Chest Pain as an Unusual First Symptom in Patients with Systemic Amyloidosis

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The summary below is from the full report titled "Systemic Amyloidosis Presenting with Angina Pectoris." It is in the 7 December 1999 issue of Annals of Internal Medicine (volume 131, pages 838-841). The authors are J. Al Suwaidi, J.L. Velianou, M.A. Gertz, R.O. Cannon III, S.T. Higano, D.R. Holmes Jr., and A. Lerman.

What is the problem and what is known about it so far?

Systemic amyloidosis is a rare disease caused by the buildup of an abnormal protein substance called amyloid in body tissues. Amyloidosis can affect many organs of the body, but involvement of the heart and blood vessels is the most common cause of illness and death in patients with this disease. In patients whose heart and blood vessels are affected by amyloidosis, shortness of breath, leg swelling, or abnormal heart rhythms are usually the first signs that they have this disease. Angina pectoris (chest pain) has not been thought of as a sign of amyloidosis.

Why did the researchers do this particular study?

The researchers wanted to see whether chest pain may be the first sign of systemic amyloidosis.

Who was studied?

The study included 153 patients who presented to the Mayo Clinic between January 1993 and February 1997 with chest pain. Study patients had to have normal cardiac catheterizations (a test that looks for blockages in the large blood vessels to the heart) and to have undergone "coronary flow reserve" testing (a special test that looks for abnormally low blood flow in the smallest blood vessels to the heart).

How was the study done?

The Mayo Clinic collects extensive computerized information on all of the patients ever seen there. The researchers reviewed that information to find patients who met the study criteria. They then identified patients who had abnormal coronary flow reserve and also were eventually diagnosed as having systemic amyloidosis.

What did the researchers find?

Of the 153 patients in the database who had chest pain, normal cardiac catheterization, and abnormal coronary flow reserve, 5 were found to have systemic amyloidosis. The diagnosis of amyloidosis was made by using special tests of biopsy samples taken from fat pads or the bone marrow. All 5 patients had initial symptoms of chest pain that seemed as if they were having a heart attack, but the results of the usual tests to look for a heart attack were normal. Results of coronary flow reserve tests were abnormal in all of these patients.

What were the limitations of the study?

Tests of biopsy samples were not done for all of the 153 patients. Because of this, it is possible that the researchers did not identify all of the patients who had systemic amyloidosis. Coronary flow reserve tests are not commonly part of the evaluation of chest pain. The reasons that these patients underwent coronary flow reserve testing are not clear, this study does not prove that patients with chest pain should have this special test.

What are the implications of the study?

Doctors should consider systemic amyloidosis as a possible diagnosis when patients present with the type of chest pain that suggests heart disease but have normal cardiac catheterizations, no other possible explanation for the pain, and abnormal coronary flow reserve.