Rituximab treatment leads to long-term remission in man with EBA
Australian case study has recorded man's years without flares since therapy
A man with severe, treatment-resistant epidermolysis bullosa acquisita (EBA) experienced sustained clinical remission for at least five years after rituximab therapy.
Rituximab (sold in the U.S. as Rituxan and biosimilars) is an antibody that suppresses immune system activity by targeting immune B-cells — responsible for making antibodies, including the self-targeting antibodies that contribute to EBA.
“This case adds to the existing evidence for rituximab in the management of EBA and demonstrates its long-term efficacy in managing severe recalcitrant [treatment-resistant] EBA,” researchers wrote.
The report, “Sustained clinical remission for 5 years in severe epidermolysis bullosa acquisita following rituximab infusions,” was published in Journal of the European Academy of Dermatology and Venereology.
EBA is an autoimmune type of epidermolysis bullosa, caused by autoantibodies against the protein type VII collagen. That leads to the loss of anchoring between the epidermis (the top layer of the skin) and the dermal layer underneath, which leads to fragile skin that easily blisters. Mucous membranes are also affected.
Treatments mainly focus on preventing and treating blisters. As with other autoimmune conditions, EBA can be treated with immunosuppressive or anti-inflammatory agents, although these may have limited efficacy.
“However, rituximab … has shown promise in inducing long-term remission for patients unresponsive to conventional therapies,” the scientists wrote.
Blistering, swallowing difficulties eased
Now, a team led by researchers in Australia described the case of a man with severe EBA. In 1994, when he was 31 years old, the patient presented with new-onset widespread blistering and was diagnosed with EBA by analysis of a skin sample (biopsy) and the presence of antibodies against type VII collagen. He had significant involvement of the esophagus, the tube that carries food from the mouth to the stomach, and that led to difficulty swallowing.
After years of unsuccessful treatment with different immunosuppressants, he started rituximab in November 2012, which reduced skin blistering and difficulty swallowing.
Over the following years, he occasionally experienced return of symptoms, which were treated three times with additional cycles of rituximab. Other than short, two-week courses of prednisone, an anti-inflammatory agent, the patient received no other immunosuppressive treatments.
In 2018, the patient also had a mild flare, which resolved spontaneously, after dental procedures. He has remained in clinical remission without therapy and with undetectable levels of autoantibodies, since June that year.
Clinical improvement was demonstrated by a sustained decrease in the EB Disease Activity and Scarring Index score. Also, the patient’s quality of life improved, as measured by the Autoimmune Bullous Disease Quality of Life score.
“To the best of our knowledge, this patient represents the first published case of sustained [clinical remission] off therapy in EBA lasting over 3 years (5 years so far) following rituximab monotherapy,” the researchers wrote.
According to the team, rituximab’s efficacy in people with EBA may be associated with a blockage in B-cell maturation.
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