title: Prevalence and clinical features of lymphedema in patients with lymphangioleiomyomatosis.
abstract: BACKGROUND
  Lymphangioleiomyomatosis (LAM) is a rare cystic lung disease predominantly affecting young women. Some of these patients develop lymphedema of the lower extremities and buttocks; however, neither the exact frequency of LAM-associated lymphedema nor the clinical features of such patients is well delineated.
  
  
  OBJECTIVE
  To document the frequency, features, and treatment of LAM-associated lymphedema.
  
  
  METHODS
  We reviewed all medical records of patients listed in the Juntendo University LAM registry for the 30 years preceding August 2010.
  
  
  RESULTS
  Of 228 patients registered with a diagnosis of LAM, eight (3.5%) had LAM-associated lymphedema of the lower extremities. All were females with sporadic LAM, and their mean age when diagnosed was 32.5 years (range 23-44). Lymphedema of the lower extremities was the chief or a prominent presenting feature in five of these LAM patients. CT scans showed that all eight patients had enlarged lymph nodes (lymphangioleiomyomas) in the retroperitoneum and/or pelvic cavity. Yet, cystic destruction of the lungs was mild in four patients, moderate in two and severe only in two. Seven of these patients were treated by administering a fat-restricted diet and complex decongestive physiotherapy, and four received a gonadotropin-releasing hormone analog. With this combined protocol, all eight patients benefitted from complete relief or good control of the lymphedema.
  
  
  CONCLUSIONS
  Lymphedema is a rare complication of LAM and may be associated with axial lymphatic involvement or dysfunction rather than severe cystic lung destruction. The combined multimodal treatments used here effectively resolved or controlled LAM-associated lymphedema.
authors.affiliation: Division of Respiratory Medicine, Juntendo University Faculty of Medicine and Graduate School of Medicine, 2-1-1 Hongo, Bunkyo-Ku, Tokyo 113-8421, Japan. yhoshika@juntendo.ac.jp
authors.forename: Yoshito
authors.initials: Y
authors.lastname: Hoshika
authors.forename: Takako
authors.initials: T
authors.lastname: Hamamoto
authors.forename: Kayoko
authors.initials: K
authors.lastname: Sato
authors.forename: Hikaru
authors.initials: H
authors.lastname: Eto
authors.forename: Sachiko
authors.initials: S
authors.lastname: Kuriyama
authors.forename: Kaku
authors.initials: K
authors.lastname: Yoshimi
authors.forename: Shin-Ichiro
authors.initials: S
authors.lastname: Iwakami
authors.forename: Kazuhisa
authors.initials: K
authors.lastname: Takahashi
authors.forename: Kuniaki
authors.initials: K
authors.lastname: Seyama
mesh_terms: D000328:Adult; D003131:Combined Modality Therapy; D058128:Compression Bandages; D018752:Diet, Fat-Restricted; D005260:Female; D007987:Gonadotropin-Releasing Hormone / Q000031:analogs & derivatives; D006801:Humans; D035002:Lower Extremity; D008175:Lung Neoplasms / Q000150:complications*; D018192:Lymphangioleiomyomatosis / Q000150:complications*; D008209:Lymphedema / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D061305:Lymphoscintigraphy; D026741:Physical Therapy Modalities; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D007987:Gonadotropin-Releasing Hormone
keywords: Complex decongestive physiotherapy; Fat-restricted diet; Gonadotropin-releasing hormone; Lymphangioleiomyomas; Lymphedema; Lymphoscintigraphy