Where we are with acquired angioedema due to C1 inhibitor deficiency: A systematic literature review

Presentation of an extraordinary colic: abdominal pain as the first and only utterance of an acquired C1-inhibitor deficiency

“…Shi et al and Zanichelli et al showed that patients with AAE secondary to a lymphoproliferative disease were also positive for autoantibodies to C1-INH in 32%–58% of the cases, respectively. 3 9 Autoantibodies in the present case were not tested routinely and are therefore unknown. Other associated diseases with AAE are monoclonal gammopathy, autoimmune disorders or other solid malignancies.…”

“… 3 The mean frequency of AAE attacks is two times per month with a diagnostic delay that varies from 2 to 5 years. 3 9 10 …”

“…AAE presents often secondary to an underlying disease, of which lymphoproliferative disorders are by far most frequently reported. 3 These lymphoproliferative disorders are often relatively indolent low-grade lymphomas, with SMZL as the frequent subtype. 9 The exact pathophysiological mechanisms responsible for the development of AAE are not entirely clarified, but evidence indicates that the C1-INH deficiency can be caused by either C1-INH autoantibodies or excessive consumption of C1-INH by neoplastic tissue.…”

“…Other associated diseases with AAE are monoclonal gammopathy, autoimmune disorders or other solid malignancies. 3 Infection with H. pylori could potentially also trigger AAE (n=2). 14 15 In these cases, eradication of H. pylori was followed by resolution of symptoms and normalisation of laboratory findings.…”

“… 2 Clinical presentation often includes self-limiting periodical oedema of the facial subcutaneous tissues, the gastrointestinal tract, the oral cavity/larynx, genitalia or extremities. 3 The oedema may present site specific or be combined and is self-limiting. A C1-INH deficiency can be hereditary (C1-INH-HAE) due to mutations affecting the SERPING1 gene or acquired (C1-INH-AAE).…”