On Thursday, we challenged Well readers to figure out why an otherwise healthy 47-year-old woman would suddenly be struck with abdominal pain, nausea and vomiting that seemed to come out of the blue every few months and resolved within days.
We got more than 300 responses from readers; 55 of you got it right. Well done. The first person to submit the answer was Pooyan Rohani, a third year medical student at Vanderbilt University. He tells me that as soon as he saw the CT scan, he knew exactly what it was.
The correct diagnosis is…
Intestinal angioedema, triggered by lisinopril, the ACE inhibitor the patient took for her high blood pressure.
The Diagnosis:
Angioedema is a localized type of swelling usually involving the mouth, tongue or upper airways. It can be part of a typical allergic reaction, with hives and itching, or it can be isolated, with swelling as the only notable finding. While there is an inherited form of this disease, most cases are acquired. And medications are the most common cause of this form of the syndrome.
The class of high blood pressure drugs known as ACE inhibitors is the most common medication linked to angioedema. This patient was taking lisinopril, one of the most widely prescribed drugs of these drugs. Although angioedema usually arises when the medication is first started, it can occur at any point during a patient's treatment.
African Americans are at a particularly high risk of this strange drug reaction. Up to one in 30 African Americans who take an ACE inhibitor will develop angioedema at some point. Women are also at somewhat higher risk than men.
And it's not just this class of medication. Painkillers such as ibuprofen and certain antibiotics are also listed as relatively frequent causes of angioedema. Indeed, there's a whole long list of drugs that have been linked to this unusual reaction.
A Rare Gut Problem:
Usually the swelling of angioedema is seen in the face: the lips, tongue or throat. And the swelling can be quite dramatic, occasionally completely blocking the upper airways.
However, this patient had a rare form of the problem that arose not in the face but in the intestine. When swelling occurs in the gut, it can block off the intestinal lumen and bring digestion to a screeching halt, causing the terrible pain and vomiting this patient experienced.
Remarkably, no matter where in the body the swelling occurs, or how severe it gets, it always resolves quickly – often within hours – even if the patient continues to take the medication.
When the angioedema happens in the G.I. tract, the diagnosis can be delayed for months or years because so many doctors don't know that this kind of reaction is even possible. When the medication is stopped, the episodic reaction also finally stops.
How the Diagnosis Was Made:
It was both the rapid resolution of the patient's symptoms and CT scan abnormalities that provided the essential clue in this case. Dr. Ajaypal Singh was the G.I. fellow who was consulted to help figure out the cause of the patient's pain. Once he saw the repeat CT scan, which — much to his astonishment — was nearly normal, the diagnosis was rapid. There are simply not very many diseases that will turn around this fast.
Dr. Singh immediately called Dr. Poonam Merai, the resident on call that day at the University of Chicago Medical Center, and told her that he thought this was a reaction to the patient's blood pressure medication.
He also recommended that the patient be tested for the inherited version of the disease. If the patient had this unusual genetic disorder, stopping the medications would not prevent the attacks of pain and vomiting. Those tests were normal.
The patient was amazed to hear that this pill she'd been taking for years was the cause of her pain. Once the doctors explained it to her, though, a light went off and the intermittent nature of her attacks suddenly made sense.
You see, one thing she hadn't told any of her doctors was that she often stopped that medication when her blood pressure seemed O.K. She had a blood pressure cuff at home and monitored it closely. She exercised regularly — she knew that brought her blood pressure down. And she tried to follow a low sodium, high potassium diet. When she did both, her blood pressure was usually well under the 140/90 measurement she knew was her target.
And she always restarted the drug when her blood pressure started to go up. That was usually predictable, occurring when she "fell off the wagon" and stopped her exercise and diet routine. Now that she thought about it, most of these episodes arose weeks after restarting her lisinopril.
How the Patient Is Doing:
The patient was given an alternative blood pressure medication and happily stopped taking her lisinopril forever. It has been six months now, and so far, so good. She is keeping her fingers crossed, but at this point it looks like a remarkably simple solution to a terribly painful problem.
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