Clinical features of 280 hospitalized patients with lymphangioleiomyomatosis in Japan.
OBJECTIVE
Lymphangioleiomyomatosis (LAM) is rare, but potentially life threatening owing to respiratory failure. However, knowledge is limited about the condition of hospitalized LAM patients. The objectives of this study were to investigate patient characteristics, comorbidities and causes of death among hospitalized LAM patients in Japan.


METHODS
Using a national inpatient database in Japan, information on 280 LAM patients hospitalized between July 2010 and March 2013 was retrospectively collected. We divided the 280 patients into three groups according to their status regarding lung transplantation.


RESULTS
For the study period, we identified 32 patients who had undergone lung transplantation ('after-transplantation' group), 12 patients admitted for lung transplantation ('for-transplantation') and 236 patients who had not undergone transplantation ('no-transplantation'). Although the clinical features of LAM patients in the 'no-transplantation' group were similar to previously reported findings, patients hospitalized in connection with transplantation showed the following: the activities of daily living score using the Barthel Index in the 'after-transplantation' group (89.4) was significantly higher than in the 'for-transplantation' group (64.6); the mortality rates in the after-transplantation group (3.1%) were significantly lower than in the for-transplantation group (25%). The most frequent comorbidity was pneumothorax, followed by respiratory failure and angiomyolipoma, although there was no significant difference in the prevalence among the three groups.


CONCLUSIONS
We determined the clinical features, comorbidities and fatalities in hospitalized LAM patients. Patients with LAM after transplantation had higher activities of daily living scores than those before transplantation, which suggests that lung transplantation may improve activities of daily living.
Department of Respiratory Medicine, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan.
Wakae
W
Hasegawa
Yasuhiro
Y
Yamauchi
Hideo
H
Yasunaga
Mitsuhiro
M
Sunohara
Taisuke
T
Jo
Hiroki
H
Matsui
Kiyohide
K
Fushimi
Kazutaka
K
Takami
Takahide
T
Nagase
D000203:Activities of Daily Living; D000328:Adult; D000368:Aged; D002423:Cause of Death; D015897:Comorbidity; D005260:Female; D006760:Hospitalization / Q000706:statistics & numerical data; D006801:Humans; D007564:Japan / Q000453:epidemiology; D008175:Lung Neoplasms / Q000453:epidemiology / Q000473:pathology / Q000503:physiopathology / Q000601:surgery; D016040:Lung Transplantation / Q000379:methods / Q000534:rehabilitation; D018192:Lymphangioleiomyomatosis / Q000453:epidemiology / Q000473:pathology / Q000503:physiopathology / Q000601:surgery; D008297:Male; D008875:Middle Aged; D011030:Pneumothorax / Q000453:epidemiology / Q000209:etiology; D015995:Prevalence; D012189:Retrospective Studies
clinical epidemiology; lung transplantation; lymphangioleiomyomatosis; rare lung disease