A New Standard for Corticosteroid Dosing for Childhood-Onset SLE with Proliferative Lupus Nephritis

Recently, an international group of pediatric rheumatologists and nephrologists met and created a consensus on a standard for a steroid-dosing regimen to treat childhood-onset systemic lupus erythematosus with proliferative lupus nephritis.

Lupus Nephritis (LN)

Lupus nephritis is a kind of kidney inflammation that occurs from a complication in people who have systemic lupus erythematosus (commonly just referred to as lupus). Lupus is an autoimmune disease in which your immune system produces proteins, called autoantibodies, that attack your own body. In lupus nephritis, lupus autoantibodies affect the parts of your kidneys that filter out waste, leading to inflammation, hematuria, proteinuria, high blood pressure, or even kidney failure. Lupus is much more common in females than in males, most often occurring during the child-bearing years. In addition, lupus is more common in people of African or Asian descent. Of these lupus cases, half of all adults and eight out of every ten children will develop lupus nephritis.

Symptoms:

Some of the symptoms of lupus nephritis include foamy or dark urine, high blood pressure, and edema, or swelling caused by excess fluid, usually in the feet, legs, or ankles. After the initial onset of these symptoms, kidney problems and associated symptoms will start, including:

  • Joint pain or swelling
  • Muscle pain
  • Uncaused fever
  • Red rash, often on the face, in the shape of a butterfly

Treatments:

Treatments for lupus nephritis are aimed at trying to suppress the immune system and reduce high blood pressure and kidney inflammation. Some medicines to help in this process are:

  • Corticosteroids
  • Immunosuppressive drugs
  • ACE inhibitors
  • Diuretics
  • Beta blockers
  • Calcium channel blockers

In addition, eating the right foods can help manage kidney disease, and eating foods with low sodium and low cholesterol may help lower high blood pressure. In severe cases, patients with lupus nephritis may need a dialysis or a kidney transplant.

The Study that Created the Consensus

Corticosteroids are often used to treat patients with childhood-onset systemic lupus erythematosus (SLE) with proliferative lupus nephritis (LN). In spite of that, there is not a lot of research that helps to advise dosing regimens. As a result, a study was done to establish a standard for these steroid-dosing regimens in patients diagnosed with childhood-onset SLE with LN.

The study, which was a six-step study, used multiple consensus formation techniques and statistical modeling of patient profile ratings of patients with childhood-onset SLE with LN. Researchers specifically analyzed the factors that affect corticosteroid dosing, the medical records of 143 patient profiles that fit specific guidelines, and the physical evaluations of patient profiles from 460 patients. Then, they created a preliminary dosing regimen which was then refined. Finally, researchers studied the physical evaluations of 66 patient profiles in order to legitimize the regimen they had come up with.

It was discovered that the factors which effect corticosteroid dosing are the extent of proteinuria, a patient’s approximate glomerular filtration rate, any changes in the patient’s renal and extrarenal disease activity, and how long has passed since a patient’s last kidney biopsy.

Overall, a group of 103 physicians examined the ratings of 5,056 patient profiles in order to create corticosteroid dosing rules. In addition, 1,838 ratings examined by 60 physicians were used to achieve consensus and validate the standardized dosing regimen.

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