Abstract Introduction Hydralazine is a common arterial vasodilator used in the treatment of hypertension and heart failure. Rarely, hydralazine can cause an overlapping syndrome of drug-induced lupus and antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). The clinical spectrum of this disease process includes features of systemic lupus erythematous and AAV Case Presentation A 62-year-old male previous smoker with 50 pack-year history and hypertension presented with an eight-month history of exertional dyspnea and pleuritic chest pain. Prior to his presentation, he was treated for pneumonia with antibiotics without improvement. His subsequent chest imaging showed a right sided pleural effusion, evaluated by thoracentesis which was exudative and lymphocytic with negative cultures. He then developed progressive ground glass opacities, anemia, lymphopenia, splenomegaly, and acute kidney injury. With concern for underlying malignancy, he underwent a PET/CT and bone marrow biopsy which were not suggestive of malignancy. Serology revealed strongly a myeloperoxidase (MP) antibody (8 U) with low titer proteinase-3 (PR3) antibody (1.2 U) and p-ANCA by immunofluorescence. Additional serology were notable for positive anti-double stranded DNA (dsDNA), positive ANA by hep2A, normal C3/C4, and creatinine peaking at 1.78 mg/dL, subnephrotic range proteinuria, and hematuria without RBC casts. Bronchoscopy noted progressively bloody return and 99% hemosiderin laden macrophages consistent with diffuse alveolar hemorrhage. Hydralazine was promptly discontinued with a clinical presentation consistent hydralazine-associated AAV and overlapping drug-induced lupus. They received remission induction therapy with IV methylprednisolone followed by tapering course of prednisone, rituximab, and avacopan. He was managed and monitored closely in the outpatient setting. With gradually improvement in renal function, a renal biopsy was deferred. Discussion Drug-induced lupus and drug-associated AAV can overlap in patients exposed to hydralazine, which can present up to several years after drug initiation. Renal and pulmonary involvement in hydralazine-induced lupus is uncommon and should prompt concern for drug-associated AAV. Compared with primary AAV, features suggestive of hydralazine-associated AAV include an adequate history of drug exposure and frequently a unique ANCA profile, hypocomplementemia, cytopenias, and additional seropositivity-including anti-histone and anti-dsDNA antibodies. Conclusion The cornerstone of management in hydralazine-induced lupus and hydralazine-associated AAV is early recognition and discontinuation of the offending medication. Induction of remission with standard immunosuppression is generally warranted, with specific therapies dictated by organ manifestations at diagnosis, similar to patients with primary AAV. Long-term outcomes in hydralazine-associated AAV appear comparable to those in primary AAV. The need for and duration of maintenance immunosuppression should be individualized based on patient presentation. This abstract is funded by: None
Peterson et al. (Fri,) studied this question.