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Q1: Definition, mechanism and classification (2 min)
- Define Sjogren's syndrome as an autoimmune epithelitis causing sicca syndrome; distinguish primary from secondary.
- Describe the pathophysiology: focal lymphocytic infiltration of exocrine glands, B-cell hyperactivity (BAFF), type I interferon signature; define the focus score on labial biopsy.
- State the key autoantibodies (anti-Ro/SSA, anti-La/SSB) and reproduce the ACR/EULAR 2016 weighted score (at least 4 points); list the exclusion criteria (HCV, HIV, sarcoidosis, IgG4-related disease, prior radiotherapy).
Q2: Clinical features and bedside assessment (2 min)
- List glandular (xerostomia, xerophthalmia, parotid swelling) and extraglandular features (fatigue, arthritis, Raynaud, vasculitic purpura, ILD, distal RTA, neuropathy).
- Demonstrate Schirmer's test (at most 5 mm in 5 minutes) and outline the ocular staining score, saliva flow and labial biopsy.
- Interpret the discordance of a high ESR with normal CRP (hypergammaglobulinaemia).
Q3: Management (3 min)
- Outline staged therapy: topical tears and saliva substitutes; secretagogues (pilocarpine, cevimeline) with contraindications (asthma, narrow-angle glaucoma).
- State the place of hydroxychloroquine (fatigue, arthralgia, skin) and that no drug convincingly improves sicca.
- Describe systemic therapy for organ-threatening disease: cyclophosphamide for severe vasculitis/CNS; mycophenolate or azathioprine for ILD/renal disease; rituximab for refractory systemic disease; bicarbonate and potassium for distal RTA.
Q4: Complications, prognosis and pregnancy (3 min)
- State the lymphoma risk (MALT, ~5 to 10 percent) and its predictors (persistent parotid enlargement, low C4, cryoglobulins, palpable purpura, falling IgM).
- Explain anti-Ro/SSA in pregnancy: congenital heart block (~2 percent, higher recurrence), fetal echocardiography 16 to 26 weeks, hydroxychloroquine reduces recurrence.
- Summarise prognosis: often stable for decades, but excess mortality from lymphoma, vasculitis and ILD; fatigue is frequently the most disabling symptom for quality of life.