Sjogren’s Syndrome
摘要
Sjogren’s syndrome is known to be the commonest autoimmune rheumatic illness. Classically a B cell-mediated disease with type I interferon signatures in the majority of cases, the role of cholinergic system especially muscarinic receptors also is coming into focus. Predominantly manifested as dry mouth and dry eyes, it has a range of extraglandular manifestations like renal tubular acidosis, demyelinating illness, and purpura which present more to the rheumatologist as a referral bias. The importance of therapy is because of its likelihood to evolve into a lymphoma in a subset of cases. While the usual investigations like Schirmer’s test, lissamine green test for dry eye, salivary flow rates for dry mouth, and autoantibodies are done, salivary ultrasound is coming to replace biopsies in recent times. The latest classification criteria have a scoring system for clinical trials. Therapy is still largely the same with topical ointments for sicca, pilocarpine for dry mouth, and immunosuppressive drugs for the more aggressive ones—ianalumab seems a promising new biologic. Prognosis is now better with earlier diagnosis and treatment.