title: Primary systemic vasculitis: treatment of difficult cases.
abstract: The primary systemic necrotizing vasculitides are a severe group of diseases, which untreated have a high mortality. The majority respond to treatment with high dose steroids and cyclophosphamide, however a significant proportion of those treated suffer morbidity due to the side effects of these agents, and a number of patients are refractory to treatment. We review and discuss alternative and emerging treatment options for patients who fail or cannot tolerate conventional therapy. An interesting subgroup of patients with systemic vasculitis and antiphospholipid antibodies provides an additional diagnostic and therapeutic challenge. We review what is known about this subgroup, and suggest screening for antiphospholipid antibodies in all patients with systemic vasculitis.
authors.affiliation: Lupus Research Unit, The Rayne Institute, St Thomas' Hospital, London, UK.
authors.forename: R
authors.initials: R
authors.lastname: Foster
authors.forename: E
authors.initials: E
authors.lastname: Rosenthal
authors.forename: S
authors.initials: S
authors.lastname: Marques
authors.forename: P
authors.initials: P
authors.lastname: Vounotrypidis
authors.forename: S
authors.initials: S
authors.lastname: Sangle
authors.forename: D
authors.initials: D
authors.lastname: D'Cruz
mesh_terms: D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood; D017152:Antibodies, Antiphospholipid / Q000097:blood; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived; D016736:Antiphospholipid Syndrome / Q000453:epidemiology; D001379:Azathioprine / Q000627:therapeutic use; D003520:Cyclophosphamide / Q000627:therapeutic use; D006801:Humans; D000069285:Infliximab; D000069283:Rituximab; D014409:Tumor Necrosis Factor-alpha / Q000037:antagonists & inhibitors; D014657:Vasculitis / Q000150:complications / Q000188:drug therapy* / Q000276:immunology
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D017152:Antibodies, Antiphospholipid; D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D014409:Tumor Necrosis Factor-alpha; D000069283:Rituximab; D003520:Cyclophosphamide; D000069285:Infliximab; D001379:Azathioprine