Corticosteroids for idiopathic pulmonary fibrosis

Richeldi, Luca; Davies, Huw Richard H R; Spagnolo, Paolo; Luppi, Fabrizio

doi:10.1002/14651858.cd002880

Cited by 179 publications

(147 citation statements)

References 21 publications

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“…In Cochrane analysis evaluating the efficiency of corticosteroids in IPF and published in 2003, no high-quality study was found and there was only one non-randomized retrospective study. This review demonstrated that there was no sufficient evidence for supporting the use of corticosteroids in IPF (17). Furthermore, in large-scale retrospective studies, any advantage for survival could not be shown in IPF patients using corticosteroids (18)(19)(20).…”

Section: Discussionmentioning

confidence: 88%

Approaches in Diagnosis and Treatment of Patients with Idiopathic Pulmonary Fibrosis: A Questionnaire Study

Anar¹,

Okutan²,

Uzun³

et al. 2015

Eurasian J Pulmonol

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Objective: As Turkish Respiratory Society Diffuse Parenchymal Lung Diseases-Pulmonary Vascular Diseases Study Group (TRS DPLD-PVD SG), we aimed to demonstrate the approaches of physicians with a questionnaire toward the patients with IPF in our country. Methods:An invitation letter including a questionnaire with 24 questions to assess the approaches they prefer in the patients with IPF and the capabilities of the departments they work at and a link for the questionnaire was directed to the mail groups of chest diseases and thoracic surgery specialists. Responses of the physicians who participated in the questionnaire were reviewed.Results: Thirty percent of the participants saw less than 5 patients with IPF in a year and 16.8% of them saw more than 21. 66.3% of participants stated that anamnesis, symptoms and clinical findings along with typical radiologic findings would be sufficient for the diagnosis, while 27.7% suggested that pathological findings should indicate IPF along with clinic and radiologic ones.When the methods used for tissue sampling were viewed; application rates of bronchoscopic transbronchial biopsy, thoracoscopic biopsy and open lung biopsy were found very close to one another.In the patients with the suspected disease of IPF, the fields in which the hesitation was the most problematic were respectively pathological examination of biopsies (73.2%) and interpretation of radiologic findings (49.5%). 37.6% of the patients with diagnosis of IPF were directed to experienced centers; medical treatment and drugless follow-up rates were respectively 33.6% and 14.8%. Among the participants, 29.7% suggested lung transplantation for all cases while 48.5% only preferred this operation in particular ones. Conclusion:Some of the results of our study is compatible with the guidelines of IPF, is to show that the quality of debate and confusion still continued in this regard.

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Section: Discussionmentioning

confidence: 88%

Approaches in Diagnosis and Treatment of Patients with Idiopathic Pulmonary Fibrosis: A Questionnaire Study

Anar¹,

Okutan²,

Uzun³

et al. 2015

Eurasian J Pulmonol

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“…In past years, patients with IPF were frequently treated with corticosteroids despite a lack of evidence for efficacy or with azathioprine despite weak evidence for efficacy; current treatment guidelines do not recommend these treatments for most patients with IPF [1,7,15]. These differences in concomitant medication use could have affected the incidence of observed TEAEs in PIPF-002 compared with those in ASCEND and CAPACITY.…”

Section: Discussionmentioning

confidence: 99%

An Open-Label, Phase II Study of the Safety of Pirfenidone in Patients with Idiopathic Pulmonary Fibrosis (PIPF-002)

