Australasian interstitial lung disease registry (AILDR): objectives, design and rationale of a bi-national prospective database

Moore, Irene; Wrobel, Jérémy; Rhodes, Jessica; Lin, Qi; Webster, Susanne; Jo, Helen; Troy, Lauren; Grainge, Christopher; Glaspole, Ian; Corte, Tamera J.

doi:10.1186/s12890-020-01297-2

Cited by 11 publications

(17 citation statements)

References 26 publications

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“…This is particularly true for idiopathic interstitial pneumonias, specifically IPF, for which there have been multiple iterations of clinical practice guidelines over the last decade (30)(31)(32). Additionally, new guidelines describing the entity of interstitial pneumonia with autoimmune features (IPAF) have led newer registries to qualify IPAF as a distinct ILD subtype, while other have collated IPAF under the broader umbrella term idiopathic interstitial pneumonia or alternatively under CTD-ILD itself (18,20,21,33). This may partially explain the geographic variability in frequency of CTD-ILD noted in the literature.…”

Section: Discussionmentioning

confidence: 99%

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Variability in Global Prevalence of Interstitial Lung Disease

et al. 2021

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There are limited epidemiologic studies describing the global burden and geographic heterogeneity of interstitial lung disease (ILD) subtypes. We found that among seventeen methodologically heterogenous studies that examined the incidence, prevalence and relative frequencies of ILDs, the incidence of ILD ranged from 1 to 31.5 per 100,000 person-years and prevalence ranged from 6.3 to 71 per 100,000 people. In North America and Europe, idiopathic pulmonary fibrosis and sarcoidosis were the most prevalent ILDs while the relative frequency of hypersensitivity pneumonitis was higher in Asia, particularly in India (10.7–47.3%) and Pakistan (12.6%). The relative frequency of connective tissue disease ILD demonstrated the greatest geographic variability, ranging from 7.5% of cases in Belgium to 33.3% of cases in Canada and 34.8% of cases in Saudi Arabia. These differences may represent true differences based on underlying characteristics of the source populations or methodological differences in disease classification and patient recruitment (registry vs. population-based cohorts). There are three areas where we feel addition work is needed to better understand the global burden of ILD. First, a standard ontology with diagnostic confidence thresholds for comparative epidemiology studies of ILD is needed. Second, more globally representative data should be published in English language journals as current literature has largely focused on Europe and North America with little data from South America, Africa and Asia. Third, the inclusion of community-based cohorts that leverage the strength of large databases can help better estimate population burden of disease. These large, community-based longitudinal cohorts would also allow for tracking of global trends and be a valuable resource for collective study. We believe the ILD research community should organize to define a shared ontology for disease classification and commit to conducting global claims and electronic health record based epidemiologic studies in a standardized fashion. Aggregating and sharing this type of data would provide a unique opportunity for international collaboration as our understanding of ILD continues to grow and evolve. Better understanding the geographic and temporal patterns of disease prevalence and identifying clusters of ILD subtypes will facilitate improved understanding of emerging risk factors and help identify targets for future intervention.

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Section: Discussionmentioning

confidence: 99%

“…The Australian Interstitial Lung Disease Registry (AILDR) is the largest longitudinal cohort study of ILD in Australia and New Zealand ( 21 ). A total of 1,061 patients were recruited from four ILD centers across the continent between 2016 and 2019 via surveys distributed to physicians.…”

Section: Comparative Epidemiology Of Interstitial Lung Diseasementioning

confidence: 99%

Variability in Global Prevalence of Interstitial Lung Disease

et al. 2021

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“…After the screening of titles and abstracts, 31 studies were qualified for further full-text analysis for eligibility criteria. Six studies were excluded due to the lack of polysomnographic data [ 18 , 19 , 20 , 21 , 22 , 23 ]. Four articles included different diagnostic criteria for OSA–other than AHI ≥ 5 [ 24 , 25 , 26 , 27 ].…”

Section: Methodsmentioning

confidence: 99%

The Association between Idiopathic Pulmonary Fibrosis and Obstructive Sleep Apnea: A Systematic Review and Meta-Analysis

