Clinical experience with pirfenidone in five patients with scleroderma-related interstitial lung disease.
نویسندگان
چکیده
Interstitial lung disease is the most common complication and cause of death among patients with scleroderma. Scleroderma-related interstitial lung disease has usually been treated with cyclophosphamide; however, its effect was evaluated to be modest and long-term administration of this drug is associated with adverse effects. Herein, we report our clinical experience of administering pirfenidone, which is an antifibrotic agent, in five patients with scleroderma-related interstitial lung disease. All patients demonstrated an increase in vital capacity.
منابع مشابه
The Assessment of Health-Related Quality of Life in Scleroderma-Interstitial Lung Disease
Introduction: Pulmonary involvement is the most common cause of mortality and disability in patients with systemic sclerosis and it significantly affects the quality of life in these patients. Therefore, early diagnosis and treatment of pulmonary involvement seems necessary in patients with SSc. In this study, we aimed to assess the health-related quality of life (HRQoL) in patients with Sclero...
متن کاملA case report: The efficacy of pirfenidone in a Chinese patient with progressive systemic sclerosis-associated interstitial lung disease
Systemic sclerosis (SSc)-associated interstitial lung disease (SSc-ILD) has become the leading SSc-related cause of death. Although various types of immunosuppressive therapy have been attempted for patients with SSc-ILD, no curative or effective treatment strategies for SSc-ILD have been developed. Therefore, management of patients with SSc-ILD remains a challenge. Here, we report a Chinese, f...
متن کاملAn Open-label, Phase II Study of the Safety and Tolerability of Pirfenidone in Patients with Scleroderma-associated Interstitial Lung Disease: the LOTUSS Trial.
OBJECTIVE Systemic sclerosis-associated interstitial lung disease (SSc-ILD) shares a number of clinical features and pathogenic mechanisms with idiopathic pulmonary fibrosis (IPF). This study was designed to evaluate the tolerability of the IPF treatment pirfenidone in SSc-ILD. The known gastrointestinal, skin, and liver adverse events (AE) of pirfenidone are of importance given the involvement...
متن کاملThe decrease of surfactant protein D in bronchoalveolar lavage fluid in patients with idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia
printing supported by . Visit Chiesi at Stand B2.10 TUESDAY, SEPTEMBER 4TH 2012 P3652 The efficacy of pirfenidone in scleroderma related interstitial lung disease Yukiko Miura1, Yoshiya Tsunoda1, Toru Tanaka1, Hiroyuki Takoi1, Yohei Yatagai1, Shigen Rin1, Akimasa Sekine1, Kenji Hayashihara1, Takefumi Saito1, Akihiko Gemma2, Arata Azuma2. 1Department of Respiratory Medicine, National Hospital Or...
متن کاملClinical Experience with Pirfenidone in Connective Tissue Diseases Related Interstitial Lung Diseases
Objective: Pirfenidone is a new, anti-fibrotic drug used for the treatmentof idiopathic pulmonary fibrosis (IPF). This study was designed to verify the effect of pirfenidone administrated in connective tissue diseases related interstitial lung disease (CTD-ILD). Methods: Twenty two patients diagnosed with CTD-ILD were administrated with a 6-months treatment with or without pirfenidone. At basel...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
- Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG
دوره 31 3 شماره
صفحات -
تاریخ انتشار 2014