Belimumab Shows Promise in Small Cohort of Adults With NPSLE

Benlysta (belimumab; GlaxoSmithKline) may be an effective treatment option for patients with neuropsychiatric systemic lupus erythematosus (NPSLE), even in refractory cases, according to new case findings.

The report, published in European Journal of Internal Medicine, is based on 5 cases of NPSLE in patients with previous immunosuppressant therapy failure.

The authors explained that a lack of an established treatment protocol, along with the frequency of resistance to general treatment, have made treatment difficult for patients with NPSLE.

Belimumab is an anti-BAFF monoclonal antibody that has been approved by the FDA to treat systemic lupus erythematosus (SLE). BAFF and interleukin 6 expression have been shown to beheightened in patients with SLE.

“To date, reports on belimumab treatment for SLE have covered various conditions, resulting in a decrease in the SLE [disease activity index] score, arthropathy, nephropathy, thrombocytopenia, and hemolytic anemia, while the effect of belimumab on refractory NPSLE is still unclear,” the investigators wrote.

The case report focuses on the experiences of 5 patients (4 females) aged 19 to 51 years. All of the patients had highly active disease and central nervous system lesions, with resistance to conventional treatment.

“Short-term intravenous high-dose dexamethasone and/or methylprednisolone and immunosuppressants, including cyclophosphamide, mycophenolate mofetil (MMF), and hydroxychloroquine, had been used for treatment before enrollment in the belimumab protocol,” the investigators reported. “In addition, 1 patient with intractable disease did not respond to the combination treatment, and thus received plasma exchange and immunoadsorption therapy as well.”

They said all of the patients had multiple organ involvement, including the kidneys, blood, and neuropsychiatric system.

On average, the patients began belimumab treatment 1.3 years after disease onset. They were given belimumab at a dosage of 10 mg/kg every 2 weeks for the first 3 doses and then every 4 weeks thereafter.

Each patient also was given moderate to high doses of corticosteroids and immunosuppressants at the start of belimumab treatment, and the use of those agents was gradually reduced over time.

The treatment had a swift impact.

“After belimumab treatment, improvement in the skin and mucocutaneous lesions was fast,” the authors wrote. “In addition, fever, fatigue, and other symptoms were alleviated.”

SLE disease activity scores, which ranged from 12 to 37 in the patients at the start of treatment, dropped to between 3 and 8 after treatment. All 5 patients had disease activity index scores of less than 6 at folloiw-ups of 6 and 12 months after therapy.

Although the treatment had an overall positive effect, the authors noted some caveats. For instance, one patient had a recurrence of central nervous system–related symptoms and another maintained low-dose steroids for 11 months and showed an increase in autoantibodies and proteinuria, they added. Four of the 5 patients remained in remission at the time of the authors’ writing.

“Our case reports, in concert with available literature evidence, suggest that belimumab could be an effective and safe option to treat NPSLE, even in refractory cases, allowing [physicians] to spare glucocorticoids and immunosuppressants, such as MMF,” the authors concluded.

They added that larger studies will be necessary to better understand the efficacy of the therapy in this patient group.

Reference

Cheng H, Zhao C-S, Yan C-L, Gao C, Wen H-Y. Efficacy of belimumab for refractory systemic lupus erythematosus (SLE) involving the central nervous system. Eur J Intern Med. Published online July 2, 2021. doi:10.1016/j.ejim.2021.06.012