Kumar B, et al. - This paper encompassed the characterization of the clinical, laboratory, and histopathologic characteristics of drug-induced lupus erythematosus (DIL) and ANCA-associated vasculitis (AAV). Findings illustrated that hydralazine-associated vasculitis was a drug-associated autoimmune syndrome characterized by interstitial lung disease, hypocomplementemia, and pauci-immune glomerulonephritis. Elements of both DIL and DIV were noted in the patients, featuring high ANA and ANCA titers along with the presence of histone and MPO antibodies.

Methods

• Following an approval from the Institutional Review Board at the University of Iowa, physicians selected all patients carrying a diagnosis of vasculitis (ICD9 code: 447.6 or ICD10 code: I77.6, I80, L95, M30, or M31) and positive ANCA lab results over the past 15 years.
• From the electronic medical record, data extraction was performed with regard to age, gender, comorbid conditions, medications taken over the prior 6 months, laboratory data, including electrolytes, urine studies and serologies, chest x-rays, CT scans, and pathologic biopsy records.

Results

• This study involved 323 cases of AAV, out of which 12 were exposed to hydralazine, all at the time of diagnosis.
• The average duration of hydralazine therapy was determined to be 22 months and mean cumulative dose was 146 g.
• The candidates were typically older (70.3 years old) with slight female preponderance (7 females).
• Dyspnea, fatigue, and unintentional weight loss was reported in 11 subjects.
• Five patients presented with polyarthralgias and 8 had lower extremity petechiae.
• Both ANA and ANCA positivity was noted among all 12 patients.
• ANA titers ranged from 1:160 and 1:2560, among these 10 were of diffuse pattern while 2 were nucleolar.
• It was noted that ANCA titers ranged from 1:320 to 1:2560.
• Eleven subjects had a pANCA pattern while one had cANCA.
• Findings revealed that all 12 patients were positive for histone and 11 were positive for myeloperoxidase antibodies.
• Eleven also had dsDNA antibodies, and 4 had anti-cardiolipin IgG or IgM antibodies.
• Furthermore, 9 individuals were hypocomplementemic (mean C3 level: 88.4 mg/dL; mean C4 level: 16.5 mg/dL).
• Variable levels of proteinuria (1+ to 3+) were disclosed in all patients and eleven had active urine sediment.
• The observation made was that urine protein:creatinine ratios ranged from 0.2 to 1.7.
• Six patients underwent kidney biopsy and illustrated pauci-immune crescentic glomerulonephritis.
• CT scan findings unveiled that 7 patients had bilateral pulmonary interstitial infiltrates and four had pleural effusions.
• Echocardiography revealed that 4 candidates had pericardial effusions.