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Thin Basement Membrane Disease — ESENeph MCQ

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EasyGlomerulonephritisThin Basement Membrane DiseaseESENeph

A 40-year-old man presents with recurrent episodes of macroscopic haematuria with mild proteinuria (0.3 g/day). Biopsy shows thin glomerular basement membranes uniformly across all glomeruli on EM. Immunofluorescence is negative. There is no family history of renal failure or deafness. What is the most likely diagnosis?

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Correct answer: A — Thin basement membrane disease

Uniform GBM thinning without splitting or lamellation on EM, negative IF, no family history of progressive kidney disease or hearing loss, and mild presentation is consistent with thin basement membrane disease (TBMD). This is a benign condition in most cases (heterozygous COL4A3/A4 carrier state). Unlike Alport syndrome, there is no progressive splitting/lamellation. Unlike IgA nephropathy, IF shows no mesangial IgA deposits. Long-term follow-up is recommended as a minority may develop proteinuria.

Reference: KDIGO 2021 – Glomerular Disease Guideline