The epidemiology of lymphangioleiomyomatosis in Japan: A nationwide cross‐sectional study of presenting features and prognostic factors

• DOI: 10.1111/j.1440-1843.2007.01101.x
• Publication Date: 2007-06-06T18:33:59Z
• Authors: Mie HAYASHIDA; Kuniaki SEYAMA; Yoshikazu INOUE; Keisaku FUJIMOTO; Keishi KUBO
• Keywords: Lymphangioleiomyomatosis

Background and objective: To evaluate the characteristics prognostic factors of Japanese patients with lymphangioleiomyomatosis (LAM). Methods: A nationwide survey to identify LAM was conducted by questionnaire. Survival probability estimated using Kaplan–Meier method, were analysed Cox regression. Results: Data collected on 173 pulmonary LAM. The major presenting features pneumothorax (43%) exertional dyspnoea (37%). survival probabilities for (Group A) 85%, 60% 47% after 5, 10 15 years, respectively, B) 95%, 89% 89%, respectively. Although age at symptom onset higher among in Group than B, regression revealed that feature a factor independent A/B hazard ratio = 5.732, P < 0.01). In subgroup whose initial FEV 1 >1000 mL, or /FVC >40%, %DL CO rate deterioration these tests greater B ( 0.01 , 0.05 ). Conclusions: There are two possible subgroups patients. One presented pneumothorax, had symptoms younger more favourable prognosis; other dyspnoea, an older poorer prognosis.