-Hodgkin’s lymphoma