Serum endostatin levels are associated with diffusion capacity and with tuberous sclerosis- associated lymphangioleiomyomatosis | Orphanet Journal of Rare Diseases | Full Text

Serum endostatin levels are associated with diffusion capacity and with tuberous sclerosis- associated lymphangioleiomyomatosis | Orphanet Journal of Rare Diseases | Full Text

Orphanet Journal of Rare Diseases

Serum endostatin levels are associated with diffusion capacity and with tuberous sclerosis- associated lymphangioleiomyomatosis

• Anthony M. Lamattina,
• Sergio Poli,
• Pranav Kidambi,
• Shefali Bagwe,
• Andrew Courtwright,
• Pierce H. Louis,
• Shikshya Shrestha,
• Benjamin Stump,
• Hilary J. Goldberg,
• Elizabeth A. Thiele,
• Ivan Rosas,
• Elizabeth P. Henske and
• Souheil El-ChemalyEmail author

Orphanet Journal of Rare Diseases201914:72

https://doi.org/10.1186/s13023-019-1050-4

©  The Author(s). 2019

• Received: 15 January 2019
• Accepted: 19 March 2019
• Published: 29 March 2019

Abstract

Endostatin is a naturally occurring collagen fragment with anti-angiogenic properties. We investigated the association between serum endostatin levels and DLCO in a cohort of patients with lymphangioleiomyomatosis (LAM). Associations of endostatin levels to clinical features of LAM were explored using logistic regression models. Endostatin levels were associated with DLCO and were higher in subjects with TSC-associated LAM compared to sporadic LAM. These data suggest that endostatin could be a predictive biomarker of decline in DLCO and that germline mutational inactivation of the TSC1 or TSC2 gene is associated with higher endostatin levels. These findings could offer novel insights into the pathogenesis of LAM.

Keywords

• LAM
• TSC
• Endostatin
• DLCO
• Isolated decrease