Assessment of Treatment response to Sirolimus in pulmonary lymphangioleiomyomatosis by longitudinal quantification of cystic changes using automated post-processing of chest CT-data
Lymphangioleiomyomatosis (LAM) is a progressive disease characterized by smooth muscle-like cell proliferation resulting in thin-walled cystic changes of the lung parenchyma. Evaluation of disease activity can be done by clinical assessment, pulmonary function tests (PFT) or by computer tomography (CT) of the chest. A conventional CT-based scan assessment of the cystic changes is challenging due both to innumerability and size-variability of lung cysts that cannot be reliably counted or longitudinally compared beside the fact that LAM may be joint by both a decrease as well as an increase in lung attenuation caused by the emphysematous changes and smooth muscle-like cell proliferation, respectively, which render density measurements inaccurate for monitoring the course of the disease. In the current article a quantitative assessment of the pulmonary cystic changes is described for longitudinal evaluation of the disease response to treatment with the mammalian Target Of Rapamycin (mTOR) (Sirolimus), which is known to potentially cause regression of neoplastic growths and thus, in some cases also improve lung function.
14 May 2020 (online)
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