2026-04-17T06:01:47Zhttps://recercat.cat/oai/request

oai:recercat.cat:11351/116752024-10-31T15:17:02Zcom_2072_378070com_2072_378040col_2072_378092

00925njm 22002777a 4500 dc Bigas, Patricia author Pereira González, Javier author Gil Serrano, Ingrid Johana author Labrador-Horrillo, Moises author GALVÁN BLASCO, PAULA author Sala Cunill, Anna author Luengo Sánchez, Olga author Cardona, Victoria author Guilarte, Mar author 2024-07-08T05:53:16Z 2024-07-08T05:53:16Z 2024-06-17 Acute phase reactants; Hereditary angioedema; Inflammation Reactants de fase aguda; Angioedema hereditari; Inflamació Reactantes de fase aguda; Angioedema hereditario; Inflamación Background: Hereditary angioedema (HAE) is a rare disease characterized by localized and self-limited angioedema (AE) attacks. A local increase of bradykinin (BK) mediates AE attacks in HAE, however the role of inflammation in HAE has been poorly explored We aim to analyze the role of inflammatory mediators in HAE patients during AE attacks. Methods: Patients with a confirmed HAE diagnosis due to C1 inhibitor deficiency (HAE-C1INH) or patients F12 gene mutations (HAE-FXII) attending to our outpatient clinic between November-2019 and May-2022 were included. Demographic and clinical characteristics were analyzed. Blood samples were collected both during symptom-free periods (baseline) and during HAE attacks, and acute phase reactants (APR), such as serum amyloid A (SAA), erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), D-Dimer and white blood cells were measured. Results: Seventy-eight patients were enrolled in the study, with a predominant representation of women (76%, n=59), and a mean age of 47.8 years (range 6–88). Among them, 67% (n=52) of patients had HAE-C1INH (46 classified as type 1 and 6 as type 2) while 33% (n=26) had HAE-FXII. During attack-free periods, the majority of patients exhibited normal levels of SAA, ESR, D-dimer, ACE and WCC. However, in a subset of patients (16% for SAA, 18% for ESR, and 14.5% for D-dimer), elevations were noted at baseline. Importantly, during HAE attacks, significant increases were observed in SAA in 88% of patients (p< 0.0001 vs. baseline), in ESR in 65% (p= 0.003 vs. baseline) and D-dimer in 71% (p=0.001 vs. baseline) of the patients. A comparison between baseline and acute attack levels in 17 patients revealed significant differences in SAA AA (p<0. 0001), ESR (p<0.0001) and D-dimer (p= 0.004). No significant differences were observed in CRP (p=0.7), ACE (p=0.67) and WCC (p=0.54). These findings remained consistent regardless of HAE type, disease activity or location of angioedema. Conclusion: The systemic increase in APR observed during HAE attacks suggests that inflammation extends beyond the localized edematous area. This finding underscores the potential involvement of inflammatory pathways in HAE and highlights the need for further investigation into their role in the pathophysiology of HAE. The author(s) declare financial support was received for the research, authorship, and/or publication of this article. Supported by Fondo de Investigación Sanitaria, Instituto de Salud Carlos III, Subdirección General de Investigación Sanitaria, Ministerio de Economía y Competitividad: PI20/01061 (MG). http://hdl.handle.net/11351/11675 Marcadors bioquímics Inflamació Malalties congènites Edema CHEMICALS AND DRUGS::Biological Factors::Biomarkers DISEASES::Congenital, Hereditary, and Neonatal Diseases and Abnormalities::Genetic Diseases, Inborn::Angioedemas, Hereditary Other subheadings::Other subheadings::/diagnosis CHEMICALS AND DRUGS::Amino Acids, Peptides, and Proteins::Peptides::Serpins::Complement C1 Inactivator Proteins::Complement C1 Inhibitor Protein DISEASES::Pathological Conditions, Signs and Symptoms::Pathologic Processes::Inflammation COMPUESTOS QUÍMICOS Y DROGAS::factores biológicos::biomarcadores ENFERMEDADES::enfermedades y anomalías neonatales congénitas y hereditarias::enfermedades genéticas congénitas::angioedemas hereditarios Otros calificadores::Otros calificadores::/diagnóstico COMPUESTOS QUÍMICOS Y DROGAS::aminoácidos, péptidos y proteínas::péptidos::serpinas::proteínas inactivadoras del complemento C1::proteína inhibidora del complemento C1 ENFERMEDADES::afecciones patológicas, signos y síntomas::procesos patológicos::inflamación Systemic inflammation biomarkers during angioedema attacks in hereditary angioedema