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You are here: Home / Articles / The Case of a 13-Year-Old Girl with Life-Threatening Lupus Onset

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

October 18, 2018 • By Charles Radis, DO

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Amanda shrieked and flailed to remain upright, as if lying down were akin to drowning. I glanced at my watch and silently counted her respiratory rate over 15 seconds. Way too high. Pain? Anxiety?

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“Welcome to my world,” Dr. Benner deadpanned. “We don’t see rheumatologists down here very often.” The lab tech emerged seconds later with a fistful of crimson tubes. Dr. Benner handed me the chart. “Okay, your turn.”

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I remained seated. “No, let’s get the portable CXR [chest X-ray] first, John. That can be cooking while I examine Amanda.”

A whiff of uncertainty flowed over me. Amanda was considerably younger than my average patient. Where’s a pediatric rheumatologist when you need one? Outside of tertiary care children’s hospitals in, say, Boston, Philadelphia or Los Angeles, pediatric rheumatologists are rare birds, averaging about one per state. In Maine, we have exactly zero. (Note: This was true at the time this story took place. Ed Fels, MD, has practiced in Portland, Maine, for the past 10 years.)

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“Fair enough.” Dr. Benner looked up from another patient’s chart and motioned to the X-ray tech.

I waited, impatiently tapping out a paradiddle with my index fingers, and caught a glimpse of myself in the reflection of the cardiac monitor: I am Dr. Average: 5’10”, 165 lbs.; tawny, brown, thinning hair, with a brown-speckled gray mustache. Eschewing the traditional thigh-length white coat, I wear a blue, button-down shirt and my favorite plaid tartan tie, with a stethoscope draped around my neck. I bared my teeth: yellow-brown. And I tucked in my shirt. Then I remembered the stain on my tie. What the heck—I’m not running for public office.

A few minutes later, I pulled back the curtain, shook hands with Mom and Dad, and patted Amanda on the ankle. I told them what I knew from Dr. Benner: “Until a week ago, I understand you were okay, Amanda. Seventh grade? New school this year?” Sometimes 13-year-olds are quite chatty. Amanda locked eyes with her mom.

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“Yes, it’s a new school. Lots of adjustments,” her mom answered.

I scanned the skin; then turned my attention to Amanda’s hands. Why the hands? They’re innocent to touch but sometimes hold the key to diagnosis. Amanda allowed me to cradle one hand in mine. Her fingers were stone cold, the knuckles slightly swollen. Beneath several nails, a thin black streak—a nail infarct—was clearly evident. Internally, I grimaced. Nail infarcts are a subtle sign of inflammation in the smallest blood vessels. They’re rarely seen in self-limited viral infections. I turned the hands palm up. A blotchy, red rash extended from the fingers to midway up the forearm.

History of Present Illness

I turned my attention to Amanda’s mom. “What was the first thing you noticed out of the ordinary?” I consciously kept my voice even, unhurried.

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Filed Under: Conditions, SLE (Lupus) Tagged With: cyclophosphamide, kidney failure, Pericarditis, SteroidsIssue: October 2018

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