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 FEV(1) was >1000 mL, or FEV(1)/FVC >40%, or %DL(CO) >40%, the rate of deterioration in these tests was greater in Group A than in Group B (P < 0.01 for FEV(1), P < 0.05 for FEV(1)/FVC and %DL(CO)). 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.
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 FEV(1) was >1000 mL, or FEV(1)/FVC >40%, or %DL(CO) >40%, the rate of deterioration in these tests was greater in Group A than in Group B (P < 0.01 for FEV(1), P < 0.05 for FEV(1)/FVC and %DL(CO)). 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.
Authors: Andrew M Courtwright; Bruno G Baldi; Pranav Kidambi; Ye Cui; Anthony M Lamattina; Julian A Villalba; Shefali Bagwe; Hilary J Goldberg; Ivan O Rosas; Elizabeth Petri Henske; Carlos R R Carvalho; Souheil El-Chemaly Journal: Sarcoidosis Vasc Diffuse Lung Dis Date: 2018-04-28 Impact factor: 0.670
Authors: Kuniaki Seyama; Keiko Mitani; Toshio Kumasaka; Shiv K Gupta; Saji Oommen; Gang Liu; Jay H Ryu; Nicholas E Vlahakis Journal: Am J Pathol Date: 2010-03-04 Impact factor: 4.307
Authors: Nishant Gupta; Hye-Seung Lee; Jay H Ryu; Angelo M Taveira-DaSilva; Gerald J Beck; Jar-Chi Lee; Kevin McCarthy; Geraldine A Finlay; Kevin K Brown; Stephen J Ruoss; Nilo A Avila; Joel Moss; Francis X McCormack Journal: Chest Date: 2018-06-22 Impact factor: 9.410
Authors: Riffat Meraj; Kathryn A Wikenheiser-Brokamp; Lisa R Young; Sue Byrnes; Francis X McCormack Journal: Front Med Date: 2012-12-07 Impact factor: 4.592