Lymphangioleiomyomatosis is a rare disease characterised by cystic destruction of the lung, lymphatic abnormalities and abdominal tumours. It affects almost exclusively females and can occur sporadically or in patients with tuberous sclerosis complex. In the past decade remarkable progress has been made in understanding of the pathogenesis of this disease leading to a new therapeutic approach. This review summarises recent advances regarding pathogenic mechanisms and clinical manifestations, and highlights the current and the most promising future therapeutic strategies.

The changing face of a rare disease: Lymphangioleiomyomatosis / S. Harari, O. Torre, R. Cassandro, J. Moss. - In: EUROPEAN RESPIRATORY JOURNAL. - ISSN 0903-1936. - 46:5(2015), pp. 1471-1485. [10.1183/13993003.00412-2015]

The changing face of a rare disease: Lymphangioleiomyomatosis

Harari S.;
2015

Abstract

Lymphangioleiomyomatosis is a rare disease characterised by cystic destruction of the lung, lymphatic abnormalities and abdominal tumours. It affects almost exclusively females and can occur sporadically or in patients with tuberous sclerosis complex. In the past decade remarkable progress has been made in understanding of the pathogenesis of this disease leading to a new therapeutic approach. This review summarises recent advances regarding pathogenic mechanisms and clinical manifestations, and highlights the current and the most promising future therapeutic strategies.
Settore MED/10 - Malattie dell'Apparato Respiratorio
EUROPEAN RESPIRATORY JOURNAL
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/2434/748962
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