Results 81 to 90 of about 1,177,456 (242)

Idiopathic pulmonary fibrosis

open access: yesOrphanet Journal of Rare Diseases, 2008
Idiopathic pulmonary fibrosis (IPF) is a non-neoplastic pulmonary disease that is characterized by the formation of scar tissue within the lungs in the absence of any known provocation.
Noble Paul W, Meltzer Eric B
doaj   +1 more source

From Interferon Signature to the Clinical Landscape: Type I Interferonopathies

open access: yesArthritis &Rheumatology, EarlyView.
Objective TypeI interferonopathies are heterogeneous diseases driven by dysregulated type I interferon (IFN‐I) signaling. Diagnosis is challenging due to clinical/molecular variability and the need for IFN‐I quantification. The aim of this study was to characterize the clinical, immunologic, genetic, molecular profiles of patients with suspected ...
Ismail Yaz   +13 more
wiley   +1 more source

Modelling Forced Vital Capacity in Idiopathic Pulmonary Fibrosis: Optimising Trial Design [PDF]

open access: yes, 2019
INTRODUCTION: Forced vital capacity is the only registrational endpoint in idiopathic pulmonary fibrosis clinical trials. As most new treatments will be administered on top of standard of care, estimating treatment response will become more challenging ...
Meyvisch, Paul   +48 more
core   +1 more source

Modified blood cell GAP model as a prognostic biomarker in idiopathic pulmonary fibrosis

open access: yesERJ Open Research
Background The Gender, Age and Physiology (GAP) model is a simple mortality prediction tool in patients with idiopathic pulmonary fibrosis that uses demographic and physiological variables available at initial evaluation.
Michael Kreuter   +9 more
doaj   +1 more source

Outcomes in Progressive Pulmonary Fibrosis in Systemic Autoimmune Rheumatic Diseases: Real‐World Data From the ILD‐PRO Registry

open access: yesArthritis &Rheumatology, EarlyView.
Objective We evaluated baseline characteristics, treatment patterns, and outcomes in patients with systemic autoimmune rheumatic disease–associated progressive pulmonary fibrosis (SARD‐PPF) and evaluated whether outcomes differed by SARD subtype. Methods The ILD‐PRO Registry is a prospective multicenter US registry of patients with PPF.
Sonali J. Bracken   +7 more
wiley   +1 more source

Exacerbation of Idiopathic Pulmonary Fibrosis

open access: yes, 2006
Samoistne włóknienie płuc (IPF, idiopathic pulmonary fibrosis) jest najczęściej obserwowaną manifestacją kliniczną spośród samoistnych śródmiąższowych zapaleń płuc (IIP, interstitial idiopathic pneumonias) [...
Dariusz Ziora
core   +1 more source

Familial interstitial pulmonary fibrosis in two different families in India: A case series

open access: yesLung India, 2017
Introduction: Idiopathic pulmonary fibrosis (IPF), a chronic progressive interstitial lung disease (ILD), Occasionally, IPF occurs in families. Familial interstitial lung disease has been reported worldwide, limited information is available on the ...
Dabhi Pradipkumar   +5 more
doaj   +1 more source

Cancer in Systemic Sclerosis: Clinical Associations and Prognostic Impact From the EUSTAR Registry

open access: yesArthritis &Rheumatology, EarlyView.
Objective Cancer represents a major cause of death in systemic sclerosis (SSc). Established risk factors are limited to specific subsets, particularly early diffuse anti‐RNA polymerase III (POLR3)–positive disease, needing further exploration. Methods We performed a nested case‐control study within the European Scleroderma Trials and Research group ...
Antonio Tonutti   +52 more
wiley   +1 more source

[Anti-fibrotics as novel therapy for idiopathic pulmonary fibrosis].

open access: yes, 2016
Recent studies have shown efficacy to slow the decrease of forced vital capacity in patients with idiopathic pulmonary fibrosis. This summary refers to recent anti-fibrotic medications and describes current studies, indication for treatment and side ...
Funke, Manuela, Geiser, Thomas
core   +1 more source

Cellular Senescence Dysregulates Antiviral Interferon Responses in Idiopathic Pulmonary Fibrosis. [PDF]

open access: yesAging Cell
Senescent idiopathic pulmonary fibroblasts (IPF) lung fibroblasts are transcriptomically uncoupled from a functional response to virus relative to healthy (HLF) lung fibroblasts. HLF lung fibroblasts require viral effectors STAT1 or IRF3 to enact a viral response while IPF lung fibroblasts do not rely on these axes in response to virus.
Hughes JB   +12 more
europepmc   +2 more sources

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