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. 2023 Jul 15;44(2):99-106.
doi: 10.2478/prilozi-2023-0030. Print 2023 Jul 1.

Glomerulopathies with Fibrillary Deposits

Pavlina Dzekova-Vidimliski  1   2 , Vlatko Karanfilovski  1   2 , Igor G Nikolov  1   2 , Irena Rambabova-Bushljetik  1   2 , Vesna Ristovska  1   2 , Gordana Petrushevska  3 , Gjulsen Selim  1   2
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Free article

Glomerulopathies with Fibrillary Deposits

Pavlina Dzekova-Vidimliski et al. Pril (Makedon Akad Nauk Umet Odd Med Nauki). .
Free article

Abstract

The glomerulopathies associated with the deposition of extracellular fibrils in the glomeruli are subdivided into Congo red positive (amyloidosis) and Congo red negative (non-amyloidotic glomerulopathies) based on Congo red staining. The non-amyloidotic glomerulopathies are divided into immunoglobulin-derived and non-immunoglobulin-derived glomerulopathies. The immunoglobulin-derived glomerulopathies: fibrillary glomerulopathy (FGn) and immunotactoid glomerulopathy (ITG) are rare glomerulopathies. The diagnosis of fibrillary-immunotactoid glomerulopathy depends on electron microscopy, which shows the presence of microfibrils in the glomeruli. The microfibrils in FGn are randomly arranged with diameters less than 30 nm. The microfibrils in ITG are larger than 30 nm with a visible lumen (microtubules), focally arranged in parallel bundles. Patients with fibrillary-immunotactoid glomerulopathy present with proteinuria (usually in the nephrotic range), microscopic hematuria, arterial hypertension, and chronic kidney disease that progresses to kidney failure over months to years. Currently, there are no guidelines for the treatment of fibrillary-immunotactoid glomerulopathy, although immunotactoid glomerulopathy could be associated with underlying hematologic disorders with the need for clone-directed therapy.

Keywords: amyloidosis; chronic kidney disease; glomerulopathy; proteinuria; treatment.

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