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Ehlers-Danlos syndromes (EDS) represent a heterogeneous group of heritable connective tissue disorders characterized by the clinical "triad" consisting in joint hypermobility, skin hyperextensibility and tissue fragility. Respiratory manifestations associated with EDS are frequent and variable. They vary mainly according to the type of EDS. In hypermobile and classical EDS, the most frequent non-vascular types, dyspnea is a common symptom. Its etiologies are wide-ranging and can coexist in the same patient: asthma, respiratory muscle weakness, chest wall abnormalities, upper and lower airway collapse. The prevalence of obstructive sleep apnea syndrome in nvEDS is high. Identification of the relevant dyspnea mechanism is essential to providing appropriate therapeutic measures. In vascular EDS (vEDS), the main pulmonary complications are pneumothorax, hemothorax and hemoptysis. As they frequently precede the diagnosis of vEDS by several years, it is imperative to raise the possibility of vEDS in a young patient with spontaneous pneumothorax or hemothorax. The presence of suggestive computed tomography parenchymal abnormalities (emphysema, clusters of calcified nodules, cavitated nodule) can be an aid to diagnosis. Treatment is based on the usual approaches, which must be carried out with caution by an experienced operator fully informed of the diagnosis. Better knowledge of respiratory manifestations of EDS by the pneumological community would improve patient care and pave the way for further research., (Copyright © 2023 SPLF. Published by Elsevier Masson SAS. All rights reserved.)

Behcet's disease (BD) is a multisystemic vasculitis involving arteries and veins of all sizes. While joint and dermatological manifestations are the most common features of BD and are associated with a good prognosis; vascular involvement, remains the principal cause of death. Arterial manifestations occur in 5-10% of cases and manifest as occlusion/thrombosis or aneurysms. Arterial aneurysms are likely multiple and the most common sites are pulmonary arteries, aorta and arteries of lower limbs. Parenchymal involvement is less frequent and may manifest as consolidation or nodules, which may evolve to excavation. Aneurysms may occur at the sites of arterial puncture; then, non-traumatic techniques are favored. Patients with arterial manifestations may present with fever and increased inflammatory markers. Artery damage is rare, serious, and may result in massive hemoptysis. The prognosis of pulmonary artery aneurysms is severe (mortality estimated up to 26%) but has been improved by earlier diagnosis and the introduction of immunosuppressants. Treatment of severe arterial manifestations is based on high-dose corticosteroids along with cyclophosphamide or anti-TNF antagonists. Anticoagulation could be added to immunosuppressants in case of venous thrombosis if a coexisting pulmonary aneurysm is ruled out. Endovascular treatment should be performed in case of severe symptomatic pulmonary aneurysms, along with an adequate medical management. Long-term maintenance therapy of these severe forms is of paramount importance because of relapse risk (40% at five years)., (Copyright © 2022 SPLF. Published by Elsevier Masson SAS. All rights reserved.)