Suggestion for Classification of Combined Pulmonary Fibrosis and Emphysema

( Yangjin Jegal ),( Soyeoun Lim ),( Jin Hyoung Kim ),( Ganghee Chae ),( Taehoon Lee ),( Kwang Won Seo ),( Seung Won Ra ),( Jong Joon Ahn ) 대한결핵 및 호흡기학회 2023 대한결핵 및 호흡기학회 추계학술대회 초록집 Vol.136 No.0

The Clinical Efficacy of GOCA Scoring System in Patients with Acute Respiratory Distress Syndrome

Yangjin Jegal,Sang Il Lee,Kyung Hee Lee,Yeon Mok Oh,Tae Sun Shim,Chae Man Lim,Sang Do Lee,Woo Sung Kim,Dong Soon Kim,Won Dong Kim,Younsuck Koh 대한의학회 2008 Journal of Korean medical science Vol.23 No.3

To explore the following hypotheses: 1) Gas exchange, Organ failure, Cause, Associated disease (GOCA) score, which reflects both general health and the severity of lung injury, would be a better mortality predictor of acute respiratory distress syndrome (ARDS) than acute physiology and chronic health evaluation (APACHE II) or simplified acute physiology score (SAPS II), which are not specific to lung injury, and lung injury score (LIS) that focuses on the lung injury; 2) the performance of APACHE II and SAPS II will be improved when reinforced by LIS, we retrospectively analyzed ARDS patients (N=158) admitted to a medical intensive care unit for five years. The overall mortality of the ARDS patients was 53.2%. Calibrations for all models were good. The area under the curve of (AUC) of LIS (0.622) was significantly less than those of APACHE II (0.743) and SAPS II (0.753). The AUC of GOCA (0.703) was not better than those of APACHE II and SAPS II. The AUCs of APACHE II and SAPS II tended to further increase when reinforced by LIS. In conclusion, GOCA was not superior to APACHE II or SAPS II. The performance of the APACHE II or SAPS II tended to improve when combining a general scoring system with a scoring system that focused on the severity of lung injury.

The role of neutrophils in the pathogenesis of IPF

Yangjin Jegal 대한내과학회 2022 The Korean Journal of Internal Medicine Vol.37 No.5

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Lymphangioleiomyomatosis

( Yangjin Jegal ) 대한결핵 및 호흡기학회 2021 대한결핵 및 호흡기학회 추계학술대회 초록집 Vol.129 No.-

Lymphangioleiomyomatosis (LAM) is a rare cystic lung disease with multisystem involvement. LAM can be either associated with Tuberous sclerosis complex (TSC-LAM) or occurs in a sporadic form (S-LAM). S-LAM occurs mainly in premenopausal women. However, TSC-LAM occurs in as many as 10-15% of men with TSC. The main pathologic characteristics are infiltration of smooth muscles cells spread via blood and lymphatics. Both S-LAM and TSC-LAM are caused by mutations in either of two TSC genes, TSC1 or TSC2. In TSC-LAM, TSC mutations are found in all cells, including the germline cells, whereas, in S-LAM, the mutations are found only in neoplastic cells. TSC1 encodes hamartin, and TSC2 encodes tuberin. Both proteins form a heterodimer that regulates cell growth, survival, and motility. Therefore, deficiency or dysfunction of those proteins can result in the up-regulated activity of mechanistic target of rapamycin (mTOR), which leads to increased protein translation, cellular proliferation, migration, and invasion. Although the symptoms are various depending on the affected organs, pulmonary manifestations are most common. Progressive dyspnea, spontaneous pneumothorax, and pleural effusion are common presenting complaints. Less common manifestations are chest pain, cough, sputum, hemoptysis, etc. Renal angiomyolipomas occur in approximately one-third of S-LAM and >80% in TSC-LAM. Lymphatic manifestations such as chylothorax, chyloperitoneum, lymphangioleiomyma of the retroperitoneum or pelvis, and lymphadenopathy are also common extrapulmonary manifestations. Clinically LAM can be diagnosed when typical HRCT findings are accompanied by other extrapulmonary findings such as TSC, renal angiomyolipoma, cystic lymphangioleiomyoma, chylous pleural effusion or ascites. The typical characteristics of pulmonary cyst in LAM are thin-walled, diffuse, round, well-defined, bilateral, and without predominance. Serum VEGF-D has been proposed as a noninvasive diagnostic test for LAM. A serum VEGF-D level of > 800pg/ml in a woman with typical HRCT is diagnostic for LAM. But it cannot distinguish TSC-LAM from the S-LAM. The two hallmark pathologic features of pulmonary LAM are lung cysts and atypical smooth muscle-like LAM cells. The common cystic lung diseases that mimic LAM are pulmonary Langerhans cell histiocytosis, lymphocytic interstitial pneumonia, Birt-Hogg-Dube syndrome, emphysema, and so on. The key treatment for LAM is inhibition of mTOR signaling by sirolimus. Sirolimus is a small molecule that forms a complex with FKBP12, which then binds to mTOR and blocks activation of downstream kinases, restores homeostasis in cells with defective TSC gene function. A double-blind, randomized, parallel-group trial [The Multicenter International LAM Efficacy of Sirolimus (MILES)] reported that sirolimus resulted in stabilization of lung function, reduced serum VEGF-D levels, and was associated with a reduction in symptoms and improvement of quality of life. Other studies also reported sirolimus treatment improved lung function, reduced the volume of angiomyolipoma or lymphangioleiomyoma and chylous accumulations. Therefore, treatment with sirolimus was recommended for symptomatic patients with abnormal/declining lung function, symptomatic chylous fluid accumulations, or evidence of rapidly progressive disease. The common adverse effects of sirolimus were mucositis, diarrhea, nausea, hypercholesterolemia, acneiform rash, and swelling in the lower extremities. Everolimus, another mTOR inhibitor, can be a second-line therapy for patients who do not tolerate or respond to sirolimus. Regarding other treatment drugs, ATS/JRS guideline for LAM suggested not using doxycycline and hormonal therapy as treatments for LAM. Lung transplantation may be required for LAM patients with progressive respiratory failure. Generally, the post-transplantation survival rates exceed those of other disease groups, although recurrent lymphangiomyomatosis after transplantation was reported.

