Efficacy and safety of pirfenidone in systemic sclerosis-related interstitial lung disease—a randomised controlled trial

Acharya, Nupoor; Sharma, Shefali; Mishra, Debashish; Dhooria, Sahajal; Dhir, Varun; Jain, Sanjay

doi:10.1007/s00296-020-04565-w

Cited by 78 publications

(70 citation statements)

References 25 publications

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“…Nine RCTs (SLS-I, 2006 [ 8 ]; SLS-II, 2016 [ 7 ]; Domiciano, D.S., 2011 [ 23 ]; Hoyles, R.K., 2006 [ 24 ]; Naidu, G.S.R.S.N.K., 2020 [ 25 ]; SENSCIS, 2019 [ 10 ]; Sircar, G., 2018 [ 26 ]; Acharya, N., 2019 [ 27 ]; Hsu, V.M., 2018 [ 28 ]) were considered eligible and were included in the final quantitative analysis (see Supplementary material 1.1, page 5 ). These studies were conducted in a total of 926 participants, comparing 8 interventions (cyclophosphamide, mycophenolate, cyclophosphamide plus high dose prednisone (CYCPRED), cyclophosphamide followed by azathioprine (CYCAZA), rituximab, pirfenidone, nintedanib, and pomalidomide), and placebo for an average follow-up of 1 year.…”

Section: Resultsmentioning

confidence: 99%

“…SSc-ILD was diagnosed using HRCT criteria in 5 RCTs (SLS-II, 2016 [ 7 ]; Naidu G.S.R.S.N.K., 2020 [ 25 ]; SENSCIS, 2019 [ 10 ]; Acharya, N., 2019 [ 27 ]; Hsu, V.M., 2018 [ 28 ]), HRCT plus biopsy, PFTs or biopsy in 3 RCTs (SLS-I, 2006 [ 8 ]; Hoyles, R.K., 2006 [ 24 ]; Sircar, G., 2018 [ 26 ]), and biopsy only in the remaining RCT (Domiciano, D.S., 2011 [ 23 ]). Domiciano, D.S., 2011 [ 23 ] enrolled only SSc-ILD patients with biopsy-proven nonspecific interstitial pneumonia (NSIP), while Naidu, G.S.R.S.N.K., 2020 [ 25 ], Sircar, G., 2018 [ 26 ], and Acharya, N., 2019 [ 27 ] enrolled patients with both NSIP and usual interstitial pneumonia (UIP). SSc-ILD pattern was not specified in the remaining RCTs (see Supplementary material 1.2, page 6 ).…”

Section: Resultsmentioning

confidence: 99%

“…Four trials were scored with a low RoB (Tashkin, D.P., 2006 [ 8 ]; Tashkin, D.P., 2016 [ 7 ]; SENSCIS, 2019 [ 10 ]; Acharya, N., 2019 [ 27 ]), whereas the remaining five trials were scored with a high RoB (Domiciano, D.S., 2011 [ 23 ]; Hoyles, R.K., 2006 [ 24 ]; Naidu, G.S.RS.N.K., 2020 [ 25 ]; Sircar, G., 2018 [ 26 ]; Hsu, V.M., 2018 [ 28 ]). No trial was scored with an intermediate RoB (see Supplementary Material 3.1–3.3, pages 30–31 ).…”

Section: Resultsmentioning

confidence: 99%

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Efficacy, Safety, and Tolerability of Treatments for Systemic Sclerosis-Related Interstitial Lung Disease: A Systematic Review and Network Meta-Analysis

Erre

Sebastiani

Fenu

et al. 2020

JCM

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Background: There is a paucity of head-to-head comparisons of the efficacy and harms of pharmacological treatments for systemic sclerosis-related interstitial lung disease (SSc-ILD). Methods: We conducted a network meta-analysis (NMA) in order to compare the effects of different treatments with the placebo on change in forced vital capacity (FVC), change in diffusion lung capacity for CO (DLCO), serious adverse events (SAEs), discontinuation for adverse events and mortality in SSc-ILD. Standardized mean difference (SMD) and log odds ratio were estimated using NMA with fixed effects. Results: Nine randomized clinical trials (926 participants) comparing eight interventions and the placebo for an average follow-up of one year were included. Compared to the placebo, only rituximab significantly reduced FVC decline (SMD (95% CI) = 1.00 (0.39 to 1.61)). Suitable data on FVC outcome for nintedanib were not available for the analysis. No treatments influenced DLCO. Safety and mortality were also not different across treatments and the placebo, although there were few reported events. Cyclophosphamide and pomalidomide were less tolerated than the placebo, mycophenolate, and nintedanib. Conclusion: Only rituximab significantly reduced lung function decline compared to the placebo. However, direct head-to-head comparison studies are required to confirm these findings and to better determine the safety profile of various treatments.

