title: Successful treatment of refractory aortitis in antineutrophil cytoplasmic antibody-associated vasculitis using tocilizumab.
abstract: A 47-year-old Japanese woman developed antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) complicated by a rare combination of aortitis and hypertrophic pachymeningitis. Despite the therapy with prednisolone and cyclophosphamide, the aortitis was not ameliorated. However, after cyclophosphamide was replaced with intravenous tocilizumab, the aortitis was improved, and the prednisolone dose was successfully tapered to 4 mg/day without elevation in C-reactive protein and myeloperoxidase ANCA (MPO-ANCA) levels. Several studies have reported that tocilizumab is effective for aortitis associated with Takayasu's arteritis and giant cell arteritis. On the other hand, we succeeded to improve the aortitis in AAV with monthly administration of tocilizumab. Moreover, we successfully controlled disease activity and enabled the tapering of prednisolone to 4 mg/day without relapses of AAV symptoms and elevated MPO-ANCA levels. It indicates that tocilizumab may be therapeutically beneficial for not only aortitis but also AAV itself. In conclusion, tocilizumab was effective in treating glucocorticoid- and cyclophosphamide-resistant AAV-associated aortitis. This is the first report demonstrating the successful treatment of AAV-associated aortitis using tocilizumab.
authors.affiliation: Department of Rheumatology, Ome Municipal General Hospital, 4-16-5 Higashi-Ome, Ome, Tokyo, 198-0042, Japan.
authors.forename: Kenchi
authors.initials: K
authors.lastname: Takenaka
authors.forename: Takehiko
authors.initials: T
authors.lastname: Ohba
authors.forename: Kozo
authors.initials: K
authors.lastname: Suhara
authors.forename: Yurie
authors.initials: Y
authors.lastname: Sato
authors.forename: Kenji
authors.initials: K
authors.lastname: Nagasaka
mesh_terms: D000893:Anti-Inflammatory Agents / Q000008:administration & dosage; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000188:drug therapy*; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood; D061067:Antibodies, Monoclonal, Humanized / Q000008:administration & dosage*; D001025:Aortitis / Q000188:drug therapy*; D002097:C-Reactive Protein / Q000378:metabolism; D003520:Cyclophosphamide / Q000008:administration & dosage; D005260:Female; D006801:Humans; D008875:Middle Aged; D011239:Prednisolone / Q000008:administration & dosage; D016896:Treatment Outcome
chemical_list: D000893:Anti-Inflammatory Agents; D019268:Antibodies, Antineutrophil Cytoplasmic; D061067:Antibodies, Monoclonal, Humanized; D003520:Cyclophosphamide; D002097:C-Reactive Protein; D011239:Prednisolone; C502936:tocilizumab