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Korean Guidelines for Diagnosis and Management of Interstitial Lung Diseases: Part 1. Introduction
박성우,백애린,이홍렬,정성환,양세훈,김용현,정만표,behalf of the Korean Interstitial Lung Diseases Study Group 대한결핵및호흡기학회 2019 Tuberculosis and Respiratory Diseases Vol.82 No.4
Idiopathic interstitial pneumonia (IIP) is a histologically identifiable pulmonary disease without a known cause that usually infiltrates the lung interstitium. IIP is largely classified into idiopathic pulmonary fibrosis, idiopathic non-specific interstitial pneumonia, respiratory bronchiolitis–interstitial lung disease (ILD), cryptogenic organizing pneumonia, desquamative interstitial pneumonia, and acute interstitial pneumonia. Each of these diseases has a different prognosis and requires specific treatment, and a multidisciplinary approach that combines chest high-resolution computed tomography (HRCT), histological findings, and clinical findings is necessary for their diagnosis. Diagnosis of IIP is made based on clinical presentation, chest HRCT findings, results of pulmonary function tests, and histological findings. For histological diagnosis, video-assisted thoracoscopic biopsy and transbronchial lung biopsy are used. In order to identify ILD associated with connective tissue disease, autoimmune antibody tests may also be necessary. Many biomarkers associated with disease prognosis have been recently discovered, and future research on their clinical significance is necessary. The diagnosis of ILD is difficult because patterns of ILD are both complicated and variable. Therefore, as with other diseases, accurate history taking and meticulous physical examination are crucial.
Korean Guidelines for Diagnosis and Management of Interstitial Lung Diseases: Part 1. Introduction
Park, Sung-Woo,Baek, Ae Rin,Lee, Hong Lyeol,Jeong, Sung Whan,Yang, Sei-Hoon,Kim, Yong Hyun,Chung, Man Pyo,Korean Interstitial Lung Diseases Study Group The Korean Academy of Tuberculosis and Respiratory 2019 Tuberculosis and Respiratory Diseases Vol.82 No.4
Idiopathic interstitial pneumonia (IIP) is a histologically identifiable pulmonary disease without a known cause that usually infiltrates the lung interstitium. IIP is largely classified into idiopathic pulmonary fibrosis, idiopathic non-specific interstitial pneumonia, respiratory bronchiolitis-interstitial lung disease (ILD), cryptogenic organizing pneumonia, desquamative interstitial pneumonia, and acute interstitial pneumonia. Each of these diseases has a different prognosis and requires specific treatment, and a multidisciplinary approach that combines chest high-resolution computed tomography (HRCT), histological findings, and clinical findings is necessary for their diagnosis. Diagnosis of IIP is made based on clinical presentation, chest HRCT findings, results of pulmonary function tests, and histological findings. For histological diagnosis, video-assisted thoracoscopic biopsy and transbronchial lung biopsy are used. In order to identify ILD associated with connective tissue disease, autoimmune antibody tests may also be necessary. Many biomarkers associated with disease prognosis have been recently discovered, and future research on their clinical significance is necessary. The diagnosis of ILD is difficult because patterns of ILD are both complicated and variable. Therefore, as with other diseases, accurate history taking and meticulous physical examination are crucial.
Lee, Jongmin,Kim, Yong Hyun,Kang, Ji Young,Jegal, Yangjin,Park, So Young,Korean Interstitial Lung Diseases Study Group The Korean Academy of Tuberculosis and Respiratory 2019 Tuberculosis and Respiratory Diseases Vol.82 No.4
Idiopathic nonspecific interstitial pneumonia (NSIP) is one of the varieties of idiopathic interstitial pneumonias. Diagnosis of idiopathic NSIP can be done via multidisciplinary approach in which the clinical, radiologic, and pathologic findings were discussed together and exclude other causes. Clinical manifestations include subacute or chronic dyspnea and cough that last an average of 6 months, most of which occur in non-smoking, middle-aged women. The common findings in thoracic high-resolution computed tomography in NSIP are bilateral reticular opacities, traction bronchiectasis, reduced volume of the lobes, and ground-glass opacity in the lower lungs. These lesions can involve diffuse bilateral lungs or subpleural area. Unlike usual interstitial pneumonia, honeycombing is sparse or absent. Pathology shows diffuse interstitial inflammation and fibrosis which are temporally homogeneous, namely NSIP pattern. Idiopathic NSIP is usually treated with steroid only or combination with immunosuppressive agents such as azathioprine, cyclophosphamide, cyclosporine, and mycophenolate mofetil. Prognosis of idiopathic NSIP is better than idiopathic pulmonary fibrosis. Many studies have reported a 5-year survival rate of more than 70%.
