Clinical behaviour of patients exposed to organic dust and diagnosed with idiopathic pulmonary fibrosis

Sadeleer, Laurens J. De; Verleden, Stijn E.; Dycker, Els De; Yserbyt, Jonas; Verschakelen, Johny; Verbeken, Eric; Nemery, Benoît; Verleden, Geert M.; Hermans, Frederik; Vanaudenaerde, Bart M.; Wuyts, Wim

doi:10.1111/resp.13342

Cited by 19 publications

(8 citation statements)

References 15 publications

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“…The concept of IPAF emphasises that there can be an overlap between IIPs and CTD-ILD and provides a framework to designate and study this condition. Such overlap between entities is common in medicine, and can be due to overlapping manifestations, as seen in overlap CTD, in which features of separate CTDs are present in the same patient, or be related to uncertain aetiology, as in patients with a condition strongly suggesting IPF but exposed to inhaled antigens known to cause chronic fibrotic hypersensitivity pneumonitis [15]. IPAF deals with the overlap between IIPs and CTD-ILD.…”

Section: Advantages Of the Ipaf Concept And Definitionmentioning

confidence: 99%

Interstitial pneumonia with autoimmune features: challenges and controversies

et al. 2021

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The presence of clinical, serological and/or radiological features suggestive, but not confirmatory, of a defined connective tissue disease in patients with interstitial lung disease is a relatively frequent occurrence. In 2015, the European Respiratory Society and the American Thoracic Society proposed classification criteria for the interstitial pneumonia with autoimmune features (IPAF) research entity to capture such patients in a standardised manner, with the intention of nurturing clinical research. This initiative resulted in the publication of several series of IPAF patients, with significant variation between cohorts in clinical characteristics, outcome and the application of IPAF criteria in patient selection. From this increasing body of published work, it has become apparent that revision of IPAF criteria is now required in order to justify the eventual designation of IPAF as a standalone diagnostic term, as opposed to a provisional entity put forward as a basis for clinical research. This review covers the current state of IPAF, conclusions that can and cannot be drawn from the IPAF evidence base, and ongoing uncertainties that require further expert group consideration.

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Section: Advantages Of the Ipaf Concept And Definitionmentioning

confidence: 99%

Interstitial pneumonia with autoimmune features: challenges and controversies

et al. 2021

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“…Such overlap between entities is common in medicine, for example between the different CTDs (overlap CTD). Among ILDs, another overlap may exist between IPF and chronic fibrotic hypersensitivity pneumonitis (“IPF with exposure to inhaled antigens”) (42).…”

Section: Areas Of Uncertainties and Controversiesmentioning

confidence: 99%

Interstitial Pneumonia With Autoimmune Features (IPAF)

et al. 2019

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A significant proportion of patients with interstitial lung disease (ILD) manifest autoimmune features, but do not fulfill the diagnostic criteria for a definite connective tissue disease (CTD). In 2015, the European Respiratory Society (ERS) and American Thoracic Society (ATS) “Task Force on undifferentiated Forms of connective tissue disease-associated interstitial lung disease” proposed classification criteria for a so-called research category of Interstitial Pneumonia with Autoimmune Features (IPAF). These classification criteria were based on a combination of features from three domains: a clinical domain consisting of extra-thoracic features; a serologic domain with specific autoantibodies; and a morphologic domain with imaging patterns, histopathological findings or multi-compartment involvement. Patients meeting IPAF criteria tend to have a history of smoking similar to patients with idiopathic pulmonary fibrosis. The most frequent clinical and serological markers of autoimmune features are Raynaud' phenomenon and positive antinuclear antibodies, respectively. Non-specific interstitial pneumonia is the predominant radiologic and histopathologic pattern, although patients meeting IPAF criteria through the clinical and serologic domains may also have a usual interstitial pneumonia pattern. Management should be carefully individualized on a case-by-case basis in keeping with the wide heterogeneity of IPAF and lack of evidence in this particular subgroup of patients. Prognosis is generally intermediate between that of idiopathic pulmonary fibrosis and connective tissue disease-associated interstitial lung disease, but substantially variable according to the predominant histologic and radiologic patterns. As acknowledged by the Task Force, the proposed classification scheme of IPAF is a research concept that will need revision and refinement based on data to better inform prognostication and patient care.

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“…Nevertheless, we examined two well-recognized prognostic indices (GAP and CPI) and the initiation of LTOT, another robust prognostic predictor, contributing to address the question of the relationship, if any, between occupational dust exposure and IPF prognosis. It should be noted that available literature on this issue at present is scarce and controversial [6, 22]. Finally, it has to be mentioned that GAP has been validated at diagnosis and not at 1 year.…”

Section: Discussionmentioning

confidence: 99%

Antifibrotic treatment response and prognostic predictors in patients with idiopathic pulmonary fibrosis and exposed to occupational dust

Casillo

Cerri²,

Ciervo

et al. 2019

BMC Pulm Med

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Background Idiopathic Pulmonary Fibrosis (IPF) is an aggressive interstitial lung disease with an unpredictable course. Occupational dust exposure may contribute to IPF onset, but its impact on antifibrotic treatment and disease prognosis is still unknown. We evaluated clinical characteristics, respiratory function and prognostic predictors at diagnosis and at 12 month treatment of pirfenidone or nintedanib in IPF patients according to occupational dust exposure. Methods A total of 115 IPF patients were recruited. At diagnosis, we collected demographic, clinical characteristics, occupational history. Pulmonary function tests were performed and two prognostic indices [Gender, Age, Physiology (GAP) and Composite Physiologic Index (CPI)] calculated, both at diagnosis and after the 12 month treatment. The date of long-term oxygen therapy (LTOT) initiation was recorded during the entire follow-up (mean = 37.85, range 12–60 months). Results At baseline, patients exposed to occupational dust [≥ 10 years ( n = 62)] showed a lower percentage of graduates (19.3% vs 54.7%; p = 0.04) and a higher percentage of asbestos exposure (46.8% vs 18.9%; p 0.002) than patients not exposed [< 10 years ( n = 53)]. Both at diagnosis and after 12 months of antifibrotics, no significant differences for respiratory function and prognostic predictors were found. The multivariate analysis confirmed that occupational dust exposure did not affect neither FVC and DLCO after 12 month therapy nor the timing of LTOT initiation. Conclusion Occupational dust exposure lasting 10 years or more does not seem to influence the therapeutic effects of antifibrotics and the prognostic predictors in patients with IPF. Electronic supplementary material The online version of this article (10.1186/s12890-019-0930-7) contains supplementary material, which is available to authorized users.

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Clinical behaviour of patients exposed to organic dust and diagnosed with idiopathic pulmonary fibrosis

Cited by 19 publications

References 15 publications

Interstitial pneumonia with autoimmune features: challenges and controversies

Interstitial pneumonia with autoimmune features: challenges and controversies

Interstitial Pneumonia With Autoimmune Features (IPAF)

Antifibrotic treatment response and prognostic predictors in patients with idiopathic pulmonary fibrosis and exposed to occupational dust

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