et al. 2018

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Introduction PIPF-002 was a phase 2, multicenter, open-label study of pirfenidone in patients with idiopathic pulmonary fibrosis (IPF) or other types of pulmonary fibrosis (PF). PIPF-002 terminated after pirfenidone became commercially available in the United States. The goal of PIPF-002 was to characterize the long-term safety of pirfenidone in these patients. Methods Between August 2003 and September 2006, 83 patients (IPF: 81, PF: 2) enrolled. Patients received pirfenidone in three divided doses daily, with the maintenance dose and schedule determined by enrollment group assignment. Treatment continued until patient withdrawal or study termination (2015). Treatment-emergent adverse events (TEAEs) were assessed by descriptive statistics. Results At baseline, median age was 70 years, mean percent predicted forced vital capacity was 67.7%, 33.7% of patients had cardiac disorders, 51.8% had gastroesophageal reflux disease, and 63.9% were receiving concomitant prednisone. Median pirfenidone dose and exposure duration were 2400 mg/day and 3.0 years, respectively. Cumulative total exposure was 279.7 patient-exposure years (PEY). Most patients (98.8%) reported ≥ 1 TEAE, with an overall incidence rate of 460.5 per 100 PEY. The most frequent TEAEs (incidence rate per 100 PEY) were nausea (23.6), IPF progression (16.1), fatigue (11.8), dyspnea (11.4), upper respiratory tract infection (11.4), and cough (10.7). Serious TEAEs were reported in 49 patients; the most frequent serious TEAEs were IPF progression and pneumonia. The most common reason for discontinuation was TEAEs (35 patients; 12.5 patients per 100 PEY), most frequently IPF progression and nausea. Overall, 21 patients died (7.5 per 100 PEY); 16 deaths were IPF-related. Conclusions Long-term safety and tolerability of pirfenidone findings in this study were consistent with the known safety profile of pirfenidone; no new safety signals were identified. These data support the continued use of pirfenidone in patients with IPF. Funding F. Hoffmann-La Roche Ltd./Genentech, Inc. Trial Registration ClinicalTrials.gov identifier, NCT00080223. Plain Language Summary Plain language summary available for this article.

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“…Recently, Richeldi et al [2003] carried out a systematic review of the efficacy of corticosteroids in the treatment of adults with IPF: no randomized controlled trial or clinical control trials were available where corticosteroid treatment was compared to placebo. The analysis did not support the efficacy of corticosteroid in the treatment of IPF.…”

Section: Treatment "Classics" Treatment Of Pulmonary Fibrosismentioning

confidence: 99%

“…In addition, the study showed that azathioprine was well tolerated and that the adverse effects observed in the study were most frequently related to prednisone use. In a recent systematic review of the use of immunosuppressive treatment in IPF [Davies et al 2003], little benefit was shown from the addition of azathioprine to oral corticosteroid therapy in the treatment of IPF. Although azathioprine does appear to be well tolerated, it appears to give minor long term survival advantage.…”

Section: Treatment "Classics" Treatment Of Pulmonary Fibrosismentioning

confidence: 99%

“…The sporadic use, or small studies with the other cytotoxic chemotherapeutic agents methotrexate [Lynch and McCune, 1997;Douglas et al 2000], chlorambucil [Brown and Turner-Warwick, 1971] and vincristine [Meuret et al 1978] have been reported with no evidence to support the use of any of these agents [Davies et al 2003]. …”

Section: Treatment "Classics" Treatment Of Pulmonary Fibrosismentioning

confidence: 99%

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Review: New perspectives in the treatment of idiopathic pulmonary fibrosis

Rogliani

Mura

Porretta

et al. 2008

Ther Adv Respir Dis

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Idiopathic pulmonary fibrosis (IPF) is the most frequent idiopathic interstitial pneumonia with a prevalence ranging from 5 to 15 per 100,000 persons, and above 175 per 100,000 in the older population. IPF is a relentlessly progressive fibrotic lung disorder leading to death within a median duration of 3 years. It was hypothesized in the 1970s that pulmonary fibrosis initiates as an "alveolitis" progressing to interstitial fibrosis with connective tissue deposition, derangement of the lung architecture and functional impairment. However, in vitro studies indicated that alveolar/bronchiolar injured epithelial cells can drive the fibrotic process in the absence of macrophages and with minimal inflammation. This, together with the inability of classic immunosuppressive therapy to cure IPF, generated new pathogenesis paradigms and intense research into the role of the lack or the excessive production of anti-fibrotic or profibrotic mediators, oxidant injury, exaggerated coagulation, thus leading to investigate new treatment strategies. Preliminary results of some of such trials have shown significant reductions in lung function decline, disease exacerbation and mortality.

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Corticosteroids for idiopathic pulmonary fibrosis

Cited by 179 publications

References 21 publications

Approaches in Diagnosis and Treatment of Patients with Idiopathic Pulmonary Fibrosis: A Questionnaire Study

Approaches in Diagnosis and Treatment of Patients with Idiopathic Pulmonary Fibrosis: A Questionnaire Study

An Open-Label, Phase II Study of the Safety of Pirfenidone in Patients with Idiopathic Pulmonary Fibrosis (PIPF-002)

Review: New perspectives in the treatment of idiopathic pulmonary fibrosis

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