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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

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Efficacy, Safety, and Tolerability of Treatments for Systemic Sclerosis-Related Interstitial Lung Disease: A Systematic Review and Network Meta-Analysis

Erre

Sebastiani

Fenu

et al. 2020

JCM

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“…Nintedanib significantly reduces the decline in forced vital capacity (FVC) and mortality rate in patients with IPF [57,58]. Pirfenidone therapy for SSc-ILD has failed to show any beneficial effect on FVC and other clinical parameters (dyspnea score, 6-minute walking distance, and modified Rodnan skin score) [59]. By contrast, nintedanib use in SSc-ILD showed better prognosis than placebo in terms of decline in FVC [60].…”

Section: A C C E P T E D a R T I C L Ementioning

confidence: 99%

Recent advances in the diagnosis and management of interstitial pneumonia with autoimmune features: the perspective of rheumatologists

Min

Kim

Lee

et al. 2021

Korean J Intern Med

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Interstitial pneumonia with autoimmune features (IPAF) is a recently established disease entity that is comprised of interstitial lung diseases with evidence of autoimmune features but does not fulfill the criteria for definite autoimmune rheumatic disease. The classification criteria for IPAF were defined by the European Respiratory Society and American Thoracic Society in 2015. However, for these classification criteria, further studies to establish IPAF subgroups and treatment modalities for each subgroup are still needed. In this review, we discuss the recent advances in the field of IPAF and raise critical points for the diagnosis and management of patients with IPAF from the perspective of rheumatologists.

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“…An open-label phase II study of pirfenidone (69) showed an acceptable tolerability profile, especially with a longer titration schedule, despite its well-recognized gastrointestinal side effect profile. A recently published randomized placebo-controlled trial of pirfenidone treatment for 6 months in patients with SSc-ILD and FVC% between 50% and 80% failed to demonstrate a significant benefit over placebo in improving/stabilizing FVC, exercise capacity, symptoms, or skin disease (70), but the study was underpowered to provide conclusive evidence. Active recruitment of study participants in a larger randomized controlled trial-SLS III (ClinicalTrials.gov identifier: NCT03221257)-is currently underway.…”

Section: Cyclophosphamide Scleroderma Lung Study I (Sls I)mentioning

confidence: 99%

Expert Perspectives On Clinical Challenges: Expert Perspectives: Challenges in Scleroderma

Simms

2020

Arthritis & Rheumatology

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You are consulted to evaluate a 56‐year‐old woman with known Raynaud's phenomenon, finger swelling of several; months’ duration, and new hypertension with a blood pressure of 160/100 mm/Hg. She also reports progressive shortness of breath. Physical examination reveals telangiectasias, sclerodactyly, and proximal skin sclerosis (thick shiny skin on the chest and upper arms), and bibasilar crackles are found on chest examination. Laboratory tests reveal evidence of microangiopathic hemolytic anemia, thrombocytopenia, and elevation of the serum creatinine level (previously normal), and chest computed tomography shows evidence of ground‐glass opacification in both lower lung fields.

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Efficacy and safety of pirfenidone in systemic sclerosis-related interstitial lung disease—a randomised controlled trial

Cited by 78 publications

References 25 publications

Efficacy, Safety, and Tolerability of Treatments for Systemic Sclerosis-Related Interstitial Lung Disease: A Systematic Review and Network Meta-Analysis

Efficacy, Safety, and Tolerability of Treatments for Systemic Sclerosis-Related Interstitial Lung Disease: A Systematic Review and Network Meta-Analysis

Recent advances in the diagnosis and management of interstitial pneumonia with autoimmune features: the perspective of rheumatologists

Expert Perspectives On Clinical Challenges: Expert Perspectives: Challenges in Scleroderma

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