Arthritis & Rheumatism, Volume 63,
November 2011 Abstract Supplement
Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Chicago, Illinois November 4-9, 2011.
Children with Ehlers-Danlos Syndrome and Airway Dysfunction.
Khiani1, Sanjay J., Lowe1, Sara, Zacharisen1, Michael, Vora2, Sheetal
Adults with hypermobility-type syndromes such as Ehlers-Danlos (EDS) have multiple issues including joint hyper-extensibility, skin laxity/scarring, hearing, voice, speech, and swallowing disorders. Recent reports suggest a predominance of atopy and respiratory symptoms. Such information is not available in children. We report respiratory symptoms in children with EDS that complicates asthma or was erroneously diagnosed as asthma.
A series of 5 children with hypermobility-type EDS (1 and 3) presented to allergy/asthma clinic for evaluation of shortness of breath, chest tightness/pain, recurrent croup or chronic cough. At the discretion of their providers, evaluations included radiographic procedures, spirometry, videostroboscopy, rhinoscopy, largyngoscopy, methacholine challenge, airway fluoroscopy, and allergy testing.
5 children ages 914 years, 3 females, 2 siblings, all had recurrent respiratory symptoms and previously diagnosed as asthma. All had normal lung function with FEV1 96124% of predicted. Only 2 had asthma confirmed with either reversible airway obstruction with albuterol or exercise pulmonary function test. One of these had 50% tracheal collapse and compression by aorta presenting as stridor. Two patients had environmental allergies confirmed by specific in vitro IgE or skin testing. Three patients had paradoxical vocal cord fold motion abnormalities on direct visualization with or without videostroboscopy. One had obstructive sleep apnea with 100% choanal obstruction by adenoid hypertrophy. Four of 5 had been diagnosed with GE reflux and 3 of these patients had g-tube placement and surgical intervention. Two patients had psychological issues such as anxiety and conversion disorder.
Children with EDS can have recurrent respiratory symptoms due to many etiologies including paradoxical vocal cord dysfunction, airway compression which may be erroneously attributed to asthma and possibly related to the underlying connective tissue abnormality.
To cite this abstract, please use the following information:
Khiani, Sanjay J., Lowe, Sara, Zacharisen, Michael, Vora, Sheetal; Children with Ehlers-Danlos Syndrome and Airway Dysfunction. [abstract]. Arthritis Rheum 2011;63 Suppl 10 :2044