Abstract: PUB262
When Lungs Wail and Kidneys Fail: Unmasking a Case of ANCA-Associated Vasculitis
Session Information
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Patra, Tumpa, Crestwood Medical Center, Huntsville, Alabama, United States
- Bag, Soumyadeep, The University of Alabama at Birmingham School of Medicine Huntsville, Huntsville, Alabama, United States
- Poojary, Indira, Crestwood Medical Center, Huntsville, Alabama, United States
- Haley, Heather L., Crestwood Medical Center, Huntsville, Alabama, United States
- Rahman, Saad, Crestwood Medical Center, Huntsville, Alabama, United States
Introduction
ANCA-associated vasculitis is a rare, systemic small-vessel vasculitis that can present with rapidly progressive glomerulonephritis and pulmonary involvement. Its acute manifestations including respiratory and kidney failure often require specialized, multidisciplinary care. Early recognition and prompt treatment are critical, though management remains challenging due to its severity and complexity.
Case Description
A 71-year-old woman with a history of congestive heart failure, type 2 diabetes mellitus, chronic kidney disease, and atrial fibrillation was transferred to the ICU of a tertiary care center with acute kidney injury (serum creatinine 7.8 mg/dL; baseline ~1.2 mg/dL) and hypoxic respiratory failure. She arrived intubated on high ventilator settings for suspected diffuse alveolar hemorrhage (DAH), which was confirmed via bronchoscopy at our facility. With high clinical suspicion for pulmonary-renal syndrome, pulse-dose corticosteroids and plasmapheresis were initiated prior to serologic and histologic confirmation. Her respiratory status improved significantly, allowing extubation within two days. Subsequent workup revealed p-ANCA positivity, and renal biopsy showed pauci-immune crescentic glomerulonephritis with 40% global glomerulosclerosis, 80% tubular injury, and 40% interstitial fibrosis. She was started on rituximab. Unfortunately her hospital course was complicated by gastrointestinal bleeding, ESBL urinary tract infection, and symptomatic anemia requiring transfusion. She completed seven sessions of plasmapheresis and two rituximab infusions. She remained dialysis-dependent and was discharged to inpatient rehabilitation on oral prednisone taper.
Discussion
ANCA-associated vasculitis with DAH carries up to 50% mortality in critically ill patients. In our case, close coordination among ICU, nephrology, and rheumatology teams enabled early diagnosis and initiation of pulse-dose steroids, plasmapheresis, and rituximab, which were critical to the patient’s survival. Despite timely treatment, she progressed to dialysis, likely due to age and comorbidities. DAH is rare but life-threatening and requires a high degree of clinical suspicion for diagnosis. Early transfer to a higher-level center, as demonstrated here, can lead to improved outcomes even in complex presentations.