Bradykinin-mediated angioedema: factors prompting ED visits.

OBJECTIVE: Bradykinin-mediated angioedema (AE) is characterized by periodic subcutaneous or submucosal swelling, with the absence of urticaria and itching. It affects the face and extremities. Attacks of abdominal pain and obstruction of the upper airways are the hallmarks of disease severity. The aim of this study was to determine the factors prompting patients to turn up at the hospital emergency department (ED) in the event of an attack. METHODS: This was a retrospective review of attacks experienced by all patients with bradykinin-mediated AE (hereditary, acquired, or drug-induced) who visited our ED between January 2002 and March 2011. Characteristics of attacks prompting ED visits and attacks when patients stayed at home were compared using logistic regression with generalized estimating equations to take into account the correlation between multiple attacks from the same patient. RESULTS: A total of 55 patients were included, of whom 39 had hereditary AE; 13, drug-induced AE; and 3, acquired C1 inhibitor deficiency. The median follow-up was 32 months (interquartile range, 10-99 months). The attack was severe in 324 (58%) of 559 cases. Thirty-two patients (58%) visited the ED for a total of 75 attacks. First attack and laryngeal edema were significantly associated with more frequent ED visits (odds ratio, 5.28 [95% confidence interval, 2.42-11.5] and 4.83 [1.54-15.5], respectively]. C1 inhibitor concentrate and icatibant treatment was significantly more often administered in the ED than at home (odds ratio, 4.9 [2.43-9.88] and 8.25 [2.4-28.3], respectively). CONCLUSIONS: First attack of bradykinin AE and swelling of the larynx were significantly associated with visits to the ED.

Acquired C1-inhibitor deficiency: 7 patients treated with rituximab.

BACKGROUND: Acquired C1-inhibitor deficiency can occur secondary to excessive C1-inhibitor consumption (type I) and be associated with a lymphoid hemopathy, or linked to the presence of anti-C1-inhibitor autoantibodies (type II) in a context of an isolated monoclonal gammopathy, sometimes associated with lymphoproliferation. Efficacy of danazol, tranexamic acid and/or corticosteroids is inconstant. Rituximab efficacy against type II angioedema has been reported. METHODS: Description of 7 rituximab-treated patients, 6 with type II acquired angioedema and 1 with type I. RESULTS: Clinical efficacy (only for type II) was complete for 3, partial for 2 and 2 were therapeutic failures. Only 2 patients had improved biological parameters, with normalization of their C1-inhibitor levels and diminished anti-C1-inhibitor autoantibodies, observed 1-9 months after the last infusion of the second rituximab cycle. An associated lymphoproliferation did not affect the response to treatment. CONCLUSION: Rituximab efficacy in the treatment of acquired angioedema is inconstant and might require repeated cycles.