Background: Angioedema (AE) is a potentially life-threatening condition with hereditary (HAE), acquired (AAE), or iatrogenic causes. A careful workup allows for the identification of the etiology of attacks and the appropriate management. In this cohort study, based on a clinical practice setting, we aimed at investigating clinical and laboratory findings concerning different features of patients with recurrent AE who were referred to a single, tertiary-level center for HAE. Methods: Clinical and laboratory data of patients fulfilling the criteria for C1-inhibitor-deficient HAE (C1-INH-HAE), C1-INH-AAE, angiotensin-converting enzyme inhibitor-related AE (ACEI-RA), and idiopathic AAE (I-AAE) were evaluated. Descriptive statistics were analyzed by means of the Mann-Whitney U test. The Fisher exact test was used for group comparisons. Results: Patients were diagnosed with type 1 HAE (n = 14), type 2 HAE (n = 1), C1-INH-AAE (n = 8), ACEI-RA (n = 16), or I-AAE (n = 26). We included only patients with concomitant autoimmune diseases from the I-AAE group (n = 8, aut-I-AAE). Age at disease onset and at diagnosis was younger in type 1 HAE than in all the other groups. The diagnostic delay was longer in type 1 HAE than in ACEI-RA. C4 and C1q levels were lower in C1-INH-AAE than in type 1 HAE, ACEI-RA, and aut-I-AAE. Both HAE and C1-INH-AAE showed lower C1-INH antigen and function compared to the other groups. Peripheral attacks were more frequent in type 1 HAE, while airway, abdominal, and oral attacks were prevalent in C1-INH-AAE. Conclusion: Investigating the clinical and laboratory features of recurrent AE without wheals represents a major topic for facilitating early diagnosis and improving treatment strategies for this heterogeneous and misdiagnosed condition.

Triggianese, P., Guarino, M.d., Pellicano, C., Borzi, M., Greco, E., Modica, S., et al. (2017). Recurrent Angioedema: Occurrence, Features, and Concomitant Diseases in an Italian Single-Center Study. INTERNATIONAL ARCHIVES OF ALLERGY AND IMMUNOLOGY, 172(1), 55-63 [10.1159/000453663].

Recurrent Angioedema: Occurrence, Features, and Concomitant Diseases in an Italian Single-Center Study

TRIGGIANESE, PAOLA;GUARINO, MARIA DOMENICA;BORZI, MAURO;GRECO, ELISABETTA;DE CAROLIS, CATERINA;PERRICONE, ROBERTO
2017

Abstract

Background: Angioedema (AE) is a potentially life-threatening condition with hereditary (HAE), acquired (AAE), or iatrogenic causes. A careful workup allows for the identification of the etiology of attacks and the appropriate management. In this cohort study, based on a clinical practice setting, we aimed at investigating clinical and laboratory findings concerning different features of patients with recurrent AE who were referred to a single, tertiary-level center for HAE. Methods: Clinical and laboratory data of patients fulfilling the criteria for C1-inhibitor-deficient HAE (C1-INH-HAE), C1-INH-AAE, angiotensin-converting enzyme inhibitor-related AE (ACEI-RA), and idiopathic AAE (I-AAE) were evaluated. Descriptive statistics were analyzed by means of the Mann-Whitney U test. The Fisher exact test was used for group comparisons. Results: Patients were diagnosed with type 1 HAE (n = 14), type 2 HAE (n = 1), C1-INH-AAE (n = 8), ACEI-RA (n = 16), or I-AAE (n = 26). We included only patients with concomitant autoimmune diseases from the I-AAE group (n = 8, aut-I-AAE). Age at disease onset and at diagnosis was younger in type 1 HAE than in all the other groups. The diagnostic delay was longer in type 1 HAE than in ACEI-RA. C4 and C1q levels were lower in C1-INH-AAE than in type 1 HAE, ACEI-RA, and aut-I-AAE. Both HAE and C1-INH-AAE showed lower C1-INH antigen and function compared to the other groups. Peripheral attacks were more frequent in type 1 HAE, while airway, abdominal, and oral attacks were prevalent in C1-INH-AAE. Conclusion: Investigating the clinical and laboratory features of recurrent AE without wheals represents a major topic for facilitating early diagnosis and improving treatment strategies for this heterogeneous and misdiagnosed condition.
Pubblicato
Rilevanza internazionale
Articolo
Esperti anonimi
Settore MED/16 - Reumatologia
English
Con Impact Factor ISI
Angioedema; Autoimmunity; Bradykinin; Complement deficiency; Lymphoproliferation; Serum complement activity; Angioedemas, Hereditary; Angiotensin-Converting Enzyme Inhibitors; Bradykinin; Cohort Studies; Complement C1 Inactivator Proteins; Complement C1 Inhibitor Protein; Early Diagnosis; Humans; Italy; Recurrence; Immunology and Allergy; Immunology
http://www.karger.com/iaa
Triggianese, P., Guarino, M.d., Pellicano, C., Borzi, M., Greco, E., Modica, S., et al. (2017). Recurrent Angioedema: Occurrence, Features, and Concomitant Diseases in an Italian Single-Center Study. INTERNATIONAL ARCHIVES OF ALLERGY AND IMMUNOLOGY, 172(1), 55-63 [10.1159/000453663].
Triggianese, P; Guarino, Md; Pellicano, C; Borzi, M; Greco, E; Modica, S; DE CAROLIS, C; Perricone, R
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2108/179981
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