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Dense Deposit Disease — ESENeph MCQ

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HardGlomerulonephritisDense Deposit DiseaseESENeph

A 35-year-old man presents with haematuria and proteinuria. C3 is very low and C4 is normal. Kidney biopsy shows a membranoproliferative pattern with bright C3 staining on IF and absence of significant immunoglobulin. Electron microscopy shows intramembranous very electron-dense deposits. What is the diagnosis?

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Correct answer: BDense deposit disease (C3 glomerulopathy)

Very low C3 with normal C4 indicates alternative complement pathway activation. The biopsy showing membranoproliferative pattern with dominant C3 staining, absent immunoglobulins, and intramembranous electron-dense deposits on EM is diagnostic of dense deposit disease (DDD), a form of C3 glomerulopathy. DDD is caused by dysregulation of the alternative complement pathway, often associated with C3 nephritic factor. KDIGO 2021 recognises C3G as a distinct entity requiring complement investigation.

Reference: KDIGO 2021 – Glomerular Disease Guideline (Complement-Mediated Diseases)