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Reflections on Living with Lupus, and on Treating It

Simon M. Helfgott, MD  |  Issue: April 2012  |  April 6, 2012

Simon M. Helfgott, MD

There are some people whose purpose in life is to entertain. Some of us are fortunate to have them as our patients. One of my favorites is Larry the Cable Guy. Before you get all excited and report me for a HIPAA violation, I am not referring to that redneck comedian with his own weekly television sitcom and a host of B-rated movies. My guy Larry was training to becoming a real cable-installation guy when one day he felt awfully short of breath. Really, really breathless. As though someone was wringing his chest like a wet sponge. He thought he was going to die. He nearly did. He was rushed to a nearby hospital and brought straight to the operating room where he underwent a lifesaving pericardiocentesis procedure. This brought Larry some temporary relief, but two days later the fluid had reaccumulated and his blood pressure began to drop. After a few more procedures to strip his heart of its pericardial lining, his condition stabilized. Larry was nineteen and thought he was following the path of his older brother who had died a year earlier from complications related to myasthenia gravis.

At first, his doctors believed that he had been exposed to a virus. Isn’t that what we tell patients when we really don’t know what their diagnosis is? His chest X-ray showed a large, globular, swollen heart bordered by a pair of healthy-looking lungs. There were no infiltrates, no pleural effusions. The blood test results demonstrated the expected moderate rise in his white-cell count, but none of his many blood and body fluid cultures grew any microorganisms, so infection seemed unlikely. His kidney and liver function studies were normal, so that ruled out organ failure as the cause of his pericardial effusion. Then came the surprise. A careful microscopic search of a sample of pericardial fluid provided the missing clue: the mysterious lupus erythematosus cell, better known as the LE cell.

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The LE cell-prep test is based on the innate ability of some lupus monocytes to phagocytize cell nuclei rendered antigenic by the disease. To this day, the finding of an LE cell remains the most specific yet the least sensitive test for diagnosing lupus. Nobody ever gets to see the LE cell anymore, except when it makes a cameo appearance during some esoteric discussion of a clinical–pathologic case. There is a good reason for its demise. First, you must shake and stir your sample fluid with some glass beads in order to disrupt cells to get them to release their nuclear material. Then you have to carefully gaze at dozens of microscopic fields to try to find the elusive white blood cell with the bulging cytoplasm that looks as though it ate far too much at the all-you-can-eat buffet. And so began Larry’s lupus journey.

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Filed under:ConditionsOpinionRheuminationsSpeak Out RheumSystemic Lupus Erythematosus Tagged with:cardiac tamponadedrugHelfgottHYDROXYCHLOROQUINELupusPathogenesispatient careprednisonerheumatologistSystemic lupus erythematosustherapeuticsTreatment

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