Restoring immune tolerance in pemphigus vulgaris
Résumé
Intravenous immunoglobulin (IVIg)-a preparation of polyclonal serum IgG pooled from thousands of blood donors-has been used for nearly three decades, and is proving to be efficient treatment for many autoimmune blistering diseases, including pemphigus vulgaris (PV). Despite its widespread use and therapeutic success, the mechanisms of action of IVIg are not completely understood. However, several hypotheses have been suggested regarding its anti-inflammatory and immunomodulatory actions. In this study, the authors present a twenty-year follow-up of 21 patients with clinical and immunopathological confirmed PV, treated with IVIg as monotherapy, according to an established published protocol. IVIg therapy produced long-term sustained, clinical, serological, and immunopathologic remission. For 20 years, these patients received no drugs and experienced no disease. This observation suggests the establishment of immune balance or restoration of immune regulation in PV. Twelve (57%) of 21 PV patients experienced no relapses during follow-up. Six patients (29%) experienced a relapse due to acute stress or post-coronavirus infection and/or vaccination. Reinstitution of IVIg resulted in prompt sustained recovery. Three patients (14.2%), in clinical and serological remission, died due to unrelated causes. No severe adverse effects from IVIg were documented in all 21 patients. The combination of several anti-inflammatory and immunomodulatory effects of IVIg may explain its beneficial influence in this autoimmune disease. This study provides a human prototype to examine the pathophysiology of autoimmunity and a model to study immune regulation and mechanisms that can facilitate restoring immune tolerance.
This research demonstrated a twenty-year clinical and serological remission in patients with recalcitrant pemphigus vulgaris (PV), a potentially fatal mucocutaneous autoimmune disease. Conventional immunosuppressive therapy was unsuccessful. Consequently, these patients were treated with intravenous immunoglobulins (IVIg) as monotherapy by a defined protocol. It requires multiple infusions at defined intervals to induce remission. The twenty-year remission was post-IVIg therapy. This would suggest restoration of immune tolerance. IVIg has antiinflammatory and immunomodulatory effects in the microenvironment. Its long-term effects are not completely studied. This long-term analytical study provides a model to examine autoimmunity and design disease-specific protocols that facilitate the mechanisms to restore immune tolerance in other autoimmune diseases.
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