Efficacy of Pirfenidone in Patients With the Advanced Stage of Idiopathic Pulmonary Fibrosis

Sakamoto, Susumu; Ishida, Fumiaki; Satoh, Keita; Sakaguchi, Shingi; Sugino, Keishi; Isobe, Kazutoshi; Homma, Sakae

doi:10.1378/chest.1114180

Cited by 8 publications

(8 citation statements)

References 7 publications

(12 reference statements)

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“…In addition, some reports [32] now suggest that a combination of pirfenidone with N-acetyl cysteine decreased the rate of decline in FVC even in severe IPF, although this was in a small group of patients (n=18). Pirfenidone treatment results in improvement in the lung functions.…”

mentioning

confidence: 93%

Idiopathic Pulmonary Fibrosis: A Study of 46 Patients from Western India: Clinical Presentations and Survival

Natarajan¹,

Subramanian²

2015

Turk Thorac J

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OBJECTIVES:Idiopathic pulmonary fibrosis (IPF) is the most common form of interstitial lung disease (ILD) diagnosed all over the world as well as in India. The objective was to study the clinical presentations, treatment options, and survival of patients diagnosed with IPF. MATERIALS AND METHODS:This was a 6-year retrospective observational study. All patients diagnosed with ILD underwent a comprehensive evaluation to confirm the diagnosis of IPF and were subsequently included in the study. Clinical data and laboratory data were recorded. Patients were treated as per current guidelines. RESULTS:Out of the total number of patients (n=123) diagnosed with ILD, 46 (37.4%) patients were included in the study [Males, n=19 (41%) and females, n=27 (59%)]. Sixty-seven percent (n=31) of the patients were in the age group of 61-80 years. The mean age of males and females was 68.36 and 66.25 years, respectively. Clubbing was observed in 67% (n=29) of the patients. The mean forced vital capacity (FVC) was 52%. High-resolution computed tomography (HRCT) of the chest showed a definite usual interstitial pneumonia (UIP) pattern of honeycombing in 60% (n=28) of the patients. Desaturation on a 6 min walk distance was noted in 60% (n=28) of the patients. Ten patients wrongly received-anti tuberculosis treatment (21%) before they were diagnosed with IPF. The median delay in diagnosis was 20 months. The 5-year survival rate was 25%; the average survival was 39 months. Patients receiving pirfenidone showed a survival benefit compared with those having received triple therapy. Survival in IPF was significantly low compared with that in other ILDs. Twenty-four (52%) patients died during the study. CONCLUSION:IPF is a disease which affects the fourth to seventh decade of the Indian population with low survival. KEYWORDS: Idiopathic pulmonary fibrosis, survival, pirfenidone INTRODUCTIONInterstitial lung diseases (ILD) are a group of disorders characterized by varying degrees of fibrosis and inflammation of the lung parenchyma or interstitium [1]. Combining clinical, radiological, and pathological information is pivotal to accurate diagnosis in interstitial lung diseases. Idiopathic pulmonary fibrosis (IPF) is the most common form of ILD diagnosed worldwide as well as in India. IPF portends a poor prognosis as well as increased morbidity and poor quality of life. In India where lung transplantation is in a nascent stage, patients presenting with IPF have a poor prognosis. We present the analysis of our data from the western Indian population.The objective was to study the clinical presentations, treatment options, and survival of patients diagnosed with IPF. MATERIAL AND METHODS Design and setting:This was a retrospective observational study of patients presented to our specialty respiratory clinics in the period from January 2008 to March 2014. Data analysis was performed subsequently.Eligibility: All patients presented to the outpatients department with a history and examination suggesting interstitial lung disease were confirmed with...

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confidence: 93%

Idiopathic Pulmonary Fibrosis: A Study of 46 Patients from Western India: Clinical Presentations and Survival

Natarajan¹,

Subramanian²

2015

Turk Thorac J

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“…There have been a number of small real-world cohort studies suggesting that those with more severe disease may actually have greater benefit from pirfenidone with a similar rate of adverse events. [47][48][49]56 A controlled study using nintedanib in severe IPF is currently recruiting patients globally (NCT02802345). Further data are required before any suggested management recommendations can be made with regard to anti-fibrotic agents in severe IPF.…”

Section: Treatment Of Ipf Patients With Varying Disease Severitymentioning

confidence: 99%

Treatment of idiopathic pulmonary fibrosis in Australia and New Zealand: A position statement from the Thoracic Society of Australia and New Zealand and the Lung Foundation Australia

Troy

Keir

et al. 2017

Respirology

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Idiopathic pulmonary fibrosis (IPF) is a fibrosing interstitial lung disease (ILD) of unknown aetiology with a median survival of only 2-5 years. It is characterized by progressive dyspnoea and worsening lung function, ultimately resulting in death. Until recently, there were no effective therapies for IPF; however, with the publication of two landmark clinical trials in 2014, the anti-fibrotic therapies, nintedanib and pirfenidone, have gained widespread approval. This position paper aims to highlight the current evidence for the treatment of IPF, with particular application to the Australian and New Zealand population. We also consider areas in which evidence is currently lacking, especially with regard to the broader IPF severity spectrum and treatment of co-morbid conditions. The utility of non-pharmacological therapies including pulmonary rehabilitation, oxygen as well as symptom management thought to be important in the holistic care of IPF patients are also discussed.

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“…The timing of initiation of therapy remains controversial 54 . It appears that their benefit is seen across a broad spectrum of patients with mild to moderate spirometric severity and, in the case of nintedanib, across various HRCT patterns [55][56][57] . As such, discussion with the patient regarding the potential benefit of early intervention seems appropriate.…”

Section: Annual Decline Of Fcv With a Cut-off Value Of 10%mentioning

confidence: 99%

Idiopathic Pulmonary Fibrosis: What Is the Best Treatment?

Wu¹,

Kaner²,

Martinez³

2017

BRN

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Efficacy of Pirfenidone in Patients With the Advanced Stage of Idiopathic Pulmonary Fibrosis

Cited by 8 publications

References 7 publications

Idiopathic Pulmonary Fibrosis: A Study of 46 Patients from Western India: Clinical Presentations and Survival

Idiopathic Pulmonary Fibrosis: A Study of 46 Patients from Western India: Clinical Presentations and Survival

Treatment of idiopathic pulmonary fibrosis in Australia and New Zealand: A position statement from the Thoracic Society of Australia and New Zealand and the Lung Foundation Australia

Idiopathic Pulmonary Fibrosis: What Is the Best Treatment?

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