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The Case of a 13-Year-Old Girl with Life-Threatening Lupus Onset

Charles Radis, DO  |  Issue: October 2018  |  October 18, 2018

Without a word, I wrapped a blood pressure cuff around Amanda’s right arm and squeezed the bulb up to l50 mm before slowly releasing the pressure. Nursing had previously charted her blood pressure. I was interested in something else. At 106 mm the faint tapping of Amanda’s pressure could be heard, but only intermittently. That’s odd. The top number, the systolic blood pressure, is usually steady like a metronome. I adjusted the earplugs and listened intently. When I held the cuff pressure steady at 106 mm, I heard the tap, tap, tap of the blood pressure only as she exhaled.

Breathing in, the rhythmic tapping disappeared. As she breathed out, the pulse rebounded. I released the pressure on the cuff ever so slowly and the pattern persisted. Finally, at 88 mm, I heard the heart rate throughout her respiratory cycle.

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The hair on the back of my neck tingled. The lab tech handed me the initial results of Amanda’s complete blood count (CBC): low white blood count and lower red blood cell count; platelets—critically low.

CXR Results

The curtain parted, and Dr. Benner motioned to me, “Dr. Radis, can I borrow you for a moment?” On the instrument tray next to the nursing station he reviewed the printout of Amanda’s ECG with me. “Classic pericarditis. Low voltage, diffuse ST segment elevation. This is pretty unusual for a teenager.”

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“Listen, John,” I replied. “If it were just pericarditis, we could take our time and work out the details. But we need to move on this. She has systemic lupus—I mean crescendo, life-threatening lupus. The full house. She needs a gram of Solu-Medrol [methylprednisolone sodium succinate] STAT, and we need to transfer her over to the medical center, like, 30 minutes ago. Check me out on this, but I think with the pericarditis, she has tamponade with a huge pulsus paradoxus. I measured it at 18.”

“Eighteen?” Dr. Benner repeated slowly.

The X-ray tech flipped the view box on and hung Amanda’s CXR on the screen. The size of the heart was breathtaking, taking up nearly three-fourths of the lower chest. We both knew the enlargement of the heart was primarily an inflammatory fluid within the sac surrounding the heart. The pressure from the fluid was slowly squeezing the heart like an external fist, triggering the abnormal pulse pressures and making it less and less effective at maintaining an effective blood pressure. I leaned forward from my seat and looked over my glasses.

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Filed under:ConditionsSystemic Lupus Erythematosus Tagged with:cyclophosphamidekidney failurePericarditisSteroids

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