Physiology Is a Stronger Predictor of Survival than Pathology in Fibrotic Interstitial Pneumonia

Jegal, Yangjin,Kim, Dong Soon,Shim, Tae Sun,Lim, Chae-Man,Do Lee, Sang,Koh, Younsuck,Kim, Woo Sung,Kim, Won Dong,Lee, Jin Seong,Travis, William D.,Kitaichi, Masanori,Colby, Thomas V. American Thoracic Society 2005 American journal of respiratory and critical care Vol.171 No.6

<P>The histopathologic pattern provides the most important prognostic marker for idiopathic interstitial pneumonia; however, studies have suggested that short-term changes in lung function may be more important. We investigated the prognostic factors for fibrotic interstitial pneumonia. The clinical features and follow-up course of 179 patients (131 with idiopathic pulmonary fibrosis and 48 with nonspecific interstitial pneumonia; 41 fibrotic types and 7 cellular) were analyzed retrospectively. The lung function indices improved or stabilized in most patients with fibrotic nonspecific interstitial pneumonia in contrast to the deterioration or stable condition of most patients with idiopathic pulmonary fibrosis. The 5-year survival of patients with fibrotic nonspecific interstitial pneumonia (76.2%) was better than for those with idiopathic pulmonary fibrosis (43.8%) (p = 0.007). Multivariate analysis at the time of presentation revealed that pathologic pattern, age, and diffusion capacity had important prognostic implications. However, after 6 months of follow-up, changes in FVC, initial diffusion capacity, and sex were the only independent prognostic factors, with no additional prognostic information conferred by the histologic diagnosis. Our data confirmed the importance of physiological parameters including short-term change in FVC. However, at the time of diagnosis, histopathology was important for the prediction of prognosis and future change in lung function.</P>

Clinical Features, Diagnosis, Management, and Outcomes of Idiopathic Pulmonary Fibrosis in Korea: Analysis of the Korea IPF Cohort (KICO) Registry

( Yangjin Jegal ),( Jong Sun Park ),( Song Yee Kim ),( Hongseok Yoo ),( Sung Hwan Jeong ),( Jin Woo Song ),( Jae Ha Lee ),( Hong Lyeol Lee ),( Sun Mi Choi ),( Young Whan Kim ),( Yong Hyun Kim ),( Hye 대한결핵 및 호흡기학회 2022 Tuberculosis and Respiratory Diseases Vol.85 No.2

Background: The Korea Interstitial Lung Disease Study Group has made a new nationwide idiopathic pulmonary fibrosis (IPF) registry because the routine clinical practice has changed due to new guidelines and newly developed antifibrotic agents in the recent decade. The aim of this study was to describe recent clinical characteristics of Korean IPF patients. Methods: Both newly diagnosed and following IPF patients diagnosed after the previous registry in 2008 were enrolled. Survival analysis was only conducted for patients diagnosed with IPF after 2016 because antifibrotic agents started to be covered by medical insurance of Korea in October 2015. Results: A total of 2,139 patients were analyzed. Their mean age at diagnosis was 67.4±9.3 years. Of these patients, 76.1% were males, 71.0% were ever-smokers, 14.4% were asymptomatic at the time of diagnosis, and 56.9% were at gender-agephysiology stage I. Occupational toxic material exposure was reported in 534 patients. The mean forced vital capacity was 74.6% and the diffusing capacity for carbon monoxide was 63.6%. Treatment with pirfenidone was increased over time: 62.4% of IPF patients were treated with pirfenidone initially. And 79.2% of patients were treated with antifiboritics for more than three months during the course of the disease since 2016. Old age, acute exacerbation, treatment without antifibrotics, and exposure to wood and stone dust were associated with higher mortality. Conclusion: In the recent Korean IPF registry, the percentage of IPF patients treated with antifibrotics was increased compared to that in the previous IPF registry. Old age, acute exacerbation, treatment without antifibrotics, and exposure to wood and stone dust were associated with higher mortality.

