Sjögren syndrome is a chronic autoimmune disorder characterized by lymphocytic infiltration of exocrine glands. It can occur as a primary disease alone or as a secondary form linked with other autoimmune disorders, especially RA, SLE, or SSc. The syndrome can present at any age and in either sex, although women in the fifth and sixth decades of life are the most commonly affected. Typical symptoms include dry eyes, dry mouth, and dry skin (xerosis). The ophthalmologist may be the first physician to see these patients because of their ocular symptoms. Parotid and lacrimal gland enlargement (Mikulicz syndrome) can occur in more severely affected individuals.
Patients with primary Sjögren syndrome may have a number of systemic manifestations, including upper-airway dryness, mucous plug development, purpuric vasculitis, and hyperglobulinemia. Although about 50% of patients report symptoms of arthralgia, arthritis is less common; and some patients may have subclinical inflammatory myopathy. Mild anemia across all cell lines is found in approximately 20% of patients, and the overall risk of non-Hodgkin lymphoma is increased. Central and peripheral neurologic involvement may be present and mimic multiple sclerosis or psychiatric disorders.
The American College of Rheumatology and European League Against Rheumatism recently updated their classification criteria for primary Sjögren syndrome based on 5 objective measures. These include specific findings on labial salivary gland biopsy, presence of anti-Ro antibodies, ocular staining, abnormal Schirmer testing, and reduced unstimulated salivary flow. The benefit of minor salivary gland biopsy is to confirm the disease, to rule out other disease processes (eg, sarcoidosis, amyloidosis), and to serve as a prognostic indicator for the development of future lymphoma. Serum level of Fms-like tyrosine kinase 3 ligand (Flt-3L) also shows promise as a potential predictor for the development of this malignancy. Ongoing research is directed at finding key biomarkers for Sjögren syndrome to provide more sensitive and specific testing. Among those being studied are profilin and carbonic anhydrase I (CA-I) in saliva and cathepsin S in tears.
Treatment is aimed at relief of symptoms and substitution or supplementation for reduced or absent secretions. Immunosuppression may be necessary in patients with systemic manifestations. (See also BCSC Section 8, External Disease and Cornea.)
Chen W, Cao H, Lin J, et al. Biomarkers for primary Sjögren’s syndrome. Genomics Proteomics Bioinformatics. 2015;13(4):219–223.
Shiboski CH, Shiboski SC, Seror R, et al; International Sjögren’s Syndrome Criteria Working Group. 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(1):9–16.
Excerpted from BCSC 2020-2021 series: Section 1 - Update on General Medicine. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.