IgG4-related disease — SCE Rheumatology MCQ

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ModerateConnective tissue diseasesIgG4-related diseaseSCE Rheumatology

A 42-year-old woman has painless submandibular swelling and obstructive jaundice. Examination shows there is lacrimal gland enlargement. Investigations show serum IgG4 is raised and biopsy shows storiform fibrosis. The admitting team asks which diagnosis best explains the whole presentation. What is the most likely diagnosis?

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Correct answer: A — IgG4-related disease

IgG4-related disease is the best answer because the pattern described is most consistent with IgG4-related disease, including the distribution of symptoms and the targeted investigation results. Antiphospholipid syndrome is a plausible distractor, but it does not account for the key discriminator in the stem. Leflunomide hepatotoxicity and Osteoporosis would be more appropriate in a different clinical pattern or at another point in the pathway, while Paget disease of bone is suboptimal for this presentation. Clinical pearl: in SCE-style questions, the decisive clue is usually the pattern across joints, extra-articular features and targeted immunology rather than a single antibody result.

Reference: BSR SLE guideline; EULAR SLE and systemic sclerosis recommendations; BNF