Unclassifiable-interstitial lung disease: Outcome prediction using CT and functional indices

Jacob, Joseph; Bartholmai, Brian J.; Rajagopalan, Srinivasan; Egashira, Ryoko; Brun, Anne; Kokosi, Maria; Nair, Arjun; Walsh, Simon L.F.; Karwoski, Ronald A.; Nicholson, Andrew G.; Hansell, David M.; Wells, Athol U.

doi:10.1016/j.rmed.2017.07.007

Cited by 48 publications

(35 citation statements)

References 29 publications

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“…In nearly all cases, high-resolution computed tomography (HRCT) is the primary tool for diagnosis [5]. HRCT (in particular, the fibrosis score) can also help determine the prognosis in ILDs, and quantitative computed tomography (CT) analysis may be useful for evaluating disease progression [17][18][19] (the article by WALSH et al [20] in this issue of the European Respiratory Review provides more detail on imaging in fibrosing ILDs that may present a progressive phenotype). ILD diagnoses should be categorised as confident, provisional or unclassifiable depending on the level of diagnostic confidence.…”

Section: Diagnostic Methodsmentioning

confidence: 99%

“…Patients with unclassifiable IIP and HRCT features suggesting fibrosis (a high fibrosis score, the presence of honeycombing or the presence of UIP or a possible UIP pattern) may have a poor prognosis, similar to patients with IPF [17]. The extent of fibrosis, which can be measured by quantitative CT analysis, may help to predict outcomes in patients with unclassifiable IIP [19].…”

Section: Diagnostic Methodsmentioning

confidence: 99%

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Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases

et al. 2018

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@ERSpublicationsOther chronic ILDs with a progressive-fibrosing phenotype may have a clinical course similar to IPF. Although challenging, identification of these patients is crucial, and requires a multidisciplinary approach, to ensure optimal diagnosis and management.ABSTRACT Although these conditions are rare, a proportion of patients with interstitial lung diseases (ILDs) may develop a progressive-fibrosing phenotype. Progressive fibrosis is associated with worsening respiratory symptoms, lung function decline, limited response to immunomodulatory therapies, decreased quality of life and, potentially, early death. Idiopathic pulmonary fibrosis may be regarded as a model for other progressive-fibrosing ILDs. Here we focus on other ILDs that may present a progressive-fibrosing phenotype, namely idiopathic nonspecific interstitial pneumonia, unclassifiable idiopathic interstitial pneumonia, connective tissue disease-associated ILDs (e.g. rheumatoid arthritis-related ILD), fibrotic chronic hypersensitivity pneumonitis, fibrotic chronic sarcoidosis and ILDs related to other occupational exposures. Differential diagnosis of these ILDs can be challenging, and requires detailed consideration of clinical, radiological and histopathological features. Accurate and early diagnosis is crucial to ensure that patients are treated optimally.

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Section: Diagnostic Methodsmentioning

confidence: 99%

Section: Diagnostic Methodsmentioning

confidence: 99%

Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases

et al. 2018

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“…This could be particularly helpful in some non-IPF-PF, where it can be particularly difficult to visually distinguish ground-glass opacities from fine reticulation. Such technology may be of particular benefit in unclassifiable ILD [83]. Other than the SLS studies, current clinical trials for non-IPF-PF have not yet included change in automated/ computer-generated quantitative CT ILD score as outcome measures, although this is likely to change in the future as more information about this technology becomes available, and depending on the findings of the SLS III study.…”

Section: Rationale For Antifibrotic Medications In Non-ipf-pfmentioning

confidence: 99%

Antifibrotic therapy for fibrotic lung disease beyond idiopathic pulmonary fibrosis

2019

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Two antifibrotic medications (nintedanib and pirfenidone) were recommended (conditionally) for the treatment of patients with idiopathic pulmonary fibrosis (IPF) in the 2015 IPF evidence-based guidelines. These medications have been shown to reduce the rate of decline in forced vital capacity among patients with IPF over time and are the only two disease-modulating pharmacological agents approved by regulatory agencies and available for clinical use worldwide. With the evolved standard of care for interstitial lung disease evaluation including routine use of high-resolution computed tomography, fibrotic lung diseases other than IPF are increasingly recognised. In addition, it is becoming evident that genetic and pathophysiological mechanisms as well as disease behaviour in patients manifesting other “non-IPF progressive fibrotic interstitial lung diseases” (non-IPF-PF) may be similar to those in patients with IPF. Thus, it is biologically plausible that pharmacological agents with antifibrotic properties may be efficacious in non-IPF-PF. Indeed, studies are underway or planned to assess the safety and efficacy of nintedanib or pirfenidone among patients with several non-IPF fibrotic lung diseases. In this review, we briefly summarise the use of pirfenidone and nintedanib in IPF as well as the rationale and potential for use of these medications in non-IPF-PF that are being investigated in ongoing and upcoming clinical trials.

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“…The proportion of ILD patients who remain unclassifiable may be lower in cohorts that have undergone a multidisciplinary discussion [13], and is particularly high (up to 45%) in an elderly ILD population [25]. [26][27][28][29][30]. Some studies report a balanced sex distribution [2,26], and others either a male [12 , [26][27][28][29][30].…”

Section: Key Pointsmentioning

confidence: 99%

“…[26][27][28][29][30]. Some studies report a balanced sex distribution [2,26], and others either a male [12 , [26][27][28][29][30]. A subgroup of patients with unclassifiable ILD have autoimmune features but cannot be assigned a specific CTD diagnosis, with a proposal that these patients be labeled as having interstitial pneumonia with autoimmune features [31].…”

Section: Key Pointsmentioning

confidence: 99%