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Trojan horses: drug culprits associated with antineutrophil cytoplasmic autoantibody (ANCA) vasculitis

Pendergraft, William F. IIIa,b,c; Niles, John L.b,c

Current Opinion in Rheumatology: January 2014 - Volume 26 - Issue 1 - p 42–49
doi: 10.1097/BOR.0000000000000014

Purpose of review Antineutrophil cytoplasmic autoantibody (ANCA) vasculitis is a systemic autoimmune disease resulting in small-vessel inflammation caused by pathogenic autoantibodies directed against proteinase 3 or myeloperoxidase. Legal drug culprits have been implicated as causative agents in secondary forms of disease, and a recent burst of reports also implicate levamisole-adulterated cocaine as a culprit.

Recent findings Here, we briefly discuss all drug culprits associated with ANCA vasculitis and then focus on clinical, serologic, therapeutic and mechanistic aspects of four main drug culprits receiving attention of late, namely hydralazine, minocycline, propylthiouracil (PTU) and levamisole-adulterated cocaine.

Summary Hydralazine, minocycline, propylthiouracil and levamisole-adulterated cocaine use should be closely considered in any patient where ANCA vasculitis is entertained given the wide use of these drugs in the community. Furthermore, medical practitioners should test urine for the presence of cocaine in any patient with presumed ANCA vasculitis, and if positive, then urine should also be tested for levamisole. Clinical features can be severe requiring not only drug cessation and supportive care, but also immunosuppression, plasma exchange in severe cases and dialysis as needed. Clinical trial investigators should strongly consider excluding patients with drug-induced forms of disease and mechanistic inroads are greatly needed in these secondary forms of disease to help elucidate the underlying cause and pathogenesis of ANCA vasculitis.

aJoint Nephrology Fellowship Program, Brigham and Women's Hospital and Massachusetts General Hospital (MGH)

bDivision of Nephrology, Department of Medicine

cVasculitis and Glomerulonephritis Clinic, Division of Nephrology, MGH, Boston, Massachusetts, USA

Correspondence to Dr John L. Niles, MD, Division of Nephrology, Department of Medicine, Massachusetts General Hospital, 151 Merrimac Street, Floor 3, Boston, MA 02114-4719, USA. Tel: +1 617 248 3811; fax: +1 617 248 3884; e-mail:

© 2014 Lippincott Williams & Wilkins, Inc.