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JAMA: SLE Review

Drs. Caroline Siegel and Lisa Sammaritano (from HSS in NYC) have published an impressive overview of systemic lupus erythematosus (SLE) in JAMA. This timely, well-written review covers the many complexities of this multi-organ disorder that affects nearly 3.4 million people worldwide.

The authors estimate that 40% of SLE patients will develop lupus nephritis, and an estimated 10% of lupus nephritis develop end-stage kidney disease after 10 years. 

Many advances in lupus research have lead to new concepts and 3 new FDA approved SLE medications, including belimumab (active SLE and lupus nephritis), voclosporin (for lupus nephritis), and anifrolumab (for active SLE).

While the article covers clinical presentations (skin, articular, neuropsychiatric, renal, hematologic, etc), classification criteria, treatment standards, prognosis, complications (cardiovascular, infectious, neoplasia) and frequently asked questions (FAQs), below we highlight a few important takeaways. Be sure to bookmark, download, print and share this article with colleagues and trainees.

  • Estimated incidence of SLE in the US is approximately 5 to 12 per 100 000 person-years
  • 2019 EULAR/ACR classification criteria have a 96.1% sensitivity and 93.4% specificity; and require a positive antinuclear antibody test at a titer of 1:80 or higher
  • 50% of patients with SLE will have acute cutaneous lupus erythematosus sometime in their disease course
  • Nonscarring alopecia occurs in 40% to 70% of SLE patients
  • DLE patients - 30% progress to SLE
  • In SLE systematic reviews: 22-42% had leukopenia (primarily lymphopenia), 20% have thrombocytopenia, and 12% had autoimmune hemolytic anemia
  • Cardiopulmonary manifestations include pleuritis, (16.5%), valve thickening, valvular regurgitation/vegetations, myocarditis, pulmonary hypertension, pneumonitis, interstitial lung disease, diffuse alveolar hemorrhage, and shrinking lung syndrome
  • Pooled prevalence of neuropsychiatric SLE was 52.2%
  • Hydroxychloroquine is the standard of care for all SLE patients regardless of organ involvement or severity
  • Standard of care for proliferative lupus nephritis (classes III and IV) is initially with pulse intravenous glucocorticoids and either mycophenolate mofetil/mycophenolic acid or intravenous cyclophosphamide, followed by mycophenolate mofetil/mycophenolic acid or azathioprine. The goal is to tapering oral glucocorticoids within 3 to 6 months (prednisone equivalent 7.5 mg/d or less)
  • Newer or more aggressive therapies (belimumab, rituximab, anifrolumab, voclosporin, tacrolimus, cyclosporine) are considered in those refractory to other immunosuppressive medications
  • SLE patients have a significantly higher all-cause mortality compared with the general population

Join The Discussion

Donald E Thomas Jr

| Apr 11, 2024 4:43 pm

I suspect that the manuscript for this excellent article by my esteemed colleagues, Dr. Siegel and Sammaritano, was written before the latest EULAR recommendations were published.

I cringed when I read on this outline (and I'd love for it to be removed) that the "SOC for LN treatment" was stated as such. Many, to most of us, would use "combination therapy" for proliferative LN (meaning CYC or MMF + belimumab and/or a calcineurin inhibitor + IV pulse steroids at lower doses than in the past followed by lower doses of oral steroids than in the past).

Reason being: the vast majority of our patients with LN do not achieve a complete renal response and are at high risk for ESRD. Once nephrons are lost: it is permanent. Combination therapy, using BEL or a CNI, immediately increases CRRs, reduces steroids, and reduces long-term renal complications.

Please consider revising this list. I'd hate to see non-lupologists reading this and thinking this statement is still the standard of care. This is a great example of how medical information changes incredibly quickly, and this statement is now outdated.

If I had LN, I'd demand BEL +/or CNI and less steroids immediately for treatment.

btw... with the above, I am referring to most cases of proliferative nephritis

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The author has no conflicts of interest to disclose related to this subject