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.
§ figuresFigures (4)
Fig. 1Hyperbaric_Treatment_Sjogren's_Syndrome
“Hyperbaric Treatment Sjogren's Syndrome” · Intermedichbo · CC BY-SA 3.0 · Wikimedia Commons
Fig. 2Secondary Raynaud's in Sjögren's syndrome
“Secondary Raynaud's in Sjögren's syndrome 2” · Intermedichbo · CC BY-SA 3.0 · Wikimedia Commons
Verified open-access images only, reproduced for education under their stated licences · no AI-generated or illustrative artwork · Sjögren Syndrome figures pending faculty review.
§ 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:
§ evidenceReferences & Evidence
Current Classification
WHO Classification of Tumours Online — Head and Neck
IARC / WHO · 2022
Continuously updated online edition of the WHO head and neck tumour classification.
Evidence summary
Highest level of evidence linked to this page: WHO / Official Classification.
- L1 · Classification — 1
- L2 · Guideline — 1
- L6 · Textbook — 3
1 linked source was published from 2024 onwards. Where guidance is limited or contested, rely on the higher-level sources above rather than this summary. Educational use only — not a substitute for clinical judgement.
Content last reviewed
September 2026
Evidence last reviewed
September 2026
Next scheduled review
September 2027
Review status
EVIDENCE REVIEWED
Search latest PubMed evidence
Last literature search: 7 September 2026
Current Classification
IARC / WHO. IARC · 2022
Continuously updated online edition of the WHO head and neck tumour classification.
Clinical Guidelines
Shiboski CH, Shiboski SC, Seror R et al.. Annals of the rheumatic diseases · 2017 · 76(1):9-16
ACR/EULAR classification criteria for primary Sjogren syndrome.
Histopathology References
Barnes L. CRC Press · 2008
Detailed histopathological reference for head and neck diagnostic criteria.
Textbook References
Neville BW, Damm DD, Allen CM et al.. Elsevier · 2024
Standard reference textbook of oral and maxillofacial pathology used throughout this atlas.
Rajendran R, Sivapathasundharam B. Elsevier · 2020
Undergraduate and postgraduate oral pathology textbook widely used in BDS and FCPS curricula.
Additional sources cited on this page
- Shiboski CH. ACR/EULAR 2016 Classification Criteria
Draft — pending faculty review. Educational use only; verify against current guidelines and primary sources before clinical application.