title: Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guidelines: Lymphangioleiomyomatosis Diagnosis and Management.
abstract: Lymphangioleiomyomatosis (LAM) is a rare cystic lung disease that primarily affects women. The purpose of these guidelines is to provide recommendations for the diagnosis and treatment of LAM.
  
  
  
  Systematic reviews were performed to summarize evidence pertinent to our questions. The evidence was summarized and discussed by a multidisciplinary panel. Evidence-based recommendations were then formulated, written, and graded using the Grading of Recommendations, Assessment, Development, and Evaluation approach.
  
  
  
  After considering the panel's confidence in the estimated effects, the balance of desirable (i.e., benefits) and undesirable (i.e., harms and burdens) consequences of treatment, patient values and preferences, cost, and feasibility, recommendations were formulated for or against specific interventions. These included recommendations for sirolimus treatment and vascular endothelial growth factor D testing and recommendations against doxycycline and hormonal therapy.
  
  
  
  Evidence-based recommendations for the diagnosis and treatment of patients with LAM are provided. Frequent reassessment and updating will be needed.
authors.forename: Francis X
authors.identifier: 0000-0001-7168-9464
authors.initials: FX
authors.lastname: McCormack
authors.forename: Nishant
authors.initials: N
authors.lastname: Gupta
authors.forename: Geraldine R
authors.initials: GR
authors.lastname: Finlay
authors.forename: Lisa R
authors.initials: LR
authors.lastname: Young
authors.forename: Angelo M
authors.initials: AM
authors.lastname: Taveira-DaSilva
authors.forename: Connie G
authors.initials: CG
authors.lastname: Glasgow
authors.forename: Wendy K
authors.initials: WK
authors.lastname: Steagall
authors.forename: Simon R
authors.initials: SR
authors.lastname: Johnson
authors.forename: Steven A
authors.initials: SA
authors.lastname: Sahn
authors.forename: Jay H
authors.initials: JH
authors.lastname: Ryu
authors.forename: Charlie
authors.initials: C
authors.lastname: Strange
authors.forename: Kuniaki
authors.initials: K
authors.lastname: Seyama
authors.forename: Eugene J
authors.initials: EJ
authors.lastname: Sullivan
authors.forename: Robert M
authors.initials: RM
authors.lastname: Kotloff
authors.forename: Gregory P
authors.initials: GP
authors.lastname: Downey
authors.forename: Jeffrey T
authors.initials: JT
authors.lastname: Chapman
authors.forename: MeiLan K
authors.initials: MK
authors.lastname: Han
authors.forename: Jeanine M
authors.initials: JM
authors.lastname: D'Armiento
authors.forename: Yoshikazu
authors.initials: Y
authors.lastname: Inoue
authors.forename: Elizabeth P
authors.initials: EP
authors.lastname: Henske
authors.forename: John J
authors.initials: JJ
authors.lastname: Bissler
authors.forename: Thomas V
authors.initials: TV
authors.lastname: Colby
authors.forename: Brent W
authors.initials: BW
authors.lastname: Kinder
authors.forename: Kathryn A
authors.initials: KA
authors.lastname: Wikenheiser-Brokamp
authors.forename: Kevin K
authors.initials: KK
authors.lastname: Brown
authors.forename: Jean F
authors.initials: JF
authors.lastname: Cordier
authors.forename: Cristopher
authors.initials: C
authors.lastname: Meyer
authors.forename: Vincent
authors.initials: V
authors.lastname: Cottin
authors.forename: Jan L
authors.initials: JL
authors.lastname: Brozek
authors.forename: Karen
authors.initials: K
authors.lastname: Smith
authors.forename: Kevin C
authors.initials: KC
authors.lastname: Wilson
authors.forename: Joel
authors.initials: J
authors.lastname: Moss
mesh_terms: D001706:Biopsy; D005260:Female; D006801:Humans; D008168:Lung / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology; D018192:Lymphangioleiomyomatosis / Q000175:diagnosis* / Q000503:physiopathology / Q000628:therapy; D008297:Male; D020123:Sirolimus / Q000627:therapeutic use; D014057:Tomography, X-Ray Computed; D042643:Vascular Endothelial Growth Factor D / Q000097:blood
chemical_list: C579459:VEGFD protein, human; D042643:Vascular Endothelial Growth Factor D; D020123:Sirolimus