# Atypical anti-glomerular basement membrane disease with membranous hyperplasia: diagnostic challenges and treatment variability

## TL;DR

A 31-year-old man with kidney symptoms was diagnosed with an atypical anti-GBM disease, highlighting diagnostic and treatment challenges in rare kidney disorders.

## Contribution

This case report adds to the understanding of atypical anti-GBM disease with membranous hyperplasia and its management complexities.

## Key findings

- The patient was diagnosed with atypical anti-GBM disease despite negative antibody tests.
- Standard treatment with corticosteroids and cyclophosphamide failed, leading to hemodialysis.
- The case emphasizes the need for tailored diagnostic and treatment approaches in rare kidney diseases.

## Abstract

This case report presents a detailed analysis of a 31-year-old male patient who presented with a complex array of clinical symptoms, including proteinuria, hematuria, edema, and kidney insufficiency. Despite undergoing multiple tests, the results for anti-glomerular basement membrane antibodies yielded negative findings. Subsequently, kidney biopsy pathology revealed a distinct diagnosis of atypical anti-glomerular basement membrane (anti-GBM) disease with membrane hyperplasia. Treatment was initiated with a comprehensive approach involving high doses of corticosteroids therapy and cyclophosphamide (CTX). However, contrary to expectations, the patient’s kidney function exhibited rapid deterioration following this therapeutic regimen. The culmination of these complications necessitated a pivotal transition to maintenance hemodialysis. This case underscores the intricate challenges associated with diagnosing and managing rare and atypical presentations of kidney disorders. The negative anti-GBM antibody results and subsequent identification of atypical anti-GBM nephropathy highlight the need for tailored diagnostic strategies to discern subtle nuances within complex clinical scenarios. Additionally, the unexpected response to the treatment regimen emphasizes the potential variability in individual patient responses, underlining the necessity for vigilant monitoring and adaptable treatment strategies. This case report contributes to the evolving understanding of atypical kidney pathologies and the complexities involved in their management.