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Semin Respir Crit Care Med 2012; 33(05): 486-497
DOI: 10.1055/s-0032-1325159
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Lymphangioleiomyomatosis: New Concepts in Pathogenesis, Diagnosis, and Treatment

Riffat Meraj
1   Division of Pulmonary, Critical Care, and Sleep Medicine, Department of Internal Medicine, University of Cincinnati School of Medicine, Cincinnati, Ohio
,
Kathryn A. Wikenheiser-Brokamp
2   Pathology and Laboratory Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio
3   Pathology and Laboratory Medicine, University of Cincinnati School of Medicine, Cincinnati, Ohio
,
Lisa R. Young
4   Division of Pediatric Pulmonary Medicine, Department of Pediatrics, Vanderbilt University, Nashville, Tennessee
,
Francis X. McCormack
1   Division of Pulmonary, Critical Care, and Sleep Medicine, Department of Internal Medicine, University of Cincinnati School of Medicine, Cincinnati, Ohio
› Author Affiliations
Further Information

Publication History

Publication Date:
21 September 2012 (online)

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Abstract

Lymphangioleiomyomatosis (LAM) is a slowly progressive lung disease that is associated with mutations in tuberous sclerosis complex genes, infiltration of the pulmonary parenchyma and lymphatics with neoplastic smooth muscle cells, extensive tissue remodeling and architectural distortion of the lung, and tumors of the chest and abdomen, including lymphangiomyomas and angiomyolipomas. LAM occurs in women in the general population and in patients of both genders with tuberous sclerosis. Overt clinical manifestations of LAM occur almost exclusively in females, however, and include progressive dyspnea on exertion, recurrent pneumothorax, and chylous effusions. The molecular basis of LAM has been extensively characterized over the past decade, resulting in the development of a targeted therapy. This article reviews emerging approaches to the diagnosis and treatment of LAM.

Keywords

lymphangioleiomyomatosis - lymphangiomyomas - angiomyolipomas - tuberous sclerosis
 

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