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IgG4-related disease — SCE Rheumatology MCQ

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HardPregnancy and Rare Rheumatic DiseaseIgG4-related diseaseSCE Rheumatology

A patient has autoimmune pancreatitis, salivary enlargement and a retroperitoneal mass, with serum IgG4 four times the upper limit. Lymph-node core biopsy shows many IgG4-positive plasma cells but no storiform fibrosis. What is the most informative next diagnostic step?

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Reveal the answer and explanation

Correct answer: E — Biopsy an involved organ or mass to assess architecture and exclude mimics

Explanation lettering: C = shown as A · D = shown as B · A = shown as C · E = shown as D · B = shown as E

B is correct. Serum IgG4 and IgG4-positive cells are supportive but not specific. A representative involved organ or mass provides the architecture needed to seek storiform fibrosis and obliterative phlebitis while excluding lymphoma, carcinoma and infection. A over-relies on serum. C overstates nodal specificity. D makes treatment response a diagnostic test. E ignores that lymph nodes often lack classic storiform fibrosis.

Reference: IgG4-related disease: lessons from the first 20 years: https://academic.oup.com/rheumatology/article/64/Supplement_1/i24/8069375