Natural History of Pediatric Idiopathic Histaminergic Angioedema: A Retrospective Monocentric Study
Background: Idiopathic histaminergic angioedema (IH-AAE) is a pathological entity poorly described in the literature. It overlaps with some forms of chronic urticaria, especially in pediatrics. Objective: This study is a descriptive analysis of this form of angioedema’s natural history and prognosis...
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2025-05-01
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| author | Vanessa Migliarino Alessandro Zago Camilla Martelossi Egidio Barbi Manuela Giangreco Irene Berti Laura Badina |
| author_facet | Vanessa Migliarino Alessandro Zago Camilla Martelossi Egidio Barbi Manuela Giangreco Irene Berti Laura Badina |
| author_sort | Vanessa Migliarino |
| collection | DOAJ |
| description | Background: Idiopathic histaminergic angioedema (IH-AAE) is a pathological entity poorly described in the literature. It overlaps with some forms of chronic urticaria, especially in pediatrics. Objective: This study is a descriptive analysis of this form of angioedema’s natural history and prognosis. The aim is to describe long-term data about the course of this clinical entity, including clinical presentation, recurrence, and response to therapy, emphasizing follow-up and outcome. Methods: We performed a retrospective monocentric descriptive study at the Allergy Unit, Department of Pediatrics of the Institute for Maternal and Child Health of Trieste, Italy. We selected pediatric patients (0–18 years old) visiting the outpatient clinic from January 2010 to December 2020 who received a diagnosis of IH-AAE. We analyzed the disease recurrence, the remission rate, the time and frequency of recurrences, and the body sites involved. Results: The median follow-up was 57 months. Among the 36 individuals examined at follow-up, 9 (25%) still had episodes of angioedema, while 27 (75%) reported the absence of attacks. Disease remission was established in 24 patients (66.6%). The median remission time was 13 months (IQR: 7–28). When comparing AE recurrence at onset and follow-up, in all children, the number of episodes decreased (in 4/9 patients) or remained unchanged over time (in 5/9 patients). Moreover, within this group, AE recurrence was recorded as high, intermediate, and low, respectively, in one (11.1%), two (22.2%), and six patients (66.7%). The median number of monthly episodes was one (IQR: 0.2–3), and eight was the maximum value. The initial recurrence of AE attacks has no impact on the time and rate of remission (<i>p</i> = 0.56). According to these data, 36% of the patients will go into remission in 1 year, 54% in 2 years, and 71% in 6.5 years, while 14% of the children will still present with AE after 8 years of disease. Conclusions: IH-AAE is a benign and self-limiting condition that can sometimes last several years. Over time, the number of episodes per month decreases or, at most, remains unchanged. No patients reported disease worsening. The frequency of attacks at onset does not correlate with the possibility of recovery or the remission time. |
| format | Article |
| id | doaj-art-185a4fbf315443bdb5a50ed482ec329c |
| institution | Kabale University |
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| publishDate | 2025-05-01 |
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| series | Children |
| spelling | doaj-art-185a4fbf315443bdb5a50ed482ec329c2025-08-20T03:47:48ZengMDPI AGChildren2227-90672025-05-0112560010.3390/children12050600Natural History of Pediatric Idiopathic Histaminergic Angioedema: A Retrospective Monocentric StudyVanessa Migliarino0Alessandro Zago1Camilla Martelossi2Egidio Barbi3Manuela Giangreco4Irene Berti5Laura Badina6Azienda Sanitaria Universitaria Friuli Centrale, San Daniele, 33100 Udine, ItalyDepartment of Medicine, Surgery and Health Sciences, University of Trieste, 34137 Trieste, ItalyDepartment of Internal Medicine and Medical Specialities—DIMI, University of Genoa, 16132 Genoa, ItalyDepartment of Medicine, Surgery and Health Sciences, University of Trieste, 34137 Trieste, ItalyInstitute for Maternal and Child Health-IRCCS “Burlo Garofolo”, 34137 Trieste, ItalyInstitute for Maternal and Child Health-IRCCS “Burlo Garofolo”, 34137 Trieste, ItalyInstitute for Maternal and Child Health-IRCCS “Burlo Garofolo”, 34137 Trieste, ItalyBackground: Idiopathic histaminergic angioedema (IH-AAE) is a pathological entity poorly described in the literature. It overlaps with some forms of chronic urticaria, especially in pediatrics. Objective: This study is a descriptive analysis of this form of angioedema’s natural history and prognosis. The aim is to describe long-term data about the course of this clinical entity, including clinical presentation, recurrence, and response to therapy, emphasizing follow-up and outcome. Methods: We performed a retrospective monocentric descriptive study at the Allergy Unit, Department of Pediatrics of the Institute for Maternal and Child Health of Trieste, Italy. We selected pediatric patients (0–18 years old) visiting the outpatient clinic from January 2010 to December 2020 who received a diagnosis of IH-AAE. We analyzed the disease recurrence, the remission rate, the time and frequency of recurrences, and the body sites involved. Results: The median follow-up was 57 months. Among the 36 individuals examined at follow-up, 9 (25%) still had episodes of angioedema, while 27 (75%) reported the absence of attacks. Disease remission was established in 24 patients (66.6%). The median remission time was 13 months (IQR: 7–28). When comparing AE recurrence at onset and follow-up, in all children, the number of episodes decreased (in 4/9 patients) or remained unchanged over time (in 5/9 patients). Moreover, within this group, AE recurrence was recorded as high, intermediate, and low, respectively, in one (11.1%), two (22.2%), and six patients (66.7%). The median number of monthly episodes was one (IQR: 0.2–3), and eight was the maximum value. The initial recurrence of AE attacks has no impact on the time and rate of remission (<i>p</i> = 0.56). According to these data, 36% of the patients will go into remission in 1 year, 54% in 2 years, and 71% in 6.5 years, while 14% of the children will still present with AE after 8 years of disease. Conclusions: IH-AAE is a benign and self-limiting condition that can sometimes last several years. Over time, the number of episodes per month decreases or, at most, remains unchanged. No patients reported disease worsening. The frequency of attacks at onset does not correlate with the possibility of recovery or the remission time.https://www.mdpi.com/2227-9067/12/5/600angioedemaidiopathic histaminergic acquired angioedemarecurrent angioedemaallergy |
| spellingShingle | Vanessa Migliarino Alessandro Zago Camilla Martelossi Egidio Barbi Manuela Giangreco Irene Berti Laura Badina Natural History of Pediatric Idiopathic Histaminergic Angioedema: A Retrospective Monocentric Study Children angioedema idiopathic histaminergic acquired angioedema recurrent angioedema allergy |
| title | Natural History of Pediatric Idiopathic Histaminergic Angioedema: A Retrospective Monocentric Study |
| title_full | Natural History of Pediatric Idiopathic Histaminergic Angioedema: A Retrospective Monocentric Study |
| title_fullStr | Natural History of Pediatric Idiopathic Histaminergic Angioedema: A Retrospective Monocentric Study |
| title_full_unstemmed | Natural History of Pediatric Idiopathic Histaminergic Angioedema: A Retrospective Monocentric Study |
| title_short | Natural History of Pediatric Idiopathic Histaminergic Angioedema: A Retrospective Monocentric Study |
| title_sort | natural history of pediatric idiopathic histaminergic angioedema a retrospective monocentric study |
| topic | angioedema idiopathic histaminergic acquired angioedema recurrent angioedema allergy |
| url | https://www.mdpi.com/2227-9067/12/5/600 |
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