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Lupus, Vol. 15, No. 3, 143-147 (2006)
DOI: 10.1191/0961203306lu2277rr

Primary systemic vasculitis: treatment of difficult cases

R Foster

Department of Immunology, St Thomas’ Hospital, London, UK

E Rosenthal

Lupus Research Unit, The Rayne Institute, St Thomas’ Hospital, London, UK

S Marques

Lupus Research Unit, The Rayne Institute, St Thomas’ Hospital, London, UK

P Vounotrypidis

Lupus Research Unit, The Rayne Institute, St Thomas’ Hospital, London, UK

S Sangle

Lupus Research Unit, The Rayne Institute, St Thomas’ Hospital, London, UK

D D’Cruz

Lupus Research Unit, The Rayne Institute, St Thomas’ Hospital, London, UK, david.d'cruz{at}kcl.ac.uk

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.

Key Words: antiphospholipid antibodies • anti-TNF alpha therapy • mycophenolate mofetil • rituximab • vasculitis


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