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Sjögren syndrome
Sjögren syndrome is an autoimmune disease of the tear and salivary glands — dry eyes and mouth with anti-Ro (SSA) or lymphocytic infiltration on a salivary gland biopsy — that greatly raises the risk of lymphoma; persistent parotid enlargement is a warning sign.
Why it matters in the exam
The question presents a middle-aged woman with a gritty feeling in the eyes, a dry mouth, difficulty swallowing dry food and dental caries, sometimes with parotid enlargement. The classification criteria are weighted: positive anti-Ro (3 points), a minor salivary gland biopsy with a focus score of 1 or more (3 points), and simple tests — Schirmer of 5 mm or less in 5 minutes, ocular staining and low saliva flow (one point each); 4 points or more classify Sjögren syndrome. It can be primary or accompany RA, lupus or scleroderma.
Manifestations outside the glands are tested quite often: joint pain, type 1 RTA with hypokalaemia, interstitial nephritis, lung disease, neuropathy, vasculitis and cryoglobulinaemia. The risk of lymphoma (mainly MALT) is several times higher, and the warning signs are persistent parotid enlargement, purpura, low C4 and cryoglobulins. Anti-Ro in a pregnant woman puts the fetus at risk of congenital heart block. Dryness is treated locally: artificial tears and saliva, with pilocarpine or cevimeline to increase secretion; hydroxychloroquine for joint pain; and for systemic disease — steroids or rituximab.
Key points
- Dry eyes and mouth + anti-Ro or a biopsy with focus score 1 or more.
- 4 points or more classify Sjögren syndrome.
- Outside the glands: joints, type 1 RTA, lung, neuropathy, vasculitis.
- Lymphoma risk: persistent parotid swelling, purpura, low C4.
- Treatment: artificial tears and saliva, pilocarpine; anti-Ro in pregnancy — heart block.
Summary table
| Topic | Finding | Meaning / action | Exam note |
|---|---|---|---|
| Classification | Anti-Ro (3), biopsy focus score 1 or more (3), Schirmer 5 mm or less, staining, low saliva (1 each) | 4 points or more = Sjögren | Primary or secondary |
| Kidney | Type 1 RTA, hypokalaemia, interstitial nephritis | Electrolytes and urinalysis | Weakness from hypokalaemia |
| Lymphoma | Persistent parotid enlargement, purpura, low C4, cryoglobulins | Biopsy and work-up | Mainly MALT |
| Pregnancy | Positive anti-Ro | Fetal heart rhythm monitoring | Congenital heart block |
| Treating dryness | Eyes and mouth | Artificial tears and saliva; pilocarpine or cevimeline | Oral hygiene and caries prevention |
| Systemic disease | Joints, lung, vasculitis | Hydroxychloroquine; steroids or rituximab | By organ |
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Sources
- ACR/EULAR Classification Criteria for Primary Sjögren's Syndrome, 2016 — Weighted criteria: anti-SSA/Ro, salivary gland histology, ocular and salivary tests
- EULAR recommendations for the management of Sjögren's syndrome, 2019 — Topical and systemic therapy
- Harrison's Principles of Internal Medicine, 21st edition (2022) — Sjögren's Syndrome
Sources are given for further reading; the text is written afresh and not copied from them.
Reviewed by the Examqo AI Professor · 2026-11-02. The text may contain errors; when in doubt, check the source.