Sjogrens

Sjögren's syndrome is a chronic autoimmune disease characterized by lymphocytic infiltration of exocrine glands, leading to dryness of the eyes and mouth (sicca). It can be primary (pSS) when it occurs alone, or secondary when associated with another autoimmune disease such as rheumatoid arthritis or lupus.

Dry eyes with a gritty sensation and reduced tear production, and dry mouth impacting speech, swallowing, and eating are typical. Salivary gland swelling may occur. Fatigue is common, and dental caries or mucosal infections can arise; extraglandular features such as arthritis or lung involvement may appear.

Pathophysiology involves autoimmune destruction of lacrimal and salivary glands with B-cell hyperactivity and autoantibodies such as anti-SSA (Ro) and anti-SSB (La). There is an increased risk of lymphoma, particularly marginal zone B-cell lymphoma of the salivary glands.

Diagnosis uses symptoms plus objective tests. Ocular tests include Schirmer or tear break-up time and staining. Salivary gland biopsy showing focal lymphocytic sialadenitis supports the diagnosis; serology often shows ANA with anti-SSA/SSB. Classification criteria also include imaging and glandular involvement.

Treatment emphasizes symptom relief and management of systemic disease. Artificial tears and saliva substitutes help; sialagogues such as pilocarpine or cevimeline aid saliva. Good dental care is important. Immunomodulatory therapies (hydroxychloroquine, methotrexate) are used for extraglandular disease; rituximab may be considered in refractory cases. Regular lymphoma monitoring is advised.

Most cases affect middle-aged women. The disorder is variable in severity, from mild dryness to systemic involvement. With appropriate care, prognosis is generally favorable, though lymphoma risk requires ongoing vigilance.