Karuga

Kaczmarski

Szmyd

et al. 2022

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The prevalence of obstructive sleep apnea (OSA) has greatly increased in recent years. Recent data suggest that severe and moderate forms of OSA affect between 6 and 17% of adults in the general population. Many papers are reporting the significantly increased prevalence of OSA in patients suffering from fibrotic diseases, including idiopathic pulmonary fibrosis (IPF). Therefore, we performed a systematic review and meta-analysis regarding the dependency between IPF and OSA. Due to the lack of papers focusing on IPF among OSA patients, we focused on the prevalence of OSA among IPF patients. In the search strategy, a total of 684 abstracts were identified, 496 after the removal of duplicates. After the screening of titles and abstracts, 31 studies were qualified for further full-text analysis for eligibility criteria. The final analysis was performed on 614 IPF patients from 18 studies, which met inclusion criteria. There were 469 (76.38%) IPF patients with OSA and 145 (23.62%) without. The mean age varied from 60.9 ± 8.1 up to 70.3 ± 7.9. The obtained prevalence was 76.4 (95% CI: 72.9–79.7) and 75.7 (95% CI: 70.1–80.9) for fixed and random effects, respectively. The median prevalence of OSA among non-IPF patients for all the ethnics groups included in this study was 16,4% (IQR: 3.4%–26.8%). The study provides strong evidence for the increased prevalence of OSA in IPF patients when comparing with the general OSA prevalence.

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“… 8 If not already enrolled, subjects with age-adjusted telomere length above the 10th centile will be offered enrolment in the Australian ILD Registry so that the impact of telomere length on the natural history of PF can be ascertained. 29 Participants will be advised of their telomere length result alongside genetic counselling. All participants with a telomere length less than or equal to the 10th centile will be offered a clinical genetics consultation.…”

Section: Methods and Analysismentioning

confidence: 99%

“…Patients with clinical features suggestive of a short telomere syndrome (eg, young age of onset, a family history of PF, early greying of the hair, haematological abnormalities) will be targeted for recruitment 8. If not already enrolled, subjects with age-adjusted telomere length above the 10th centile will be offered enrolment in the Australian ILD Registry so that the impact of telomere length on the natural history of PF can be ascertained 29. Participants will be advised of their telomere length result alongside genetic counselling.…”

Section: Methods and Analysismentioning

confidence: 99%

TELO-SCOPE study: a randomised, double-blind, placebo-controlled, phase 2 trial of danazol for short telomere related pulmonary fibrosis

et al. 2021

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IntroductionRecent discoveries have identified shortened telomeres and related mutations in people with pulmonary fibrosis (PF). There is evidence to suggest that androgens, including danazol, may be effective in lengthening telomeres in peripheral blood cells. This study aims to assess the safety and efficacy of danazol in adults and children with PF associated with telomere shortening.Methods and analysisA multi-centre, double-blind, placebo-controlled, randomised trial of danazol will be conducted in subjects aged >5 years with PF associated with age-adjusted telomere length ≤10th centile measured by flow fluorescence in situ hybridisation; or in children, a diagnosis of dyskeratosis congenita. Adult participants will receive danazol 800 mg daily in two divided doses or identical placebo capsules orally for 12 months, in addition to standard of care (including pirfenidone or nintedanib). Paediatric participants will receive danazol 2 mg/kg/day orally in two divided doses or identical placebo for 6 months. If no side effects are encountered, the dose will be escalated to 4 mg/kg/day (maximum 800 mg daily) orally in two divided doses for a further 6 months. The primary outcome is change in absolute telomere length in base pairs, measured using the telomere shortest length assay (TeSLA), at 12 months in the intention to treat population.Ethics and disseminationEthics approval has been granted in Australia by the Metro South Human Research Ethics Committee (HREC/2020/QMS/66385). The study will be conducted and reported according to Standard Protocol Items: Recommendations for Interventional Trials guidelines. Results will be published in peer-reviewed journals and presented at international and national conferences.Trial registration numbersNCT04638517; Australian New Zealand Clinical Trials Registry (ACTRN12620001363976p).

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Australasian interstitial lung disease registry (AILDR): objectives, design and rationale of a bi-national prospective database

Cited by 11 publications

References 26 publications

Variability in Global Prevalence of Interstitial Lung Disease

Variability in Global Prevalence of Interstitial Lung Disease

The Association between Idiopathic Pulmonary Fibrosis and Obstructive Sleep Apnea: A Systematic Review and Meta-Analysis

TELO-SCOPE study: a randomised, double-blind, placebo-controlled, phase 2 trial of danazol for short telomere related pulmonary fibrosis

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