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Prediction of therapeutic response before and during i.v. cyclophosphamide pulse therapy for interstitial lung disease in systemic sclerosis: A longitudinal observational study
Author(s) -
Sumida Hayakazu,
Asano Yoshihide,
Tamaki Zenshiro,
Aozasa Naohiko,
Taniguchi Takashi,
Toyama Tetsuo,
Takahashi Takehiro,
Ichimura Yohei,
Noda Shinji,
Akamata Kaname,
Saigusa Ryosuke,
Miyazaki Miki,
Kuwano Yoshihiro,
Yanaba Koichi,
Yoshizaki Ayumi,
Sato Shinichi
Publication year - 2018
Publication title -
the journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.9
H-Index - 65
eISSN - 1346-8138
pISSN - 0385-2407
DOI - 10.1111/1346-8138.14669
Subject(s) - dlco , medicine , diffusing capacity , interstitial lung disease , gastroenterology , cyclophosphamide , lung , therapeutic effect , lung function , chemotherapy
There have been no established parameters to predict responsiveness to i.v. cyclophosphamide ( IVCY ) pulse therapy in combination with corticosteroids in patients with interstitial lung disease ( ILD ) related to systemic sclerosis ( SS c). This retrospective study was conducted to determine predictive factors for efficacy of IVCY at the time of before and during the treatment. Thirty‐two Japanese SS c patients, ever treated for ILD with IVCY in combination with prednisolone, were analyzed retrospectively. We performed detailed time‐course analyses of parameters derived from blood samples and pulmonary function tests. With the exclusion of eight unclassified patients, 24 patients were classified into 14 good responders ( GR ) or 10 poor responders ( PR ) on the basis of changes in percent predicted diffusing capacity for carbon monoxide ( DL co). Pretreatment percent predicted DL co was significantly reduced in PR compared with GR . In addition, serum parameters such as Krebs von den Lungen‐6 ( KL ‐6), surfactant protein D ( SP ‐D) and C‐reactive protein were significantly higher in PR than in GR . Furthermore, our time‐course analyses revealed a transient increase in serum KL ‐6 levels with a peak at 3 months after the first infusion of cyclophosphamide, which showed no relation to therapeutic efficacy. Moreover, continuously high serum KL ‐6 levels (>2000 U/mL) and rapid decrease in SP ‐D levels (<200 ng/mL) during IVCY were remarkably characteristic of PR and GR , respectively. ILD severity/activity before treatment and variability of serum KL ‐6 and SP ‐D levels during treatment may be useful to predict therapeutic effects of IVCY on SS c‐ILD.