All Outputs (8)

Cross talk between LAM cells and fibroblasts may influence alveolar epithelial cell behavior in lymphangioleiomyomatosis (2022)
Journal Article
Clements, D., Miller, S., Babaei-Jadidi, R., Adam, M., Potter, S. S., & Johnson, S. R. (2022). Cross talk between LAM cells and fibroblasts may influence alveolar epithelial cell behavior in lymphangioleiomyomatosis. AJP - Lung Cellular and Molecular Physiology, 322(2), L283-L293. https://doi.org/10.1152/AJPLUNG.00351.2021

Lymphangioleiomyomatosis (LAM) is a female-specific cystic lung disease in which tuberous sclerosis complex 2 (TSC2)-deficient LAM cells, LAM-associated fibroblasts (LAFs), and other cell types infiltrate the lungs. LAM lesions can be associated with... Read More about Cross talk between LAM cells and fibroblasts may influence alveolar epithelial cell behavior in lymphangioleiomyomatosis.

Mast Cell Tryptase Release Contributes to Disease Progression in Lymphangioleiomyomatosis (2021)
Journal Article
Babaei-Jadidi, R., Dongre, A., Miller, S., Castellanos Uribe, M., Stewart, I. D., Thompson, Z. M., …Johnson, S. R. (2021). Mast Cell Tryptase Release Contributes to Disease Progression in Lymphangioleiomyomatosis. American Journal of Respiratory and Critical Care Medicine, 204(4), 431-444. https://doi.org/10.1164/rccm.202007-2854OC

Rationale: Lymphangioleiomyomatosis (LAM) is a multisystem disease that causes lung cysts and respiratory failure. Loss of TSC (tuberous sclerosis complex) gene function results in a clone of “LAM cells” with dysregulated mTOR (mechanistic target of... Read More about Mast Cell Tryptase Release Contributes to Disease Progression in Lymphangioleiomyomatosis.

Machine learning can predict disease manifestations and outcomes in lymphangioleiomyomatosis (2020)
Journal Article
Chernbumroong, S., Johnson, J., Gupta, N., Miller, S., Mccormack, F. X., Garibaldi, J. M., & Johnson, S. R. (2021). Machine learning can predict disease manifestations and outcomes in lymphangioleiomyomatosis. European Respiratory Journal, 57(6), Article 2003036. https://doi.org/10.1183/13993003.03036-2020

Background: Lymphangioleiomyomatosis (LAM) is a rare multisystem disease with variable clinical manifestations and differing rates of progression that make management decisions and giving prognostic advice difficult. We used machine learning to ident... Read More about Machine learning can predict disease manifestations and outcomes in lymphangioleiomyomatosis.

Cross-sectional study of reversible airway obstruction in LAM: better evidence is needed for bronchodilator and inhaled steroid use (2019)
Journal Article
Johnson, J., & Johnson, S. R. (2019). Cross-sectional study of reversible airway obstruction in LAM: better evidence is needed for bronchodilator and inhaled steroid use. Thorax, 74(10), 999-1002. https://doi.org/10.1136/thoraxjnl-2019-213338

Lymphangioleiomyomatosis can be associated with reversible airflow obstruction and although no guidelines around reversibility testing or inhaled therapy exist, many patients receive bronchodilators and inhaled corticosteroids. To better identify tho... Read More about Cross-sectional study of reversible airway obstruction in LAM: better evidence is needed for bronchodilator and inhaled steroid use.

Lung function response and side effects to rapamycin for lymphangioleiomyomatosis: A prospective national cohort study (2017)
Journal Article
Bee, J., Fuller, S., Miller, S., & Johnson, S. R. (2018). Lung function response and side effects to rapamycin for lymphangioleiomyomatosis: A prospective national cohort study. Thorax, 73(4), 369-375. https://doi.org/10.1136/thoraxjnl-2017-210872

© Article author(s) 2018. Rationale Mechanistic target of rapamycin inhibitors reduce loss of lung function in lymphangioleiomyomatosis (LAM), although their benefit varies between individuals. We examined lung function response and side effects to r... Read More about Lung function response and side effects to rapamycin for lymphangioleiomyomatosis: A prospective national cohort study.

Cardiovascular and inflammatory effects of simvastatin therapy in patients with COPD: a randomized controlled trial. (2015)
Journal Article
John, M., Cockcroft, J. R., McKeever, T. M., Coward, W. R., Shale, D. J., Johnson, S. R., …Bolton, C. E. (2015). Cardiovascular and inflammatory effects of simvastatin therapy in patients with COPD: a randomized controlled trial. International Journal of Chronic Obstructive Pulmonary Disease, 10(1), https://doi.org/10.2147/COPD.S76061

BACKGROUND: There is excess cardiovascular mortality in patients with chronic obstructive pulmonary disease. Aortic stiffness, an independent predictor of cardiovascular risk, and systemic and airway inflammation are increased in patients with the... Read More about Cardiovascular and inflammatory effects of simvastatin therapy in patients with COPD: a randomized controlled trial..

A 2-year randomised placebo-controlled trial of doxycycline for lymphangioleiomyomatosis (2014)
Journal Article
Chang, W. Y., Cane, J. L., Kumaran, M., Lewis, S., Tattersfield, A. E., & Johnson, S. R. (2014). A 2-year randomised placebo-controlled trial of doxycycline for lymphangioleiomyomatosis. European Respiratory Journal, 43(4), https://doi.org/10.1183/09031936.00167413

Lymphangioleiomyomatosis (LAM) is characterised by lung cysts and airflow obstruction. Matrix metalloproteinases have been implicated in lung destruction in LAM. We performed a randomised, double-blind trial, comparing the matrix metalloproteinases i... Read More about A 2-year randomised placebo-controlled trial of doxycycline for lymphangioleiomyomatosis.

The role of inflammation resolution speed in airway smooth muscle mass accumulation in asthma: insight from a theoretical model (2014)
Journal Article

Despite a large amount of in vitro data, the dynamics of airway smooth muscle (ASM) mass increase in the airways of patients with asthma is not well understood. Here, we present a novel mathematical model that describes qualitatively the growth dynam... Read More about The role of inflammation resolution speed in airway smooth muscle mass accumulation in asthma: insight from a theoretical model.