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Published on: February 8, 2019
Treatment of refractory giant cell arteritis with cyclophosphamide:a retrospective analysis of 35 patients from three
Jan Loock1, Jörg Henes, Ina Kötter
1University of Lübeck, Department of Rheumatology, Vasculitis Center & Klinikum Bad Bramstedt, Germany. jan.loock@live.de
Insights
Cyclophosphamide effectively induces remission in giant cell arteritis (GCA) patients resistant to standard treatments, reducing steroid dependence and adverse effects. This offers a viable therapeutic option for refractory GCA.
Area of Science:
- Rheumatology
- Immunosuppressive Therapy
- Clinical Trials
Background:
- Giant cell arteritis (GCA) poses challenges for patients refractory to standard immunosuppressive therapy.
- High-dose glucocorticoids in GCA treatment increase the risk of adverse effects.
- Need for effective and safe alternatives for GCA remission induction exists.
Purpose of the Study:
- To assess the efficacy and safety of cyclophosphamide for inducing remission in GCA patients.
- To evaluate cyclophosphamide's role in reducing glucocorticoid dependence in refractory GCA.
Main Methods:
- A study involving 35 patients with persistently active GCA unresponsive to standard treatments.
- Patients received cyclophosphamide due to inability to reduce daily prednisolone dose below 10 mg.
- Disease activity, glucocorticoid dose changes, relapse rates, adverse events, and survival were monitored.
Main Results:
- Cyclophosphamide treatment led to disease activity improvement in 90.3% of 31 analyzed patients.
- Sustained reduction of daily prednisolone intake to below 10 mg was achieved in 90.3% of responders.
- Relapses occurred in 12 patients after a median of 20.5 months; 5-year survival was comparable to the general population.
Conclusions:
- Cyclophosphamide is a potential therapeutic option for inducing remission in GCA.
- The drug demonstrates an acceptable safety profile for refractory GCA patients.
- Cyclophosphamide aids in reducing steroid dependency in difficult-to-treat GCA cases.
Unlabelled:
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.
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.
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