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IgG4 Retroperitoneal Fibrosis — ESENeph MCQ

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ModerateAcute Kidney InjuryIgG4 Retroperitoneal FibrosisESENeph

A 50-year-old man presents with AKI and is found to have bilateral ureteric obstruction from retroperitoneal fibrosis. His creatinine is 550 umol/L. After bilateral nephrostomy insertion, his creatinine improves to 150 umol/L. Biopsy of the retroperitoneal mass shows dense fibrosis with IgG4-positive plasma cells. What is the diagnosis and treatment?

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Correct answer: D — IgG4-related retroperitoneal fibrosis – Glucocorticoids are first-line treatment

IgG4-related disease is an important cause of retroperitoneal fibrosis. The biopsy showing dense fibrosis with abundant IgG4-positive plasma cells, along with elevated serum IgG4 (typically >1.35 g/L), is diagnostic. First-line treatment is Glucocorticoids (typically Prednisolone 0.6-1 mg/kg tapered over months), which often produces dramatic improvement. Rituximab is used for relapsing or steroid-refractory disease. Ureteric stenting or nephrostomy may be needed for acute obstruction relief.

Reference: Stone et al 2012 – IgG4-Related Disease; ACR/EULAR 2019 Classification Criteria