Acquired Non-histaminergic Angioedema With C1q Autoantibody and Urticaria: A Case Report
- PMID: 37736455
- PMCID: PMC10510307
- DOI: 10.7759/cureus.43841
Acquired Non-histaminergic Angioedema With C1q Autoantibody and Urticaria: A Case Report
Abstract
Acquired angioedema (AAE) is a rare disease with life-threatening complications. This pathology has classically been associated with medication use and B cell lymphoproliferative disorders. In this report, we describe a 61-year-old man with a six-year history of angioedema, unrelated to any known triggers or malignancy. Extensive workup has led to a diagnosis of idiopathic nonhistaminergic AAE with normal C1 inhibitor. The patient is currently being treated with lanadelumab, which has resolved the patient's symptoms. This case provides insight into the onset, exploration, treatment, and outcomes of an extremely rare disease process.
Keywords: aae; acquired angioedema; anti-c1q antibody; autoimmune disease; bradykinin; c1q autoantibody; icatibant; inh-aae; lanadelumab; urticaria.
Copyright © 2023, Kochvar et al.
Conflict of interest statement
The authors have declared financial relationships, which are detailed in the next section.
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References
-
- Angioedema due to bradykinin dysregulation. Cicardi M, Zuraw BL. J Allergy Clin Immunol Pract. 2018;6:1132–1141. - PubMed
-
- Classification, diagnosis, and approach to treatment for angioedema: consensus report from the Hereditary Angioedema International Working Group. Cicardi M, Aberer W, Banerji A, et al. Allergy. 2014;69:602–616. - PubMed
-
- Clinical and biological distinctions between type I and type II acquired angioedema. Bouillet-Claveyrolas L, Ponard D, Drouet C, et al. Am J Med. 2003;115:420–421. - PubMed
-
- Autoimmune C1 inhibitor deficiency: report of eight patients. Cicardi M, Bisiani G, Cugno M, et al. Am J Med. 1993;95:169–175. - PubMed
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