scholarly journals Diagnosis of idiopathic pulmonary fibrosis

2021 ◽  
Vol 64 (4) ◽  
pp. 248-255
Author(s):  
Hye Sook Choi
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Bronchoalveolar Lavage ◽  
Lung Biopsy ◽  
Usual Interstitial Pneumonia ◽  
High Resolution Computed Tomography ◽  
Exertional Dyspnea ◽  
Surgical Lung Biopsy ◽  
Typical Distribution ◽  
Hrct Patterns

Interstitial lung disease (ILD) is a group of diseases, involving the inflammation and fibrosis of the interstitium of the lung. ILD is classified according to whether or not the cause is known. Known causes of ILDs include inhalation of environmental substances, drugs, infection, and related connective tissue disease. ILD of unknown cause is called idiopathic ILD. The most common form of idiopathic ILD is idiopathic pulmonary fibrosis (IPF). IPF is a chronic progressive fibrosing ILD that results in the decline of lung function with exertional dyspnea, cough, bibasilar inspiratory crackles, and digital clubbing. The incidence of IPF increases with age, and is predominant in men. The most characteristic feature of IPF is a usual interstitial pneumonia (UIP) pattern detected on high-resolution computed tomography (HRCT). The typical HRCT pattern in case of UIP is honeycombing, with or without traction bronchiectasis or bronchiolectasis; this may be superimposed with fine reticulation. The typical distribution of UIP is subpleural, and there is basal predominance with heterogeneity. A definitive diagnosis of IPF in patients with clinically suspected IPF is made when there is presence of a UIP pattern on HRCT. Bronchoalveolar lavage or surgical lung biopsy is not recommended if a UIP pattern is detected on HRCT. However, bronchoalveolar lavage and surgical lung biopsy are required if probable UIP pattern, indeterminate UIP pattern, or an alternative diagnosis pattern are found on HRCT in order to diagnose IPF. A specific combination of HRCT patterns and histopathological patterns requiring multidisciplinary discussion is necessary to rule in IPF or rule it out.

scholarly journals Clinical diagnosis of patients subjected to surgical lung biopsy with a probable usual interstitial pneumonia pattern on high-resolution computed tomography

2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Regina Celia Carlos Tibana ◽  
Maria Raquel Soares ◽  
Karin Mueller Storrer ◽  
Gustavo de Souza Portes Meirelles ◽  
Katia Hidemi Nishiyama ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Clinical Diagnosis ◽  
Lung Biopsy ◽  
Usual Interstitial Pneumonia ◽  
High Resolution Computed Tomography ◽  
Surgical Lung Biopsy

Abstract Background Usual interstitial pneumonia can present with a probable pattern on high-resolution computed tomography (HRCT), but the probability of identifying usual interstitial pneumonia by surgical lung biopsy in such cases remains controversial. We aimed to determine the final clinical diagnosis in patients with a probable usual interstitial pneumonia pattern on HRCT who were subjected to surgical lung biopsy. Methods HRCT images were assessed and categorized by three radiologists, and tissue slides were evaluated by two pathologists, all of whom were blinded to the clinical findings. The final clinical diagnosis was accomplished via a multidisciplinary discussion. Patients with a single layer of honeycombing located outside of the lower lobes on HRCT were not excluded. Results A total of 50 patients were evaluated. The most common final clinical diagnosis was fibrotic hypersensitivity pneumonitis (38.0%) followed by idiopathic pulmonary fibrosis (24.0%), interstitial lung disease ascribed to gastroesophageal reflux disease (12.0%) and familial interstitial lung disease (10.0%). In the group without environmental exposure (n = 22), 10 patients had a final clinical diagnosis of idiopathic pulmonary fibrosis (45.5%). Irrespective of the final clinical diagnosis, by multivariate Cox analysis, patients with honeycombing, dyspnoea and fibroblastic foci on surgical lung biopsy had a high risk of death. Conclusions The most common disease associated with a probable usual interstitial pneumonia pattern on HRCT is fibrotic hypersensitivity pneumonitis followed by idiopathic pulmonary fibrosis and interstitial lung disease ascribed to gastroesophageal reflux disease. In patients without environmental exposure, the frequencies of usual interstitial pneumonia and a final clinical diagnosis of idiopathic pulmonary fibrosis are not sufficiently high to obviate the indications for surgical lung biopsy.

scholarly journals Clinical diagnosis of patients subjected to surgical lung biopsy with a probable usual interstitial pneumonia pattern on high-resolution computed tomography

2020 ◽  
Author(s):  
Regina Tibana ◽  
Maria Raquel Soares ◽  
Karin Mueller Storrer ◽  
Gustavo de Souza Portes Meirelles ◽  
Katia Nishiyama ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Lung Disease ◽  
Clinical Diagnosis ◽  
Lung Biopsy ◽  
Hypersensitivity Pneumonitis ◽  
Usual Interstitial Pneumonia ◽  
High Resolution Computed Tomography ◽  
Surgical Lung Biopsy

