Tuberculosis and Respiratory Diseases

The Korean Academy of Tuberculosis and Respiratory Diseases (대한결핵및호흡기학회)
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Clinical Features of Pulmonary Langerhans Cell Histiocytosis in Korea

국내 폐 랑게르한스세포 조직구증(Pulmonary Langerhans Cell Histiocytosis)의 임상양상

  • Kim, Chul (Department of Internal Medicine, Soonchunhyang University College of Medicine) ;
  • Jeong, Sung Hwan (Department of Internal Medicine, Gachon University Gil Medical Center) ;
  • Shim, Jae Jeong (Department of Internal Medicine, Guro Hospital, Korea University Medical Center) ;
  • Cha, Seung-Ick (Department of Internal Medicine, Kyungpook National University School of Medicine) ;
  • Son, Choonhee (Department of Internal Medicine, Dong-A University College of Medicine) ;
  • Chung, Man Pyo (Department of Internal Medicine, Sungkyunkwan University School of Medicine) ;
  • Park, Hye Yoon (Department of Internal Medicine, Sungkyunkwan University School of Medicine) ;
  • Kim, Young Whan (Department of Internal Medicine, Seoul National University College of Medicine) ;
  • Park, Jong Sun (Department of Internal Medicine, Seoul National University College of Medicine) ;
  • Uh, Soo-Taek (Department of Internal Medicine, Soonchunhyang University College of Medicine) ;
  • Park, Choon Sik (Department of Internal Medicine, Soonchunhyang University College of Medicine) ;
  • Kim, Dong Soon (Department of Internal Medicine, Asan Medical Center, Ulsan University College of Medicine) ;
  • Cho, Kyung Wook (Department of Internal Medicine, Asan Medical Center, Ulsan University College of Medicine) ;
  • Song, Jin Woo (Department of Internal Medicine, Asan Medical Center, Ulsan University College of Medicine) ;
  • Jegal, Yang Jin (Department of Internal Medicine, Ulsan University College of Medicine) ;
  • Park, Moo Suk (Department of Internal Medicine, Yonsei University College of Medicine) ;
  • Park, Byung Hoon (Department of Internal Medicine, Yonsei University College of Medicine) ;
  • Lee, Jin Hwa (Department of Internal Medicine, Ewha Womans University School of Medicine) ;
  • Hur, Jin Won (Department of Internal Medicine, College of Medicine, Inje University) ;
  • Yum, Ho-Kee (Department of Internal Medicine, College of Medicine, Inje University) ;
  • Lee, Hong-Lyeol (Department of Internal Medicine, College of Medicine, Inha University) ;
  • Park, Yong Bum (Department of Internal Medicine, College of Medicine, Hallym University)
  • Received : 2009.01.20
  • Accepted : 2009.02.09
  • Published : 2009.02.28
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Abstract

Background: Pulmonary Langerhans cell histiocytosis (PLCH) is characterized by a proliferation of Langerhans cells and this results in granulomas that involve multiple organs of the body. Because the incidence of PLCH is very low in Korea and worldwide, collecting the clinical data of patients with PLCH nationwide is needed to determine the clinical features of Korean patients with PLCH. Methods: The patients with PLCH confirmed by biopsy at any body site were included and the patients should have lung lesions present. A questionnaire that had items on the symptoms, lung function tests, the roentgenographic findings and the treatment was collected retrospectively at a Korean ILD Research Meeting. Results: A total of 56 cases were collected. The number of males and females was 48 and 8, respectively, and their median age was 43 years (range: 18-67 years). The patients were current or ex-smokers in 79% of the cases. The most frequent symptom was coughing (39%), followed in decreasing order by dyspnea (38%), sputum (20%) and chest pain (20%). Pneumothorax was observed in 16 (29%) patients. Lung function tests showed a normal, restrictive, mixed or obstructive pattern in 26 (61%), 7 (16%), 7 (16%) and 3 patients (7%), respectively. Nodular-cystic lesion was most frequently observed in 59% of the patients on HRCT. The lung lesions were located in the middle and upper lobes in almost the cases. The median follow-up period was 90 months (range: 1-180 months) and only two patients died during this period. Conclusion: This study provides a national survey of the patients with PLCH during a long follow-up period.

연구배경: 폐 랑게르한스세포 조직구증(pulmonary Lan gerhans cell histiocytosis, PLCH)은 랑게르한스세포의 증식으로 형성된 육아종이 여러 장기에 침범하여 발생하는 질환이다. 주로 폐에 침범하며, 무증상에서부터 호흡곤란등의 다양한 임상증상을 가진다. 국외뿐만 아니고 국내에서도 빈도가 적은 것으로 알려져 있어, 다기관에서 국내 증례를 모아 그 임상양상을 알아보고자 하였다. 방 법: 부위에 관계없이 조직학적으로 진단되고 폐의 병변이 있는 PLCH 환자의 임상증상을 ILD 연구회를 통하여 후향적으로 조사하였다. 결 과: 수집된 증례는 총 56명으로 남자 48명, 여자 8명이었고, 중간나이는 43세(18-67세)였다. 진단 당시 흡연자와 과거 흡연자는 79%로 흡연과 관계가 있었다. 가장 흔한 증상은 기침(39%)이었고, 이어 호흡곤란(38%), 객담(20%), 흉통(20%) 순이었다. 기흉은 16명의 환자에서 관찰되었다. 폐기능 검사에서 정상은 26명(61%), 제한성 7명(16%), 혼합형 7명(16%), 폐쇄성은 3명(7%)에서 관찰 되었다. 방사선 소견은 대부분의 환자에서 병변이 중엽과 상엽에 있었으며, 결절과 낭종이 같이 있는 경우가 59%로 가장 많았다. 결 론: 이 연구는 PLCH 환자에 대한 국가적 그리고 장기적 추적 연구의 필요성을 시사한다.

Keywords

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