Visceral angioedema D84.-

Visceral angioedema is a rare disease that may present with or without skin and upper respiratory tract involvement. The disease is bradykinin-mediated. In this respect, anaphylactic therapy is not indicated.

ACE inhibitor-induced visceral angioedema: The occurrence of ACE inhibitor-induced visceral angioedema is associated with a probability of 0.3-0.7% under ACE inhibitor therapy. In this case, the skin and upper respiratory tract are affected in addition to the gastrointestinal tract.

Visceral hereditary angioedema (HAE): Hereditary visceral angioedema (see Angioedema hereditary below) usually manifests in childhood or young adulthood. In addition to the gastrointestinal tract, the skin and upper respiratory tract are affected. The development of visceral hereditary angioedema is often preceded by a stressful situation (surgery, psychological stress) (regarding the classification of visceral hereditary angioedema, see below Angioedema hereditary).

Visceral angioedema is bradykinin-mediated and therefore, unlike mast cell-mediated angioedema, is not accompanied by pruritus and urticaria. Bradykinin is a potent vasodilator, which also increases capillary permeability. Thus, increased fluid can transfer to the submucosal tissues, resulting in the edema.

Visceral angioedema is characterized by localized, self-limiting swelling of the mucosa. The leading symptom of visceral angioedema is colicky abdominal pain. These may be accompanied by nausea, vomiting, and/or diarrhea. The jejunum is most commonly involved, followed by the ileum and duodenum. Intestinal angioedema may be visualized as swelling of the intestinal wall by computed tomography or ultrasound, where dilated intestinal loops, cocardia, thickened mucosal folds, perihepatic fluid, and/or ascites are typically found.

The diagnosis of visceral angioedema is often difficult and relies primarily on the patient's clinic. Many often report a history of disease for years before the correct diagnosis is made. In case of recurrent colicky abdominal pain with vomiting, diarrhea and possibly ascites and possible angioedema of the skin and upper respiratory tract, complement factor 4 (C4) as well as C1INH and functional C1INH should be determined by laboratory chemistry. Depending on this, HAE type 1 and HAE type 2 as well as C1INH-AAE can be diagnosed. The diagnosis of the other forms is to be derived purely from anamnesis and by means of imaging.

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