Case Presentation: A 71-year-old Caucasian female with history of atrial fibrillation, gastroesophageal reflux disease, chronic kidney disease stage 3a was admitted for 2-week history of nausea, vomiting, and decreased urinary output. The patient had a recent admission for atrial fibrillation with rapid ventricular rate without causative etiology identified and was discharged on amiodarone and apixaban. She denied NSAID use and review of systems was otherwise negative. Her workup was significant for creatinine elevated to 8.0 (baseline 1.0), hematuria, and bacteriuria. Of note, she had new hematuria compared to urinalysis 3 months prior. Computed tomography of the chest and abdomen revealed new pericardial effusion, pleural effusions and a 4mm stone in the lower pole of the left kidney. Workup for glomerulonephritis was performed with labs significant for C-ANCA 1:2560 and PR3 antibodies >3285 with normal complement levels. During hospitalization she required hemodialysis and her respiratory status declined. She developed hemoptysis requiring admission to the ICU for intubation and bronchoscopy which revealed diffuse alveolar hemorrhage. Renal biopsy was performed and resulted with florid subacute pauci-immune crescentic glomerulonephritis with 48/76 glomeruli with crescent formation. Findings from biopsy and clinical picture were consistent with granulomatosis with polyangiitis with severe disease. High dose steroids, cyclophosphamide, trimethoprim-sulfamethoxazole and PLEX were initiated. She was extubated and discharged from the hospital on rituximab and she remained dialysis dependent.
Discussion: Granulomatosis with polyangiitis (GPA) is an ANCA-associated vasculitis which affects medium and small sized vessels. Although rare, GPA should be considered in patients with signs of glomerulonephritis and severe AKI. Classically patients present with sinopulmonary involvement including hemoptysis, nosebleeds, and cough however many patients present with nonspecific symptoms including fever, fatigue, weight loss, arthralgias without evidence of specific organ involvement. Only 10-15% of patients with GPA will present with renal insufficiency, however renal impairment both portends rapid disease progression and is also associated with worsened mortality and worse prognosis than either pulmonary or sinus involvement with dialysis dependence associated with the worst mortality. Identifying rapidly progressive renal involvement is imperative given the aggressive nature and involvement with other organ systems. This patient did not present with the classically associated symptoms of GPA and had other compelling explanations for her renal dysfunction and hematuria. Amongst elderly patients, rituximab and cyclophosphamide have been shown to limit permanent organ damage. This case demonstrates both the rapid nature of disease progression and the necessity of timely diagnosis to prevent severe complications such as dialysis.
Conclusions: In appropriate patients, it is critical to include vasculitis on the differential in rapidly progressive renal failure even without other systemic symptoms to prevent further damage.