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IgG4-related disease — ABIM Board MCQ

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HardRheumatology/Allergy/ImmunologyIgG4-related diseaseABIM Board

A 63-year-old man has biopsy-confirmed IgG4-related autoimmune pancreatitis with obstructive jaundice and salivary-gland involvement. Cultures are negative, and careful evaluation finds no pancreatic malignancy. Which induction therapy is most appropriate?

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Correct answer: D — Begin systemic glucocorticoid therapy with planned response assessment

The best answer is “Begin systemic glucocorticoid therapy with planned response assessment”. Active symptomatic IgG4-related disease, particularly autoimmune pancreatitis causing biliary obstruction, generally responds rapidly to systemic glucocorticoids. Response should be assessed clinically, biochemically and radiographically. Rituximab is useful for selected relapsing, refractory or steroid-intolerant disease, but surgery is inappropriate once malignancy has been adequately excluded.

Reference: IgG4-Related Disease: Current Challenges and Future Prospects: https://pmc.ncbi.nlm.nih.gov/articles/PMC6583577/