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Hereditary angioedema: A rare cause of pancreatitis
Clinics and Research in Hepatology and Gastroenterology (2016) 40, e48—e49
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LETTER TO THE EDITOR
Hereditary angioedema: A rare cause of pancreatitis Hereditary angioedema (HAE) is an autosomal dominant disorder usually characterized by a mutation in one of the two alleles of the C1-inhibitor (C1-INH) gene, leading to low levels or dysfunctional C1-INH protein. Symptoms include attacks of mucosal and subcutaneous edema involving face, tongue, larynx, extremities, trunk, gastrointestinal and genitourinary systems [1]. Pancreas is rarely involved in those patients. A 55-years-old man admitted to our hospital with recurrent left upper quadrant pain for six months. At his first attack he had also swelling of face, lips and tongue. However, swelling of those organs did never recur at the following attacks. He had no vomiting, nausea or fever during the pain attacks. The abdominal pain was colicky and lasted for few hours. At each attack, serum amylase and lipase were found to be increased for at least 4—5 times. Other biochemical tests including serum lipids and calcium levels were normal. Radiological examinations demonstrated abdominal free fluid and bowel wall thickness during the attacks. Serum pancreatic amylase and lipase levels returned to the normal levels rapidly when the pain resolved. Also, radiological examinations became normal at the attack-free periods. He had no alcohol consumption, smoking, drug usage or gallstone story. He underwent diagnosing laporotomy and partial omentectomy. Pathology was congestion and minimal inflammation. Magnetic resonance cholangiopancreatography showed mild main pancreatic duct dilatation and side branch duct ectasia consistent with chronic pancreatitis (Fig. 1). Because of angioedema at first admission and recurrent edematous pancreatitis attacks, C1-INH was measured detecting very low serum level of C1-INH. He was diagnosed with hereditary angioedema and danazol was initiated (200 mg/day p.o.). Plasma-derived C1INH was also prescribed for acute episodes. He was free of symptoms at the 3-month follow-up. Abdominal symptoms occur up to 93% of patients with HAE and may be the only manifestation of the disease. They are characterized by crampy pain, nausea, vomiting, diarrhoea, and other features. Appendicitis, hepatitis, biliary obstruction, diverticulitis are the possible gastrointestinal http://dx.doi.org/10.1016/j.clinre.2016.02.002 2210-7401/© 2016 Published by Elsevier Masson SAS.
Figure 1 MRCP image shows mild main pancreatic duct dilatation and side branch duct ectasia consistent with chronic pancreatitis.
disorders [2]. Although the high frequency of abdominal attacks, pancreatitis due to HAE is very rare. The possible mechanism for pancreatitis is the partial ductal obstruction because of pancreatic edema. Bork et al. reported the detailed clinical data and course of abdominal attacks in patients with HAE. They documented a total of 33,671 abdominal attacks in 153 patients with HAE and pancreatitis was not described as a reason [3]. This is to our knowledge only the fifth case reported in the literature [4—6]. Our patient is unique regarding not having a diagnosis of HAE previously and, also, he did not have concomitant visible swelling of subcutaneous and mucosal tissues other than first attack. Also our patient developed chronic pancreatitis in a short time. In conclusion, HAE should be always in mind in patients suffering from idiopathic recurrent pancreatitis with or without visible angioedema. The diagnosis must be confirmed by evaluation of C1-INH serum levels.
Ethical statement The paper has ethical adherence.
Letter to the editor
Disclosure of interest The authors declare that they have no competing interest.
References [1] Jalaj S, Scolapio JS. Gastrointestinal manifestations, diagnosis, and management of hereditary angioedema. J Clin Gastroenterol 2013;47(10):817—23. [2] Ali MA, Borum ML. Hereditary angioedema: what the gastroenterologist needs to know. Clin Exp Gastroenterol 2014;7:435—45. [3] Bork K, Staubach P, Eckardt AJ, Hardt J. Symptoms, course, and complications of abdominal attacks in hereditary angioedema due to C1 inhibitor deficiency. Am J Gastroenterol 2006;101:619—27. [4] Cutler AF, Yousif EA, Blumenkehl ML. Hereditary angioedema associated with pancreatitis. South Med J 1992;85:1149—50. [5] Brickman CM, Tsokos GC, Balow JE, et al. Immunoregulatory disorders associated with hereditary angioedema. J Allergy Clin Immunol 1986;77:749—57.
e49 [6] Matesic D, Fernández Pérez ER, Vlahakis NE, Hagan JB. Acute pancreatitis due to hereditary angioedema. Ann Allergy Asthma Immunol 2006;97(5):611—4.
