Treatment of refractory giant cell arteritis with cyclophosphamide: a retrospective analysis of 35 patients from three centres

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Rheumatology ArticleJ. Loock, J. Henes, I. Kötter, T. Witte, P. Lamprecht, M. Schirmer, W. Gross

University of Lübeck, Department of Rheumatology, Vasculitis Center & Klinikum Bad Bramstedt, Germany. jan.loock@live.de

CER5457 Submission on line 2012 Vol.30, N°1 ,Suppl.70 - PI 0070, PF 0076
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Patients with giant cell arteritis (GCA) refractory to standard immunosuppressive therapy may constitute a significant clinical problem with a high risk of glucocorticoid-related adverse effects. OBJECTIVES:
To evaluate efficacy and safety of cyclophosphamide for remission induction in GCA patients with persistent disease activity despite standard immunosuppressive treatment.
Thirty-five individuals from 3 tertiary rheumatological centres treated for persistently active GCA unresponsive to treatment with glucocorticoids plus at least either methotrexate or azathioprine for a minimum of 3 months and unable to reduce daily glucocorticoid dose to <10 mg prednisolone equivalent. We recorded signs of disease activity (clinical, laboratory, imaging); course of glucocorticoid doses during cyclophosphamide treatment and follow-up; relapse rate; treatment-related adverse events; and survival. Since all patients had been refractory to standard therapy, a matched control group could not be defined.
Data from 31 patients completing cyclophosphamide treatment were available for analysis. Twenty-eight patients (90.3%) responded with improved disease activity and sustained reduction of daily prednisolone intake to <10 mg (mean reduction -13.1 mg or -51.6%, p<0.001). Twelve months later, doses <7.5 or <5 mg were achieved in 89.3% and 67.7% of these patients on maintenance immunosuppressive treatment, respectively. Relapses occurred in 12 patients after a median of 20.5 months. Survival over 5 years was similar to expected rates of the general population. Adverse events comprised transient leucopenia, infections and 1 case of haemorrhagic cystitis.
Cyclophosphamide can be considered a therapeutic option with an acceptable safety profile for remission induction in GCA refractory to standard immunosuppressive treatment.

PMID: 22640650 [PubMed]

Received: 13/02/2012 - Accepted : 13/04/2012 - In Press: 14/05/2012 - Published: 11/05/2012