Researchers presented the case of a pregnant patient with hereditary angioedema but normal C1 inhibitor levels, as well as systemic lupus erythematosus (SLE), in the Journal of Allergy and Clinical Immunology: Global.

The case report details a 24-year-old female patient who had been having recurrent angioedema attacks since the age of 12 years. The most prominent symptoms during these episodes were abdominal pain, nausea, and vomiting.

The attacks were brought on by stress and hormonal fluctuations, occurred around 4 times a month, and lasted for 2 to 4 days. She did not respond to antihistamines, corticosteroids, fresh frozen plasma, or tranexamic acid. 


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“At the age of 19, the patient started to develop new symptoms of a malar rash, photosensitivity with sun exposure, and cold fingertips with color changes,” the authors of the study wrote. 

Read more about hereditary angioedema etiology 

In addition, the patient also began to experience stiff lower limb joints with knee swelling, as well as proteinuria and microscopic hematuria. Laboratory investigations confirmed a diagnosis of SLE. She continued to experience recurring episodes of angioedema attacks. 

When the patient became pregnant, her episodes became more frequent and severe. She also experienced an SLE flare with proteinuria, high blood pressure, and low levels of C3 and C4. 

During early labor, the patient developed acute tongue, laryngeal, and vaginal swelling, which was treated with a 30-mg dose of subcutaneous icatibant. The attack was aborted within 30 minutes. She went on to have a normal vaginal delivery of a healthy boy. 

Postdelivery, the patient was started on subcutaneous human C1-esterase inhibitor at a dose of 3300 IU as prophylaxis. During the early postpartum period, she developed several acute attacks, which were successfully treated with icatibant. She continued to be administered 3300 IU of human C1-esterase inhibitor twice a week and experienced less frequent and less severe edema attacks. 

“This case demonstrates the effectiveness of human C1-esterase inhibitors as prophylactic therapy, decreasing the frequency and severity of angioedema episodes after labor,” the authors of the study wrote. 

Reference

Taha OS, Abi-Melhem R, Taha Y, Meyer D, Assaad M. A patient with hereditary angioedema (HAE) with normal C1-INH, and systemic lupus erythematosus with pregnancy. J Allergy Clin Immunol Glob. Published online October 27, 2022. doi:10.1016/j.jacig.2022.09.005