SHM Converge Scientific Abstract ePoster Gallery-Granulomatosis With Polyangiitis Presenting as Severe Renal Failure

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This case describes a 72-year-old woman with chronic kidney disease, atrial fibrillation, and GERD who presented with two weeks of severe nausea, vomiting, poor oral intake, and decreased urine output. She was found to have a marked rise in creatinine from 1.0 to 8.0 mg/dL, new hematuria, and imaging showing pleural and pericardial effusions. Despite diuresis, she developed volume overload requiring hemodialysis.<br /><br />Further workup revealed a strongly positive C-ANCA and proteinase 3 antibody, and kidney biopsy showed florid active and chronic pauci-immune crescentic glomerulonephritis, consistent with granulomatosis with polyangiitis (GPA). Her illness then progressed to acute hypoxemic respiratory failure with hemoptysis and new bilateral infiltrates. Bronchoscopy demonstrated hemorrhagic secretions throughout the airways, confirming diffuse alveolar hemorrhage. She was treated with inhaled tranexamic acid, cyclophosphamide, and plasma exchange, with eventual resolution of pulmonary symptoms. She was discharged on a prednisone taper but remained dialysis-dependent.<br /><br />The report emphasizes that GPA can present initially as isolated severe renal failure, even without classic upper or lower respiratory symptoms. Although renal involvement occurs in many patients, only a minority present initially with renal insufficiency. When renal dysfunction is present at diagnosis, outcomes are worse, especially if dialysis is required. Because progression can be rapid, early recognition and treatment are essential to prevent life- and organ-threatening complications.<br /><br />In summary, this case highlights the importance of maintaining a high index of suspicion for GPA in patients with unexplained rapidly progressive renal failure and hematuria, even when other possible causes of AKI are present.

Asset Subtitle

Christopher N. Eaton

Meta Tag

Author List Christopher N. Eaton, Elizabeth Allen

Category Clinical Vignettes

Concept Granulomatosis with Polyangiitis

Concept Rapidly Progressive Glomerulonephritis

Concept Acute Kidney Injury

Concept Pauci-Immune Crescentic Glomerulonephritis

Concept Diffuse Alveolar Hemorrhage

Distinguished Non-Finalist

Presenter Organization OhioHealth Riverside Methodist Hospital

Presenting Author Christopher N. Eaton

Track Adult

Keywords

granulomatosis with polyangiitis

GPA

rapidly progressive glomerulonephritis

pauci-immune crescentic glomerulonephritis

C-ANCA

proteinase 3 antibody

acute kidney injury

diffuse alveolar hemorrhage

hemodialysis

hematuria

Granulomatosis with Polyangiitis

Rapidly Progressive Glomerulonephritis

Acute Kidney Injury

Pauci-Immune Crescentic Glomerulonephritis

Diffuse Alveolar Hemorrhage