A current viewpoint of lymphangioleiomyomatosis supporting immunotherapeutic treatment options

Daniel F. Dilling, Emily R. Gilbert, Maria M. Picken, Jonathan M. Eby, Robert B. Love, I. Caroline Le Poole*

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

15 Scopus citations


Lymphangioleiomyomatosis (LAM) leads to hyperproliferation of abnormal smooth muscle cells in the lungs, associated with diffuse pulmonary parenchymal cyst formation and progressive dyspnea on exertion. The disease targets women of child-bearing age. Complications include pneumothoraces and chylous pleural effusions. Ten year survival is estimated at 70%, and lung transplantation remains the only validated treatment. It has been observed that LAM cells express markers associated with melanocytic differentiation, including gp100 and MART-1. Other melanocytic markers have also been observed. The same proteins are targeted by T cells infiltrating melanoma tumors as well as by T cells infiltrating autoimmune vitiligo skin, and these antigens are regarded as relatively immunogenic. Consequently, vaccines have been developed for melanoma targeting these and other immunogenic melanocyte differentiation proteins. Preliminary data showing susceptibility of LAM cells to melanoma derived T cells suggest that vaccines targeting melanosomal antigens can be successful in treating LAM.

Original languageEnglish (US)
Pages (from-to)1-5
Number of pages5
JournalAmerican Journal of Respiratory Cell and Molecular Biology
Issue number1
StatePublished - Jan 1 2012


  • Immunotherapy
  • Lymphangioleiomyomatosis
  • Melanoma associated antigens
  • TSC1
  • TSC2

ASJC Scopus subject areas

  • Molecular Biology
  • Pulmonary and Respiratory Medicine
  • Clinical Biochemistry
  • Cell Biology

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