Biological therapy of autoimmune blistering diseases

ABSTRACT

Introduction: Autoimmune blistering skin diseases are a group of disorders subdivided according to the location of blister formation: intraepidermal blistering in the pemphigus group and subepidermal in the pemphigoid group. These conditions are clinically heterogeneous and are treated with systemic corticosteroids and/or other forms of immunosuppression on the basis of clinical subtype and disease severity. These approaches may not be effective for the induction and maintenance of clinical response or need to be stopped because of intolerable side effects.

Areas covered: Biological therapies can represent a valid alternative strategy in various autoimmune blistering disorders and this review article will address this issue with a special focus on pemphigus vulgaris and bullous pemphigoid. These biological approaches are designed to target B cells, autoantibodies, complement proteins, and several cytokines.

Expert opinion: Innovative strategies for the treatment of autoimmune blistering conditions primarily depend on the use of drugs with a high degree of specificity targeting crucial steps in the immunopathology of these disorders. Novel biological agents offer treatment alternatives to patients with autoimmune blistering conditions by targeting B cells, pathogenic autoantibodies, complement and cytokines.

KEYWORDS:

• Autoimmune blistering diseases
• pemphigus vulgaris
• pemphigoid
• biological agents
• autoimmunity

Article highlights

• Selective depletion of B cells is a well-established treatment autoimmune blistering diseases.

• Rituximab associated with short-course prednisone should be considered the first-line treatment in newly diagnosed moderate-to-severe pemphigus.

• Alive T cells that express a chimeric autoantibody receptor (CAAR-T cells) are able to deplete anti-DSG3-specific B cells in vitro and in a pemphigus mice model.

• BP patients can be treated successfully with omalizumab, an anti-IgE mAb.

• Targeting the neonatal Fc receptor for reducing pathogenic IgG autoantibodies is an attractive option for the treatment of autoimmune blistering conditions.

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Declaration of interest

The author has no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial relationships or otherwise to disclose.

Additional information

Funding

This paper was not funded.