title: Comprehensive evaluation of 35 patients with lymphangioleiomyomatosis.
abstract: OBJECTIVE
  To evaluate comprehensively the characteristics of lymphangioleiomyomatosis (LAM), with emphasis on the application of imaging and immunohistochemical methods.
  
  
  METHODS
  Prospective study.
  
  
  METHODS
  Thirty-five female subjects with LAM.
  
  
  METHODS
  Clinical Center, National Institutes of Health.
  
  
  METHODS
  BAL, pulmonary function test, ventilation/perfusion lung scans, CT of the chest and abdomen, ultrasonography of abdomen, and immunohistochemical study of lung biopsy specimens.
  
  
  RESULTS
  Most patients had exertional dyspnea (83%) and pneumothorax (69%). BAL did not show diagnostic changes. The most common abnormalities on pulmonary function tests were decreased diffusing capacity of carbon monoxide (83%), hypoxemia (57%), and airway obstruction (51%). Bronchodilator response was found in 26% of patients. CT, which is almost pathognomonic, showed numerous thin-walled cysts throughout both lungs in all patients. Thirty-four patients (97%) had abnormal ventilation and/or perfusion lung scans. An unusual "speckling" pattern was observed on ventilation scans of 74% of patients. Common extrapulmonary features were retroperitoneal adenopathy (77%) and renal angiomyolipomas (60%). The percentage of abnormal smooth muscle cells (LAM cells), reactive with HMB45, varied from 17 to 67% in 10 lung biopsy specimens.
  
  
  CONCLUSIONS
  Improved diagnostic methods have defined the abnormalities in patients with pulmonary LAM and increased the potential for early recognition and treatment of this disorder. Patients with LAM should be evaluated for bronchodilator responsiveness and may benefit from a trial of bronchodilators.
authors: Pulmonary-Critical Care Medicine Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD 20892-1590, USA.
authors: S C
authors: SC
authors: Chu
authors: K
authors: K
authors: Horiba
authors: J
authors: J
authors: Usuki
authors: N A
authors: NA
authors: Avila
authors: C C
authors: CC
authors: Chen
authors: W D
authors: WD
authors: Travis
authors: V J
authors: VJ
authors: Ferrans
authors: J
authors: J
authors: Moss
mesh_terms: D000005:Abdomen / Q000000981:diagnostic imaging; D000293:Adolescent; D000328:Adult; D001992:Bronchoalveolar Lavage Fluid / Q000166:cytology; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008168:Lung / Q000737:chemistry / Q000000981:diagnostic imaging; D018192:Lymphangioleiomyomatosis / Q000175:diagnosis*; D008875:Middle Aged; D011446:Prospective Studies; D011860:Radiography, Abdominal; D011877:Radionuclide Imaging; D015656:Respiratory Mechanics; D014057:Tomography, X-Ray Computed; D014463:Ultrasonography