Pulmonary lymphangioleiomyomatosis: a report of 46 patients including a clinicopathologic study of prognostic factors.
The clinical and pathologic features of 46 patients from Japan, Korea, and Taiwan with pulmonary lymphangioleiomyomatosis (LAM) were studied. Only two (5%) among 40 evaluable treatments were assessed to be effective. Some prognostic factors of LAM were recognized. A reduction of the forced expiratory volume in one second/forced vital capacity (FEV1/FVC) ratio was a poor prognostic factor at 2 yr after the first examination, with a statistically significant difference (p < 0.05). An increase in the percentage of predicted total lung capacity (%TLC) correlated with a poor prognosis at 2, 3, and 5 yr after the first examination, with statistically significant differences (p < 0.05). Histologically, two types of pulmonary lesions were observed; a predominantly cystic type and a predominantly muscular type. Patients with predominantly cystic LAM lesions showed a tendency to a poor prognosis from 2 to 5 yr after the biopsy. Among open lung biopsy findings, higher grades of abnormal areas were unfavorable as a prognostic factor from 2 to 5 yr after the biopsy, with statistically significant differences (p < 0.05). Higher grades of cystic lesions correlated inversely with survival at 2, 4, and 5 yr after the lung biopsy, with statistically significant differences (p < 0.05).
Department of Laboratory Medicine, Chest Disease Research Institute Hospital, Kyoto, Japan.
M
M
Kitaichi
K
K
Nishimura
H
H
Itoh
T
T
Izumi
D000328:Adult; D001706:Biopsy; D005260:Female; D006801:Humans; D008168:Lung / Q000473:pathology; D008175:Lung Neoplasms / Q000401:mortality / Q000473:pathology* / Q000503:physiopathology / Q000628:therapy; D018192:Lymphangioleiomyomatosis / Q000401:mortality / Q000473:pathology* / Q000503:physiopathology / Q000628:therapy; D008875:Middle Aged; D011379:Prognosis; D015656:Respiratory Mechanics; D012189:Retrospective Studies