# A Unique Case of Rapidly Progressive Glomerulonephritis in a Patient With Anti-neutrophil Cytoplasmic Antibody (ANCA)-Positive Vasculitis Presenting With Ocular and Cardiac Manifestations

## TL;DR

A 33-year-old man with no prior health issues developed a rare kidney disease linked to a type of vasculitis, showing eye and heart symptoms, and was treated with immunosuppressive drugs.

## Contribution

This case highlights a rare and rapidly progressing form of GPA with ocular and cardiac features in a young patient.

## Key findings

- The patient presented with RPGN and PR3-ANCA positivity, consistent with GPA.
- Treatment with cyclophosphamide and glucocorticoids was initiated to prevent irreversible kidney damage.
- Upper airway symptoms, pulmonary lesions, and renal failure were key diagnostic features.

## Abstract

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAVs) can be divided into three distinct clinical entities. Of the three subgroups, granulomatosis with polyangiitis (GPA) is the most common AAV. We present a 33-year-old Hispanic male with no past medical history who presented to the ER with acute-onset pleuritic chest pain and dyspnea. The patient had chronic sinusitis with occasional epistaxis. Initial lab work was significant for hematuria and proteinuria, as well as worsening acute kidney injury (AKI). A renal biopsy confirmed the presence of rapidly progressive glomerulonephritis (RPGN). Autoimmune panels were significant for PR3-ANCA positivity. The patient’s clinical picture was most compatible with that of GPA, given the prominence of upper airway symptoms in the setting of new-onset renal failure with erythrocyturia and proteinuria, as well as pulmonary lesions. Cyclophosphamide-based treatment was initiated, in conjunction with glucocorticoids, mesna, and atovaquone. High suspicion for GPA with RPGN should be considered in the younger population who present with new-onset renal failure and pulmonary lesions to present irreversible kidney injury.