Overview of the management and prognosis of Sjögren's syndrome
- Alan N Baer, MD, FACP
Alan N Baer, MD, FACP
- Associate Professor of Medicine
- Director, Jerome L. Greene Sjogren’s Syndrome Center
- Johns Hopkins University School of Medicine
- Frederick B Vivino, MD, MS, FACR
Frederick B Vivino, MD, MS, FACR
- Director, Penn Sjögren's Syndrome Center
- Professor of Clinical Medicine
- Perelman School of Medicine, University of Pennsylvania
Sjögren's syndrome (SS) is a chronic, multisystem autoimmune disease characterized by lacrimal and salivary gland inflammation, with resultant dryness of the eyes and mouth and occasional glandular enlargement. In addition, a variety of systemic (so-called "extraglandular") manifestations may occur, including fatigue, musculoskeletal symptoms, rashes, and internal organ (eg, pulmonary, renal, hepatic, and neurologic) disease. There is also increased risk of non-Hodgkin B-cell lymphoma.
SS occurs in a primary form, not associated with other autoimmune rheumatic diseases, and in a secondary form, often associated with rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), or systemic sclerosis.
An overview of the systemic treatment and prognosis of SS will be reviewed here. The clinical manifestations and diagnosis of SS and the treatment of dry eyes, dry mouth (including the use of muscarinic agonists such as pilocarpine and cevimeline as secretagogues), other nonocular sicca symptoms, and manifestations affecting other organs and tissues are described in detail separately. (See "Clinical manifestations of Sjögren's syndrome: Exocrine gland disease" and "Clinical manifestations of Sjögren's syndrome: Extraglandular disease" and "Diagnosis and classification of Sjögren's syndrome" and "Treatment of dry eye in Sjögren's syndrome: General principles and initial therapy" and "Treatment of dry mouth and other non-ocular sicca symptoms in Sjögren's syndrome" and "Treatment of Sjögren's syndrome: Constitutional and non-sicca organ-based manifestations".)
TREATMENT GOALS AND PRINCIPLES
The goals of therapy in patients with Sjögren's syndrome (SS) are to ameliorate symptoms of dry eye and mouth, prevent complications of mucosal dryness (such as dental decay, corneal ulceration, or oral candidal infection), and detect and manage systemic manifestations and glandular lymphoproliferative disease. The following general principles apply to the management of patients with SS:
●Evaluation and management should be provided by a multidisciplinary team; a rheumatologist, an eye care professional, and a dentist or oral medicine specialist are often required for optimal care.
- Vivino FB, Gala I, Hermann GA. Change in final diagnosis on second evaluation of labial minor salivary gland biopsies. J Rheumatol 2002; 29:938.
- Baer AN, McAdams DeMarco M, Shiboski SC, et al. The SSB-positive/SSA-negative antibody profile is not associated with key phenotypic features of Sjögren's syndrome. Ann Rheum Dis 2015; 74:1557.
- Rasmussen A, Radfar L, Lewis D, et al. Previous diagnosis of Sjögren's Syndrome as rheumatoid arthritis or systemic lupus erythematosus. Rheumatology (Oxford) 2016; 55:1195.
- Seror R, Bootsma H, Saraux A, et al. Defining disease activity states and clinically meaningful improvement in primary Sjögren's syndrome with EULAR primary Sjögren's syndrome disease activity (ESSDAI) and patient-reported indexes (ESSPRI). Ann Rheum Dis 2016; 75:382.
- Seror R, Theander E, Brun JG, et al. Validation of EULAR primary Sjögren's syndrome disease activity (ESSDAI) and patient indexes (ESSPRI). Ann Rheum Dis 2015; 74:859.
- Ramos-Casals M, Brito-Zerón P, Solans R, et al. Systemic involvement in primary Sjogren's syndrome evaluated by the EULAR-SS disease activity index: analysis of 921 Spanish patients (GEAS-SS Registry). Rheumatology (Oxford) 2014; 53:321.
- Shiboski CH, Shiboski SC, Seror R, et al. 2016 American College of Rheumatology/European League Against Rheumatism classification criteria for primary Sjögren's syndrome: A consensus and data-driven methodology involving three international patient cohorts. Ann Rheum Dis 2017; 76:9.
- Yazdany J, Schmajuk G, Robbins M, et al. Choosing wisely: the American College of Rheumatology's Top 5 list of things physicians and patients should question. Arthritis Care Res (Hoboken) 2013; 65:329.
- Tzioufas AG, Boumba DS, Skopouli FN, Moutsopoulos HM. Mixed monoclonal cryoglobulinemia and monoclonal rheumatoid factor cross-reactive idiotypes as predictive factors for the development of lymphoma in primary Sjögren's syndrome. Arthritis Rheum 1996; 39:767.
- Risselada AP, Kruize AA, Goldschmeding R, et al. The prognostic value of routinely performed minor salivary gland assessments in primary Sjögren's syndrome. Ann Rheum Dis 2014; 73:1537.
