Life expectancy in Italian patients with hereditary angioedema due to C1-inhibitor deficiency

Perego, Francesca; Gidaro, Antonio; Zanichelli, Andrea; Cancian, Mauro; Arcoleo, Francesco; Senter, Riccardo; Bova, María; Pasquale, Tiziana De; Guarino, Maria Domenica; Pizzo, Mariangela Lo; Frigerio, Chiara; Duca, P.; Cicardi, Marco; Itaca,

doi:10.1016/j.jaip.2020.01.007

Cited by 8 publications

(9 citation statements)

References 8 publications

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“…The study showed that mortality rate among Italian patients with HAE did not differ from that of the general population and that the use of attenuated androgens was not associated with increased prevalence of neoplasms. None of the 5 patients with liver cancer in their study had been on attenuated androgens [8]. Although the number of patients was small, these findings were reassuring to those of us who have limited access to other options of prophylactic therapy for HAE patients.…”

Section: Discussion/conclusionmentioning

confidence: 83%

“…Despite the favorable outcomes of our intervention, patients continued to present severe attacks, albeit at a significant lower frequency. In a recent publication, Perego et al [8] reported on mortality rates of Italian HAE patients followed at a reference center since 1973, with causes of deaths available for 88/90 HAE patients who died within this period. The study showed that mortality rate among Italian patients with HAE did not differ from that of the general population and that the use of attenuated androgens was not associated with increased prevalence of neoplasms.…”

Section: Discussion/conclusionmentioning

confidence: 99%

“…Hereditary angioedema (HAE) is a rare disease with autosomal dominant inheritance characterized by recurrent episodes of angioedema of subcutaneous tissue, gastrointestinal tract, and upper airways [1-5]. Angioedema may be life-threatening due to potential obstruction of upper airways, leading to death by asphyxia [6-8]. Recurrent angioedema episodes often result in significant burden to patients and families, including increased risk for mental disorders [9-14].…”

Section: Introductionmentioning

confidence: 99%

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Decreasing Attacks and Improving Quality of Life through a Systematic Management Program for Patients with Hereditary Angioedema

Nunes

Ferriani

Moreno

et al. 2021

Int Arch Allergy Immunol

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Introduction: Prevention of attacks is a major goal in management of patients with hereditary angioedema (HAE). We aimed to investigate the effects of a systematic intervention for HAE patients. Methods: Thirty-three patients with HAE with C1-inhibitor deficiency, belonging to a single family, participated in a management program coordinated by an allergist/immunologist. Angioedema attacks before intervention were ascertained by interviews and emergency room charts and recorded prospectively by patients or caregivers after enrollment. Mean number of attacks/month was compared at 12 months preintervention and 8 and 14 months within intervention. Patient-reported outcome instruments were used to assess quality of life, including HAE Quality of Life (HAE-QoL) questionnaire, psychological conditions, and work impairment, at baseline and 8 and 14 months within intervention. Data were stored in REDCap platform and analyzed by adjusted Bayesian models of double Poisson regression. Results: Mean number of attacks/month significantly decreased (95% credible interval [CrI] excluding 0) from 1.15 preintervention to 0.25 and 0.23, 8 and 14 months within intervention, with mean decreases of −0.89 (95% CrI: −1.21 to −0.58) and −0.92 (95% CrI: −1.22 to −0.60), respectively. HAE-QoL scores showed mean total increases of 15.2 (95% CrI: 1.23–29.77) and 26 (95% CrI: 14.56–39.02) at 8 and 14 months within the study, as compared to baseline, revealing marked improvement in quality of life. Significant increase in role-emotional and reduction of depression, stress, and anxiety were observed at 14 months. Conclusion: A systematic approach integrating HAE-specific care with effective handling of psychological issues decreased the number of attacks and improved quality of life, targets for best practice in HAE.

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Section: Discussion/conclusionmentioning

confidence: 83%

Section: Discussion/conclusionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Decreasing Attacks and Improving Quality of Life through a Systematic Management Program for Patients with Hereditary Angioedema

Nunes

Ferriani

Moreno

et al. 2021

Int Arch Allergy Immunol

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“…In our center, three patients (from two families, one female, two They found that their life expectancy did not differ significantly from that of the general population; suffocation was preceded by death by malignant and cerebrovascular diseases, which could be explained by the availability of on-demand treatment. 27 The first UAE occurs at a young age, mostly in the second or third decade; however, it can also occur in children. In the literature the youngest age when UAE occurred was 3 years.…”

Section: Incidence Of Uae In Case Of Hereditary Angioedema Patients With Normal C1-inh Levelsmentioning

confidence: 99%

The characteristics of upper airway edema in hereditary and acquired angioedema with C1‐inhibitor deficiency

Balla

Andrási

Pólai

et al. 2021

Clinical & Translational All

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Background: Angioedemas localized in the upper airway are potentially life threatening, and without proper treatment, they may lead to death by suffocation.Upper airway edemas (UAE) in bradykinin-mediated angioedemas can even be the first symptoms of the disease. Methods: Our survey was performed with a retrospective long-term follow-up method from the medical history of 197 hereditary (C1-INH-HAE) and 20 acquired C1-inhibitor deficiency (C1-INH-AAE), 3 factor XII and 3 plasminogen gene mutation (FXII-HAE, PLG-HAE) patients treated at our center between 1990 and 2020. The UAE group included edemas localized to the mesopharynx, hypopharynx, and larynx, as narrowing of these anatomical regions can lead to suffocation. Results: 98/197 C1-INH-HAE (47 families) and 13/20 C1-INH-AAE, 1/3 PLG-HAE, 1/ 3 FXII-HAE patients had experienced UAE at least once according to their medical history. In case of C1-INH-HAE patients, in 6/47 families who had undiagnosed ancestors had 13 members who died of suffocation. After the diagnosis, 1-1 member of two families died of UAE. 44/64 C1-INH-HAE patients did not smoke, 20/64 did. The occurrence of UAE was significantly higher in smoker patients. We analyzed 7607 HAE attacks of 56/98 patients. Out of all attacks, the incidence of UAE in the C1-INH-HAE group was 4%, and 9.5% in the C1-INH-AAE group, respectively. Conclusion:Early diagnosis is key in bradykinin-mediated angioedemas cases, since the patient must be provided with adequate treatment; and also it is essential to inform patients about the importance of avoiding the trigger factors and the early symptoms of UAE, as these measures could significantly decrease the incidence of lethal UAEs.

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“…Vale ressaltar a variabilidade clínica da doença é um fator notável. O mesmo paciente pode passar períodos longos e variados sem crises de angioedema e então novamente apresentar sintomas graves e frequentes (PEREGO et al, 2020).…”

Section: Diagnósticounclassified

Diminuição das crises e melhora da qualidade de vida através de um programa de manejo sistemático para pacientes com angioedema hereditário

Nunes¹

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Life expectancy in Italian patients with hereditary angioedema due to C1-inhibitor deficiency

Cited by 8 publications

References 8 publications

Decreasing Attacks and Improving Quality of Life through a Systematic Management Program for Patients with Hereditary Angioedema

Decreasing Attacks and Improving Quality of Life through a Systematic Management Program for Patients with Hereditary Angioedema

The characteristics of upper airway edema in hereditary and acquired angioedema with C1‐inhibitor deficiency

Diminuição das crises e melhora da qualidade de vida através de um programa de manejo sistemático para pacientes com angioedema hereditário

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