For their part, rheumatologists see their lupus patients for decades, through periods of remission and relapse, blending medications according to disease activity, striving to control the disease without oversuppressing the immune system.
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Explore This IssueOctober 2018
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I liken my job as a rheumatologist to adjusting the damper in a fireplace: Undertreating lupus is akin to opening the damper wide. The fire of uncontrolled lupus rages. Close the damper too tightly, and the immune system is smothered. I want an even flame, a competent immune system. It can be a tough balance to maintain.
But how does one manage lupus? There is no simple recipe. For mild disease—and there are many patients with mild disease—strict sun avoidance may control sun-sensitive rashes. Lupus patients are advised to wear sun-protective clothing and sun block, and to avoid outdoor activities in the middle of bright, sunny days. For more resistant skin disease or arthritis, the anti-malarial medication hydroxychloroquine (Plaquenil) is often prescribed.
Belimumab, azathioprine, rituximab, mycophenolate mofetil and cyclophosphamide have found their way into SLE management for more severe disease.3 And, of course, there are steroids.
Acting like a wet blanket on the immune system, prednisone and other members of the corticosteroid family downregulate multiple gene products responsible for inflammation. Given in heroic doses, steroids act quickly, and if an injured organ has a wisp of function remaining, the drug may stave off organ failure. All too often though, corticosteroids’ side effects soon emerge—weight gain, acne, anxiety, insomnia, hypertension, diabetes—and office visits become a war of wills. My patients want less. I want to prescribe less, but the disease demands more.
Amanda needed a second medication, a slower-acting immune suppressant that could gradually allow us to taper prednisone.
One morning on my way to the ICU, I stopped at the pathology office to review the biopsy slides of the kidney. I was interested in the basic question: What type of inflammation was present on Amanda’s kidney biopsy? As luck would have it, John Parker, the nephrologist who’d performed the kidney biopsy, arrived at the same time. We sat down with the pathologist and peered through a three-headed teaching scope, entering the microscopic world of cellular infiltrates, glomeruli and basement membranes. Histology was not my forte in medical school. I figured (correctly) that’s why you have pathologists. But even I could see the normal architecture on the kidney biopsy was distorted almost beyond recognition.
“Diffuse membranoproliferative glomerulonephritis—Class IV SLE nephritis,” the pathologist said finally, peering over her glasses. “What a shame. Thirteen years old?”