IgG4 Renal Disease — SCE Rheumatology MCQ
Instant feedback + full explanation. One question, done properly.
Educational content. Not a substitute for clinical judgement or local policy.
Reveal the answer and explanation
Correct answer: A — Retroperitoneal fibrosis causing bilateral ureteric obstruction and hydronephrosis
The best answer is “Retroperitoneal fibrosis causing bilateral ureteric obstruction and hydronephrosis”. IgG4-related tubulointerstitial nephritis is the most common renal manifestation of IgG4-RD. It presents with renal impairment bilateral renal enlargement on imaging and may mimic lymphoma or renal infiltrative disease. Histology shows the typical IgG4-RD pattern: dense lymphoplasmacytic infiltrate storiform fibrosis and elevated IgG4/IgG ratio. Other renal manifestations include membranous nephropathy and retroperitoneal fibrosis causing ureteric obstruction. Treatment involves glucocorticoids (high response rate) with Rituximab for relapsing disease. Serum IgG4 levels correlate with disease activity and guide treatment monitoring. The competing options represent related diagnoses, tests or treatments, but they do not match the decisive phenotype, safety constraint or management point in this stem.
Reference: International IgG4-related disease pathology consensus: https://pmc.ncbi.nlm.nih.gov/articles/PMC3434018/