이종민,김용현,강지영,제갈양진,박소영,on behalf of Korean Interstitial Lung Diseases Study Group 대한결핵및호흡기학회 2019 Tuberculosis and Respiratory Diseases Vol.82 No.4
Idiopathic nonspecific interstitial pneumonia (NSIP) is one of the varieties of idiopathic interstitial pneumonias. Diagnosis of idiopathic NSIP can be done via multidisciplinary approach in which the clinical, radiologic, and pathologic findings were discussed together and exclude other causes. Clinical manifestations include subacute or chronic dyspnea and cough that last an average of 6 months, most of which occur in non-smoking, middle-aged women. The common findings in thoracic high-resolution computed tomography in NSIP are bilateral reticular opacities, traction bronchiectasis, reduced volume of the lobes, and ground-glass opacity in the lower lungs. These lesions can involve diffuse bilateral lungs or subpleural area. Unlike usual interstitial pneumonia, honeycombing is sparse or absent. Pathology shows diffuse interstitial inflammation and fibrosis which are temporally homogeneous, namely NSIP pattern. Idiopathic NSIP is usually treated with steroid only or combination with immunosuppressive agents such as azathioprine, cyclophosphamide, cyclosporine, and mycophenolate mofetil. Prognosis of idiopathic NSIP is better than idiopathic pulmonary fibrosis. Many studies have reported a 5-year survival rate of more than 70%.
Koo, So-My,Kim, Song Yee,Choi, Sun Mi,Lee, Hyun-Kyung,Korean Interstitial Lung Diseases Study Group The Korean Academy of Tuberculosis and Respiratory 2019 Tuberculosis and Respiratory Diseases Vol.82 No.4
Connective tissue disease (CTD) is a collection of disorders characterized by various signs and symptoms such as circulation of autoantibodies in the entire system causing damage to internal organs. Interstitial lung disease (ILD) which is associated with CTD is referred to as CTD-ILD. Patients diagnosed with ILD should be thoroughly examined for the cooccurrence of CTD, since the treatment procedures and prognosis of CTD-ILD are vary from those of idiopathic interstitial pneumonia. The representative types of CTD which may accompany ILD include rheumatoid arthritis, systemic sclerosis (SSc), Sjogren's syndrome, mixed CTD, idiopathic inflammatory myopathies, and systemic lupus erythematous. Of these, ILD most frequently co-exists with SSc. If an ILD is observed in the chest, high resolution computed tomography and specific diagnostic criteria for any type of CTD are met, then a diagnosis of CTD-ILD is made. It is challenging to conduct a properly designed randomized study on CTD-ILD, due to low incidence. Therefore, CTD-ILD treatment approach is yet to been established in absence of randomized controlled clinical trials, with the exception of SSc-ILD. When a patient is presented with acute CTD-ILD or if symptoms occur due to progression of the disease, steroid and immunosuppressive therapy are generally considered.
