title: A nationwide survey of lymphangioleiomyomatosis in Korea: recent increase in newly diagnosed patients.
abstract: In 2007, the Korean Interstitial Lung Disease Society had collected clinical data of patients who have diagnosed as Lymphangioleiomyomatosis (LAM) since 1990 through nationwide survey, which showed that LAM patients had increased sharply after 2004. The present study was performed to show the clinical features of Korean patients with LAM, and to establish the reason for the recent increase in the diagnosis. All 63 patients were women and the mean age at diagnosis was 36 yr. The most common presenting symptom was dyspnea and 8 patients had tuberous sclerosis complex. The survival rate at 5 yr after diagnosis was 84%. Compared with patients diagnosed after 2004 (n=34), the patients diagnosed before 2004 (n=29) complained with dyspnea more (P=0.016) and had lower FEV(1)% predicted (P=0.003), and DLco% predicted (P=0.042). The higher proportion of patients diagnosed after 2004 showed the normal chest radiography, and they were detected by routine chest CT screening (P=0.016). This study showed that clinical features of Korean patients with LAM were not different from those reported elsewhere. It is concluded that the reason for the increase of newly diagnosed patients is the result of increase in detection of the early stage LAM by the widespread use of chest CT screening.
authors.affiliation: Division of Pulmonary and Critical Care Medicine, Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
authors.forename: Hye Yun
authors.initials: HY
authors.lastname: Park
authors.forename: Hae-Seong
authors.initials: HS
authors.lastname: Nam
authors.forename: Man Pyo
authors.initials: MP
authors.lastname: Chung
authors.forename: Sung Hwan
authors.initials: SH
authors.lastname: Jeong
authors.forename: Yu Jin
authors.initials: YJ
authors.lastname: Kim
authors.forename: Seung-Ick
authors.initials: SI
authors.lastname: Cha
authors.forename: Young Whan
authors.initials: YW
authors.lastname: Kim
authors.forename: Jong Sun
authors.initials: JS
authors.lastname: Park
authors.forename: Soo-Taek
authors.initials: ST
authors.lastname: Uh
authors.forename: Choon-Sik
authors.initials: CS
authors.lastname: Park
authors.forename: Moo Suk
authors.initials: MS
authors.lastname: Park
authors.forename: Ji Ae
authors.initials: JA
authors.lastname: Moon
authors.forename: Kyung Soo
authors.initials: KS
authors.lastname: Jung
authors.forename: Yang Jin
authors.initials: YJ
authors.lastname: Jegal
authors.forename: Dong Soon
authors.initials: DS
authors.lastname: Kim
authors.forename: Jin Woo
authors.initials: JW
authors.lastname: Song
authors.forename: Ho-Kee
authors.initials: HK
authors.lastname: Yum
authors.forename: Young Bum
authors.initials: YB
authors.lastname: Park
mesh_terms: D000328:Adult; D000368:Aged; D042241:Early Diagnosis; D005260:Female; D006801:Humans; D008175:Lung Neoplasms / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000401:mortality; D018192:Lymphangioleiomyomatosis / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000401:mortality; D008875:Middle Aged; D056910:Republic of Korea; D012129:Respiratory Function Tests; D015996:Survival Rate; D014057:Tomography, X-Ray Computed