THBS1 mediates hypoxia driven EndMT in pulmonary hypertension

Pulmonary hypertension (PH) is a frequent complication of chronic lung disease (CLD). However, PH is difficult to diagnose early since accompanying symptoms overlap and are similar to those of the underlying CLD. In most cases the PH is mild to moderate and therefore physical signs may be absent or subtle.

To the editor,
Autoimmune pulmonary alveolar proteinosis (aPAP) is a rare disease characterized by alveolar surfactant accumulation, progressive dyspnea, and hypoxemic respiratory insufficiency, and in some patients, secondary infections, pulmonary fibrosis, respiratory failure, and death.1 GM-CSF (granulocyte/macrophage colony-stimulating factor) autoantibodies cause aPAP by blocking GM-CSF signaling, which impairs alveolar macrophage functions including surfactant clearance.

The PERFECT study, a randomized, controlled, double-blind study of inhaled treprostinil in patients with COPD and associated pulmonary hypertension (PH-COPD) was a negative trial that was terminated early. The reason(s) for the negative outcome remains uncertain.

Tuberculosis (TB) may cause significant long-term cardiorespiratory complications, of which pulmonary vascular disease is most under-recognized. TB is rarely listed as a cause of pulmonary hypertension (PH) in most PH guidelines, yet PH may develop at various stages in the time course of TB, from active infection through to the post-TB period.