[Lymphangioleiomyomatosis - report of three cases].

Rev Port Pneumol

Pulmonology, Hospitais da Universidade de Coimbra, Av. Bissaya Barreto e Praceta Prof. Mota Pinto, 3000 -075 Coimbra.

Published: April 2010

AI Article Synopsis

  • Pulmonary lymphangioleiomyomatosis (LAM) is a rare lung disease marked by abnormal smooth muscle cell growth, mainly affecting women of reproductive age and often linked to tuberous sclerosis complex (TSC).
  • The article discusses three female patients diagnosed with LAM through clinical and imaging methods.
  • Additionally, it provides a concise overview of the disease's characteristics and associations.

Article Abstract

Pulmonary lymphangioleiomyomatosis (LAM) is a rare disease of unknown aetiology. It is characterized by proliferation of abnormal smooth -muscle cells throughout the peribronchial, perivascular and perilymphatic regions of the lung. LAM may occur sporadically, in association with tuberous sclerosis complex (TSC) or inheritable multiorgan hamartomatosis. In either situation, LAM occurs almost exclusively in women of reproductive age, and approximately one third of the patients with TSC have LAM2. The authors review the cases of three female patients diagnosed with LAM based on clinical and radiological findings. A brief review of the disease is then presented.

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http://dx.doi.org/10.1016/s0873-2159(15)30017-9DOI Listing

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