Rheumatology Advance Access originally published online on June 4, 2006
Rheumatology 2007 46(1):124-130; doi:10.1093/rheumatology/kel112
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Intravenous cyclophosphamide therapy for progressive interstitial pneumonia in patients with polymyositis/dermatomyositis
1Division of Rheumatology and Allergy, Department of Internal Medicine, 2Department of Radiology, 3Division of Thoracic Surgery, Center for Respiratory Disease, St Marianna University School of Medicine, 2-16-1 Sugao, Miyamae, Kawasaki, Kanagawa 216-8511, Japan and 4Division of Rheumatology and Clinical Immunology, Department of Medicine, University of Florida, USA.
Correspondence to: Yoshioki Yamasaki, Division of Rheumatology and Allergy, Department of Medicine, St Marianna University School of Medicine, 2-16-1 Sugao, Miyamae, Kawasaki, Kanagawa 216-8511, Japan. E-mail: yams{at}marianna-u.ac.jp
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Abstract |
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Objectives. To study the efficacy and safety of monthly intravenous pulse cyclophosphamide (IVCYC) therapy for progressive interstitial pneumonia in polymyositis/dermatomyositis (PM/DM).
Methods. Seventeen patients with PM/DM/amyopathic DM (mean age 51.4 ± 10.4, mean follow-up 32 months) who received IVCYC for progressive interstitial pneumonia between August 1993 and October 2002 were studied. Nine patients had failed to respond to previous treatment with high-dose steroid and/or immunosuppressant. Cyclophosphamide (300–800 mg/m2) was given at least six times every 4 weeks. Oral prednisolone (0.5–1 mg/kg/day) was administered for the first 2 weeks and was gradually tapered. Response to treatment was evaluated based on the degree of exertional dyspnea, pulmonary function test and high-resolution computed tomography (HRCT).
Results. Eleven of 17 patients showed improvement in their dyspnea; six out of seven patients who had required oxygen treatment before IVCYC no longer did so after IVCYC. Eight of 17 patients had 
10% improvement of vital capacity (VC)% and 9/17 had 10 point reduction in their HRCT score. Twelve patients had exhibited at least one result. Two patients with anti-Jo-1 antibodies showed a flare-up of interstitial pneumonia or myositis. After the IVCYC therapy, mean VC% improved by 15% (from 68 to 83%, P = 0.0034). The extent of abnormal lesions in HRCT was reduced from 24 to 13% (P = 0.0055). There was neither death nor severe toxicities observed.
Conclusions. In this open-label study, IVCYC improved symptoms, pulmonary function tests and HRCT findings in patients with PM/DM. Longitudinal controlled studies are required to further confirm the efficacy of IVCYC.
KEY WORDS: Interstitial pneumonia, Intravenous pulse, Cyclophosphamide, Polymyositis, Dermatomyositis