Rheumatology Advance Access published online on June 4, 2006
Rheumatology, doi:10.1093/rheumatology/kel112
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1 Division of Rheumatology and Allergy, Department of Internal Medicine, St Marianna University School of Medicine, 2-16-1 Sugao, Miyamae, Kawasaki, Kanagawa 216-8511, Japan
* To whom correspondence should be addressed. 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 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.
Received September 29, 2005
Accepted February 28, 2006
Original Papers
Intravenous cyclophosphamide therapy for progressive interstitial pneumonia in patients with polymyositis/dermatomyositis
Y. Yamasaki 1 *,
H. Yamada 1,
M. Yamasaki 1,
M. Ohkubo 1,
K. Azuma 1,
S. Matsuoka 2,
Y. Kurihara 2,
H. Osada 3,
M. Satoh 4,
and
S. Ozaki 1
2 Department of Radiology, St Marianna University School of Medicine, 2-16-1 Sugao, Miyamae, Kawasaki, Kanagawa 216-8511, Japan
3 Division of Thoracic Surgery, Center for Respiratory Disease, St Marianna University School of Medicine, 2-16-1 Sugao, Miyamae, Kawasaki, Kanagawa 216-8511, Japan
4 Division of Rheumatology and Clinical Immunology, Department of Medicine, University of Florida, USA
Y. Yamasaki, E-mail: yams{at}marianna-u.ac.jp
Abstract 
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.
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