ArticleEuropean respiratory review : an official journal of the European Respiratory Society2014
Differential diagnosis of usual interstitial pneumonia: when is it truly idiopathic?
Article in European respiratory review : an official journal of the European Respiratory Society, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 47 papers.
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Who cites it
47 citing papers in PubMed, 121 citations in OpenAlex.
- Trial
- The 2025 ATS/ERS update of the international multidisciplinary classification of the interstitial pneumonias: implications for the pathologist.Histopathology · 2026Review
- Radiological Insights into UIP Pattern: A Comparison Between IPF and Non-IPF Patients.Journal of clinical medicine · 2025Article
- Usual interstitial pneumopathy in a patient with hypersensitivity pneumonitis and microscopic polyangiitis. Case report.Respiratory medicine case reports · 2024Article
- Diabetic Pneumopathy- A Novel Diabetes-associated Complication: Pathophysiology, the Underlying Mechanism and Combination Medication.Endocrine, metabolic & immune disorders drug targets · 2024Review
- Plasma extracellular vesicle proteins as promising noninvasive biomarkers for diagnosis of idiopathic pulmonary fibrosis.Journal of extracellular biology · 2023Article
- Diagnosis and Pharmacologic Management of Fibrotic Interstitial Lung Disease.Life (Basel, Switzerland) · 2023Review
- Diagnostic Value of Connective Tissue Disease Related CT Signs in Usual Interstitial Pneumonia Pattern of Interstitial Lung Disease.The Indian journal of radiology & imaging · 2023Article
- Multidisciplinary teams in the clinical care of fibrotic interstitial lung disease: current perspectives.European respiratory review : an official journal of the European Respiratory Society · 2022Review
- Enhancing Imagistic Interstitial Lung Disease Diagnosis by Using Complex Networks.Medicina (Kaunas, Lithuania) · 2022Article
- Histopathologic Insights into Distal Lung Injury and Inflammation following Military Deployment.American journal of respiratory and critical care medicine · 2022Article
- Clinical and radiological features of lung disorders related to connective-tissue diseases: a pictorial essay.Insights into imaging · 2022Review
- Usual interstitial pneumonia: a clinically significant pattern, but not the final word.Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc · 2022Review
- A Trans-Agency Workshop on the Pathophysiology of Radiation-Induced Lung Injury.Radiation research · 2022Article
- A Trans-Agency Workshop on the Pathophysiology of Radiation-Induced Lung Injury.Radiation research · 2022Article
- Relationship between Mechanical Ventilation and Histological Fibrosis in Patients with Acute Respiratory Distress Syndrome Undergoing Open Lung Biopsy.Journal of personalized medicine · 2022Article
- Demystifying idiopathic interstitial pneumonia: time for more etiology-focused nomenclature in interstitial lung disease.Expert review of respiratory medicine · 2022Review
- Idiopathic pulmonary fibrosis: Disease mechanisms and drug development.Pharmacology & therapeutics · 2021Review
- Clinical and radiological criteria for the differential diagnosis between asbestosis and idiopathic pulmonary fibrosis: Application in two cases.La Medicina del lavoro · 2021Article
- Hermansky-Pudlak syndrome pulmonary fibrosis: a rare inherited interstitial lung disease.European respiratory review : an official journal of the European Respiratory Society · 2021Article
Corrections and comments
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- Erratum issued
Authors and funding
6 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Idiopathic pulmonary fibrosis (IPF), the most common and lethal of the idiopathic interstitial pneumonias, is defined by a radiological and/or pathological pattern of usual interstitial pneumonia (UIP). However, UIP is not synonymous with IPF as other clinical conditions may be associated with UIP, including chronic hypersensitivity pneumonitis, collagen vascular disease, drug toxicity, asbestosis, familial IPF and Hermansky-Pudlak syndrome. Differentiating IPF ("idiopathic UIP") from conditions that mimic IPF ("secondary UIP") has substantial therapeutic and prognostic implications. A number of radiological and histological clues may help distinguish IPF from other conditions with a UIP pattern of fibrosis, but their appreciation requires extensive expertise in interstitial lung disease as well as an integrated multidisciplinary approach involving pulmonologists, radiologists and pathologists. In addition, multidisciplinary discussions may decrease the time to initial IPF diagnosis and, thus, enable more timely management. This concept was strongly emphasised by the 2011 ATS/ERS/JRS/ALAT guidelines. This article highlights, with the aid of a clinical case, the difficulties in making a diagnosis of IPF in clinical practice. Yet, an accurate diagnosis is critical, particularly given the availability of drugs that may reduce the pace of functional decline and disease progression in IPF.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.