SjögrenSyndrom

Sjögren's syndrome, sometimes written as SjögrenSyndrom, is a chronic autoimmune disorder characterized by lymphocytic infiltration of exocrine glands, leading to dry mouth and dry eyes. It can occur as a primary condition or secondarily to other autoimmune diseases such as rheumatoid arthritis, SLE, or scleroderma.

The hallmark symptoms are sicca: xerostomia (dry mouth) causing difficulties with speaking, swallowing, and dental caries; xerophthalmia from reduced tear production with irritation, gritty sensation, and photophobia. Many patients also experience fatigue and arthralgia. Extraglandular features can include arthritis, lung involvement, kidney tubulointerstitial nephritis, vasculitis, and neuropathies.

Pathophysiology involves B-cell hyperactivity and autoantibody production, especially anti-SSA/Ro and anti-SSB/La. Diagnosis relies on objective tests such as the Schirmer test for tear production, tear breakup time, ocular surface staining, and measurement of saliva flow, together with salivary gland biopsy showing focal lymphocytic sialadenitis. Classification criteria combine laboratory tests and glandular involvement to establish a diagnosis.

Treatment is largely symptomatic and, when systemic involvement is present, disease-modifying. Artificial tears and ocular lubricants help eye symptoms; saliva substitutes and sialogogues such as cevimeline or pilocarpine aid xerostomia. Immunosuppressive therapy (for example hydroxychloroquine, methotrexate, or rituximab) may be used for systemic disease or severe glandular involvement, with management tailored to organ involvement and comorbidities.

Prognosis is variable; Sjögren's syndrome is typically a chronic condition with a risk of lymphoma, particularly mucosa-associated lymphoid tissue (MALT) lymphoma. Regular monitoring for extraglandular manifestations is advised. Epidemiology shows a higher prevalence in women, with onset commonly in middle age; estimates vary by diagnostic criteria and population.