The unmet care needs of progressive fibrosing interstitial lung disease

Teague²,

Nicholson³

et al. 2022

Front. Med.

Despite conventional treatment, a proportion of interstitial lung disease (ILD) patients develop a progressive phenotype known as “fibrosing ILD with a progressive phenotype” (PF-ILD), characterized by worsening respiratory symptoms, decline in lung function, and early mortality. This review describes the epidemiology, and the humanistic and economic burden of PF-ILDs other than idiopathic pulmonary fibrosis (non-IPF PF-ILD). A structured review of the literature was conducted, using predefined search strategies in Ovid MEDLINE and EMBASE, and supplemented with gray literature searches. The search identified 3,002 unique articles and an additional 3 sources were included from the gray literature; 21 publications were included. The estimated prevalence of non-IPF PF-ILD ranges from 6.9 to 70.3/100,000 persons and the estimated incidence from 2.1 to 32.6/100,000 person-years. Limited evidence demonstrates that PF-ILD has a significant impact on patients' quality of life, affecting their daily lives, psychological well-being, careers, and relationships. PF-ILD is also associated with significant economic burden, demonstrating higher healthcare resource use and direct costs compared with the non-progressive phenotype, and indirect costs, which include job losses. This review indicates that PF-ILD places a considerable humanistic burden on both patients and caregivers, and a substantial economic burden on healthcare systems, patients, and society.

Section: Discussionmentioning

confidence: 99%

The Burden of Progressive-Fibrosing Interstitial Lung Diseases

Teague²,

Nicholson³

et al. 2022

Front. Med.

“…Relative prevalence of progressive fibrosing interstitial lung diseases by cause according to the reports of the different published cohorts. Source: Faverio et al [26] in 2020, Sweeney et al [27] in 2020, Chen et al [28] in 2021, Komatsu et al [29] in 2021, Kwon et al [30] in 2021, Nasser et al [25 ▪ ] in 2021, Simpson et al [31] in 2021, Gagliardi et al [32] in 2021, Hambly et al [33 ▪ ] in 2022 and Takei et al [34] in 2022.…”

Section: Real-life Cohort Studiesmentioning

confidence: 99%

“…Sweeney et al [27] explored the characteristics, healthcare implications and cost of PF-ILD in an Australian cohort of 118 patients with non-IPF-fibrosing ILDs, out of them 56 (47.5%) met the INBUILD trial criteria of progression.…”

Section: Real-life Cohort Studiesmentioning

confidence: 99%

Epidemiology and real-life experience in progressive pulmonary fibrosis

Valenzuela

Current Opinion in Pulmonary Medicine

2022

Purpose of reviewIdiopathic pulmonary fibrosis (IPF), characterized by relentless disease progression from the time of diagnosis, is part of a larger group of chronic fibrosing interstitial lung diseases (ILDs). A proportion of patients with non-IPF ILDs may develop, despite conventional treatment, a progressive pulmonary fibrosis (PPF), also referred to as ILD with a progressive fibrosing phenotype (PF-ILD). These patients experience worsening of respiratory symptoms, decline in lung function, and early mortality. The goal of this review is to describe the epidemiology and recent real-life cohorts of PF-ILD, with implications for management.Recent findingsThe relatively new concept of PF-ILD has aroused active clinical research over the past years. To understand risk factors for progression and the real burden of the disease is crucial to improve management. In the last 2 years, different cohort studies have addressed these questions. They showed that almost one-third of the non-IPF fibrotic ILD patients develop PF-ILD or PPF.SummaryEmerging data show similarities in prognosis between patients with IPF or with non-IPF PF-ILD patients. Early detection and appropriate treatment of this group of patients is a priority. Further research is needed to identify risk factors of progression, to clarify the assessment of progression in clinical practice, for a better management of patients with PF-ILD in a real-world setting.

“…This speculation is amply supported by clinical studies of individual disorders, although it should be stressed that these were expert center patient cohorts [23,24]. In summary, the PFP can be anticipated to manifest in 25–30% of patients with fibrotic ILDs other than IPF, excluding patients with pulmonary sarcoidosis, with baseline risk modified by baseline CT, biopsy and disease severity data, a conclusion broadly supported by other estimates [23–26]. The exact definition of progression remains to be defined but in general, progression has been viewed as a variable composite of lung function decline, symptomatic change and increasing disease extent on imaging.…”

Section: Prevalence Of the Progressive Fibrotic Phenotypementioning

confidence: 90%

The progressive fibrotic phenotype in current clinical practice

Wells

Brown

Current Opinion in Pulmonary Medicine

2021

Purpose of reviewThe progressive fibrotic phenotype (PFP), a term that covers large sub-groups of patients with fibrotic lung diseases that clinically progress despite appropriate usual management, is now an everyday problem for patients and clinicians alike. This review covers recent data that are relevant to major clinical uncertainties. Recent findingsThe clinical relevance of the PFP is covered by a brief review of data from which this entity was constructed. Estimates of the prevalence of the PFP are cited. The importance of an accurate initial diagnosis is emphasized -with refutation of the belief that diagnosis now matters less because of recent antifibrotic trial data. Pivotal trials are reviewed briefly with emphasis on the range of diseases studied and the efficacy signals. Included in this section are analyses of treatment effects in individual diseases and data that validate the progression criteria that define the PFP.