Integration and Application of Clinical Practice Guidelines for the Diagnosis of Idiopathic Pulmonary Fibrosis and Fibrotic Hypersensitivity Pneumonitis

Marinescu, D; Raghu, Ganesh; Rémy‐Jardin, Martine; Travis, William D.; Adegunsoye, Ayodeji; Beasley, Mary Beth; Chung, Jonathan H.; Churg, Andrew; Cottin, Vincent; Egashira, Ryoko; Pérez, Evans R. Fernández; Inoue, Yoshikazu; Johannson, Kerri A.; Kazerooni, Ella A.; Khor, Yet H.; Lynch, David A.; Müller, Néstor L.; Nicholson, Andrew G.; Rajan, Sujeet; Saito, Ryoko; Troy, Lauren; Walsh, Simon L.F.; Wells, Athol U.; Wijsenbeek, Marlies; Wright, Joanne L.; Ryerson, Christopher J.

doi:10.1016/j.chest.2022.06.013

Cited by 32 publications

(12 citation statements)

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“…The predominant diagnosis in White patients was IPF, while fHP was most commonly diagnosed among Hispanic patients and CTD-ILD was 3 to 4 times more common in Black patients compared with White patients. As autoimmune disease is more frequently reported in Black individuals and pet birds are often present in Hispanic households, this raises the possibility that confirmation bias in the medical diagnosis–making process results in the observed diagnostic differences across racial and ethnic groups . For example, in a study that examined US decedents with IPF within the National Center for Health Statistics database, Black decedents were less likely to be coded with IPF than were White decedents .…”

Section: Discussionmentioning

confidence: 99%

“…As autoimmune disease is more frequently reported in Black individuals 25,26 and pet birds are often present in Hispanic households, 27 this raises the possibility that confirmation bias in the medical diagnosis-making process results in the observed diagnostic differences across racial and ethnic groups. [28][29][30][31] For example, in a study that examined US decedents with IPF within the National Center for Health Statistics database, Black decedents were less likely to be coded with IPF than were White decedents. 15 Conversely, Hispanic decedents were more likely to be coded with IPF at the time of death.…”

Section: Race and Ethnicitymentioning

confidence: 99%

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Evaluation of Pulmonary Fibrosis Outcomes by Race and Ethnicity in US Adults

et al. 2023

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ImportancePulmonary fibrosis (PF) is characterized by progressive scarring of lung tissue and poor survival. Racial and ethnic minority populations face the greatest risk of morbidity and mortality from disparities impacting respiratory health, but the pattern of age at clinically relevant outcomes across diverse racial and ethnic populations with PF is unknown.ObjectiveTo compare the age at PF-related outcomes and the heterogeneity in survival patterns among Hispanic, non-Hispanic Black, and non-Hispanic White participants.Design, Setting, and ParticipantsThis cohort study included adult patients with a PF diagnosis and used data from prospective clinical registries: the Pulmonary Fibrosis Foundation Registry (PFFR) for the primary cohort and registries from 4 geographically distinct tertiary hospitals in the US for the external multicenter validation (EMV) cohort. Patients were followed between January 2003 and April 2021.ExposuresRace and ethnicity comparisons between Black, Hispanic, and White participants with PF.Main Outcomes and MeasuresAge and sex distribution of participants were measured at the time of study enrollment. All-cause mortality and age at PF diagnosis, hospitalization, lung transplant, and death were assessed in participants over 14 389 person-years. Differences between racial and ethnic groups were compared using Wilcoxon rank sum tests, Bartlett 1-way analysis of variance, and χ2 tests, and crude mortality rates and rate ratios were assessed across racial and ethnic categories using Cox proportional hazards regression models.ResultsIn total, 4792 participants with PF were assessed (mean [SD] age, 66.1 [11.2] years; 2779 [58.0%] male; 488 [10.2%] Black, 319 [6.7%] Hispanic, and 3985 [83.2%] White); 1904 were in the PFFR and 2888 in the EMV cohort. Black patients with PF were consistently younger than White patients (mean [SD] age at baseline, 57.9 [12.0] vs 68.6 [9.6] years; P &lt; .001). Hispanic and White patients were predominantly male (Hispanic: PFFR, 73 of 124 [58.9%] and EMV, 109 of 195 [55.9%]; and White: PFFR, 1090 of 1675 [65.1%] and EMV, 1373 of 2310 [59.4%]), while Black patients were less likely to be male (PFFR, 32 of 105 [30.5%] and EMV, 102 of 383 [26.6%]). Compared with White patients, Black patients had a lower crude mortality rate ratio (0.57 [95% CI, 0.31-0.97), but for Hispanic patients, the mortality rate ratio was similar to that of White patients (0.89; 95% CI, 0.57-1.35). Mean (SD) hospitalization events per person were highest among Black patients compared with Hispanic and White patients (Black: 3.6 [5.0]; Hispanic, 1.8 [1.4]; and White, 1.7 [1.3]; P &lt; .001). Black patients were consistently younger than Hispanic and White patients at first hospitalization (mean [SD] age: Black, 59.4 [11.7] years; Hispanic, 67.5 [9.8] years; and White, 70.0 [9.3] years; P &lt; .001), lung transplant (Black, 58.6 [8.6] years; Hispanic, 60.5 [6.1] years; and White, 66.9 [6.7] years; P &lt; .001), and death (Black, 68.7 [8.4] years; Hispanic, 72.9 [7.6] years; and White, 73.5 [8.7] years; P &lt; .001). These findings remained consistent in the replication cohort and in sensitivity analyses within prespecified deciles of age groups.Conclusions and RelevanceIn this cohort study of participants with PF, racial and ethnic disparities, especially among Black patients, were found in PF-related outcomes, including earlier onset of death. Further research is essential to identify and mitigate the underlying responsible factors.

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Section: Discussionmentioning

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Section: Race and Ethnicitymentioning

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Evaluation of Pulmonary Fibrosis Outcomes by Race and Ethnicity in US Adults

et al. 2023

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“…Diagnosis of HP is often challenging given the heterogeneity in clinical presentation, in addition to overlap with other non‐fibrotic and fibrotic ILDs. Establishing a clinical profile may help inform pre‐test probability when distinguishing between fibrotic ILDs, though an indeterminate clinical profile with overlapping features that may be compatible with both idiopathic pulmonary fibrosis (IPF) and fHP (such as male >60 years of age, former smoker with a possible exposure) can make distinction difficult 4 . As a result, misdiagnosis is not infrequent, as a proportion of patients labelled as having IPF are often found to have fHP on explant studies or case re‐evaluation 5–7 .…”

Section: Figurementioning

confidence: 99%

“…Establishing a clinical profile may help inform pre-test probability when distinguishing between fibrotic ILDs, though an indeterminate clinical profile with overlapping features that may be compatible with both idiopathic pulmonary fibrosis (IPF) and fHP (such as male >60 years of age, former smoker with a possible exposure) can make distinction difficult. 4 As a result, misdiagnosis is not infrequent, as a proportion of patients labelled as having IPF are often found to have fHP on explant studies or case re-evaluation. [5][6][7] Furthermore, a causative antigen exposure is not identified in up to 60% of patients with HP, despite thorough evaluation.…”

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confidence: 99%

Hypersensitivity pneumonitis: Principles in diagnosis and management

Kadura

Raghu

2023

Respirology

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“…It is noteworthy that BAL was added to the diagnostic algorithm figure in the IPF/PPF guidelines (1). Indeed, the need to integrate and apply clinical practice guidelines for the diagnosis of IPF and fibrotic HP has been recently discussed (6).…”

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confidence: 99%