ASN's Mission

ASN leads the fight to prevent, treat, and cure kidney diseases throughout the world by educating health professionals and scientists, advancing research and innovation, communicating new knowledge, and advocating for the highest quality care for patients.

learn more

Contact ASN

1401 H St, NW, Ste 900, Washington, DC 20005

email@asn-online.org

202-640-4660

The Latest on Twitter

Kidney Week

Abstract: PO1953

Sabotaged: Hydralazine-Induced ANCA Glomerulonephritis

Session Information

Category: Trainee Case Report

  • 1203 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Kruger gomes, Larissa, Beth Israel Deaconess Medical Center, Boston, Massachusetts, United States
  • Sula Karreci, Esilida, Beth Israel Deaconess Medical Center, Boston, Massachusetts, United States
  • Agarwal, Krishna A., Beth Israel Deaconess Medical Center, Boston, Massachusetts, United States
  • Schulman, Ruth, Beth Israel Deaconess Medical Center, Boston, Massachusetts, United States
  • William, Jeffrey H., Beth Israel Deaconess Medical Center, Boston, Massachusetts, United States
  • Lecker, Stewart H., Beth Israel Deaconess Medical Center, Boston, Massachusetts, United States
  • Stillman, Isaac Ely, Beth Israel Deaconess Medical Center, Boston, Massachusetts, United States
Introduction

While usually well known to cause SLE-like syndrome, hydralazine (HZ) can also be involved in a different clinical scenario with ANCA vasculitis.

Case Description

77y/o woman with hypertension and COPD presents with 2 days of hemoptysis and hematuria, requiring urgent intubation. CT scan reveals multifocal infiltrates and bronchoscopy shows diffuse alveolar hemorrhage. Labs showed Hb of 6.8 and Cr of 1.9. Dysmorphic RBCs were seen in the urine sediment and proteinuria at 1.2g/g. Given concern for anti-GBM, she received 1g of methylprednisolone and plasma exchange. ANA 1:1280 with MPO-ANCA levels >8.0 U, along with positive anti-histone and SCL-70 antibodies, but with negative anti-GBM and ds-DNA. Kidney biopsy showed pauci-immune crescentic GN with trace staining for IgA, IgM, C3, kappa and lambda. As she had been on HZ, the diagnosis of HZ-induced ANCA-associated vasculitis was made. Offending agent was held and cyclophosphamide was started. After 2 months, kidney function returned to baseline, with resolution of proteinuria and hematuria.

Discussion

In patients with HAV, unusually high titers of MPO are present and can be used to differentiate drug-associated and spontaneous cases. A minority of patients can also present with other positive antibodies, such as ds-DNA, anti-histone, or Scl-70. As HZ can also cause SLE-like pattern of injury, it can be difficult to obtain a diagnosis based on serologies alone; biopsy is essential for diagnosis and prognosis. The treatment of HAV involves not only removing the inciting agent, but also further immunosuppression, as HZ is thought to lead to increased expression of MPO and PR3 breaking neutrophils tolerance and leading to auto-antibody formation. When choosing a treatment strategy, guidelines for spontaneous ANCA should be followed; in this particular case, as she had severe lung involvement, cyclophosphamide was chosen.

(a) Masson thrichrome - Crescent with compression of capilary tuft. Tubules with RBC casts. (b) IF 0 to trace IgG (C) EM Occasional electron dense deposits in mesangium only.