Pulmonary lymphangioleiomyomatosis: a report of 46 patients including a clinicopathologic study of prognostic factors

Am J Respir Crit Care Med. 1995 Feb;151(2 Pt 1):527-33. doi: 10.1164/ajrccm.151.2.7842216.

Abstract

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).

Publication types

  • Multicenter Study

MeSH terms

  • Adult
  • Biopsy
  • Female
  • Humans
  • Lung / pathology
  • Lung Neoplasms / mortality
  • Lung Neoplasms / pathology*
  • Lung Neoplasms / physiopathology
  • Lung Neoplasms / therapy
  • Lymphangioleiomyomatosis / mortality
  • Lymphangioleiomyomatosis / pathology*
  • Lymphangioleiomyomatosis / physiopathology
  • Lymphangioleiomyomatosis / therapy
  • Middle Aged
  • Prognosis
  • Respiratory Mechanics
  • Retrospective Studies