Hydralazine-induced antineutrophil cytoplasmic antibody-associated vasculitis with pulmonary–renal syndrome: a case report

Abstract

Background: Hydralazine is a common vasodilator which has been used for the treatment of hypertension and heart failure. Hydralazine can induce antineutrophil cytoplasmic antibody-associated vasculitis due to its autoimmunogenic capability and one of the very rare presentations is pulmonary-renal syndrome. Case presentation: We report a case of a 64-year-old African American woman, who presented to our emergency room with shortness of breath, orthopnea, paroxysmal nocturnal dyspnea, leg swelling, fatigue, loss of appetite, cough with clear sputum, and lightheadedness. On admission, she developed acute hypoxic respiratory failure requiring intubation and acute renal failure requiring hemodialysis. A serologic workup was positive for antineutrophil cytoplasmic antibody, antinuclear antibody, anti-histone, anti-cardiolipin IgM, and anti-doublestranded DNA antibodies. A renal biopsy was done due to persistent deterioration in kidney function and demonstrated classic crescentic (pauci-immune) glomerulonephritis. Hydralazine was empirically discontinued early in the admission and she was started on corticosteroids and cyclophosphamide following biopsy results. She was clinically stable but remained dependent on hemodialysis after discharge. Conclusion: Hydralazine-induced antineutrophil cytoplasmic antibody-associated vasculitis with pulmonary-renal syndrome is a rare occurrence. In the setting of hydralazine use, multiple positive antigens, and multisystem involvement, clinicians should consider this rare condition requiring prompt cessation of offending drug, early evaluation with biopsy, and contemplate empiric immunosuppressive therapy while biopsy confirmation is pending.

Rare complication of a commonly used antihypertensive agent: A case of hydralazine‐induced ANCA‐associated vasculitis presenting as rapidly progressive glomerulonephritis

“…7 Moreover, anti-histone antibody positivity is not independently diagnostic of DIL, particularly in the absence of other criteria supporting a diagnosis of lupus. 3,4,7,13,[19][20][21][22] Additionally, complement pathway activation and resulting hypocomplementemia have been described in both drug-induced vasculitis and drug-induced lupus. 7,23 In fact, recent studies have demonstrated the existence of complement pathway activation in AAV pathogenesis 24,25 and further connect it with more severe renal disease and poor survival outcomes.…”

Not Lupus Nephritis but a Rare Case of Drug-Induced Pauci-Immune Glomerulonephritis

“…The incidence of hydralazine-induced ANCA-mediated vasculitis is proportional to the dose and duration of use. The incidence rate can be up to 10.4% in patients taking a dose of 200 mg/day for greater than three years [8]. Serum complement levels can be beneficial in differentiating the underlying pathogenesis since C3 and C4 levels are usually normal in PIGN and anti-GBM disease as opposed to the low levels seen in another glomerulonephritis [9,10].…”

“…There is a higher incidence of drug-induced nephropathy in patients who are greater than 60 years old, patients with diabetes, heart failure, and those with pre-existing renal insufficiency [7,8]. It has been reported it is more common in females and patients with thyroid disease with 80% of these patients having renal involvement on initial presentation [5].…”

Anti-neutrophil Cytoplasmic Antibody-Associated Glomerulonephritis Secondary to Hydralazine: A Case Report

“…AAV secondary to hydralazine is directly dependent on the duration and dose of hydralazine use. The use of hydralazine for more than three years can have an annual incidence of up to 5.4% and 10.4% in patients taking 100mg/day and 200mg/day, respectively [ 8 ].…”

“…There are reports of patients developing end-stage kidney disease requiring maintenance hemodialysis. In reported cases, renal outcomes for this disease have been variable, with one case study claiming full recovery in all patients and another reporting that one-third to half of the patients still needed long-term hemodialysis following immunosuppressive treatment [ 5 , 8 , 9 ].…”