The prevalence of hereditary angioedema in a Chinese cohort with decreased complement 4 levels

Abstract Background Hereditary angioedema (HAE) is a rare disease with potential life-threatening risks. To study the natural course of HAE under therapy-free conditions throughout patient life is essential for practitioners and patients to avoid possible risk factors and guide treatment. Objectives Describe the natural course of HAE and explore possible risk factors, providing new clues for guiding clinical prevention and treatment. Methods A web-based survey was conducted in 103 Chinese patients with type 1 HAE. Disease progression at different age stages was provided by each participant. The data for exploring the natural course of HAE composed of two parts: one came from the participants who had never adopted any prophylactic drug for HAE; the other was from the patients with a history of medication, but only the periods before they got confirmed diagnosis and received medications were analyzed. The demographic characteristics, lifestyles, disease severity, and family history were also collected. Results Among 103 patients, 14 (13.6%) had their first HAE attack before ten years old and 51 (49.5%) between 10 and 19. The disease worsened in 83.3% of the patients in their twenties. The proportion of patients with symptoms alleviated increased after the age of 30 years old, but the disease maintained relatively severe in most cases before 50. The participants also reported 233 members shared similar symptoms of angioedema in their family and 30 had died of laryngeal edema with the median death age of 46 years old. The disease severity was not observed to be affected significantly by gender, BMI, alcohol or smoking. Conclusions We summarized HAE progression patterns under therapy-free conditions, showing the natural course of HAE development along with aging. Long-term prophylaxis and symptomatic treatment are recommended for all HAE patients, especially young and middle-aged and might be adjusted depending on the disease progression.

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The Natural Course of Hereditary Angioedema in a Chinese Cohort

10.21203/rs.3.rs-25286/v1 ◽

2020 ◽

Author(s):

Yang Cao ◽

Shuang Liu ◽

Yuxiang Zhi

Keyword(s):

Risk Factors ◽

Disease Progression ◽

Disease Severity ◽

Hereditary Angioedema ◽

Symptomatic Treatment ◽

Natural Course ◽

Chinese Patients ◽

Laryngeal Edema ◽

Chinese Cohort ◽

Clinical Prevention

Abstract Background Hereditary angioedema (HAE) is a rare disease with potential life-threatening risks. Until now, few studies have focused on the natural course of HAE, which is essential for practitioners and patients to predict disease progression and avoid possible risk factors. Objectives Describe the natural course of HAE and explore possible risk factors, providing new clues for guiding clinical prevention and treatment. Methods A web-based survey was conducted in 103 Chinese patients with type 1 HAE. Disease progression under therapy-free conditions was collected at different age stages. The demographic characteristics, lifestyles, disease severity and family history were also provided by each participant. Results Among 103 patients, 14 (13.6%) had their first HAE attack before ten years old and 51 (49.5%) between 10 and 19. The disease worsened in 83.3% of the patients in their twenties. The proportion of patients with symptoms alleviated increased after the age of 30 years old, but the disease maintained relatively severe in most cases before 50. The participants also reported 233 members shared similar symptoms of angioedema in their family and 30 had died of laryngeal edema with the median death age of 46 years old. The disease severity was not observed to be affected significantly by gender, BMI, alcohol or smoking. Conclusions We summarized HAE progression patterns in therapy-free conditions, showing the natural course of disease development along with aging. Long-term prophylaxis and symptomatic treatment are recommended for all HAE patients, especially young and middle-aged and might be adjusted depending on the disease progression.

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The role of C1 inhibitor and complement as acute phase reactants: are we missing the diagnosis of hereditary angioedema?

Allergy Asthma & Clinical Immunology ◽

10.1186/s13223-021-00607-5 ◽

2021 ◽

Vol 17 (1) ◽

Author(s):

Peter Stepaniuk ◽

Ana-Maria Bosonea ◽

Persia Pourshahnazari ◽

Adrienne Roos ◽

Amin Kanani

Keyword(s):

Acute Phase ◽

Hereditary Angioedema ◽

Delayed Diagnosis ◽

Case Series ◽

Upper Respiratory Tract ◽

C1 Inhibitor ◽

Acute Phase Reactants ◽

Reactive Protein ◽

Clinically Significant ◽

Complement 4

Abstract Background C1 inhibitor (C1-INH) and complement 4 (C4) have historically been referred to as positive acute phase reactants, however this has never been evaluated in hereditary angioedema (HAE) patients. Low function of C1-INH and low levels of C4 are important in the diagnosis of HAE type 1 and 2. If C1-INH and/or C4 are significant acute phase reactants, their levels may be falsely “normal” in patients with HAE when measured during times of infection or inflammation resulting in missed or delayed diagnosis. Case presentation We present a case series of four HAE patients who had C4, C1-INH, c-reactive protein (CRP) and ferritin measured at baseline and again during a self-reported upper respiratory tract infection (URTI) or flu-like illness. We did not identify any HAE patients who had a significant change in their C1-INH functional level in the context of a mild infection. However, the C4 level did increase into the normal range on three occasions (2 patients, with 1 patient having elevation during two separate illnesses). Conclusions C1 inhibitor may not be a clinically significant acute phase protein and appears to still be a reliable diagnostic marker of hereditary angioedema, even in times of modest acute inflammation, unlike complement C4 which can be elevated in this setting.