- Theander E, Vasaitis L, Baecklund E, et al. Lymphoid organisation in labial salivary gland biopsies is a possible predictor for the development of malignant lymphoma in primary Sjögren's syndrome. Ann Rheum Dis 2011; 70:1363.
- Howlett DC, Menezes LJ, Lewis K, et al. Sonographically guided core biopsy of a parotid mass. AJR Am J Roentgenol 2007; 188:223.
- Theander E, Mandl T. Primary Sjögren's syndrome: diagnostic and prognostic value of salivary gland ultrasonography using a simplified scoring system. Arthritis Care Res (Hoboken) 2014; 66:1102.
- Hackett KL, Deane KH, Strassheim V, et al. A systematic review of non-pharmacological interventions for primary Sjögren's syndrome. Rheumatology (Oxford) 2015; 54:2025.
- Bartoloni E, Baldini C, Schillaci G, et al. Cardiovascular disease risk burden in primary Sjögren's syndrome: results of a population-based multicentre cohort study. J Intern Med 2015; 278:185.
- Ezra N, Jorizzo J. Hydroxychloroquine and smoking in patients with cutaneous lupus erythematosus. Clin Exp Dermatol 2012; 37:327.
- Leroux G, Costedoat-Chalumeau N, Hulot JS, et al. Relationship between blood hydroxychloroquine and desethylchloroquine concentrations and cigarette smoking in treated patients with connective tissue diseases. Ann Rheum Dis 2007; 66:1547.
- Bhargava R, Kumar P, Kumar M, et al. A randomized controlled trial of omega-3 fatty acids in dry eye syndrome. Int J Ophthalmol 2013; 6:811.
- Ariza-Ariza R, Mestanza-Peralta M, Cardiel MH. Omega-3 fatty acids in rheumatoid arthritis: an overview. Semin Arthritis Rheum 1998; 27:366.
- De Carolis S, Salvi S, Botta A, et al. The impact of primary Sjogren's syndrome on pregnancy outcome: our series and review of the literature. Autoimmun Rev 2014; 13:103.
- Petruzzi LM, Vivino FB. Sjögren's syndrome--implications for perioperative practice. AORN J 2003; 77:612.
- Belenguer R, Ramos-Casals M, Brito-Zerón P, et al. Influence of clinical and immunological parameters on the health-related quality of life of patients with primary Sjögren's syndrome. Clin Exp Rheumatol 2005; 23:351.
- Ioannidis JP, Vassiliou VA, Moutsopoulos HM. Long-term risk of mortality and lymphoproliferative disease and predictive classification of primary Sjögren's syndrome. Arthritis Rheum 2002; 46:741.
- Brito-Zerón P, Kostov B, Solans R, et al. Systemic activity and mortality in primary Sjögren syndrome: predicting survival using the EULAR-SS Disease Activity Index (ESSDAI) in 1045 patients. Ann Rheum Dis 2016; 75:348.
- Singh AG, Singh S, Matteson EL. Rate, risk factors and causes of mortality in patients with Sjögren's syndrome: a systematic review and meta-analysis of cohort studies. Rheumatology (Oxford) 2016; 55:450.
- Ramos-Casals M, Anaya JM, García-Carrasco M, et al. Cutaneous vasculitis in primary Sjögren syndrome: classification and clinical significance of 52 patients. Medicine (Baltimore) 2004; 83:96.
- Ramos-Casals M, Font J, Garcia-Carrasco M, et al. Primary Sjögren syndrome: hematologic patterns of disease expression. Medicine (Baltimore) 2002; 81:281.
- Voulgarelis M, Dafni UG, Isenberg DA, Moutsopoulos HM. Malignant lymphoma in primary Sjögren's syndrome: a multicenter, retrospective, clinical study by the European Concerted Action on Sjögren's Syndrome. Arthritis Rheum 1999; 42:1765.
- Ramos-Casals M, Brito-Zerón P, Yagüe J, et al. Hypocomplementaemia as an immunological marker of morbidity and mortality in patients with primary Sjogren's syndrome. Rheumatology (Oxford) 2005; 44:89.
- TREATMENT GOALS AND PRINCIPLES
- PRETREATMENT EVALUATION
- Confirmation of the diagnosis
- Assessment of disease activity and severity
- - Laboratory evaluation
- Testing for all patients at initial evaluation
- Testing in confirmed SS to define subset, activity, and prognosis
- Additional studies
- Determining disease subset
- NONPHARMACOLOGIC AND PREVENTIVE INTERVENTIONS
- GENERAL APPROACH TO DRUG THERAPY
- THERAPY FOR ORGAN-BASED DISEASE AND CONSTITUTIONAL SYMPTOMS
- SPECIAL CONSIDERATIONS
- Management during pregnancy
- General anesthesia
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS