On July 27, 2022, GSK announced that the US Food and Drug Administration (FDA) has approved Benlysta (belimumab) as an intravenous treatment for lupus and active lupus nephritis (LN) in children between the ages of 5 and 17 who are receiving standard therapy. This approval makes Benlysta the first and only biologic treatment approved for both adults and children with lupus or LN. Although Benlysta was first approved in 2011, its novel use for pediatric LN will be beneficial as LN is responsible for increased complications, hospitalizations and mortality rates in patients.
Systemic lupus erythematosus (SLE) is an incurable autoimmune disease that causes a variety of painful symptoms in those affected, such as swollen joints, fatigue, random fever, rashes and organ damage. Lupus nephritis (LN) is one of the organ-related manifestations of SLE and causes serious inflammation of the kidneys that can lead to end-stage kidney disease where dialysis or a kidney transplant is needed.
With LN, the small blood vessels that filter waste through the kidney (glomeruli) become swollen and scarred as the immune system attacks them like it would a disease. In children with lupus, LN can cause serious complications — the first case of LN usually occurs within the first two years after initial lupus diagnosis and despite improvements in diagnosis and treatment over the past year, LN indicates poor prognosis.
How Benlysta (belimumab) Works
Benlysta (belimumab) is a biologic therapy, a human monoclonal antibody. It is made to be taken in addition to other lupus treatments and is available as an autoinjector or pre-filled syringe to inject by the patient themselves, or as an intravenous infusion administered by a healthcare professional. The latter option is the only one available for children with LN.
B cells are a type of white blood cell important for the functioning of the immune system. In those affected by lupus, autoreactive B cells (those that react against the body), remain in the body long after the “threat” to the immune system is gone. Autoreactive B cells release antibodies like other B cells do, except they release autoantibodies. The autoantibodies do not fight actual infection or bacteria, but rather the normal tissue in the body. When this behaviour is prolonged, it results in inflammation.
B cells require a specific protein for growth called BLyS (B-lymphocyte stimulator), which is implicated in the pathogenesis of SLE. Benlysta is a BLyS-specific inhibitor that binds to BLyS and inhibits the survival of B cells (including autoreactive B cells), and decreases the differentiation of B cells into immunoglobulin-producing plasma cells. This reduces abnormal immune system activity that contributes to lupus.
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Managing Lupus Nephritis
Before Benlysta was approved, the treatment options for LN were limited to non-selective immunosuppressants and corticosteroids. Stevan W. Gibson, President and CEO of the Lupus Foundation of America, stated that “this approval marks a significant step forward in providing treatment options to these children at risk of incurring kidney damage early on in life.”
Ultimately, the long-term goal of LN management in both children and adults is to preserve kidney function and ensure minimal treatment-related toxicities and morbidities. Adding Benlysta to the list of treatment options for pediatric patients may reduce the incidence of kidney damage and result in a better quality of life for those affected.