Background Interstitial lung disease (ILD) is the leading cause of death in patients with systemic sclerosis (SSc). Treatment with cyclophosphamide (CYC) for 1 year has been shown to preserve pulmonary function. However, long-term efficacy and safety of CYC treatment for more than 1 year remains to be known.
Objectives To investigate efficacy and tolerability of long-term CYC treatment in SSc patients with ILD.
Methods All of the SSc-associated ILD patients who have been treated with CYC for more than 1 year at Rheumatology clinic of Seoul National University Hospital between 1978 and 2013 were enrolled. The information on clinical characteristics and pulmonary function test results were obtained from medical record review. Chest computed tomography (CT) was reviewed by a radiologist and classified into 4 grades depending on the extent of lung involvement. Mortality data were obtained from medical record or mortality record from Statistics Korea.
Results A total of 32 SSc patients with ILD were treated with CYC for more than 1 year with the mean treatment duration of 28.0 months (range: 12.0- 70.5 months). The mean age at diagnosis was 47.5±9.4 years and women were dominant at 84.4%. The mean functional vital capacity (FVC) at baseline was 66.0±16.0% of predicted. At baseline 18 (64.3%) patients had CT grade of 2 or more. After mean (standard deviation) follow up period of 4.1 (3.1) years, FVC remained stable with the mean increase of 2.3 (2.5) % (P=0.365, by paired t test). Furthermore, the degree of pulmonary involvement on CT did not progress. Six patients died during the follow-up; pneumonia (n=1), pulmonary hypertension (n=1), subdural hemorrhage (n=1), heart failure (n=2) and malignancy (n=1). Adverse events during CYC treatment included severe infection (n=4), cytopenia (n=4), hemorrhagic cystitis (n=1) and alopecia (n=3).
Conclusions Prolonged treatment with CYC for more than 1 year is associated with stabilization of ILD in the respect of pulmonary function and extent of lung involvement on CT. Long-term maintenance treatment with CYC may be an option to treat severe ILD in SSc patients.
Disclosure of Interest None declared