ArticleScientific reports2016
Pirfenidone in patients with rapidly progressive interstitial lung disease associated with clinically amyopathic dermatomyositis.
Article in Scientific reports, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to 2 registered trials, which are not on this map. Cited by 63 papers, 10 of them syntheses that pooled it.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Evaluation of the Efficacy of Pirfenidone in Progressive Chronic Hypersensitivity Pneumonitis
Nintedanib Plus Standard of Care Immunosuppression Versus Standard of Care Immunosuppression Alone in Patients With Progressive Fibrotic Myositis Associated - Interstitial Lung Disease: A Randomized, Double-Blind, Exploratory Trial
Who cites it
63 citing papers in PubMed, 10 syntheses or guidelines pooled it, 106 citations in OpenAlex.
- Clinically Amyopathic Dermatomyositis: Epidemiology, Clinical Presentation, Pathophysiology, Treatment, and Prognosis: A Systematic Review.Rheumatology and therapy · 2026Pooled it
- ERS/EULAR clinical practice guidelines for connective tissue disease-associated interstitial lung disease.The European respiratory journal · 2026Guideline
- Non-targeted immunosuppressive and immunomodulatory therapies for idiopathic inflammatory myopathies.The Cochrane database of systematic reviews · 2025Pooled it
- Targeted immunosuppressive and immunomodulatory therapies for idiopathic inflammatory myopathies.The Cochrane database of systematic reviews · 2025Pooled it
- Pooled it
- 2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) Guideline for the Treatment of Interstitial Lung Disease in People with Systemic Autoimmune Rheumatic Diseases.Arthritis care & research · 2024Guideline
- 2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) Guideline for the Treatment of Interstitial Lung Disease in People with Systemic Autoimmune Rheumatic Diseases.Arthritis & rheumatology (Hoboken, N.J.) · 2024Guideline
- Rituximab for the treatment of connective tissue disease-associated interstitial lung disease: A systematic review and meta-analysis.Frontiers in pharmacology · 2022Pooled it
- Systematic review and meta-analysis of prognostic factors for idiopathic inflammatory myopathy-associated interstitial lung disease.BMJ open · 2018Pooled it
- Impact of antifibrotic therapy on disease progression, all-cause mortality, and risk of acute exacerbation in non-IPF fibrosing interstitial lung diseases: evidence from a meta-analysis of randomized controlled trials and prospective controlled studies.Therapeutic advances in respiratory diseasePooled it
- High frequency of interstitial lung disease in Indian patients with adult- onset idiopathic inflammatory myopathy: Insights from a prospective observational cohort.Clinical rheumatology · 2026Observational
- Shaping the landscape of interstitial lung disease in idiopathic inflammatory myopathies: state of the art, evidence gaps and a need for clinical trials.Clinical and experimental rheumatology · 2026Review
- Association of serological markers and interstitial lung disease in patients with suspected myositis.Medicine · 2025Observational
- Treatment Strategies for Acute Exacerbation of Interstitial Lung Disease Associated with Systemic Autoimmune Rheumatic Diseases in Chinese Patients: A Scoping Review.Rheumatology and therapy · 2025Review
- Predictive Biomarkers and Novel Treatments for the Progressive Fibrosing Phenotype in Interstitial Lung Disease Associated with Connective Tissue Disease.Biomedicines · 2025Review
- Korean Guidelines for Diagnosis and Management of Interstitial Lung Diseases: Connective Tissue Disease Associated Interstitial Lung Disease.Tuberculosis and respiratory diseases · 2025Article
- Recognition of Idiopathic Inflammatory Myopathies Underlying Interstitial Lung Diseases.Diagnostics (Basel, Switzerland) · 2025Review
- Different faces of anti-melanoma differentiation-associated gene 5 antibody-positive myositis.Reumatologia · 2025Article
- Idiopathic Inflammatory Myopathies-Associated Interstitial Lung Disease in Adults.Tuberculosis and respiratory diseases · 2025Article
- Acute exacerbation of anti-Ha antibody-positive antisynthetase syndrome-associated interstitial lung disease: a case report.Frontiers in immunology · 2025Article
3 more citing papers are in PubMed but not listed here.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
9 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
To evaluate the efficacy of pirfenidone in patients with rapidly progressive interstitial lung disease (RPILD) related to clinically amyopathic dermatomyositis (CADM), we conducted an open-label, prospective study with matched retrospective controls. Thirty patients diagnosed with CADM-RPILD with a disease duration <6 months at Renji Hospital South Campus from June 2014 to November 2015 were prospectively enrolled and treated with pirfenidone at a target dose of 1800 mg/d in addition to conventional treatment, such as a glucocorticoid and/or other immunosuppressants. Matched patients without pirfenidone treatment (n = 27) were retrospectively selected as controls between October 2012 and September 2015. We found that the pirfenidone add-on group displayed a trend of lower mortality compared with the control group (36.7% vs 51.9%, p = 0.2226). Furthermore, the subgroup analysis indicated that the pirfenidone add-on had no impact on the survival of acute ILD patients (disease duration <3 months) (50% vs 50%, p = 0.3862); while for subacute ILD patients (disease duration 3-6 months), the pirfenidone add-on (n = 10) had a significantly higher survival rate compared with the control subgroup (n = 9) (90% vs 44.4%, p = 0.0450). Our data indicated that the pirfenidone add-on may improve the prognosis of patients with subacute ILD related to CADM.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.