Current monitoring and treatment of progressive fibrosing interstitial lung disease: a survey of physicians in Japan, the United States, and the European Union

Takizawa, Ayako; Kamita, Mitsutoyo; Kondoh, Yasuhiro; Bando, Masashi; Kuwana, Masataka; Inoue, Yoshikazu

doi:10.1080/03007995.2020.1860920

Cited by 8 publications

(5 citation statements)

References 56 publications

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“…The variation in these epidemiological estimates is a result of factors including the variation in study designs, heterogenous referral bias between centers, definitions of progression in fibrosing ILDs applied and populations (studies were conducted across several distinct geographies and varied in underlying ILD subgroup proportions). This highlights, that despite increasing recognition of the PF phenotype among ILD specialists, there are difficulties in identifying patients with PF-ILDs other than IPF in practice due to an absence of standardized diagnostic criteria, treatment guidelines, and specialist education in the field (35).…”

Section: Discussionmentioning

confidence: 99%

The Burden of Progressive-Fibrosing Interstitial Lung Diseases

Cottin

Teague²,

Nicholson³

et al. 2022

Front. Med.

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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.

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Section: Discussionmentioning

confidence: 99%

The Burden of Progressive-Fibrosing Interstitial Lung Diseases

Cottin

Teague²,

Nicholson³

et al. 2022

Front. Med.

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“…Gleiches gilt für die RELIEF-Studie zu PF-ILD, hier erhielten 81 % der Patienten Glukokortikoide und/oder Immunmodulatoren in Kombination [13]. In einer jüngsten internationalen Online-Befragungen gaben Experten an, dass sie 25-70 % [105] ihrer PF-ILD-Patienten nicht antiinflammatorisch behandeln würden, v. a. traf dies auf Patienten mit idiopathischer NSIP und unklassifizierbarer ILD zu [106].…”

Section: Sollen Ipf-patienten Mit Dem Pde5-hemmer Sildenafil Behandel...unclassified

Pharmakotherapie der idiopathischen Lungenfibrose (ein Update) und anderer progredienter pulmonaler Fibrosen

et al. 2023

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“…Pulmonary function and HRCT have largely been used in clinical trials for monitoring progression in F-ILDs [ 2 , 37 , 67 – 69 ]. Sequential assessments and other appropriate investigations, when necessary, are recommended for a) detecting progression of disease and b) assessing complications and comorbidities.…”

Section: Section 2: Prognostication and Monitoringmentioning

confidence: 99%

“…PFTs (at least FVC and D LCO ) are suggested in the first year at a frequency of at least every 3–4 months [ 69 , 70 ]. The course of lung function decline in patients with IPF is progressive, but variable [ 38 ].…”

Section: Section 2: Prognostication and Monitoringmentioning

confidence: 99%

“…Repeat HRCT scans should be directed by clinical context and lung function decline, and their frequency can vary from patient to patient. In the majority of patients, repeat imaging can be done annually (and even less frequently if the patient is clinically stable or improving), although certain situations may require more frequent HRCTs [ 69 , 75 ].…”

Section: Section 2: Prognostication and Monitoringmentioning

confidence: 99%

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Progressive pulmonary fibrosis: an expert group consensus statement

Rajan

Cottin

Dhar³

et al. 2022

Eur Respir J

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This expert group consensus statement emphasises the need for standardising the definition of progressive fibrosing interstitial lung diseases (F-ILDs), with an accurate initial diagnosis being of paramount importance in ensuring appropriate initial management. Equally, case-by-case decisions on monitoring and management are essential, given the varying presentations of F-ILDs and the varying rates of progression. The value of diagnostic tests in risk stratification at presentation and, separately, the importance of a logical monitoring strategy, tailored to manage the risk of progression, are also stressed. The term “progressive pulmonary fibrosis” (PPF) exactly describes the entity that clinicians often face in practice. The importance of using antifibrotic therapy early in PPF (once initial management has failed to prevent progression) is increasingly supported by evidence. Artificial intelligence software for high-resolution computed tomography analysis, although an exciting tool for the future, awaits validation. Guidance is provided on pulmonary rehabilitation, oxygen and the use of non-invasive ventilation focused specifically on the needs of ILD patients with progressive disease. PPF should be differentiated from acute deterioration due to drug-induced lung toxicity or other forms of acute exacerbations. Referral criteria for a lung transplant are discussed and applied to patient needs in severe diseases where transplantation is not realistic, either due to access limitations or transplantation contraindications. In conclusion, expert group consensus guidance is provided on the diagnosis, treatment and monitoring of F-ILDs with specific focus on the recognition of PPF and the management of pulmonary fibrosis progressing despite initial management.

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Current monitoring and treatment of progressive fibrosing interstitial lung disease: a survey of physicians in Japan, the United States, and the European Union

Cited by 8 publications

References 56 publications

The Burden of Progressive-Fibrosing Interstitial Lung Diseases

The Burden of Progressive-Fibrosing Interstitial Lung Diseases

Pharmakotherapie der idiopathischen Lungenfibrose (ein Update) und anderer progredienter pulmonaler Fibrosen

Progressive pulmonary fibrosis: an expert group consensus statement

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