Abstract Background: Usual interstitial pneumonia (UIP) can present with a probable pattern on high-resolution computed tomography (HRCT), but the probability of UIP by surgical lung biopsy in such cases remains controversial. We aimed to determine the final clinical diagnosis in patients with a probable UIP pattern on HRCT subjected to surgical lung biopsy.Methods: HRCT images were assessed and categorized by three radiologists, and tissue slides were evaluated by two pathologists, all of whom were blinded to the clinical findings. The final clinical diagnosis was accomplished via a multidisciplinary discussion. Patients with a single layer of honeycombing located outside of the lower lobes on HRCT were not excluded.Results: A total of 50 patients were evaluated. The most common final clinical diagnosis was fibrotic hypersensitivity pneumonitis (38.0%; 95% CI: 24.7% to 52.8%), followed by idiopathic pulmonary fibrosis (24.0%; 95% CI: 13.1% to 38.2%) and interstitial lung disease ascribed to gastroesophageal reflux disease (12.0%; 95% CI: 4.5% to 24.3%) and familial interstitial lung disease (10.0%; 95% CI: 3.3% to 21.8%). In the group without environmental exposure (n = 22), 10 patients had a final clinical diagnosis of idiopathic pulmonary fibrosis (45.5%; 95% CI: 24.4% to 67.8%). Irrespective of the final clinical diagnosis, by multivariate Cox analysis, patients with honeycombing, dyspnoea and the presence of fibroblastic foci on surgical lung biopsy had a high risk of death.Conclusions: The most common disease associated with a probable UIP pattern on HRCT is fibrotic hypersensitivity pneumonitis, followed by idiopathic pulmonary fibrosis and interstitial lung disease ascribed to gastroesophageal reflux disease. In patients without environmental exposure, the frequency of UIP and a final clinical diagnosis of idiopathic pulmonary fibrosis are not sufficiently high to obviate the indication for surgical lung biopsy.

scholarly journals Concordance between sequential transbronchial lung cryobiopsy and surgical lung biopsy in patients with diffuse interstitial lung disease

2019 ◽  
Vol 14 (1) ◽  
Author(s):  
Yoshiaki Zaizen ◽  
Yasuo Kohashi ◽  
Kishio Kuroda ◽  
Kazuhiro Tabata ◽  
Yuka Kitamura ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Lung Disease ◽  
Lung Biopsy ◽  
Usual Interstitial Pneumonia ◽  
Airway Disease ◽  
Surgical Lung Biopsy ◽  
High Concordance ◽  
Transbronchial Lung Cryobiopsy

Abstract Background Increasing evidence indicates the utility of transbronchial lung cryobiopsy (TBLC) for the diagnosis of interstitial lung disease (ILD). However, only one study has compared TBLC and surgical lung biopsy (SLB) performed on the same patients. Methods We identified seven patients with ILD with TBLC and SLB. We evaluated the clinical characteristics and made a pathological diagnosis based on the official ATS/ERS/JRS/ALAT clinical practice guideline of idiopathic pulmonary fibrosis with both TBLC and SLB. Results Six cases were diagnosed as Usual interstitial pneumonia (UIP) in both TBLC and SLB. One case was diagnosed as indeterminate for UIP with TBLC and probable UIP with SLB. Etiological diagnosis with TBLC and SLB were concordant in 2 cases of idiopathic pulmonary fibrosis (IPF) but discordant for other diagnoses. Major histological findings of UIP including dense fibrosis, peripheral distribution, and fibroblastic foci showed high concordance between TBLC and SLB, which implies that TBLC can reliably detect these features. In contrast, loose fibrosis, cellular infiltration, and airway disease showed poor concordance between the two methods. Conclusion Our study showed that TBLC is useful for UIP diagnosis but not for other ILD. With a multidisciplinary approach, diagnosis of IPF may be determined by TBLC, whereas ILD other than IPF may require SLB.

scholarly journals Idiopathic pulmonary fibrosis: possibilities of multidisciplinary diagnostic approach

2018 ◽  
Vol 28 (5) ◽  
pp. 622-625
Author(s):  
A. М. Kardangusheva ◽  
Н. A. Sabanchieva
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Lung Biopsy ◽  
Poor Prognosis ◽  
Lung Diseases ◽  
Multidisciplinary Approach ◽  
Usual Interstitial Pneumonia ◽  
Interstitial Lung Diseases ◽  
High Resolution Computed Tomography ◽  
Antifibrotic Drugs

Idiopathic pulmonary fibrosis (IPF) is the commonest form of idiopathic interstitial pneumonias with very poor prognosis. Currently, diagnostic and treatment approaches to this disease have been revised. Confirmation of the diagnosis requires careful exclusion of other known causes of interstitial lung diseases and the presence of usual interstitial pneumonia (UIP) pattern on high-resolution computed tomography (HRTC) and/or on lung biopsy. Also, multidisciplinary discussion involving experts with experience in the diagnosis of interstitial lung diseases is recommended. Given recent knowledge on pathogenesis of IPF antifibrotic drugs are recommended for the therapy of this disease. A clinical case that demonstrates the multidisciplinary approach to diagnosis of IPF is reported in this article.

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