The Journal of Allergy and Clinical Immunology: In Practice
Original ArticleDiagnosis, Course, and Management of Angioedema in Patients With Acquired C1-Inhibitor Deficiency
Section snippets
Patients
Patients diagnosed with C1-INH-AAE from 1976 and followed until 2015 were included in this study. Diagnosis was based on personal history of recurrent angioedema without wheals and decreased plasma levels of C1-INH antigen and/or functional activity of C1-INH and C4 antigen below 50% of normal, similar to patients with C1-INH-HAE type I/II.13 Differently from C1-INH-HAE, all patients with C1-INH-AAE had no family history of angioedema and a later onset of symptoms (after age 40 years). When
Results
From 1976 until 2015, 77 patients (58% females) were diagnosed with C1-INH-AAE at our angioedema center in Milan. During the same period, 675 patients were diagnosed with C1-INH-HAE, giving a relative frequency of 1 patient with C1-INH-AAE for every 8.8 patients with C1-INH-HAE. Median (interquartile range) age was 70 years (63.5-77.5) and the median (interquartile range) delay between symptom onset and diagnosis was 2 years (1.0-5.0) (Table I).
Lymphoproliferative neoplasms were present in 24
Discussion
This is the first study from Italy to examine the incidence and clinical characteristics in a large group of patients with C1-INH-AAE who were diagnosed and followed at a single center for nearly 40 years. On comparing the incidence of newly diagnosed acquired versus hereditary C1-INH deficiency in this center from 1975 to 2015, we found 1 patient with C1-INH-AAE for every 8.8 patients with C1-INH-HAE. We do not know how often this condition remains unrecognized; therefore, we cannot estimate
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This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.
Conflicts of interest: S. Caccia has received research support from Cariplo Foundation. M. Cicardi is on the boards for Shire/Dyax, CSL Behring, Alnylam, and SOBI; has received research support and payment for developing educational presentations from Shire; and has received lecture fees from Shire, SOBI, and CSL Behring. The rest of the authors declare that they have no relevant conflicts of interest.