Incidence and prevalence of interstitial lung diseases worldwide: a systematic literature review

Gupta, Rikisha Shah; Koteci, Ardita; Morgan, Ann; George, Peter M.; Quint, Jennifer

doi:10.1136/bmjresp-2022-001291

Cited by 22 publications

(8 citation statements)

References 107 publications

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“…Idiopathic pulmonary fibrosis (IPF) is a progressively fibrotic lung disease with a median survival time after diagnosis of 3-5 years 1 . In the USA and Europe, IPF is estimated to have a disease prevalence of 0.63 to 7.6 per 100,000 people 2 . The number of people diagnosed with IPF is increasing and males are more likely to be diagnosed than women 3,4 .…”

Section: Introductionmentioning

confidence: 99%

Genome-wide SNP-sex interaction analysis of susceptibility to idiopathic pulmonary fibrosis

Leavy,

Goemans,

Stockwell

et al. 2024

Preprint

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Background Idiopathic pulmonary fibrosis (IPF) is a chronic lung condition that is more prevalent in males than females. The reasons for this are not fully understood, with differing environmental exposures due to historically sex-biased occupations, or diagnostic bias, being possible explanations. To date, over 20 independent genetic variants have been identified to be associated with IPF susceptibility, but these have been discovered when combining males and females. Our aim was to test for the presence of sex-specific associations with IPF susceptibility and assess whether there is a need to consider sex-specific effects when evaluating genetic risk in clinical prediction models for IPF. Methods We performed genome-wide single nucleotide polymorphism (SNP)-by-sex interaction studies of IPF risk in six independent IPF case-control studies and combined them using inverse-variance weighted fixed effect meta-analysis. In total, 4,561 cases (1,280 females and 2,281 males) and 23,500 controls (8,360 females and 14,528 males) of European genetic ancestry were analysed. We used polygenic risk scores (PRS) to assess differences in genetic risk prediction between males and females. Findings Three independent genetic association signals were identified. All showed a consistent direction of effect across all individual IPF studies and an opposite direction of effect in IPF susceptibility between females and males. None had been previously identified in IPF susceptibility genome-wide association studies (GWAS). The predictive accuracy of the PRSs were similar between males and females, regardless of whether using combined or sex-specific GWAS results. Interpretation We prioritised three genetic variants whose effect on IPF risk may be modified by sex, however these require further study. We found no evidence that the predictive accuracy of common SNP-based PRSs varies significantly between males and females.

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Section: Introductionmentioning

confidence: 99%

Genome-wide SNP-sex interaction analysis of susceptibility to idiopathic pulmonary fibrosis

Leavy,

Goemans,

Stockwell

et al. 2024

Preprint

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“…[ 14 , 15 ] Internationally there has been a lack of standardisation in diagnostic coding, leading to an estimated reported prevalence of IPF ranging from 7 to 1650 per 100 000 persons. [ 16 ] Patients living with a diagnosis of IPF have high unmet care needs and require a multi-disciplinary team approach to care which should include supports such as multi-disciplinary team discussion at the time of diagnosis [ 1 , 17 ].…”

Section: Introductionmentioning

confidence: 99%

A scoping review of the unmet needs of patients diagnosed with idiopathic pulmonary fibrosis (IPF)

Bramhill,

Langan,

Mulryan

et al. 2024

PLoS ONE

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Aims Patients diagnosed with idiopathic pulmonary fibrosis (IPF) have a high symptom burden and numerous needs that remain largely unaddressed despite advances in available treatment options. There is a need to comprehensively identify patients’ needs and create opportunities to address them. This scoping review aimed to synthesise the available evidence and identify gaps in the literature regarding the unmet needs of patients diagnosed with IPF. Methods The protocol for the review was registered with Open Science Framework (DOI 10.17605/OSF.IO/SY4KM). A systematic search was performed in March 2022, in CINAHL, MEDLINE, Embase, PsychInfo, Web of Science Core Collection and ASSIA Applied Social Science Index. A comprehensive review of grey literature was also completed. Inclusion criteria included patients diagnosed with IPF and date range 2011–2022. A range of review types were included. Data was extracted using a data extraction form. Data was analysed using descriptive and thematic analysis. A total of 884 citations were reviewed. Ethical approval was not required. Results 52 citations were selected for final inclusion. Five themes were identified: 1.) psychological impact of an IPF diagnosis. 2.) adequate information and education: at the right time and in the right way. 3.) high symptom burden support needs. 4.) referral to palliative care and advance care planning (ACP). 5.) health service provision-a systems approach. Conclusion This review highlights the myriad of needs patients with IPF have and highlights the urgent need for a systems approach to care, underpinned by an appropriately resourced multi-disciplinary team. The range of needs experienced by patients with IPF are broad and varied and require a holistic approach to care including targeted research, coupled with the continuing development of patient-focused services and establishment of clinical care programmes.

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“…Systemic sclerosis (SSc) is an autoimmune disease characterized by diffuse fibrosis and vasculopathy that has the potential to affect nearly every organ system. Interstitial lung disease (ILD) is present in up to 90% of SSc patients with many presenting early in their disease course with variable trajectories (1)(2)(3). Despite advances in the field, ILD remains the leading cause of SSc morbidity and mortality accounting for up to 35% of SSc-related deaths and a 5year mortality rate ranging between 18% and 44% (4,5).…”

Section: Introductionmentioning

confidence: 99%

Gene expression meta-analysis reveals aging and cellular senescence signatures in scleroderma-associated interstitial lung disease

Yang,

Lee,

Neely

et al. 2024

Front. Immunol.

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Aging and cellular senescence are increasingly recognized as key contributors to pulmonary fibrosis. However, our understanding in the context of scleroderma-associated interstitial lung disease (SSc-ILD) is limited. To investigate, we leveraged previously established lung aging- and cell-specific senescence signatures to determine their presence and potential relevance to SSc-ILD. We performed a gene expression meta-analysis of lung tissues from 38 SSc-ILD and 18 healthy controls and found that markers (GDF15, COMP, and CDKN2A) and pathways (p53) of senescence were significantly increased in SSc-ILD. When probing the established aging and cellular senescence signatures, we found that epithelial and fibroblast senescence signatures had a 3.6- and 3.7-fold enrichment, respectively, in the lung tissue of SSc-ILD and that lung aging genes (CDKN2A, FRZB, PDE1A, and NAPI12) were increased in SSc-ILD. These signatures were also enriched in SSc skin and associated with degree of skin involvement (limited vs. diffuse cutaneous). To further support these findings, we examined telomere length (TL), a surrogate for aging, in the lung tissue and found that, independent of age, SSc-ILD had significantly shorter telomeres than controls in type II alveolar cells in the lung. TL in SSc-ILD was comparable to idiopathic pulmonary fibrosis, a disease of known aberrant aging. Taken together, this study provides novel insight into the possible mechanistic effects of accelerated aging and aberrant cellular senescence in SSc-ILD pathogenesis.

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Incidence and prevalence of interstitial lung diseases worldwide: a systematic literature review

Cited by 22 publications

References 107 publications

Genome-wide SNP-sex interaction analysis of susceptibility to idiopathic pulmonary fibrosis

Genome-wide SNP-sex interaction analysis of susceptibility to idiopathic pulmonary fibrosis

A scoping review of the unmet needs of patients diagnosed with idiopathic pulmonary fibrosis (IPF)

Gene expression meta-analysis reveals aging and cellular senescence signatures in scleroderma-associated interstitial lung disease

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