Atypical Cardiopulmonary Manifestations in pediatric Behçet`s Disease
2Rappaport Faculty of Medicine, Technion–Israel Institute of Technology
3Pediatric Radiology Unit, Rappaport Children's Hospital, Rambam Health Care Campus
4Pediatric Cardiology Institute, Rappaport Children's Hospital, Rambam Health Care Campus
5Pediatric Rheumatology Unit, Rappaport Children's Hospital, Rambam Health Care Campus
Background: Behcet`s disease (BD) is a multisystem auto inflammatory disease of unknown etiology. Cardio-pulmonary involvement is rare, especially in young patients and carries high morbidity and mortality rates.
Aim: Out of 26 patients with pediatric BD enlisted in our center, we encountered three patients with severe atypical cardiorespiratory presentations. Our aim was to describe the manifestations and course that led to diagnosis of BD.
Results: Three adolescents presented with intracardiac thrombi causing myocardial infarction, pulmonary artery aneurysm with pulmonary embolism (PE) in situ, and suspected epiglottitis, respectively. In two patients the diagnosis of BD was delayed. All three patients had a good response to anti-inflammatory agents.
Conclusions: Pediatric BD is associated with atypical pulmonary manifestations which may be life-threatening. Diagnosis may be challenging; a high index of suspicion is needed, especially in young patients, to promptly diagnose and treat these complications. Cardiopulmonary manifestations, though uncommon, may be the first manifestation and the clue to diagnosis in this rare disease