Table 3.

Management of Common Respiratory Manifestations in EDS and HSD.

Respiratory manifestations	Management strategies
Dyspnea	Physical activity and exercise, compression vests and garments, and management of dysautonomia
Cough	Management of rhinitis, MCAS, and gastroesophageal reflux disease 18 , 20
Pneumothorax	Intrapleural drain, needle aspiration, pleural rubbing, chemical pleurodesis, total pleural covering techniques, and bullectomy 13,21 –23
Hemothorax	Pleural drainage and vascular embolization 25 Celiprolol to reduce vascular stress in vEDS 26 Lifestyle changes to minimize risk of trauma, individualized emergency plans, centralized management, and blood pressure control Tranexamic acid (for recurrent hemoptysis) 44 Annual monitoring of the vascular tree utilizing ultrasound, CT-arteriography and nuclear magnetic resonance 27
Tissue Fragility and Risk	Avoid use of fluoroquinolones, bronchoscopy, arterial blood gases, and careful discussion regarding surgery
Chest Wall Abnormalities	Physiotherapy, compression vests/garments to improve postural stability
Respiratory Muscle Weakness	Inspiratory muscle training 15
Diaphragmatic Rupture	Surgical correction of diaphragmatic rupture/reduction of herniation of abdominal contents 28,29 Aggressive management: oxygenation, ventilation, and fluid resuscitation 30
Lung Herniation	Thoracic surgical repair of herniation zone 31
Asthma	Review of symptoms, risk factors and triggers (i.e. smoking, allergens, etc.) Evaluation of comorbidities (i.e. obesity, GERD, OSA, MCAS, etc.) Physical activity Action Plan
Mast Cell Activation Syndrome (MCAS)	Identify potential symptom triggers (i.e. dietary, chemicals, allergens) 32 Identify other triggers: alcohol, heat, radiocontrast dye, physical stimuli, exercise, and emotional stress Desensitization therapy and pharmacological therapy (i.e., H1 and H2 antihistamines, sodium cromoglycate, ketotifen, omalizumab and leukotriene receptor blockers)
Dysphonia	Speech therapy 9 Exercises to strengthen vocal cords
Tracheal Stenosis	Surgical correction 33 Mechanical dilation 34
Obstructive Sleep Apnea	Continuous Positive Airway Pressure
Hematoma Cavitary Lesions Fibrous Nodules	Conservative approach with observation