Ten percent of patients with chronic spontaneous urticaria (CSU) have isolated angioedema. Although, antileukotriene agents are beneficial in NSAID-intolerant CSU patients, the evidence for benefit in other CSU patients is mixed. We assessed the efficacy of antileukotrienes in CSU-isolated angioedema phenotype.
Retrospective review of CSU-isolated angioedema patients evaluated at Johns Hopkins Allergy Clinic between October 2008 and April 2017. Each patient’s symptoms before and after LTRA treatment was graded on a 7-point scale based on frequency and severity of episodes.
46 patients were identified and 17 were lost to follow-up after the initial visit. Of the 29 patients included in the study, mean age was 57 years, and 16 (55%) were female. 24 (83%) had received antileukotriene agent in conjunction with antihistamines. 17 (71%) of these 24 patients had sought urgent treatment for an episode of swelling while 2 (7%) had been intubated due to swelling prior to the initial visit. 22 (92%) had significant improvement in their symptoms following addition of anti-leukotriene agent (p<0.0001) with 15 (62.5%) having had complete resolution of symptoms over the follow-up period ranging 4 months to 7 years. Of the 22 who had response to antileukotriene agent, only 5 (22%) had reported NSAID-intolerance. 1 of the patients with no response to antileukotriene had response to doxepin while the other was placed on anti-immunoglobulin E therapy.
In patients with CSU-isolated angioedema, antileukotriene therapy appear to control symptoms when added to anti-histamines. However, larger prospective studies are needed to validate these results and identify predictive markers.