AKT · Musculoskeletal/Connective tissue diseaselow yield

Sjögren syndrome

Autoimmune lymphocytic infiltration of exocrine glands (anti-Ro/anti-La)

Overview

An autoimmune disease in which lymphocytic infiltration destroys exocrine (especially lacrimal and salivary) glands, causing dry eyes and dry mouth (sicca). It may be primary or secondary to another connective tissue disease (RA, SLE). Anti-Ro (SSA) and anti-La (SSB) antibodies are characteristic; the major long-term risk is a markedly increased incidence of B-cell (MALT) lymphoma.

Recognise

Dry eyes (keratoconjunctivitis sicca — gritty sensation) and dry mouth (xerostomia, dental caries, difficulty swallowing dry food)
Bilateral parotid swelling, fatigue, arthralgia, Raynaud's, vaginal dryness
Anti-Ro/anti-La antibodies; anti-Ro crosses the placenta → risk of congenital heart block / neonatal lupus

Red flags

Persistent unilateral parotid enlargement/lymphadenopathy → exclude B-cell (MALT) lymphoma (markedly increased risk)
Pregnancy with anti-Ro → fetal heart-block surveillance

Differentials & how to tell them apart

Sicca from drugs/ageanticholinergics, ageing — no autoantibodies or glandular infiltration

Sarcoidosis / IgG4 diseaseother causes of glandular swelling — different histology/markers

Secondary Sjögren'soccurs with RA/SLE — look for the primary CTD

Investigations

Anti-Ro (SSA)/anti-La (SSB), ANA, RF; Schirmer test (reduced tear production); reduced salivary flow; labial gland biopsy (focal lymphocytic sialadenitis) if needed; assess for an associated CTD.

Management

Symptomatic sicca care (tears/saliva substitutes) ± pilocarpine; hydroxychloroquine for systemic features

1Confirm with anti-Ro/anti-La, Schirmer test and reduced salivary flow (± labial biopsy). Treat sicca symptoms with artificial tears/saliva substitutes and dental care; pilocarpine for significant dryness.Gate: Persistent unilateral parotid swelling or lymphadenopathy → investigate for B-cell (MALT) lymphoma — the risk is markedly increased in Sjögren's.
2Hydroxychloroquine for arthralgia/fatigue; immunosuppression for systemic involvement; in pregnancy with anti-Ro, arrange fetal heart-block surveillance.

Symptomatic: artificial tears and saliva substitutes, meticulous oral/dental care — mainstay for sicca symptoms

Pilocarpine (muscarinic agonist) — stimulates secretions in significant dryness

Hydroxychloroquine for arthralgia/fatigue — systemic features

Immunosuppression for systemic/organ involvement — and treat any associated CTD

Key points

Dry eyes + dry mouth + anti-Ro/anti-La = Sjögren's → symptomatic sicca care + pilocarpine. Two high-yield risks: B-cell (MALT) LYMPHOMA (persistent parotid swelling) and, with anti-Ro in pregnancy, congenital heart block.

Monitor & prognosis

Sicca symptoms, dental health, lymphadenopathy (lymphoma surveillance), associated CTD.

Generally good; lymphoma is the main serious complication.

Source: BSR Sjögren's; cross-ref ophthalmology (dry eye)