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Published on: May 19, 2020
C-reactive protein levels in hereditary angioedema
Z L M Hofman1, A Relan, C E Hack
1Laboratory for Translational Immunology, University Medical Centre Utrecht, Utrecht, The Netherlands.
Hereditary angioedema (HAE) involves systemic inflammation, with elevated C-reactive protein (CRP) observed in asymptomatic patients. Abdominal attacks show a significant CRP increase, suggesting inflammation precedes and influences HAE symptom onset.
Area of Science:
- Immunology
- Genetics
- Pathophysiology
Background:
- Hereditary angioedema (HAE) is characterized by recurrent, potentially life-threatening swelling attacks.
- HAE pathogenesis is linked to C1-inhibitor (C1INH) deficiency and bradykinin overproduction.
- The role of systemic inflammation in HAE attacks requires further elucidation.
Purpose of the Study:
- To investigate the presence and dynamics of systemic inflammation in HAE patients.
- To analyze C-reactive protein (CRP) levels in relation to HAE attack status and clinical presentation.
- To explore the potential link between inflammation and HAE attack triggers.
Main Methods:
- Analysis of CRP levels in 68 HAE patients across asymptomatic, attack, and follow-up periods.
- Correlation of CRP levels with clinical manifestations, particularly attack location.
- Utilizing data from a clinical database of recombinant human C1-inhibitor (rhC1INH) studies.
Main Results:
- Elevated CRP levels were found in 20% of asymptomatic HAE patients.
- CRP levels significantly increased during abdominal HAE attacks and in the subsequent 24 hours.
- Post-attack CRP levels were higher in patients with abdominal attacks compared to other locations.
Conclusions:
- A significant proportion of HAE patients exhibit elevated CRP even when asymptomatic, indicating low-grade systemic inflammation.
- Abdominal HAE attacks are associated with a pronounced inflammatory response, evidenced by significant CRP elevation.
- These findings suggest that systemic inflammatory processes may precede and contribute to the onset of HAE attacks.
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