scholarly journals The 2018 Diagnosis of Idiopathic Pulmonary Fibrosis Guidelines: Surgical Lung Biopsy for Radiological Pattern of Probable Usual Interstitial Pneumonia Is Not Mandatory

2019 ◽  
Vol 200 (9) ◽  
pp. 1089-1092 ◽  
Author(s):  
Ganesh Raghu ◽  
Martine Remy-Jardin ◽  
Jeffrey Myers ◽  
Luca Richeldi ◽  
Kevin C. Wilson
2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Regina Celia Carlos Tibana ◽  
Maria Raquel Soares ◽  
Karin Mueller Storrer ◽  
Gustavo de Souza Portes Meirelles ◽  
Katia Hidemi Nishiyama ◽  
...  

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.


2019 ◽  
Vol 14 (1) ◽  
Author(s):  
Yoshiaki Zaizen ◽  
Yasuo Kohashi ◽  
Kishio Kuroda ◽  
Kazuhiro Tabata ◽  
Yuka Kitamura ◽  
...  

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.


2021 ◽  
Vol 64 (4) ◽  
pp. 248-255
Author(s):  
Hye Sook Choi

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.


2020 ◽  
Author(s):  
Regina Tibana ◽  
Maria Raquel Soares ◽  
Karin Mueller Storrer ◽  
Gustavo de Souza Portes Meirelles ◽  
Katia Nishiyama ◽  
...  

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.


Respiration ◽  
2020 ◽  
pp. 1-8
Author(s):  
Pierre-Henri Aussedat ◽  
Nader Chebib ◽  
Kais Ahmad ◽  
Jean-Charles Glerant ◽  
Gabrielle Drevet ◽  
...  

<b><i>Background:</i></b> Video-assisted surgical lung biopsy (SLB) is performed in 10–30% of cases to establish the diagnosis of idiopathic pulmonary fibrosis (IPF). <b><i>Objectives:</i></b> The aim of the study was to analyze the impact of SLB on lung function in patients eventually diagnosed with IPF. <b><i>Methods:</i></b> This is an observational, retrospective, monocentric study of all consecutive patients eventually diagnosed with IPF in multidisciplinary discussion who underwent SLB over 10 years in a specialized center. The primary end point was the variation in forced vital capacity (FVC) before and after the SLB. The secondary end points were the variations in forced expiratory volume in one second (FEV1), total lung capacity (TLC), carbon monoxide diffusion capacity (DLCO), and morbidity and mortality associated with the SLB. <b><i>Results:</i></b> In 118 patients who underwent SLB and were diagnosed with IPF, a relative decrease in FVC of 4.8% (<i>p</i> &#x3c; 0.001) was found between measurements performed before and after the procedure. The mean FVC decrease was 156 ± 386 mL in an average period of 185 days, representing an annualized decline of 363 ± 764 mL/year. A significant decrease was also observed after SLB in FEV1, TLC, and DLCO. Complications within 30 days of SLB occurred in 14.4% of patients. Two patients (1.7%) died within 30 days, where one of them had poor lung function. Survival at 1 year was significantly poorer in patients with FVC &#x3c;50% at baseline. <b><i>Conclusion:</i></b> In this uncontrolled study in patients ultimately diagnosed with IPF, SLB was followed by a significant decline in FVC, which appears to be numerically greater than the average decline in the absence of treatment in the literature. <b><i>Summary at a Glance:</i></b> This study evaluated the change in lung function in 118 consecutive patients diagnosed with idiopathic pulmonary fibrosis by surgical lung biopsy. Forced vital capacity decreased by 156 ± 386 mL in a mean of 185 days between the last measurement before and first measurement after biopsy, representing an annualized decline of 363 ± 764 mL/year.


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