Researchers reported the case of a patient with hereditary angioedema (HAE) type 2 who had recurrent abdominal pain as the only clinical manifestation of the disease, according to a letter to the editor published in The Spanish Journal of Gastroenterology. 

The case as reported details a 19-year-old male who was followed up for recurrent abdominal pain in the last 3 years. He had no other relevant personal or family medical history. 

According to the patient, he did not experience weight loss or signs of gastrointestinal bleeding. In addition, he did not have any ocular or skin manifestations, nor did he have oral aphthae or arthromyalgia. 

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All physical examinations were normal. An ultrasound and computed tomography scan conducted during symptomatic episodes revealed a thickened enteral wall, inflamed mesenteric fat, locoregional adenopathy, and abdominal free fluid. These findings were consistent with jejunitis, ileitis, and colitis. Blood and fecal microbiological studies were negative. Upper and lower gastrointestinal scope examinations revealed no relevant findings. 

Read more about HAE epidemiology 

The patient was hospitalized for a new abdominal pain episode, with computed tomography scans revealing inflammation of the gastrointestinal tract. Upon further history taking, the patient mentioned that each of his abdominal pain episodes occurred after a viral infection. However, protein electrophoresis and immunoglobulin examinations returned normal. 

A study of the patient’s complement system revealed C4 levels of 0.04 g/L (low) and C1 inhibitor levels of 0.41 g/L (high), with 8% functional activity (low). The patient was diagnosed with HAE type 2 and was referred to allergology, who prescribed him C1 esterase inhibitors should new episodes occur. 

“It is necessary to rule out [hereditary angioedema] by quantifying C4 levels and if they are low, request activity and levels of [C1 inhibitors] in patients with inexplicable recurrent abdominal pain, even in absence of cutaneous manifestations, since a specific treatment could be administered, and unnecessary examinations avoided,” the authors concluded. 


Muíño-Domínguez D, Carballo-Folgoso L, Martínez Camblor L, et al. Recurrent abdominal pain as the only clinical manifestation of hereditary angiodema type IIRev Esp Enferm Dig. 2022;10.17235/reed.2022.9119/2022. doi:10.17235/reed.2022.9119/2022