Abstract: TH-PO959
Hydralazine-Induced Crescentic Pauci-Immune Glomerulonephritis Associated with Multi-Antigenicity
Session Information
- Glomerular Trainee Case Reports
November 07, 2019 | Location: Exhibit Hall, Walter E. Washington Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Trainee Case Report
- 1202 Glomerular Diseases: Immunology and Inflammation
Authors
- Tharian, Antonia, NYU Winthrop, Manhasset, New York, United States
- Drakakis, James, NYU Winthrop, Manhasset, New York, United States
Introduction
Hydralazine use is common for treatment of hypertension, and heart failure with the ability to induce lupus as a rare complication. Though mechanisms are not understood, it may also be a causal factor in antinuclear cytoplasmic antibody (ANCA) vasculitis (AAV). This emerging syndrome is characterized by crescentic pauci-immune glomerulonephritis and classically positive antibodies, including anti myeloperoxidase (MPO) and anti-histone. We report a case notable for the wide array of auto antibodies, the combination of which has not been previously reported.
Case Description
A 71 year old male presented to the hospital with a two month history of fatigue. His past medical history included hypertension for which he was taking Hydralazine, and an ischemic cardiomyopathy. His lab results showed an elevation in serum creatinine of 3.2 mg/dL from a baseline of 1.2 – 1.3 mg/dL. Urinalysis revealed proteinuria and hematuria with accompanying red blood cells. Serologies returned positive not only anti-histone antibodies, but also for both MPO and anti-proteinase 3 (PR3) antibodies. In addition, was a high titer ANA, double stranded DNA, low C3, presence of lupus anticoagulant, and anti-cardiolipin IgG. Kidney biopsy revealed focal segmental necrotizing and crescentic glomerulonephritis, pauci-immune type, acute and subacute. CT chest was negative for pulmonary hemorrhage. Taken together, these findings seemed consistent with a drug induced AAV. Deemed to be the culprit, Hydralazine was discontinued and treatment rendered with pulse dose steroids, transitioning to tapering Prednisone and two doses of Rituximab. This led to a marked clinical and serologic improvement, which was not only rapid, but also proved durable.
Discussion
Drug induced AAV has been linked to certain agents, including Hydralazine. The presentation may be severe, and often associated antibody positivity such as MPO and anti-histone may hold the key to the diagnosis. Our case illustrates a vaster splay of antibodies, the combination of which has not been previously described. The mechanism by which these are induced and the implication to disease response and progression need be investigated further. Thorough medication history to identify those at risk, rapid discontinuation of the drug and prompt treatment may all lead to improved clinical outcomes.