Intravenous cyclophosphamide therapy for progressive interstitial pneumonia in patients with polymyositis/dermatomyositis.
OBJECTIVE
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 >or=10% improvement of vital capacity (VC)% and 9/17 had >or=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.
Yamasaki
Y
Y
Division of Rheumatology and Allergy, Department of Medicine, St Marianna University School of Medicine, 2-16-1 Sugao, Miyamae, Kawasaki, Kanagawa 216-8511, Japan. yams@marianna-u.ac.jp
Yamada
H
H
Yamasaki
M
M
Ohkubo
M
M
Azuma
K
K
Matsuoka
S
S
Kurihara
Y
Y
Osada
H
H
Satoh
M
M
Ozaki
S
S
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D003520:Cyclophosphamide / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D003882:Dermatomyositis / Q000150:complications; D018450:Disease Progression; D004334:Drug Administration Schedule; D004417:Dyspnea / Q000188:drug therapy / Q000209:etiology; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D007275:Injections, Intravenous; D017563:Lung Diseases, Interstitial / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000209:etiology / Q000503:physiopathology; D008875:Middle Aged; D017285:Polymyositis / Q000150:complications*; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D014797:Vital Capacity / Q000187:drug effects
D007166:Immunosuppressive Agents; D003520:Cyclophosphamide