Risk Predictors for Lymphoma Development in Sjogren Syndrome - A Systematic Review

Abstract

Introduction: Sjogren syndrome is a systemic autoimmune disease characterized by lymphocytic infiltrates of salivary and tears glands, leading to oral and ocular dryness and by autoantibody secretion. The association between primary Sjogren syndrome and lymphoma, mostly non-Hodgkin ́s lymphoma or lymphoproliferative disease, has been documented for the past 40 years. The aim of our systematic review was to evaluate the available evidence regarding the identification of serological and hematological predictors for lymphoma in Sjogren syndrome patients. Materials and Methods: Articles were searched and selected through PubMed. Hand search from the relevant journals was also performed. Articles were reviewed and analyzed. The articles were reviewed based on serological and hematological risk predictors such as Anti-Ro antibodies, Anti-La,C3 levels, C4 levels, Cryoglobulins, Lymphopenia, anemia, antinuclear antibodies (ANA), and Rheumatoid Factor positivity. Results: Review of these articles showed significant associations of prognostic factors with development of lymphoma. Within the limitations, Identification of a high-risk phenotype for lymphoma development at the time of Sjogren Syndrome diagnosis has been a major diagnostic challenge over years. Conclusion: All these factors act as a guide to identify patients with high risk at the time of diagnosis leading to an increase in disease free survival rate.