Kidney Week

Abstract: PUB421

Hydralazine-Induced ANCA-Associated Vasculitis with Pulmonary Involvement

Session Information

• Publication Only

Category: Hypertension and CVD

• 1602 Hypertension and CVD: Clinical

Authors

• Mark, Shemrine, Long Island Community Hospital, Patchogue, New York, United States

Shemrine Mark, MD, MPH

• London, Jonathan, Long Island Community Hospital, Patchogue, New York, United States

Jonathan London, MD

• Mekhail, Mario, Long Island Community Hospital, Patchogue, New York, United States

Mario Mekhail, MBChB, MSc

• Bolotova, Olena, Brookhaven Nephrology Associates, Patchogue, New York, United States

Olena Bolotova, MD

• Durgun, Ayse S., NYU Langone Pulmonary Associates, East Patchogue, Patchogue, New York, United States

Ayse S. Durgun, MD

• Rahman, Mohammed S., Brookhaven Nephrology Associates, Patchogue, New York, United States

Mohammed S. Rahman, MD

Introduction

Hydralazine is a frequently used antihypertensive associated with ANCA vasculitis. Clinical symptoms range from benign to multiorgan involvement like alveolar hemorrhage and glomerulonephritis which can be fatal if not treated in a timely manner. It is important to be aware of this association for timely treatment and management.

Case Description

A 64-year-old male with a pmh of ESRD on hemodialysis for 5 years due to hypertensive nephropathy, Coronary artery disease presented to the emergency department for low hemoglobin. The patient complained of chronic fatigue, cough and denied any other complaints. Vitals were stable with conjunctival pallor on exam. The patient has had frequent hospital visits for weekly blood transfusions. Laboratory results showed hemoglobin of 6.2(13.7-17.5 g/dL). CT chest showed bilateral ground glass opacities. Evaluation of anemia included endoscopy, colonoscopy and bone marrow biopsy which were negative. Treatment included iron, darbepoetin alfa, weekly blood transfusion and pantoprazole. Due to recurrent transfusions and inflammatory changes on imaging autoimmune workup was done. Results showed ESR 51(0-15mm/hr), CRP 28.8(0-3mg/L), positive ANA, MPO ANCA >8(<1AI), anti-histone antibodies 2.1(0-0.9 units), low complement level and normal thyroid function. Hydralazine was discontinued and the patient was treated with pulse steroids. Patient had a lung biopsy which showed bronchiolar fibrosis with hemosiderin laden macrophages, perivascular chronic inflammation with necrosis consistent with treated granulomatosis with polyangiitis. Patient was reported to be on hydralazine for at least 2.5 years maximum dose 100 mg three times daily. Patient reported improvement in symptoms with stabilization of hemoglobin on follow up appointment.

Discussion

ANCA vasculitis is a rare disease with an incidence of 10-20 cases per million people. Of the patients with hydralazine induced vasculitis only 18% had pulmonary involvement. Mechanism is unclear. However, risk factors include thyroid disease, increased dose and duration of drug use, female sex and slow acetylators. Diagnosis is made through history, serology for anti-histone antibodies, ANCA, low complement and biopsy of affected organs. Treatment includes discontinuation of hydralazine and immunosuppressive therapy with pulse steroids, rituximab and or cyclophosphamide.