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Sjögren

Rheumatology/ImmunologyExocrine glands (lacrimal, salivary)Immune systemMusculoskeletal systemRenal systemPulmonary systemPeripheral nervous system

Summary

Sjögren syndrome is a chronic autoimmune disorder characterized by lymphocytic infiltration of exocrine glands, classically causing dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia). It predominantly affects middle-aged women and can occur alone (primary) or with other autoimmune diseases like RA or SLE (secondary).

Detail

Pathophysiology: Autoimmune destruction of lacrimal and salivary glands due to lymphocytic (CD4+ T-cell predominant) infiltration leads to decreased tear and saliva production. Chronic inflammation can cause glandular fibrosis. B-cell hyperactivity is prominent, increasing risk of lymphoma (particularly MALT lymphoma of the parotid gland - the classic complication to know for boards).

Clinical features: Classic triad includes xerophthalmia (dry eyes, foreign body sensation), xerostomia (dry mouth, dysphagia, dental caries), and often bilateral parotid gland enlargement. Extraglandular manifestations include arthralgia, Raynaud phenomenon, interstitial lung disease, renal tubular acidosis (distal RTA type I), peripheral neuropathy, and vasculitis (palpable purpura).

Diagnosis: - Anti-SSA (Ro) and Anti-SSB (La) antibodies (SSA is also important in neonatal lupus - can cause congenital heart block if mother is positive) - Positive Schirmer test (decreased tear production) - Labial salivary gland biopsy showing focal lymphocytic sialadenitis (gold standard) - Elevated rheumatoid factor and ANA are common but nonspecific

Associations: Increased risk of non-Hodgkin lymphoma (MALT type) - suspect with new unilateral parotid swelling or persistent lymphadenopathy. Secondary Sjögren occurs with RA, SLE, systemic sclerosis, and other autoimmune conditions.

Treatment: Symptomatic (artificial tears, saliva substitutes, pilocarpine/cevimeline for gland stimulation), immunosuppressants for systemic disease, and monitoring for lymphoma development.

High-yield boards pearls: Anti-Ro/SSA antibody association with neonatal lupus and congenital heart block; MALT lymphoma risk; distinguishing primary vs. secondary; parotid gland enlargement differential.

Sources

  • First Aid for the USMLE Step 1
  • Robbins Basic Pathology
  • UpToDate: Sjögren syndrome
  • Kaplan USMLE Step 1 Immunology

Reviewed by Medusa editorial — medical student review. Information here is for study reference only and is not medical advice. Spotted an error? Let us know.