Rituximab treatment may be safe, effective for EBA: Review study
About three-quarters of treated patients saw disease remission
About three-quarters of people with epidermolysis bullosa acquisita (EBA) treated with rituximab experienced disease remission, according to a review study. Almost all patients responded to treatment.
Overall, nearly 40% of rituximab-treated patients experienced relapses — when symptoms return after a period of improvement — over almost two years of follow-up. Treatment-related side effects were mainly mild and temporary infusion reactions.
Rituximab, sold in the U.S. as Rituxan and biosimilars, is an antibody that suppresses the activity of the immune system, by targeting B-cells — those responsible for producing antibodies, including self-targeting antibodies that cause EBA.
Rituximab “is safe and effective in patients with EBA,” the researchers wrote in the study, “Rituximab in the Treatment of Epidermolysis Bullosa Acquisita: A Systematic Review,” published in the Journal of Clinical and Aesthetic Dermatology. “This biological treatment modality can be routinely used in managing EBA.”
EBA is a rare skin disorder caused by self-reactive antibodies against a protein called type VII collagen, essential for maintaining skin structure. It is characterized by skin blistering mostly in the mucous membranes — tissue that lines body cavities such as the mouth, nose, and eyes — but also in the hands, feet, knees, elbows, and buttocks.
Rituximab treatment used for difficult cases
EBA treatment may involve immunosuppressive agents, corticosteroids such as prednisone, or anti-inflammatory medications such as dapsone. However, “patients are commonly refractory to conventional immunosuppressive agents, and many fail to attain disease remission,” the researchers wrote.
As EBA is rare, no properly designed clinical trial has assessed the efficacy and safety of medications for this type of epidermolysis bullosa. Previous studies indicated that only rituximab and intravenous immunoglobulin, a type of therapy that delivers specific antibodies obtained from healthy people to restore or help regulate immune responses in patients, may lead to complete disease remission.
Researchers in Iran, along with a collaborator in the U.S., aimed to summarize current evidence on the benefits of rituximab in EBA. A total of 31 studies were analyzed, involving EBA 68 patients with a mean age of 52.9.
Of the 43 patients whose EBA subtype was available, most had mechanobullous (classical) EBA (53.5%) — with erosions and fluid-filled blisters at trauma sites — and 44.2% had non-mechanobullous (inflammatory) EBA. One patient had a mixed form of the disease.
The main reason for rituximab use was difficult-to-treat disease (38 patients, or 55.9%). Most (63 patients, or 92.7%) had a clinical response to rituximab. Among 28 studies considering disease remission a goal of treatment, this outcome was reported in 73.8% of patients.
During a mean follow-up of 23 months, 15 patients (39.5%) had disease relapses and 11 (28.2%) experienced rituximab-related side effects. The data came from 17 studies with 38 EBA patients.
Most side effects were mild and transient infusion reactions. Of those with more severe reactions, two patients had pneumonia, an infection of the lungs, that caused their deaths. One patient had a deep vein thrombosis, a blood clot that forms in a deep vein, usually in the legs.
“The only major safety concern is the higher risk of infection, which should be considered before administration in vulnerable patients,” the researchers wrote.
Additional studies “could improve our insights into the effectiveness and action mechanisms of [rituximab] therapy in patients with EBA,” they wrote. Combination therapy with intravenous immunoglobulin may provide further clinical benefits, they said.
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