Acute pancreatitis due to hereditary angioedema.
BACKGROUND
Hereditary angioedema (HAE) is an infrequent disorder characterized by abnormalities in the levels and/or function of complement C1 esterase inhibitor. Clinical manifestations of HAE are due to recurrent episodic swelling of the subcutaneous or submucosal tissue. When swelling involves the gastrointestinal mucosa, patients may present with nausea, vomiting, diarrhea, and severe abdominal pain. However, HAE is almost never suspected as a potential cause of acute pancreatitis.


OBJECTIVE
To describe a patient with HAE-associated pancreatitis requiring intensive medical care that responded favorably to conservative and supportive measures.


METHODS
Various tests were performed, including abdominal imaging, measurement of pancreatic enzymes levels, liver function tests, measurement of complement levels, and endoscopic retrograde cholangiopancreatography.


RESULTS
The results of these tests confirmed the diagnosis of HAE-associated acute pancreatitis. No other obvious origin, such as gallstones or alcohol use, was identified.


CONCLUSIONS
This case illustrates the need for a high clinical suspicion of acute pancreatitis when caring for patients with HAE who present with abdominal symptoms. There continues to be an urgent need for better and additional therapeutic options for HAE patients, including those to prevent and abort ongoing attacks.
Matesic
Damir
D
Department of Pulmonary and Critical Care Medicine, Mayo Clinic College of Medicine, Rochester, Minnesota 55901, USA.
Fernández Pérez
Evans R
ER
Vlahakis
Nicholas E
NE
Hagan
John B
JB
D000328:Adult; D000799:Angioedema / Q000150:complications* / Q000188:drug therapy / Q000235:genetics; D002760:Cholangiopancreatography, Endoscopic Retrograde; D003613:Danazol / Q000627:therapeutic use; D004965:Estrogen Antagonists / Q000627:therapeutic use; D030342:Genetic Diseases, Inborn / Q000150:complications* / Q000188:drug therapy; D006801:Humans; D008297:Male; D010179:Pancreas / Q000473:pathology; D019283:Pancreatitis, Acute Necrotizing / Q000097:blood / Q000175:diagnosis / Q000209:etiology*; D014057:Tomography, X-Ray Computed
D004965:Estrogen Antagonists; D003613:Danazol