title: Lymphangioleiomyomatosis and multifocal micronodular pneumocyte hyperplasia in Japanese patients with tuberous sclerosis complex.
abstract: BACKGROUND
  Pulmonary involvement in tuberous sclerosis complex (TSC) includes lymphangioleiomyomatosis (LAM) and multifocal micronodular pneumocyte hyperplasia (MMPH). This retrospective study investigated pulmonary involvement in Japanese TSC patients and pulmonary function testing in TSC-LAM.
  
  
  METHODS
  The study subjects included 59 TSC patients (age range: 13-66, 15 males and 44 females). Female patients were divided into 3 groups (Group 1: symptomatic LAM, Group 2: asymptomatic LAM, Group 3: without LAM) and 3 cystic grades according to increasing cyst numbers on computed tomography images (patients without LAM, Grade 1 patients, and Grade 2+Grade 3 patients). The results of pulmonary function tests were compared among the groups and the grades.
  
  
  RESULTS
  One male (6.7%) patient and 19 female (43.2%) patients were diagnosed with LAM and 7 male (43.2%) and 23 female (52.3%) patients were diagnosed with MMPH. Patients with multiple pulmonary nodules had higher rates of renal angiomyolipoma and history of seizures than patients without nodules. Although all 6 adolescent patients displayed no pulmonary symptoms, MMPH was found in 3 patients and LAM was found in a 13-year-old girl. Carbon monoxide diffusing capacity (DLco) differed significantly among the 3 groups and DLco and carbon monoxide diffusing capacity divided by the alveolar volume (DLco/VA) differed significantly among the 3 cystic grades.
  
  
  CONCLUSIONS
  There was no difference in the prevalence of pulmonary involvement in TSC patients among countries. LAM and MMPH occur even during adolescence in TSC patients. DLco and the number of cysts are useful predictors of onset and progression of TSC-LAM.
authors.affiliation: Department of Dermatology, Course of Integrated Medicine, Graduate School of Medicine, Osaka University, 2-2 Yamada-oka, Suita, Osaka 565-0871, Japan. Electronic address: maritanaka@derma.med.osaka-u.ac.jp.
authors.forename: Mari
authors.initials: M
authors.lastname: Tanaka
authors.affiliation: Department of Respiratory Medicine, Allergy and Rheumatic Diseases, Graduate School of Medicine, Osaka University, 2-2 Yamada-oka, Suita, Osaka 565-0871, Japan. Electronic address: charhirata@imed3.med.osaka-u.ac.jp.
authors.forename: Haruhiko
authors.initials: H
authors.lastname: Hirata
authors.affiliation: Department of Dermatology, Course of Integrated Medicine, Graduate School of Medicine, Osaka University, 2-2 Yamada-oka, Suita, Osaka 565-0871, Japan. Electronic address: mkaneda@derma.med.osaka-u.ac.jp.
authors.forename: Mari
authors.initials: M
authors.lastname: Wataya-Kaneda
authors.affiliation: Department of Respiratory Medicine, Allergy and Rheumatic Diseases, Graduate School of Medicine, Osaka University, 2-2 Yamada-oka, Suita, Osaka 565-0871, Japan. Electronic address: hiroinyoshicli@gmail.com.
authors.forename: Mitsuhiro
authors.initials: M
authors.lastname: Yoshida
authors.affiliation: Department of Dermatology, Course of Integrated Medicine, Graduate School of Medicine, Osaka University, 2-2 Yamada-oka, Suita, Osaka 565-0871, Japan. Electronic address: Katayama@derma.med.osaka-u.ac.jp.
authors.forename: Ichiro
authors.initials: I
authors.lastname: Katayama
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000368:Aged; D015897:Comorbidity; D018450:Disease Progression; D005260:Female; D006801:Humans; D006965:Hyperplasia / Q000000981:diagnostic imaging; D007564:Japan / Q000453:epidemiology; D008175:Lung Neoplasms / Q000000981:diagnostic imaging / Q000453:epidemiology*; D018192:Lymphangioleiomyomatosis / Q000000981:diagnostic imaging / Q000453:epidemiology*; D008297:Male; D008875:Middle Aged; D055613:Multiple Pulmonary Nodules / Q000000981:diagnostic imaging / Q000453:epidemiology*; D015995:Prevalence; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed; D014402:Tuberous Sclerosis / Q000453:epidemiology*; D055815:Young Adult
keywords: Adolescent; Lymphangioleiomyomatosis; Multifocal micronodular pneumocyte hyperplasia; Respiratory function tests; Tuberous sclerosis complex