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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 a mass-like retroperitoneal lesion, raised serum IgG4 and obstructed ureters. Lymphoma and other malignancy remain plausible. What should establish the diagnosis before immunosuppression?

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Correct answer: D — Image-guided core biopsy with histology and clonality assessment

Serum IgG4 elevation and imaging are not specific. A mass-like lesion with a malignant differential requires adequate tissue architecture, immunostaining and assessment for clonality before glucocorticoids obscure the diagnosis; urgent decompression of obstruction proceeds separately when needed.

Reference: 2019 ACR/EULAR classification criteria for IgG4-related disease (Published 2020): https://ard.bmj.com/content/79/1/77; International consensus guidance for management of IgG4-related disease (Published 2015): https://ard.bmj.com/content/74/9/1688