Red Flags
- Persistent unilateral parotid swelling, palpable mass, or rapidly enlarging gland — exclude lymphoma (5-fold lifetime risk in primary Sjögren's); imaging and biopsy[1]
- Severe extraglandular disease (interstitial lung disease, vasculitis, peripheral neuropathy, glomerulonephritis, severe cytopenia) — admit and immunosuppression[1]
- Cryoglobulinaemic vasculitis — palpable purpura, neuropathy, glomerulonephritis; investigate hepatitis C; rituximab consideration[1]
- Pregnancy with anti-Ro/anti-La positive — fetal echocardiography 16–28 weeks; risk of congenital heart block; multidisciplinary care[1]
First-line treatment
Interventions
- Symptomatic management of sicca[1]Eyes: artificial tears, lubricating gels at night, ciclosporin 0.05% drops, punctal plugs, autologous serum drops in severe disease. Mouth: frequent sips of water, sugar-free gum/lozenges, saliva substitutes, fluoride toothpaste, regular dental review
- Patient education and lifestyle[1]Avoid smoking, dry environments; hydration; dental hygiene; fatigue management; manage comorbidities; physiotherapy for fatigue and musculoskeletal symptoms
- Stepped pharmacotherapy for sicca[1]Step 1: lubricants. Step 2: pilocarpine or cevimeline if residual function. Step 3: corticosteroid topical for severe inflammation. Step 4: systemic immunosuppression for severe refractory glandular disease
- Vaccination and infection prevention[1]Pneumococcal, influenza annual, recombinant zoster, HPV, hepatitis B; defer live vaccines once on biologic; counsel about increased infection susceptibility
First-line drug therapy
| Drug | Class | Adult | Paediatric | Notes |
|---|---|---|---|---|
| Pilocarpine[1] | Muscarinic agonist (cholinergic) | 5 mg PO QDS | — | Stimulates residual salivary and lacrimal function; sweating, GI upset; contraindicated in narrow-angle glaucoma, asthma, severe COPD |
| Cevimeline (where available)[1] | Muscarinic M3-selective agonist | 30 mg PO TDS | — | Alternative cholinergic agonist; more M3-selective than pilocarpine; better cardiac tolerability |
| Hydroxychloroquine[1] | Antimalarial / immunomodulator | 200–400 mg PO daily (max 5 mg/kg actual body weight) | — | Useful for arthralgia, fatigue, cutaneous lupus features; safety established; baseline retinal exam and annual screening from year 5 |
| Prednisolone (severe extraglandular)[1] | Systemic corticosteroid | 0.5–1 mg/kg/day with rapid taper to ≤7.5 mg/day; preceded by IV pulse 250–500 mg × 3 days for severe organ disease | — | Bridge to immunosuppressant; minimise duration; bone protection (calcium, vitamin D, bisphosphonate per fracture risk) |
| Rituximab[1] | Anti-CD20 monoclonal antibody | 1 g IV × 2 doses 2 weeks apart, repeat at 6 months by clinical response | — | First-line biologic for severe refractory extraglandular disease (cryoglobulinaemic vasculitis, peripheral neuropathy, ILD); HBV screen and prophylaxis; vaccinate before |
| Mycophenolate mofetil[1] | Inosine monophosphate dehydrogenase inhibitor | 1–3 g/day PO in divided doses | — | Useful for ILD, neurological, glomerulonephritis; teratogenic — strict contraception |
Pilocarpine[1]
Muscarinic agonist (cholinergic)
- Adult
- 5 mg PO QDS
- Paediatric
- —
Stimulates residual salivary and lacrimal function; sweating, GI upset; contraindicated in narrow-angle glaucoma, asthma, severe COPD
Cevimeline (where available)[1]
Muscarinic M3-selective agonist
- Adult
- 30 mg PO TDS
- Paediatric
- —
Alternative cholinergic agonist; more M3-selective than pilocarpine; better cardiac tolerability
Hydroxychloroquine[1]
Antimalarial / immunomodulator
- Adult
- 200–400 mg PO daily (max 5 mg/kg actual body weight)
- Paediatric
- —
Useful for arthralgia, fatigue, cutaneous lupus features; safety established; baseline retinal exam and annual screening from year 5
Prednisolone (severe extraglandular)[1]
Systemic corticosteroid
- Adult
- 0.5–1 mg/kg/day with rapid taper to ≤7.5 mg/day; preceded by IV pulse 250–500 mg × 3 days for severe organ disease
- Paediatric
- —
Bridge to immunosuppressant; minimise duration; bone protection (calcium, vitamin D, bisphosphonate per fracture risk)
Rituximab[1]
Anti-CD20 monoclonal antibody
- Adult
- 1 g IV × 2 doses 2 weeks apart, repeat at 6 months by clinical response
- Paediatric
- —
First-line biologic for severe refractory extraglandular disease (cryoglobulinaemic vasculitis, peripheral neuropathy, ILD); HBV screen and prophylaxis; vaccinate before
Mycophenolate mofetil[1]
Inosine monophosphate dehydrogenase inhibitor
- Adult
- 1–3 g/day PO in divided doses
- Paediatric
- —
Useful for ILD, neurological, glomerulonephritis; teratogenic — strict contraception
Safety-net
- Dental review every 6 months — Sjögren's accelerates dental caries; fluoride toothpaste, mouthwash, frequent water sips and sugar-free gum reduce damage[1]
- Persistent gland enlargement, especially unilateral, deserves evaluation — small lymphoma risk warrants vigilance[1]
- Plan pregnancy with rheumatologist — anti-Ro positivity needs fetal cardiac surveillance to detect heart block early[1]
Referral criteria
- All suspected Sjögren's syndromeRheumatology; combined with ophthalmology and dental input[1]
- Severe extraglandular disease (ILD, vasculitis, neuropathy, glomerulonephritis, severe cytopenia)Tertiary rheumatology and relevant subspecialty[1]
- Suspected lymphoma (persistent unilateral parotid mass, B symptoms, monoclonal protein)Haematology / oncology with imaging and biopsy[1]
- Pregnancy with anti-Ro/anti-La positivityJoint rheumatology and fetal medicine clinic[1]
Clinical summary
Diagnosis and stepwise management of sicca symptoms and systemic manifestations of primary Sjögren's syndrome in adults.