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


OBJECTIVE
To evaluate efficacy and safety of cyclophosphamide for remission induction in GCA patients with persistent disease activity despite standard immunosuppressive treatment.


METHODS
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.


RESULTS
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.


CONCLUSIONS
Cyclophosphamide can be considered a therapeutic option with an acceptable safety profile for remission induction in GCA refractory to standard immunosuppressive treatment.
University of Lübeck, Department of Rheumatology, Vasculitis Center & Klinikum Bad Bramstedt, Germany. jan.loock@live.de
Jan
J
Loock
Jörg
J
Henes
Ina
I
Kötter
Torsten
T
Witte
Peter
P
Lamprecht
Michael
M
Schirmer
Wolfgang L
WL
Gross
D000284:Administration, Oral; D000368:Aged; D003520:Cyclophosphamide / Q000008:administration & dosage* / Q000009:adverse effects; D004351:Drug Resistance; D004359:Drug Therapy, Combination; D005260:Female; D005858:Germany; D013700:Giant Cell Arteritis / Q000188:drug therapy* / Q000401:mortality; D005938:Glucocorticoids / Q000008:administration & dosage; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage* / Q000009:adverse effects; D007262:Infusions, Intravenous; D008297:Male; D008875:Middle Aged; D012008:Recurrence; D012189:Retrospective Studies; D016019:Survival Analysis; D013997:Time Factors; D016896:Treatment Outcome
D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide