Primary Sjogren Syndrome: Clinical Features and Management

Abstract

Primary Sjögren syndrome (SS) is a chronic inflammatory disease affecting the lacrimal and salivary glands, resulting in dry eyes and dry mouth. It may also lead to systemic manifestations, including fatigue, arthritis, lung involvement, kidney disease, neuropathy, and vasculitis, and predisposes to B cell lymphoma. The diagnosis of primary SS is based on a composite of clinical, serologic, and pathologic features, namely, objective evidence of dry eyes and dry mouth and either a positive test for anti-SSA (Ro) antibodies or a positive labial salivary gland biopsy. Primary SS may be confused with other conditions affecting the lacrimal and salivary glands, such as chronic hepatitis C virus infection, sarcoidosis, and a new entity, IgG4-related disease. The management of patients with primary SS is largely symptomatic and consists of tear supplementation, regular dental hygiene, and liberal use of moisturizers. Cyclosporine 0.05% ophthalmic emulsion and lifitegrast 5% ophthalmic solution are approved agents for the treatment of dry eyes. Two oral secretagogues, pilocarpine and cevimeline, have been shown in trials to significantly reduce the symptoms of dry eyes and dry mouth. Although hydroxychloroquine may reduce fatigue and joint pain, the results of a randomized controlled trial failed to confirm its efficacy in this clinical setting. More serious organ system involvement may be managed with corticosteroids and other immunomodulatory drugs. Although no disease-modifying drugs are currently approved for the treatment of primary SS, a robust pipeline of promising therapies for this disease is currently in development. This review contains 3 figures, 5 tables, and 69 references. Key words: anti-Ro/SSA antibodies, B cell lymphoma, classification criteria, EULAR Sjögren’s syndrome Patient Reported Index (ESSPRI), EULAR Sjögren’s Syndrome Disease Activity Index (ESSDAI), hydroxychloroquine,  keratoconjunctivitis sicca, rituximab, Sjögren syndrome