Manifestations respiratoires dans le syndrome d'Ehlers-Danlos (SED). Nouveaux apports thérapeutiques

The respiratory pathophysiology and oxygenation take up a very important space in the symptomatology as well as the treatment of Ehlers-Danlos syndrome. This aspect had been neglected and nearly forgotten in the initial descriptions of EDS, which had been dominated by articular hypermobility and skin hyperextensibility. The data presented here is part of a cohort study, initiated 17 years ago, counting today 2450 cases of EDS. The most frequent respiratory tract related symptoms are dyspnea (76%), inspiratory bradypnea (64%), bronchitis (64%), upper respiratory tract infections, especially in children (74%), dysphonia, false passages, especially salivary. Intercostal pain (71%), which seems to be at the origin of respiratory crises, reacts well to local injections of Lidocaine. Persistent coughs as well as hick-ups may be observed together with gastro-esophageal reflux (72% of cases), as well as, infrequently, pneumothorax. These last ones are the consequence of tissue fragility, which becomes more frequent with endoscopies. The other manifestations are the consequence of generalized troubles with proprioception characterising Ehlers-Danlos syndrome by the absence or the disinformation of signalling from receptors, mainly mecanoreceptors, bedded in less reactive, read mutated, connective tissue, towards the respiratory centres. A weak abdominal wall that offers little resistance to the contracting diaphragm and frequently disturbed by meteorism adds its proper effects to the problem. Preventive treatments are streamlined towards the gastrointestinal system and thoracic area (wearing compressive clothing that acts on proprioception, respiratory exercises, singing). Daily or several times per week oxygen and percussionaire therapy have considerably improved the respiratory symptoms and have a remarkable effect on fatigue and migraines. This was shown by a retrospective study on 100 cases and a prospective study on 30 cases with a very significant improvement in 47% cases for the fatigue and 57% of cases of the migraine symptomatology.