On this page
Study tools
Write your answer
Saved on this device. No marking — you are the marker.
Stem
A 52-year-old woman presents with a 2-year history of progressively gritty, dry eyes and a dry mouth, requiring her to sip water to swallow dry food. She has had rapid dental caries (several extractions in the last year), recurrent oral candidiasis, fatigue, and intermittent bilateral parotid swelling. She also reports intermittent palpable purpura over the shins. There is no history of head/neck radiotherapy. Examination reveals a furrowed, dry tongue, bilateral non-tender parotid enlargement, and palpable purpura on the lower legs.
Questions
a) What is the most likely diagnosis, and is this primary or secondary? Give two reasons. (2 marks)
Most likely diagnosis: primary Sjogren's syndrome. It is primary because there is no clinical or serological evidence of another defined connective-tissue disease (no features of RA, SLE or systemic sclerosis), and the patient has the classic sicca syndrome (xerophthalmia + xerostomia) with extraglandular manifestations (parotid enlargement, palpable purpura, fatigue).
b) List four objective investigations you would use to confirm the diagnosis and state the ACR/EULAR 2016 threshold. (4 marks)
- Anti-Ro/SSA antibodies (key serology, 3 weighted points).
- Labial (minor) salivary gland biopsy — focal lymphocytic sialadenitis, focus score at least 1 (3 points).
- Schirmer's test — at most 5 mm wetting in 5 minutes (1 point).
- Unstimulated whole saliva flow — at most 0.1 mL per minute (1 point); plus ocular staining score at least 5 (1 point).
- Screen and exclude HCV and HIV. A weighted score of at least 4 (with no exclusions) classifies primary Sjogren's syndrome.
c) Outline the stepwise management of her sicca symptoms, naming a key drug and a contraindication. (3 marks)
- Ocular: preservative-free artificial tears/lubricants first line; punctal plugs for moderate disease.
- Oral: saliva substitutes, frequent water sips, sugar-free sialogogues, rigorous oral hygiene, topical fluoride; avoid anticholinergic drugs.
- Secretagogues if topical measures fail: pilocarpine 5 mg four times daily (or cevimeline 30 mg three times daily), a muscarinic M3 agonist. Contraindicated in asthma and narrow-angle glaucoma (causes bronchospasm and raises intraocular pressure); side-effects include sweating, flushing and gastrointestinal upset.
d) Name two complications of this disease and the single most important one requiring long-term surveillance, with two predictors. (1 mark)
Complications include rapid dental caries with tooth loss, corneal ulceration/keratitis, interstitial lung disease, and distal (type 1) renal tubular acidosis. The most important to surveil for is B-cell (MALT) lymphoma of the salivary glands; predictors include persistent parotid enlargement/lymphadenopathy, low complement C4, cryoglobulinaemia, palpable purpura, and a falling IgM or rheumatoid factor.