Sjögrens

Sjögren's syndrome is a chronic autoimmune disease characterized by lymphocytic infiltration and dysfunction of exocrine glands, leading to reduced moisture production. It can be classified as primary when it occurs alone, or secondary when it accompanies another autoimmune disease such as rheumatoid arthritis, systemic lupus erythematosus, or systemic sclerosis.

The hallmark symptoms are dryness of the eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia), which can cause eye irritation, a gritty sensation, dental problems, and difficulties with swallowing or speaking. Additional signs may include fatigue, joint pain, and swelling of the salivary glands. The condition is more common in women and typically presents in middle age, though it can occur at other ages.

Pathophysiologically, Sjögren's syndrome involves autoimmune attack on moisture-producing glands, with lymphocytic infiltration and B-cell hyperactivity. Autoantibodies such as anti-Ro/SSA and anti-La/SSB are commonly present. Extraglandular involvement can occur, affecting skin, lungs, kidneys, nerves, and blood vessels in some patients.

Diagnosis combines clinical features with objective tests. These may include assessments of tear production (Schirmer test), ocular surface staining, and salivary flow measurements, as well as serologic testing for autoantibodies and inflammatory markers. A lip biopsy showing focal lymphocytic sialadenitis can support the diagnosis. Classification criteria distinguish primary Sjögren's from secondary forms.

Treatment is focused on symptom relief and management of systemic disease, as there is no cure. Management strategies include artificial tears and saliva substitutes, stimulants such as pilocarpine or cevimeline, and thorough dental care. For systemic or extraglandular involvement, therapies may include hydroxychloroquine, methotrexate, or other immunosuppressants; biologics like rituximab may be used in select cases.