A 28‐year‐old woman presented with an acute exacerbation of chronic, intermittent abdominal pain. The patient described intense cramping and colicky periumbilical pain lasting 1‐2 days and occurring 2‐3 times per year for 9 years with no known triggers. Associated symptoms frequently included nausea, vomiting, and watery diarrhea. The patient denied fever, chills, skin changes, arthralgias, and other physical symptoms. Over the course of many prior presentations, numerous diagnostic entities had been explored, from gastroenteritis to ischemia and from irritable bowel syndrome to lymphoma. Blood counts, chemistries, and serologies, as well as CT and MR studies, were repeatedly normal. Upper and lower endoscopies with biopsies had been unrevealing. The patient had undergone cholecystectomy and sphinctorotomy without relief. Chronic functional pain syndrome and somatization disorder were the leading diagnostic considerations. On this occasion, the patient experienced a witnessed episode of typical abdominal pain accompanied by lip and facial swelling. The patient had not been knowingly exposed to causative toxins or medications. Hereditary angioedema (HAE) was suspected. For the first time, a CT scan demonstrated marked edema in the small bowel, gastric antrum, and cecum. Biopsy revealed gastric edema with otherwise normal histology. The patient was discharged and referred to allergy and pain specialists. Autoimmune serology and complement studies were normal, prompting a diagnosis of HAE type III. Standard therapies for HAE types I and II failed to provide sustained relief. However, with a comprehensive approach to pain control and her ongoing doctor‐patient relationships, the patient achieved an improved functional status, starting both a family and a rewarding career.
Hereditary angioedema is a rare inherited disorder resulting in rapid swelling of the skin, mucosa, and submucosal tissues. A high estimated mortality of 15%‐33% is attributed to acute laryngeal edema and asphyxiation. Although angioedema is more commonly associated with drug allergies, notably to ACE inhibitors, the less common hereditary form has been characterized since Heinrich Quincke and Sir William Osier studied several familial clusters more than 120 years ago. Current research implicates the kinin pathway, wherein kinins are enzymatically cleaved into potent vasodilators. Type III, or normal‐complement HAE, an especially uncommon x‐linked variant, was first identified in 2000.
This case illustrates the importance of perseverance at the bench and the bedside and of combining scientific understanding with compassionate understanding for patients who endure the burden of rare diseases. Learning objectives include: understanding the pathophysiology of angioedema, exploring issues of trust and suspicion for hospitalists and their patients with chronic, unexplained pain, and formulating a framework for diagnosis of rare causes of chronic abdominal pain.
J. Morrow, Durham Regional Hospital/Duke University, employment.
To cite this abstract:Morrow J. When to Depend on the Kinins of Strangers: An Unusual Case of Chronic Abdominal Pain. Abstract published at Hospital Medicine 2009, May 14-17, Chicago, Ill. Abstract 174. Journal of Hospital Medicine. 2009; 4 (suppl 1). https://www.shmabstracts.com/abstract/when-to-depend-on-the-kinins-of-strangers-an-unusual-case-of-chronic-abdominal-pain/. Accessed June 17, 2019.