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Vitamin D binding protein: a new biomarker of disease severity in lymphangioleiomyomatosis

Lymphangioleiomyomatosis (LAM) is a rare destructive pulmonary neoplasm that occurs primarily in women, with onset typically during the child-bearing years. LAM is caused by hyperproliferation of abnormal smooth muscle-like “LAM cells” carrying inactivating mutations in the tumour suppressor genes encoding tuberous sclerosis complex protein 1 or 2 (TSC1 or TSC2) [1–4]. LAM cells are believed to destroy the surrounding lung parenchyma, leading to cystic lung destruction, pneumothorax and progressive lung function decline [5]. A pivotal clinical trial has demonstrated that rapamycin (sirolimus) can stabilise lung function in LAM [6]. Vitamin D binding protein is a novel biomarker of disease severity in LAM http://ow.ly/qGdc30ml0EE