Ehlers-Danlos and respiratory function. Clinical data on a cohort of 5,700 patients: oxygen therapy and physical rehabilitation medicine (P.R.M.)

Abstract

Ehlers-Danlos syndrome (EDS) appears today to be very common but is still, paradoxically, considered as rare because of a very scarce and belated diagnosis. Respiratory manifestations (exertional dyspnea, painful inspiratory bradypnea and sleep apnea are among the poorly understood manifestations of this diffuse inherited collagen disease. They can cause diagnostic errors, especially with asthma, which can lead to worsening, in these patients with very fragile tissues (including bones), contraindicating corticosteroid therapy. EDS should be systematically discussed in case of respiratory disorders in children but also in adults. There are very beneficial effects observed with oxygen therapy which considerably helps with symptoms such as fatigue, migraine, cognition, by correcting cerebral anoxia which accompanies it. Pneumologists should be made aware of the benefits of oxygen therapy when faced with an EDS patient as they may have to prescribe it.