구소미,김송이,최선미,이현경,on behalf of Korean Interstitial Lung Diseases Study Group 대한결핵및호흡기학회 2019 Tuberculosis and Respiratory Diseases Vol.82 No.4
Connective tissue disease (CTD) is a collection of disorders characterized by various signs and symptoms such as circulation of autoantibodies in the entire system causing damage to internal organs. Interstitial lung disease (ILD) which is associated with CTD is referred to as CTD-ILD. Patients diagnosed with ILD should be thoroughly examined for the co-occurrence of CTD, since the treatment procedures and prognosis of CTD-ILD are vary from those of idiopathic interstitial pneumonia. The representative types of CTD which may accompany ILD include rheumatoid arthritis, systemic sclerosis (SSc), Sjögren’s syndrome, mixed CTD, idiopathic inflammatory myopathies, and systemic lupus erythematous. Of these, ILD most frequently co-exists with SSc. If an ILD is observed in the chest, high resolution computed tomography and specific diagnostic criteria for any type of CTD are met, then a diagnosis of CTD-ILD is made. It is challenging to conduct a properly designed randomized study on CTD-ILD, due to low incidence. Therefore, CTD-ILD treatment approach is yet to been established in absence of randomized controlled clinical trials, with the exception of SSc-ILD. When a patient is presented with acute CTD-ILD or if symptoms occur due to progression of the disease, steroid and immunosuppressive therapy are generally considered.
이상훈,여유미,김태형,이홍렬,이진화,박용범,박종선,김이형,송진우,전병우,김현정,박진경,어수택,김영환,김동순,박무석,on behalf of Korean Interstitial Lung Diseases Study Group 대한결핵및호흡기학회 2019 Tuberculosis and Respiratory Diseases Vol.82 No.2
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive fibrosing interstitial pneumonia, which presents with a progressive worsening dyspnea, and thus a poor outcome. The members of the Korean Academy of Tuberculosis and Respiratory Diseases as well as the participating members of the Korea Interstitial Lung Disease Study Group drafted this clinical practice guideline for IPF management. This guideline includes a wide range of topics, including the epidemiology, pathogenesis, risk factors, clinical features, diagnosis, treatment, prognosis, and acute exacerbation of IPF in Korea. Additionally, we suggested the PICO for the use of pirfenidone and nintendanib and for lung transplantation for the treatment of patients with IPF through a systemic literature review using experts’ help in conducting a meta-analysis. We recommend this guideline to physicians, other health care professionals, and government personnel in Korea, to facilitate the treatment of patients with IPF.
( Joo Han Song ),( Korean Interstitial Lung Diseases Research Group ),( Kyung Soo Chung ),( Song Yee Kim ),( Eun Young Kim ),( Ji Ye Jung ),( Young Ae Kang ),( Young Sam Kim ),( Se Kyu Kim ),( Joon Ch 대한내과학회 2014 대한내과학회 추계학술대회 Vol.2014 No.1
Background: Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by abnormal accumulation of lipoproteinaceous material within the alveoli. This study aims to compare clinical and laboratory features and therapeutic approach of PAP over the years. Methods: A total of 78 patients with PAP from 13 university hospitals registered in Korean Interstitial Lung Disease Research Group were included in this study. The patients were divided into two groups, based on the year of diagnosis: group A withpatients diagnosed from 1993 to 2007 (n= 41) and group B diagnosed from 2008 to 2013 (n = 37). We compared group A and B using the data on demographics, smoking status, diagnostic methods, symptom at presentation, pulmonary function tests, and occupation and exposed dusts. Results: The median age at diagnosis was 41 years (range, 15-81) in group A and 48 years (range, 15-68) in group B (p=0.349). Male to female ratio was similar. In recent years, less number of patients received BAL and/or TBLB (90.2% vs 75.7%; p=0.085), while surgical lung biopsy (SLB) was performed in similar numbers (43.9% vs 45.9%; p=0.856). Although not significant, the incidence of PAP in never smoker was increased after 2008. A history of dust exposure was seen in 43.6% (39.0% vs. 48.6%; p=0.392). Thirty-five (44.9%) patients underwent whole lung lavage (WLL), which was used less in recent years (58.5% vs. 29.7%; p=0.011). Oxygenation (PaO2: n=23, p=0.016; DLco: n=13, p=0.089) markedly improved after WLL. Anti-GM CSF antibody treatment was used for 1 patient in group A (2.4%) and 3 patients in group B (8.1%; p=0.341). Overall survival was 96% (n=75). Conclusions: PAP is increasing in Korea. Dust exposure seems an important predisposingfactor. WLL is a safe procedure and yields dramatic improvements in oxygenation in PAP.