Is It Idiopathic Pulmonary Fibrosis or Not?

Salvatore, Mary M.; Ishikawa, Genta; Padilla, María L.

doi:10.3122/jabfm.2018.01.170288

Cited by 21 publications

(23 citation statements)

References 27 publications

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“…A 10-year mortality rate of 16% has been reported in patients with lung fibrosis [106], which, allowing for comorbidities and other causes of death, may suggest that a small subset of patients have the progressive-fibrosing phenotype despite appropriate therapy. Acute exacerbations have not been reported in stage IV pulmonary sarcoidosis [105].…”

Section: Sarcoidosismentioning

confidence: 90%

“…Extensive parenchymal destruction and mycetoma are also occasionally observed. A UIP pattern of lung injury is uncommonly seen in patients with fibrosing pulmonary sarcoidosis, although honeycombing may be seen in the upper zones of the lungs [105].…”

Section: Sarcoidosismentioning

confidence: 99%

“…Approximately 20% of patients with pulmonary sarcoidosis develop pulmonary fibrosis (stage IV sarcoidosis), which is associated with significant morbidity and mortality [102]. Pulmonary fibrosis predominantly affects the upper lobes ( posterior segments) and is airway-centred with no air trapping; the fibrosis tends to follow the bronchovascular bundles [105]. The extent and type of fibrosis are highly variable.…”

Section: Sarcoidosismentioning

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: Sarcoidosismentioning

confidence: 90%

Section: Sarcoidosismentioning

confidence: 99%

Section: Sarcoidosismentioning

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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“…Accurate diagnosis requires a multidisciplinary approach with incorporation of clinical, pathophysiological, immunological and imaging information. This comprehensive testing contributes to the cost of healthcare [15][16][17][18][19][20]. It has been reported that up to 34% of ILD patients do not receive a final diagnosis for ⩾2 years during which time we anecdotally observe repetition of diagnostic tests and consultations.…”

Section: Diagnosis and Prognosismentioning

confidence: 99%

Healthcare utilisation and costs in the diagnosis and treatment of progressive-fibrosing interstitial lung diseases

et al. 2018

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There are over 200 interstitial lung diseases (ILDs). In addition to patients with idiopathic pulmonary fibrosis (IPF), a percentage of patients with other ILDs also develop progressive fibrosis of the lung during their disease course. Patients with progressive-fibrosing ILDs may show limited response to immunomodulatory therapy, worsening symptoms and lung function and, ultimately, early mortality. There are few data for ILDs that may present a progressive fibrosing phenotype specifically, but we believe the burden and healthcare costs associated with these conditions may be comparable to those reported in IPF. This review discusses the burden of ILDs that may present a progressive fibrosing phenotype and the factors impacting healthcare utilisation.

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“…16 From the family physician perspective, the original diagnosis is often the quandary-when is it pulmonary fibrosis with its poor prognosis, and when is it something else that is more currently treatable, and which treatment should be used? Salvatore et al 16 provide a helpful review of current diagnosis and treatment. A reader also provides us a letter about the importance of being an ABFM diplomate.…”

Section: Othermentioning

confidence: 99%

Interventions Must Be Realistic to Be Useful and Completed in Family Medicine

Bowman¹,

Seehusen

Neale³

2018

J Am Board Fam Med

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Being realistic while helping our patients is this issue's theme. Given the volume of tasks required in family medicine, recommendations for improvements in direct care or care measurement cannot just be evidence-based but must also be realistic. On the list of realistic: ordering antipsychotics for symptoms of dementia in the elderly, despite recommendations to not do so; ordering antidepressants without fear that the patient could develop hypertension; mental health care providers in primary care offices; forced choice for opioid management; plus agenda setting for visit efficiency. In this issue we group articles into general categories based on our assessment of how realistic the implied actions are, related to current provider training, finances, and organizational factors.

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Is It Idiopathic Pulmonary Fibrosis or Not?

Cited by 21 publications

References 27 publications

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

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

Healthcare utilisation and costs in the diagnosis and treatment of progressive-fibrosing interstitial lung diseases

Interventions Must Be Realistic to Be Useful and Completed in Family Medicine

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