Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content + Download Section PDF Listen ++ For further information, see CMDT Part 20-14: Sjögren Syndrome + Key Features Download Section PDF Listen +++ +++ Essentials for Diagnosis ++ Dryness of eyes and dry mouth (sicca components); they occur alone or with rheumatoid arthritis or other connective tissue disease Rheumatoid factor and antinuclear autoantibodies common Increased incidence of lymphoma +++ General Considerations ++ A systemic autoimmune disorder Clinical presentation is usually dominated by dryness of the eyes and mouth due to immune-mediated dysfunction of the lacrimal and salivary glands Can occur in isolation ("primary" Sjögren syndrome) or in association with another rheumatic disease, most frequently rheumatoid arthritis +++ Associated conditions ++ Rheumatoid arthritis Systemic lupus erythematosus (SLE) Primary biliary cirrhosis Scleroderma Polymyositis Hashimoto thyroiditis Polyarteritis Interstitial pulmonary fibrosis +++ Demographics ++ Predominantly a disease of women, with a female:male ratio of 9:1 Greatest incidence between ages 40 and 60 years + Clinical Findings Download Section PDF Listen +++ +++ Symptoms and Signs ++ Eyes Ocular burning, itching, ropy secretions "Grain of sand in the eye" sensation Parotid glands Enlargement may be chronic or relapsing Develops in one-third of patients Dryness of the mouth (xerostomia) leads to difficulty in swallowing dry foods (like crackers), to constant thirst for fluids, and to severe dental caries There may be loss of taste and smell Systemic manifestations Dysphagia, pancreatitis Pleuritis, obstructive airways disease and interstitial lung disease (in the absence of smoking) Neuropsychiatric dysfunction Small vessel vasculitis Kidney Renal tubular acidosis (type I, distal) occurs in 20% of patients Chronic interstitial nephritis, which may result in impaired kidney function, may be seen +++ Differential Diagnosis ++ Sicca complex associated with other autoimmune disease, eg, sarcoidosis, rheumatoid arthritis, SLE, scleroderma Other causes of dry mouth or eyes, eg, anticholinergics, mumps, irradiation, seasonal allergies, irritation from smoking IgG4-related systemic disease + Diagnosis Download Section PDF Listen +++ +++ Laboratory Tests ++ Rheumatoid factor is found in 70% of patients Antibodies against SS-A and SS-B are often present (Table 20–7) When SS-A antibodies are present, extraglandular manifestations are far more common +++ Diagnostic Procedures ++ Lip biopsy reveals characteristic lymphoid foci in accessory salivary glands Biopsy of the parotid gland should be reserved for patients with atypical presentations such as unilateral gland enlargement The Schirmer test measures the quantity of tears secreted + Treatment Download Section PDF Listen +++ +++ Medications ++ Topical ocular 0.05% cyclosporine improves ocular symptoms and signs of dryness Pilocarpine (5 mg four times daily) and the acetylcholine derivative cevimeline (30 mg three times daily) are helpful for severe xerostomia Prednisone or various immunosuppressive medications are used to treat severe systemic inflammatory manifestations Atropinic drugs and decongestants decrease salivary secretions and should be avoided Extraglandular disease, including arthritis, vasculitis, or pulmonary manifestations, are treated with similar immunosuppressive medications as SLE or rheumatoid arthritis +++ Therapeutic Procedures ++ Treatment of sicca symptoms is symptomatic and supportive Artificial tears applied frequently will relieve ocular symptoms and avert further desiccation Sipping water frequently or using sugar-free gums and hard candies usually relieves dry mouth symptoms A program of oral hygiene is essential to preserve dentition + Outcome Download Section PDF Listen +++ +++ Complications ++ A spectrum of lymphoproliferation ranging from benign to malignant may be found Malignant lymphomas and Waldenström macroglobulinemia occur nearly 50 times more frequently than can be explained by chance alone in primary Sjögren syndrome +++ Prognosis ++ Usually benign and consistent with a normal life span Prognosis is mainly influenced by the nature of the associated disease The patients (3–10% of the total Sjögren population) at greatest risk for developing lymphoma have Severe dryness Marked parotid gland enlargement Splenomegaly Vasculitis Peripheral neuropathy Anemia Mixed monoclonal cryoglobulinemia +++ When to Refer ++ Presence of systemic symptoms or signs Ocular dryness not responsive to artificial tears +++ When to Admit ++ Presence of severe systemic signs such as vasculitis unresponsive to outpatient management + References Download Section PDF Listen +++ + +Brito-Zerón P et al. Sjögren syndrome. Nat Rev Dis Primers. 2016 Jul 7;2:16047. [PubMed: 27383445] + +Mariette X et al. Primary Sjögren's syndrome. N Engl J Med. 2018 Mar 8;378(10):931–9. [PubMed: 29514034] + +Ramos-Casals M et al. EULAR recommendations for the management of Sjögren's syndrome with topical and systemic therapies. Ann Rheum Dis. 2020 Jan;79(1):3–18. [PubMed: 31672775] + +Verstappen GM et al. The value of rituximab treatment in primary Sjögren's syndrome. Clin Immunol. 2017 Sep;182:62–71. [PubMed: 28478105]