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The Natural Course of Hereditary Angioedema in a Chinese Cohort

10.21203/rs.3.rs-28783/v3 ◽

2020 ◽

Author(s):

Yang Cao ◽

Shuang Liu ◽

Yuxiang Zhi

Keyword(s):

Risk Factors ◽

Disease Progression ◽

Disease Severity ◽

Hereditary Angioedema ◽

Symptomatic Treatment ◽

Natural Course ◽

Chinese Patients ◽

Laryngeal Edema ◽

Chinese Cohort ◽

Clinical Prevention

Abstract Background: Hereditary angioedema (HAE) is a rare disease with potential life-threatening risks. To study the natural course of HAE under therapy-free conditions throughout patient life is essential for practitioners and patients to avoid possible risk factors and guide treatment.Objectives: Describe the natural course of HAE and explore possible risk factors, providing new clues for guiding clinical prevention and treatment.Methods: A web-based survey was conducted in 103 Chinese patients with type 1 HAE. Disease progression at different age stages was provided by each participant. The data for exploring the natural course of HAE composed of two parts: one came from the participants who had never adopted any prophylactic drug for HAE; the other was from the patients with a history of medication, but only the periods before they got confirmed diagnosis and received medications were analyzed. The demographic characteristics, lifestyles, disease severity, and family history were also collected.Results: Among 103 patients, 14 (13.6%) had their first HAE attack before ten years old and 51 (49.5%) between 10 and 19. The disease worsened in 83.3% of the patients in their twenties. The proportion of patients with symptoms alleviated increased after the age of 30 years old, but the disease maintained relatively severe in most cases before 50. The participants also reported 233 members shared similar symptoms of angioedema in their family and 30 had died of laryngeal edema with the median death age of 46 years old. The disease severity was not observed to be affected significantly by gender, BMI, alcohol or smoking.Conclusions: We summarized HAE progression patterns under therapy-free conditions, showing the natural course of HAE development along with aging. Long-term prophylaxis and symptomatic treatment are recommended for all HAE patients, especially young and middle-aged and might be adjusted depending on the disease progression.

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The Natural Course of Hereditary Angioedema in a Chinese Cohort

10.21203/rs.3.rs-28783/v1 ◽

2020 ◽

Author(s):

Yang Cao ◽

Shuang Liu ◽

Yuxiang Zhi

Keyword(s):

Risk Factors ◽

Disease Progression ◽

Disease Severity ◽

Hereditary Angioedema ◽

Symptomatic Treatment ◽

Natural Course ◽

Chinese Patients ◽

Laryngeal Edema ◽

Chinese Cohort ◽

Clinical Prevention

Abstract Background: Hereditary angioedema (HAE) is a rare disease with potential life-threatening risks. Until now, few studies have focused on the natural course of HAE, which is essential for practitioners and patients to predict disease progression and avoid possible risk factors. Objectives: Describe the natural course of HAE and explore possible risk factors, providing new clues for guiding clinical prevention and treatment. Methods: A web-based survey was conducted in 103 Chinese patients with type 1 HAE. Disease progression under therapy-free conditions was collected at different age stages. The demographic characteristics, lifestyles, disease severity and family history were also provided by each participant. Results: Among 103 patients, 14 (13.6%) had their first HAE attack before ten years old and 51 (49.5%) between 10 and 19. The disease worsened in 83.3% of the patients in their twenties. The proportion of patients with symptoms alleviated increased after the age of 30 years old, but the disease maintained relatively severe in most cases before 50. The participants also reported 233 members shared similar symptoms of angioedema in their family and 30 had died of laryngeal edema with the median death age of 46 years old. The disease severity was not observed to be affected significantly by gender, BMI, alcohol or smoking. Conclusions: We summarized HAE progression patterns in therapy-free conditions, showing the natural course of disease development along with aging. Long-term prophylaxis and symptomatic treatment are recommended for all HAE patients, especially young and middle-aged and might be adjusted depending on the disease progression.

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The natural course of hereditary angioedema in a Chinese cohort

Orphanet Journal of Rare Diseases ◽

10.1186/s13023-020-01526-1 ◽

2020 ◽

Vol 15 (1) ◽

Author(s):

Yang Cao ◽

Shuang Liu ◽

Yuxiang Zhi

Keyword(s):

Risk Factors ◽

Disease Progression ◽

Disease Severity ◽

Hereditary Angioedema ◽

Symptomatic Treatment ◽

Natural Course ◽

Chinese Patients ◽

Laryngeal Edema ◽

Chinese Cohort ◽

Clinical Prevention

Abstract Background Hereditary angioedema (HAE) is a rare disease with potential life-threatening risks. To study the natural course of HAE under therapy-free conditions throughout patient life is essential for practitioners and patients to avoid possible risk factors and guide treatment. Objectives Describe the natural course of HAE and explore possible risk factors, providing new clues for guiding clinical prevention and treatment. Methods A web-based survey was conducted in 103 Chinese patients with type 1 HAE. Disease progression at different age stages was provided by each participant. The data for exploring the natural course of HAE composed of two parts: one came from the participants who had never adopted any prophylactic drug for HAE; the other was from the patients with a history of medication, but only the periods before they got confirmed diagnosis and received medications were analyzed. The demographic characteristics, lifestyles, disease severity, and family history were also collected. Results Among 103 patients, 14 (13.6%) had their first HAE attack before 10 years old and 51 (49.5%) between 10 and 19. The disease worsened in 83.3% of the patients in their twenties. The proportion of patients with symptoms alleviated increased after the age of 30 years old, but the disease maintained relatively severe in most cases before 50. The participants also reported 233 members shared similar symptoms of angioedema in their family and 30 had died of laryngeal edema with the median death age of 46 years old. The disease severity was not observed to be affected significantly by gender, BMI, alcohol or smoking. Conclusions We summarized HAE progression patterns under therapy-free conditions, showing the natural course of HAE development along with aging. Long-term prophylaxis and symptomatic treatment are recommended for all HAE patients, especially young and middle-aged and might be adjusted depending on the disease progression.

Download Full-text