Acquired Non-histaminergic Angioedema With C1q Autoantibody and Urticaria: A Case Report.

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.

Hereditary Angioedema with Normal C1 Esterase Inhibitor Refractory to Long-Term Prophylaxis: A Case Report.

Hereditary angioedema (HAE) was classically thought to be related to a deficiency in the C1 esterase inhibitor (C1-INH). However, HAE with a normal C1 esterase inhibitor (HAE nC1-INH) is a rare disease that has been recently characterized. In this case, we describe a woman with a 21-year history of angioedema who, in the last two years, has experienced severe exacerbations that led to the formal diagnosis of HAE nC1-INH. The patient has been treated with current HAE long-term prophylaxis (LTP) and on-demand therapies but is still experiencing severe, frequent attacks. Our case describes the timeline of this patient's presentation and provides valuable insight into the presentation and management of HAE nC1-INH.

Pregnancy-Induced Exacerbation of Hereditary Angioedema in a Multiparous Caucasian Female.

Hereditary angioedema (HAE) manifests due to a deficiency of the C1-esterase inhibitor and can present with life-threatening swelling of multiple body regions such as the face, hands, upper respiratory tract, and intestinal walls. The present case describes the manifestation and symptomatic exacerbation of HAE in a multiparous Caucasian female. Very few trials and cases are available on HAE exacerbations during pregnancy, and our case describes the timeline and treatment in order to add to the clinical awareness of the disease. It is necessary to treat these patients rapidly to avoid unnecessary morbidity and interventions. For the time being, our patient has been appropriately managed with icatibant.