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Study of systemic sclerosis

Pathophysiological study of a rare disease

The Air Liquide Foundation provides support for a research project led by Professor Anh Tuan Dinh-Xuan at Cochin Hospital in partnership with Paris Descartes University. It focuses on a rare disease known as systemic sclerosis. The research examines the potential therapeutic action of enzymes involved in nitric oxide (NO) transmission on lung diseases associated with systemic sclerosis.

Systemic sclerosis

Systemic sclerosis is a rare disease of unknown cause. The prevalence of this disease is on the rise. It is characterized by a progressive thickening of the skin and visceral organs, including the lungs. Two types of lung disease have been observed: diffuse interstitial lung disease, which is caused and made worse by pulmonary inflammation, and pulmonary arterial hypertension. These lung diseases are now the leading cause of systemic sclerosis-related mortality.

Paris Descartes University

Poorly understood lung diseases

The pathophysiology of these lung diseases is only partially understood because access to human lung tissue is restricted. Therefore, Professor Dinh-Xuan's team carries out its research on a mouse model in which systemic sclerosis is induced. The team has confirmed the involvement of nitric oxide in the pathophysiological processes of systemic sclerosis. Thus, the team hopes to study the severity of the pulmonary vascular disease and test for the presence of pulmonary arterial hypertension in the animal. The researchers also hope to assess the therapeutic effect of the activation or inhibition of key enzymes involved in the transmission of nitric oxide1.

1. System for information transmission between cells

Institut Cochin

The Air Liquide Foundation's involvement

The Air Liquide Foundation is helping to fund the purchase of a device for measuring exhaled nitric oxide as well as the acquisition of the molecules and products necessary for the research. The Foundation has provided a €60,000 grant.