Evrim K. Aksoy a,∗ Seyfettin Koklu a Omer Ozturk a Hayretdin Koklu a Musturay Karcaaltincaba b Orhan Sezgin c a Department of Gastroenterology, Hacettepe University School of Medicine, 06100 Sıhhıye, Ankara, Turkey b Department of Radiology, Hacettepe University School of Medicine, 06100 Sıhhıye, Ankara, Turkey c Department of Gastroenterology, Mersin University School of Medicine, Mersin, Turkey ∗ Corresponding author. E-mail address: [email protected] (E.K. Aksoy) Available online 27 August 2016
Available online at
ScienceDirect www.sciencedirect.com
LETTER TO THE EDITOR
Hereditary angioedema: A rare cause of pancreatitis Hereditary angioedema (HAE) is an autosomal dominant disorder usually characterized by a mutation in one of the two alleles of the C1-inhibitor (C1-INH) gene, leading to low levels or dysfunctional C1-INH protein. Symptoms include attacks of mucosal and subcutaneous edema involving face, tongue, larynx, extremities, trunk, gastrointestinal and genitourinary systems [1]. Pancreas is rarely involved in those patients. A 55-years-old man admitted to our hospital with recurrent left upper quadrant pain for six months. At his first attack he had also swelling of face, lips and tongue. However, swelling of those organs did never recur at the following attacks. He had no vomiting, nausea or fever during the pain attacks. The abdominal pain was colicky and lasted for few hours. At each attack, serum amylase and lipase were found to be increased for at least 4—5 times. Other biochemical tests including serum lipids and calcium levels were normal. Radiological examinations demonstrated abdominal free fluid and bowel wall thickness during the attacks. Serum pancreatic amylase and lipase levels returned to the normal levels rapidly when the pain resolved. Also, radiological examinations became normal at the attack-free periods. He had no alcohol consumption, smoking, drug usage or gallstone story. He underwent diagnosing laporotomy and partial omentectomy. Pathology was congestion and minimal inflammation. Magnetic resonance cholangiopancreatography showed mild main pancreatic duct dilatation and side branch duct ectasia consistent with chronic pancreatitis (Fig. 1). Because of angioedema at first admission and recurrent edematous pancreatitis attacks, C1-INH was measured detecting very low serum level of C1-INH. He was diagnosed with hereditary angioedema and danazol was initiated (200 mg/day p.o.). Plasma-derived C1INH was also prescribed for acute episodes. He was free of symptoms at the 3-month follow-up. Abdominal symptoms occur up to 93% of patients with HAE and may be the only manifestation of the disease. They are characterized by crampy pain, nausea, vomiting, diarrhoea, and other features. Appendicitis, hepatitis, biliary obstruction, diverticulitis are the possible gastrointestinal http://dx.doi.org/10.1016/j.clinre.2016.02.002 2210-7401/© 2016 Published by Elsevier Masson SAS.
Figure 1 MRCP image shows mild main pancreatic duct dilatation and side branch duct ectasia consistent with chronic pancreatitis.
disorders [2]. Although the high frequency of abdominal attacks, pancreatitis due to HAE is very rare. The possible mechanism for pancreatitis is the partial ductal obstruction because of pancreatic edema. Bork et al. reported the detailed clinical data and course of abdominal attacks in patients with HAE. They documented a total of 33,671 abdominal attacks in 153 patients with HAE and pancreatitis was not described as a reason [3]. This is to our knowledge only the fifth case reported in the literature [4—6]. Our patient is unique regarding not having a diagnosis of HAE previously and, also, he did not have concomitant visible swelling of subcutaneous and mucosal tissues other than first attack. Also our patient developed chronic pancreatitis in a short time. In conclusion, HAE should be always in mind in patients suffering from idiopathic recurrent pancreatitis with or without visible angioedema. The diagnosis must be confirmed by evaluation of C1-INH serum levels.
Ethical statement The paper has ethical adherence.
Letter to the editor
Disclosure of interest The authors declare that they have no competing interest.
References [1] Jalaj S, Scolapio JS. Gastrointestinal manifestations, diagnosis, and management of hereditary angioedema. J Clin Gastroenterol 2013;47(10):817—23. [2] Ali MA, Borum ML. Hereditary angioedema: what the gastroenterologist needs to know. Clin Exp Gastroenterol 2014;7:435—45. [3] Bork K, Staubach P, Eckardt AJ, Hardt J. Symptoms, course, and complications of abdominal attacks in hereditary angioedema due to C1 inhibitor deficiency. Am J Gastroenterol 2006;101:619—27. [4] Cutler AF, Yousif EA, Blumenkehl ML. Hereditary angioedema associated with pancreatitis. South Med J 1992;85:1149—50. [5] Brickman CM, Tsokos GC, Balow JE, et al. Immunoregulatory disorders associated with hereditary angioedema. J Allergy Clin Immunol 1986;77:749—57.
e49 [6] Matesic D, Fernández Pérez ER, Vlahakis NE, Hagan JB. Acute pancreatitis due to hereditary angioedema. Ann Allergy Asthma Immunol 2006;97(5):611—4.
Evrim K. Aksoy a,∗ Seyfettin Koklu a Omer Ozturk a Hayretdin Koklu a Musturay Karcaaltincaba b Orhan Sezgin c a Department of Gastroenterology, Hacettepe University School of Medicine, 06100 Sıhhıye, Ankara, Turkey b Department of Radiology, Hacettepe University School of Medicine, 06100 Sıhhıye, Ankara, Turkey c Department of Gastroenterology, Mersin University School of Medicine, Mersin, Turkey ∗ Corresponding author. E-mail address: [email protected] (E.K. Aksoy) Available online 27 August 2016