AtlasSalivarySjögren Syndrome

Salivary Gland Disorders

Sjögren Syndrome

aka SS

Chronic autoimmune exocrinopathy targeting lacrimal and salivary glands, producing xerostomia and xerophthalmia; carries a 15–20× increased risk of MALT lymphoma.

Antibody
Anti-Ro/SSA
Biopsy
Focus score ≥1
Malignancy
MALT lymphoma ↑15–20×

§ overviewOverview

Systemic autoimmune disease characterised by lymphocytic infiltration and progressive destruction of exocrine glands, principally salivary and lacrimal.

§ icdICD Classification

M35.0

§ epidemiologyEpidemiology

F:M 9:1; peak 40–60 y; second most common autoimmune rheumatic disease.

§ clinicalClinical Features

  • 01Xerostomia: burning mouth, difficulty swallowing dry food, rampant caries
  • 02Xerophthalmia: gritty eyes, keratoconjunctivitis sicca
  • 03Bilateral parotid enlargement
  • 04Fissured/depapillated tongue
  • 05Extra-glandular: arthralgia, Raynaud, interstitial lung disease, neuropathy

§ histopathHistopathology

  • 01Labial minor salivary gland biopsy: focal lymphocytic sialadenitis with ≥1 focus (50 lymphocytes) per 4 mm² of glandular tissue

§ investigationsInvestigations

  • 01ACR/EULAR 2016 criteria (score ≥ 4): Focus score ≥ 1 on labial gland biopsy (3), anti-SSA/Ro (3), ocular staining score ≥ 5 (1), Schirmer ≤ 5 mm/5 min (1), unstimulated whole salivary flow ≤ 0.1 mL/min (1)
  • 02Anti-Ro/SSA (60–70%), anti-La/SSB (40%)
  • 03Elevated ESR, hypergammaglobulinaemia
  • 04Sialography (fruit-laden branchless tree), sialoscintigraphy, US of parotid

§ classificationClassification

  • 01Primary SS (isolated)
  • 02Secondary SS (associated with RA, SLE, systemic sclerosis)

§ treatmentTreatment

  • 01Symptomatic sicca: artificial tears, saliva substitutes, sugar-free gum
  • 02Muscarinic agonists (pilocarpine 5 mg qid, cevimeline)
  • 03Systemic: hydroxychloroquine for arthralgia/fatigue; immunosuppression (methotrexate, rituximab) for severe extra-glandular disease
  • 04Aggressive caries prevention: fluoride, chlorhexidine, sialogogues

§ complicationsComplications

  • 01Rampant cervical caries
  • 02Candidiasis
  • 03Non-Hodgkin MALT lymphoma of parotid (15–20× risk) — persistent unilateral swelling warrants biopsy
  • 04Corneal ulceration

§ prognosisPrognosis

Chronic; excellent with symptom control. Life expectancy near normal unless lymphoma develops.

§ examKey Examination Points

  • 01Salivary flow test, Schirmer test, focus score biopsy
  • 02Persistent unilateral parotid mass → exclude lymphoma

§ revisionQuick Revision Summary

  • 01Focus score ≥1 · anti-Ro/SSA · pilocarpine · watch for MALT lymphoma

§ vivaBDS Viva Questions

  • 01ACR/EULAR criteria?
  • 02Focus score?
  • 03Lymphoma risk?

§ mcqsMCQs — Assessment (3)

Question 1

Autoantibodies most specific for SS:

Question 2

A minor salivary gland focus consists of ≥:

Question 3

Increased risk of which malignancy:

References

  1. Shiboski CH. ACR/EULAR 2016 Classification Criteria

Draft — pending faculty review. Educational use only; verify against current guidelines and primary sources before clinical application.