An Unexpected Culprit of ANCA-Associated Vasculitis With Pulmonary-Renal Syndrome

Document Type

Conference Proceeding

Publication Date

10-2025

Publication Title

Chest

Abstract

INTRODUCTION: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of small vessel vasculitides that can have varied systemic manifestations. Renal and pulmonary manifestations are the most common and include hemoptysis, dyspnea, and hematuria. Rarely, ANCA-associated vasculitis can be drug-induced; most frequently by minocycline, propylthiouracil, hydralazine, and levamisole-adulterated cocaine. The mechanism and incidence of hydralazineinduced AAV is largely unknown but the risk for the disease is dose-dependent. CASE PRESENTATION: An 80-year-old male with history of hypertension, type 2 diabetes mellitus, CKD 3, and untreated obstructive sleep apnea presented to a small community hospital with several weeks of intermittent hemoptysis. Initial diagnostic findings included acute kidney injury, progressively worsening anemia, and bilateral pulmonary infiltrates on radiography. Empiric treatment with steroids as well as antibiotics for possible community acquired pneumonia were initiated. Subsequently, the patient was transferred to a larger hospital for nephrology evaluation due to acuity and necessity for dialysis. CT chest showed diffuse groundglass opacities and intralobular septal thickening. Upon further investigation, it was discovered that the patient was chronically on hydralazine but had a recent dosage increase thus it was discontinued. Despite this, fevers persisted and respiratory status declined therefore patient was ultimately transferred to a tertiary care hospital for renal biopsy and intensive-level care where he unfortunately progressed to intubation and hemodialysis. Plasmapheresis and high-dose steroids were initiated for suspected pulmonary-renal syndrome. Further testing demonstrated ANA (1:1280) with positive Scl70, p-ANCA with positive MPO, and negative anti-histone antibodies. Bronchoalveolar lavage showed diffuse alveolar hemorrhage and renal biopsy demonstrated pauci-immune crescentic glomerulonephritis which were both consistent with pulmonary-renal syndrome secondary to hydralazine-induced AAV. Shortly after completing plasmapheresis, patient was able to be extubated and rituximab was initiated. Unfortunately, the patient remained hemodialysis-dependent post-discharge. DISCUSSION: Diagnostic testing for AAV includes ANCA, anti-histone antibodies, chest imaging, and urinalysis. Ultimately, if there is high clinical suspicion, renal biopsy showing pauci-immune glomerulonephritis is the gold-standard for confirmation of disease. Treatment consists of high-dose steroids and immunosuppression with rituximab or cyclophosphamide and should not be delayed for biopsy. Our patient received plasmapheresis due to lack of glucocorticoid response, severity of renal dysfunction, and prior literature supporting that plasmapheresis may reduce risk of end-stage renal disease. In contrast, the PEXIVAS trial in 2024 demonstrated no additional benefit with addition of plasmapheresis to standard glucocorticoid and immunosuppressive therapy in patients with severe AAV. This is in alignment with the American College of Rheumatology guidelines which conditionally recommend against the routine addition of plasma exchange to remission induction therapy. Interestingly, a subgroup analysis of patients with AAV and DAH did show possible benefit in the PEXIVAS trial. CONCLUSIONS: In patients with pulmonary-renal syndrome, initial evaluation should include a focused review of systems including constitutional symptoms, rashes, hemoptysis, and hematuria as well as detailed medication reconciliation and substance use history. Early recognition, medication discontinuation, and prompt initiation of glucocorticoids or immunosuppressive therapy are essential. The PEXIVAS trial raises important questions about the optimal treatment approach for AAV with DAH and renal involvement, highlighting the need for further research in this area.

Volume

168

Issue

4S

First Page

6369A

Last Page

6370A

Comments

American College of Chest Physicians CHEST Annual Meeting, October 19-22, 2025, Chicago, IL

DOI

10.1016/j.chest.2025.07.3560

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