Based on the consensus of an international group of pediatric rheumatologists and nephrologists, a standardized steroid-dosing regimen has been developed for the treatment of childhood-onset systemic lupus erythematosus (SLE) with proliferative lupus nephritis (LN). The study was published in Arthritis & Rheumatology.

Although corticosteroids are commonly used in the treatment of patients with childhood-onset SLE with LN, strong medical evidence to guide dosing regimens is lacking.

The current study was undertaken to establish consensus for a standardized steroid-dosing regimen in patients with childhood-onset SLE with LN. 


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Researchers used consensus formation techniques and statistical modeling of patient profile ratings of a cohort of patients with childhood-onset SLE with LN. The study design included 6 steps:

  1. Literature review to identify factors affecting corticosteroid dosing.
  2. Medical record review of 143 patients to develop patient profiles between 2 consecutive visits within the first 24 months of LN diagnosis.
  3. Physician evaluation of 460 patient profiles to rate the course of LN and extrarenal SLE between the 2 visits and to adjudicate oral corticosteroid and/or intravenous corticosteroid dosage for the next 30 days considering prior corticosteroid use.
  4. Development of a preliminary standardized steroid dosing regimen.
  5. Consensus refinement of preliminary standardized steroid dosing regimen.
  6. Physician evaluation of 66 patient profiles describing the disease course for up to 6 months after LN diagnosis to validate the standardized steroid dosing regimen.

Factors that increased corticosteroid dosing included degree of proteinuria, estimated glomerular filtration rate, changes in renal and extrarenal disease activity, and the time since kidney biopsy.

In total, 103 physicians provided 5056 patient profile ratings to develop LN and extrarenal SLE course definitions and corticosteroid dosing rules. An additional 1838 ratings by 60 physicians achieved consensus and validated the standardized dosing regimen.

Limitations of the study included the limited application for some patients with severe disease or those who require kidney replacement therapy and the assumption that patients were adherent to their medications.

“The SSR is anticipated to be used for clinical care and to standardize CS dosage during clinical trials,” the researchers concluded. “To enhance the widespread use of the SSR, a web-based calculator is in development.”

Reference

Chalhoub NE, Wenderfer SE, Levy DM, et al. International consensus for the dosing of corticosteroids in childhood-onset systemic lupus erythematosus with proliferative lupus nephritis. Arthritis Rheumatol. Published online July 19, 2021. doi:10.1002/art.41930