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Immunotactoid Glomerulopathy — ESENeph MCQ

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HardGlomerulonephritisImmunotactoid GlomerulopathyESENeph

A 50-year-old man presents with nephrotic syndrome. Kidney biopsy shows organised microtubular deposits (>30 nm diameter) on EM arranged in parallel arrays. IF shows monotypic IgG1-kappa. Congo red is negative. What is the diagnosis?

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Correct answer: D — Immunotactoid glomerulopathy

Immunotactoid glomerulopathy (ITG) is characterised by organised microtubular deposits >30 nm in diameter (larger than fibrillary GN fibrils which are 12-24 nm, and larger than amyloid fibrils which are 8-12 nm) arranged in parallel arrays. Congo red is negative. ITG is almost always associated with an underlying lymphoproliferative disorder (CLL, lymphoma, monoclonal gammopathy). Monotypic immunoglobulin staining supports the clonal association. Treatment targets the underlying haematological disease.

Reference: Nasr et al 2015 – Immunotactoid Glomerulopathy; KDIGO 2021 – GN Guideline