Figure 8. Schematic representation of respiratory muscle failure in CF.

A greater inherent sensitivity of CF muscle cells to pro-inflammatory stimuli, when combined with the higher levels of pulmonary and systemic inflammation found in CF, leads to exaggerated NF-kB and cytokine activation within diaphragmatic muscle fibers. This in turn provokes diaphragmatic wasting and weakness in CF, thus contributing to the pathogenesis of respiratory failure.