Evidence map›Paper›PMID 30758708›Full record

ArticleLung2019

Pirfenidone Therapy for Familial Pulmonary Fibrosis: A Real-Life Study.

David Bennett, Rosa Metella Refini, Maria Lucia Valentini, Annalisa Fui, Antonella Fossi, Maria Pieroni, Maria Antonietta Mazzei, Paola Rottoli

Abstract readComparative Study
PubMed Publisher
In one paragraph

Article in Lung, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 7 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
7citing papers in PubMed, 1 pooled it
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

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.

2 · The registry

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.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

7 citing papers in PubMed, 1 synthesis or guideline pooled it.

  1. Pooled it
  2. Article
  3. Article
  4. Article
  5. Telomere biology disorders.NPJ genomic medicine · 2021
    Review
  6. Article
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4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

8 authors.

David BennettRespiratory Diseases and Lung Transplantation Unit, Azienda Ospedaliera Universitaria Senese (AOUS) - Department of Medical and Surgical Sciences & Neurosciences, University of Siena, Viale Bracci, 16, 53100, Siena, Italy. david.btt@gmail.com.ORCID 0000-0001-5354-152X
Rosa Metella RefiniRespiratory Diseases and Lung Transplantation Unit, Azienda Ospedaliera Universitaria Senese (AOUS) - Department of Medical and Surgical Sciences & Neurosciences, University of Siena, Viale Bracci, 16, 53100, Siena, Italy.
Maria Lucia ValentiniRespiratory Diseases and Lung Transplantation Unit, Azienda Ospedaliera Universitaria Senese (AOUS) - Department of Medical and Surgical Sciences & Neurosciences, University of Siena, Viale Bracci, 16, 53100, Siena, Italy.
Annalisa FuiRespiratory Diseases and Lung Transplantation Unit, Azienda Ospedaliera Universitaria Senese (AOUS) - Department of Medical and Surgical Sciences & Neurosciences, University of Siena, Viale Bracci, 16, 53100, Siena, Italy.
Antonella FossiRespiratory Diseases and Lung Transplantation Unit, Azienda Ospedaliera Universitaria Senese (AOUS) - Department of Medical and Surgical Sciences & Neurosciences, University of Siena, Viale Bracci, 16, 53100, Siena, Italy.
Maria PieroniRespiratory Diseases and Lung Transplantation Unit, Azienda Ospedaliera Universitaria Senese (AOUS) - Department of Medical and Surgical Sciences & Neurosciences, University of Siena, Viale Bracci, 16, 53100, Siena, Italy.
Maria Antonietta MazzeiDiagnostic Imaging Unit, Azienda Ospedaliera Universitaria Senese (AOUS) - Department of Medical and Surgical Sciences & Neurosciences, University of Siena, Siena, Italy.
Paola RottoliRespiratory Diseases and Lung Transplantation Unit, Azienda Ospedaliera Universitaria Senese (AOUS) - Department of Medical and Surgical Sciences & Neurosciences, University of Siena, Viale Bracci, 16, 53100, Siena, Italy.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

introductionFamilial pulmonary fibrosis (FPF) is defined as an idiopathic diffuse parenchymal lung disease affecting two or more members of the same primary biological family. The aim of this study was to compare disease progression and tolerance to pirfenidone in a population of FPF patients who presented with radiological and/or histological evidence of UIP, and a group of idiopathic pulmonary fibrosis (IPF) patients.

methodsSeventy-three patients (19 with FPF and 54 with IPF) were enrolled and data were collected retrospectively at 6, 12 and 24 months follow-up.

resultsFPF patients were statistically younger and more frequently females. A significantly greater decline in FVC and DLCO was recorded in FPF than in IPF patients at 24 months follow-up. At the 6-min walking test, walked distance declined significantly in FPF patients than IPF at 24 months. No statistically significant differences in drug tolerance or side effects were recorded between groups.

conclusionDifferent rate of progression was observed in patients with IPF and FPF on therapy with pirfenidone; our findings may not be due to lack of effectiveness of therapy, but to the different natural history and evolution of these two conditions. Pirfenidone was well tolerated by FPF and IPF patients. Specific unbiased randomized clinical trials on larger populations to validate our preliminary exploratory results are needed.

Indexed as

AgedDisease ProgressionFemaleHumansIdiopathic Pulmonary FibrosisLungMaleMiddle AgedPyridonesRetrospective StudiesTime FactorsTreatment OutcomepirfenidonePyridonesFamilial pulmonary fibrosisIdiopathic pulmonary fibrosisPirfenidoneTherapy

Identifiers

PMID30758708

What OpenQuestion holds

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Registered trials

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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.