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.
Recent advances in pathogenesis, clinical manifestations and therapeutic strategies of lymphangioleiomyomatosis http://ow.ly/Rbmlh
Previous articles in this series: No. 1: Collard HR, Bradford WZ, Cottin V, et al. A new era in idiopathic pulmonary fibrosis: considerations for future clinical trials. Eur Respir J 2015; 46: 243–249. No. 2: Ryerson CJ, Cottin V, Brown KK, et al. Acute exacerbation of idiopathic pulmonary fibrosis: shifting the paradigm. Eur Respir J 2015; 46: 512–520.
Conflict of interest: None declared.
- Received March 13, 2015.
- Accepted July 11, 2015.
- Copyright ©ERS 2015