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

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.