The epidemiology of lymphangioleiomyomatosis in Japan: A nationwide cross-sectional study of presenting features and prognostic factors
Background and objective: To evaluate the characteristics and prognostic factors of Japanese patients with lymphangioleiomyomatosis (LAM). Methods: A nationwide survey to identify patients with LAM was conducted by questionnaire. Survival probability was estimated using the Kaplan–Meier method, an...
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Published in | Respirology (Carlton, Vic.) Vol. 12; no. 4; pp. 523 - 530 |
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Main Authors | , , , , |
Format | Journal Article |
Language | English |
Published |
Melbourne, Australia
Blackwell Publishing Asia
01.07.2007
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Subjects | |
Online Access | Get full text |
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Summary: | Background and objective: To evaluate the characteristics and prognostic factors of Japanese patients with lymphangioleiomyomatosis (LAM).
Methods: A nationwide survey to identify patients with LAM was conducted by questionnaire. Survival probability was estimated using the Kaplan–Meier method, and the prognostic factors were analysed by Cox regression.
Results: Data were collected on 173 patients with pulmonary LAM. The major presenting features were pneumothorax (43%) and exertional dyspnoea (37%). The survival probabilities for patients presenting with exertional dyspnoea (Group A) were 85%, 60% and 47% after 5, 10 and 15 years, respectively, and for patients presenting with pneumothorax (Group B) were 95%, 89% and 89%, respectively. Although the age at symptom onset was higher among patients in Group A than in Group B, Cox regression revealed that the presenting feature was a prognostic factor independent of age at symptom onset (Group A/B hazard ratio = 5.732, P < 0.01). In the subgroup of patients whose initial FEV1 was >1000 mL, or FEV1/FVC >40%, or %DLCO >40%, the rate of deterioration in these tests was greater in Group A than in Group B (P < 0.01 for FEV1, P < 0.05 for FEV1/FVC and %DLCO).
Conclusions: There are two possible subgroups of LAM patients. One subgroup that presented with pneumothorax, had onset of symptoms at a younger age and a more favourable prognosis; the other presented with exertional dyspnoea, had onset of symptoms at an older age and a poorer prognosis. |
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Bibliography: | ark:/67375/WNG-CCLZFVJ6-N ArticleID:RESP1101 istex:3041EFDC5AB9D167D1F132EB72AD234B74545F05 ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23 |
ISSN: | 1323-7799 1440-1843 |
DOI: | 10.1111/j.1440-1843.2007.01101.x |