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IgG4-related disease — SCE Rheumatology MCQ

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HardPregnancy in Rheumatic Disease, Rare ConditionsIgG4-related diseaseSCE Rheumatology

A patient has autoimmune pancreatitis, bilateral submandibular enlargement and retroperitoneal fibrosis. Biopsy shows storiform fibrosis and obliterative phlebitis with abundant IgG4-positive plasma cells. What is the diagnosis?

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Correct answer: A — IgG4-related disease

The combination of characteristic organ involvement and storiform fibrosis, obliterative phlebitis and IgG4-positive plasma cells is typical of IgG4-related disease. Serum IgG4 alone is neither necessary nor sufficient, and mimics including lymphoma and infection must be excluded.

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