Risk, Predictors, and Clinical Characteristics of Lymphoma Development in Primary Sjögren's Syndrome

• Published in: Seminars in arthritis and rheumatism Vol. 41; no. 3; pp. 415 - 423
• Main Authors: Solans-Laqué, Roser, MD, PhD, López-Hernandez, Andrés, MD, PhD, Angel Bosch-Gil, Josep, MD, PhD, Palacios, Andrés, MD, PhD, Campillo, Mercedes, MD, PhD, Vilardell-Tarres, Miquel, MD, PhD
• Format: Journal Article
• Language: English
• Published: New York, NY Elsevier Inc 01-12-2011; Elsevier
• Subjects: Adolescent; Adult; Aged; Aged, 80 and over; Biological and medical sciences; Diseases of the osteoarticular system; Female; Hematologic and hematopoietic diseases; Humans; hypocomplementemia; Incidence; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis; lymphocytopenia; lymphoma; Lymphoma - epidemiology; Lymphoma - etiology; Male; Medical sciences; Middle Aged; mortality; Predictive Value of Tests; primary Sjögren syndrome; prognostic factors; Rheumatology; Risk; Risk Factors; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Sjogren's Syndrome - complications; Sjogren's Syndrome - epidemiology; Survival Rate; primary Sjögren syndrome; mortality; lymphocytopenia; prognostic factors; hypocomplementemia; lymphoma; Immunopathology; Prognosis; Leukopenia; Stomatology; Mortality; Rheumatology; Autoimmune disease; Malignant hemopathy; Epidemiology; Lymphoma; Sjögren syndrome; Symptomatology; Eye disease; Hypocomplementemia; Lymphoproliferative syndrome; Systemic disease; Risk factor; Predictive factor; Cancer; Lymphocytopenia
• ISSN: 0049-0172; 1532-866X
• DOI: 10.1016/j.semarthrit.2011.04.006

Objective To assess the risk and predictors of lymphoma development in a large cohort of patients with primary Sjögren's syndrome (pSS). Methods Cox-regression analyses were used to study the predictive value of clinical and laboratory findings at pSS diagnosis, and Kaplan-Meier survival curves to compare survival probability between patients who developed lymphoma and the total cohort. Expected risk for lymphoma was calculated by comparison with the background population. Results Eleven (4.5%) from 244 patients developed a non-Hodgkin lymphoma (NHL). Diffuse large B-cell and mucosa-associated lymphoid tissue lymphomas occurred at a similar frequency. Three (27.3%) patients died: 2 due to transformation from mucosa-associated lymphoid tissue to diffuse large B-cell. Purpura (HR 8.04, 95% confidence interval [CI] 2.33-27.67), parotidomegaly (HR 6.75, 95%CI 1.89-23.99), anemia (HR 3.43, 95%CI 1.04-11.35), leukopenia (HR 8.70, 95%CI 2.38-31.82), lymphocytopenia (HR 16.47, 95%CI 3.45-78.67), hypergammaglobulinemia (HR 4.06, 95%CI 1.06-15.58), low C3 (HR 36.65, 95%CI 10.65-126.18), and low C4 (HR 39.70, 95%CI 8.85-126.18) levels at pSS diagnosis were significant predictors of NHL development, but only hypocomplementemia and lymphocytopenia were independent risk factors. Hypocomplementemia was related to earlier development of NHL and higher mortality. The cumulative risk of developing lymphoma ranged from 3.4% in the first 5 years to 9.8% at 15 years. Standardized incidence ratio (95%CI) for NHL development was 15.6 (95%CI 8.7-28.2). Conclusions Patients with pSS have a 16-fold increased risk of developing lymphoma. This risk increases with time. Hypocomplementemia and lymphocytopenia at pSS diagnosis are the strongest predictors. Survival is clearly reduced in patients with hypocomplementemia. Indolent lymphomas tend to evolve over time toward a more aggressive histologic type.