Abstract: TH-PO684
Late-Onset Hydralazine-Associated Immune-Complex Glomerulonephritis with Overlap ANCA and Lupus Nephritis Features
Session Information
- Glomerular Diseases: Epidemiology and Case Reports
November 02, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Trials
Author
- Srisawitri, Liana, Indiana University School of Medicine, Indianapolis, Indiana, United States
Introduction
Hydralazine is one medication that has been long associated with drug-induced lupus and also antinuclear cytoplasmic antibodies (ANCA) vasculitis. We present an unusual case of an 83-year-old patient with hydralazine-associated glomerulonephritis (GN) with overlap features of ANCA vasculitis and lupus nephritis.
Case Description
The patient presented with hemoptysis with blood clot and estimated half a cup of red blood. She has history of hypertension and atrial fibrillation. Patient has had dyspnea on exertion, cough, nausea, and fatigue for 3 months. CT chest showed bronchial opacification, airspace disease, and mild fibrosis. Patient had remote 20 pack-year history of smoking. Her medications include hydralazine 50 mg 3 times a day for more than 10 years.
Hemoglobin (Hgb) was 6.4 g/dL (down from 9.3 g/dL 1 month prior). Serum creatinine (Cr) was 2.3 mg/dL up from 1.4 one month prior and 0.7 six months prior. C3 and C4 70 and <8 mg/dL. Urinalysis showed 6-10 WBC and 50-100 RBC/hpf with dysmorphic RBCs and RBC casts. Urine protein/Cr ratio was 0.62 g/g. ANA 1:640, anti-dsDNA indeterminate, p-ANCA 1:640, MPO Ab 7.2 AI, PR-3 Ab >8.0 AI, anti-histone Ab 4.4 U. Kidney biopsy showed mild mesangial hypercellularity on light microscopy and mild mesangial deposits on electron microscopy. Immunofluorescence showed 1-2+ IgA, IgM, C3 diffuse granular deposits. Patient was given pulse IV methylprednisolone for 3 days, followed by slow taper prednisone. Cr peaked at 3.3 mg/dL and improved to 2.1 mg/dL upon discharge.
Discussion
Patient’s presentation of pulmonary renal syndrome is common in ANCA vasculitis, supported by the high p-ANCA, MPO Ab, and PR-3 Ab However, the classical findings of ANCA vasculitis with purely pauci-immune necrotizing and/or crescentic GN were not found. Instead, glomeruli showed mild IgA-predominant deposits. Low complements and elevated ANA and anti-histone Ab suggest hydralazine-induced GN. Ten years of hydralazine use confers a high cumulative dose, in contrast to a review of 12 cases of hydralazine-associated ANCA vasculitis showed median drug duration of 22 months. In a review of 7 cases of drug-induced lupus nephritis, the duration was >12 months for most patients. In summary, this case may be an atypical hydralazine-induced GN with overlapping lupus and ANCA serology but with dominant IgA deposits.