ORIGINAL ARTICLE

Intermittent intravenous cyclophosphamide pulse therapy for the treatment of active interstitial lung disease associated with collagen vascular diseases

Authors

Makoto Okada¹, Kimihiro Suzuki¹, Mitsuyo Matsumoto¹, Masahiro Nakashima¹, Takashi Nakanishi¹, Kunio Takada¹, Hideyuki Horikoshi¹, Osamu Matsubara² and Fumitaka Ohsuzu³

1. Department of Internal Medicine, Division of Rheumatology, National Defense Medical College, 3-2 Namiki, Tokorozawa 359-8513, Japan
2. Department of Pathology II, National Defense Medical College, Tokorozawa, Japan
3. Department of Internal Medicine, Division of Cardiology, National Defense Medical College, Tokorozawa, Japan

Received:

13 October 2006

Accepted:

22 January 2007

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Abstract

The availability of intravenous cyclophosphamide (CYC) pulse therapy for collagen vascular diseases (CVD)-associated interstitial lung disease (ILD) has been indicated. However, the standard protocol concerning the dosage and the interval of CYC infusion has not yet been established. The aim of this study is to elucidate the efficacy and the safety of our “divided administration” protocol of CYC for the treatment of CVD-ILD. The treatment protocol consists of two steps: step 1, CYC 400-500mg at 10-day intervals for at least 30 days, and step 2, CYC 500mg at 14-day intervals for at least 4 weeks. The ILD activities were monitored by respiratory symptoms, serum levels of KL-6 (a serological marker of IP), chest computed tomography (CT), and pulmonary function tests. Seventeen patients [nonspecific interstitial pneumonia (NSIP), 12 patients; usual interstitial pneumonia (UIP), 4; lymphocytic interstitial pneumonia (LIP), 1] accomplished the study protocol. The sessions of CYC infusion ranged from 5 to 20 (mean, 8.3). In all patients, respiratory symptoms were improved and the serum levels of KL-6 were decreased (from 1572 ± 904 to 978 ± 392U/ml; P < 0.01). Chest CT findings were improved in 4 patients (23.5%): they were all classified as NSIP; not deteriorated, 13 patients (76.5%). An improvement in the vital capacity percentage (%VC) was recognized in 10 patients (78.6%) and in diffusing capacity of carbon monoxide (%DLco) in 8 patients (61.5%). Nevertheless, mean %VC and mean %DLco did not change significantly. No major adverse event(s) occurred. The efficacy and safety of our “divided administration” protocol of CYC for CVD-ILD was demonstrated.

Key words

Computed tomography - KL-6 - Pulmonary functioning test