DOI: https://doi.org/10.36347/sjmcr.2024.v12i11.036

Pages: 1994-1998

Fibrillary glomerulonephritis (FGN) is a rare pseudoamyloid glomerulopathy characterized by an extracellular deposit of DNAJB9 positive fibrils. Diagnosis of FGN is challenging, since there is no clinical specificity, and pathologically there are others glomerulopathies with organized deposits. Diagnosis of FGN is even more problematic in developing countries, where the necessary diagnostic tools aren’t available. Diagnosis of FGN requires DNAJB9 immunostaining or electron microscopy examination. Until today, there is no standard therapeutic regimen for FGN. The prognosis is still unclear and it is unpredictable. More than half of cases progress to the end-stage kidney disease. We report, in this paper, a case of FGN diagnosed in Moroccan woman. She was treated by steroids and rituximab induction therapy. She didn’t receive rituximab maintenance therapy due to a sustained CD19 depletion. We suspect here an association with an immunodeficiency condition. The clinical course was favourable. Nephrologists and pathologists, especially in developing countries, should be aware about this entity.