토치작업에서 노출된 이산화질소에 의한 폐부종 1례

안종준,제갈양진,서광원,권운정,최나리,김양호 대한산업의학회 2007 대한직업환경의학회지 Vol.19 No.3

Is Bronchial Washing Cytology Helpful for the Diagnosis of Lung Cancer?

( Sun Young Park ),( Yangjin Jegal ),( Byung Ju Kang ),( Jin Hyoung Kim ),( Taehoon Lee ),( Seung Won Ra ),( Hyeon Hui Kang ),( Kwang Won Seo ),( Jong Joon Ahn ) 대한결핵 및 호흡기학회 2021 대한결핵 및 호흡기학회 추계학술대회 초록집 Vol.129 No.-

Background Although the diagnostic yield of bronchial washing cytology is generally much lower than that of forceps biopsy, it has been routinely used to diagnose lung cancer. Recently, new diagnostic Methods such as transbronchial lun g biopsy with endobronchial ultrasonography using a guided sheath (EBUS-GS) and endobronchial ultrasoundguided transbronchial needle aspiration(EBUS-TBNA) were developed. Therefore, the diagnostic value of washing cytology must be changed. This study aims to evaluate the efficacy of bronchial washing cytology for diagnosing primary and secondary neoplasms of the lung. Methods The medical records of 728 patients (M:F = 471:257; mean age = 66.4 years) who were performed bronchial washing cytology at Ulsan university hospital from January 1, 2020, to December 31, 2020, were retrospectively reviewed. For interpretation of cytology, atypical cells were considered malignancy unless the reactive change was strongly suspected. Results Of the 728 patients, 280 patients were diagnosed with lung cancer or metastasis. The sensitivity of washing cytology was 50.7 %, specificity was 91.1% overall. However, the sensitivity decreased to 37.9% when only patients without endobronchial lesion were analyzed. In addition, all patients except one were confirmed lung malignancy by other diagnostic Methods than cyto logy (forcep biopsy for 90 patients, EBUS-GS for 58 patients, EBUS-TBNA for 51 patients, and surgery for 11 patients). Only one patient with multiple lung metastases of colon cancer was diagnosed by washing cytology. However, the case could be diag nosed easily by clinical information and chest CT without cytology. Interestingly, the cytology was positive for 54.0% of EBUSGS biopsied patients, although there was no endobronchial lesion. It may be because thin bronchoscope that can approach closer to the lesion than conventional bronchoscope was used, and the washing was performed after the biopsy. Conclusion Bronchial washing cytology seems unnecessary for diagnosing lung cancer due to newly developed variable diagnostic tools.

The Combination of Real-Time PCR and HPLC for the Identification of Non-Tuberculous Mycobacteria

박재선,최정인,임지훈,안종준,Yangjin Jegal,서광원,나승원,전재범,이선호,김성렬,정조셉,정윤성 대한진단검사의학회 2013 Annals of Laboratory Medicine Vol.33 No.5

We used HPLC and AdvanSure real-time PCR (LG Life Sciences, Korea) to retrospectively analyze non-tuberculous mycobacteria (NTM) in 133 clinical specimens. The specimens were culture-positive for NTM and the HPLC method identified 130 strains of mycobacteria from the cultures (97.7%) at the species level. Among the isolates, 48 Mycobacterium. kansasii (36.1%), 39 M. intracellulare (29.3%), 17 M. avium (12.8%), 16 M. abscessus (12.0%), 6 M. fortuitum (4.5%), 2 M. szulgai (1.5%), 2 M. gordonae (1.5%), and 3 unclassified NTM strains (2.3%) were identified. The real-time PCR assay identified 60 NTM-positive specimens (45.1%), 65 negative specimens (48.9%), and 8 M. tuberculosis (TB)-positive specimens (6.0%). The real-time PCR assay is advantageous because of its rapid identification of NTM. However, in our study, the real-time PCR assay showed relatively low sensitivity (45.1%) when using direct specimens including sputum and bronchoalveolar lavage (BAL) fluid. HPLC is useful as it discriminates NTM at the species level, although it is time-consuming and requires specific equipment and technical expertise. A combination of both methods will be helpful for the rapid and accurate identification of mycobacteria in clinical laboratories.

탄화수소 섭취 후 발생한 화학성 폐렴 1예

김석환(Suk Hwan Kim),제갈양진(Yangjin Jegal),최나리(Nari Choy),김양호(Yangho Kim) 대한직업환경의학회 2009 대한직업환경의학회지 Vol.21 No.4