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Glomerulopathies with Fibrillary Deposits.

Pavlina Dzekova-Vidimliski^1,2, Vlatko Karanfilovski^1,2, Igor G Nikolov^1,2

¹1University Hospital of Nephrology, Skopje, RN Macedonia.

Prilozi (Makedonska Akademija Na Naukite I Umetnostite. Oddelenie Za Medicinski Nauki)

|July 15, 2023

Summary

Fibrillary and immunotactoid glomerulopathies are rare kidney diseases characterized by microfibril deposition. Diagnosis relies on electron microscopy, differentiating them by microfibril size and arrangement, with no current treatment guidelines.

Keywords:

amyloidosis chronic kidney disease glomerulopathy proteinuria treatment

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Analysis of β-Amyloid-induced Abnormalities on Fibrin Clot Structure by Spectroscopy and Scanning Electron Microscopy

Analysis of β-Amyloid-induced Abnormalities on Fibrin Clot Structure by Spectroscopy and Scanning Electron Microscopy

Published on: November 30, 2018

Area of Science:

Nephrology
Pathology
Electron Microscopy

Background:

Glomerulopathies involve extracellular fibril deposition, classified by Congo red staining as amyloidosis or non-amyloidotic types.
Non-amyloidotic glomerulopathies are further divided into immunoglobulin-derived and non-immunoglobulin-derived categories.
Fibrillary glomerulopathy (FGn) and immunotactoid glomerulopathy (ITG) are rare immunoglobulin-derived conditions.

Purpose of the Study:

To differentiate fibrillary glomerulopathy (FGn) and immunotactoid glomerulopathy (ITG) based on ultrastructural morphology.
To highlight the clinical presentation and diagnostic challenges of these rare glomerulopathies.

Main Methods:

Congo red staining to differentiate amyloidosis from non-amyloidotic glomerulopathies.
Electron microscopy to analyze microfibril characteristics (size, arrangement, lumen presence) in FGn and ITG.
Clinical data review for patient presentation and disease progression.

Main Results:

FGn microfibrils are randomly arranged, <30 nm in diameter.
ITG microfibrils are >30 nm, possess a visible lumen (microtubules), and are arranged in parallel bundles.
Patients present with nephrotic-range proteinuria, hematuria, hypertension, and progressive chronic kidney disease.

Conclusions:

Electron microscopy is crucial for distinguishing FGn from ITG based on distinct microfibril ultrastructure.
Fibrillary-immunotactoid glomerulopathy presents with significant renal dysfunction and lacks established treatment guidelines.
Immunotactoid glomerulopathy may require clone-directed therapy if associated with hematologic disorders.