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Loeys-Dietz Syndrome and Asthma: Pathophysiological Insights and Clinical Dilemmas
Ella Brockwell-Mole1, John D Blakey1,2,3, Vidya V Navaratnam1,2,3
1Sir Charles Gairdner Hospital, Respiratory Medicine Perth Western Australia Australia.
Abstract:
A 23-year-old man presented with a life-threatening asthma exacerbation following a respiratory infection. Although he had a childhood history of asthma, he had remained asymptomatic in adulthood and reported regular use of both vapes and marijuana. His medical history included Type 2 Loeys-Dietz syndrome (LDS), requiring multiple vascular surgeries, rendering standard asthma therapies relatively contraindicated. Due to the severity of his condition, nebulised beta-agonists were cautiously administered, and his beta-blocker therapy was substituted. Imaging and forced oscillation technique (FOT) demonstrated severe obstructive airways disease. Marked and persistent eosinophilia was noted. While asthma is a recognised comorbidity in LDS, earlier recognition and intervention, such as inhaled corticosteroids, smoking cessation support, and judicious use of beta-blockers, may have reduced the severity. Increased awareness of the prevalence and severity of asthma in LDS could support earlier diagnosis, preventive strategies and timely escalation of therapy, thereby reducing the risk of catastrophic respiratory events.
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