Combination Therapy of Plasma Exchange and Rituximab to Treat Cicatricial Pemphigoid and Bullous Pemphigoid

Abstract

The pemphigoid group of subepidermal autoimmune blistering diseases can affect both cutaneous and mucosal tissues. Therapy of this group of diseases, including cicatricial pemphigoid (CP) and bullous pemphigoid (BP), consists of systemic steroids and immunomodulatory agents. Recalcitrant cases have typically been treated with plasmapheresis or rituximab individually. This report describes two patients with severe, rapidly progressive CP and BP refractory to high-dosage systemic steroids and immunomodulatory agents. Both patients were treated with a combination of plasmapheresis and rituximab. In addition to these cases, one retrospective study showed the effectiveness of other immunosuppressants in combination with plasmapheresis in 17 patients with pemphigus refractory to corticosteroids and immunosuppressants alone. No major adverse events occurred in the study. Similar studies employing immunoadsorption and rituximab with various combinations of intravenous immune globulin (IVIg), corticosteroids, and other conventional immunosuppressants have shown promising results in other autoimmune blistering diseases. The successful response in the patients described here, as well as those described in the literature who underwent similar management, provides a possible combination treatment option for patients with severe, recalcitrant pemphigoid. A further trial with a larger group of pemphigoid patients is warranted.

Efficacy of therapeutic plasma exchange in a case of severe, generalised canine pemphigus foliaceus

“…10 The decision to discontinue TPE in human patients is based on monitoring the clinical improvement and/or anti-desmoglein titers; it is often carried out over several weeks to months in human pemphigus patients. 9,11,12 In our case, TPE was discontinued after 1 week based on improved clinical response and as a consequence of financial constraints limiting additional TPE sessions. The extended application of TPE alongside adjunctive interventions such as human immunoglobulin therapy may have resulted in a better outcome in this patient.…”

“…In human medicine, the application of TPE for the treatment of pemphigus is considered for cases where standard therapies have been ineffective. The procedure has yielded significant reductions in circulating autoantibodies, leading to clinical improvement and a reduction in the severity of the disease 9–12 . There is a scarcity of literature published on the use of TPE for the treatment of pemphigus in veterinary medicine.…”

“…Thus, the primary objective of TPE is to lower circulating autoantibody levels, attain rapid disease control and provide immunomodulation until systemic immunosuppressive therapy takes effect 10 . The decision to discontinue TPE in human patients is based on monitoring the clinical improvement and/or anti‐desmoglein titers; it is often carried out over several weeks to months in human pemphigus patients 9,11,12 . In our case, TPE was discontinued after 1 week based on improved clinical response and as a consequence of financial constraints limiting additional TPE sessions.…”

Interest of therapeutic plasmapheresis in a chronic hemodialysis patient with severe bullous pemphigoid

“…In contrast to the experience of Chang et al, Kitabata et al, and Nasu et al who used systemic steroids and plasma exchange concomitantly, in our case, we observed that therapeutic plasma exchange could obviate the use of systemic immunosuppression. [10][11][12][15][16][17] In our patient, the extremely high levels of autoantibodies recognized after dilution may have played a role in this patient's relapse, highlighting the efficacy of therapeutic plasmapheresis in quickly reducing the high titer of pathogenic autoantibodies and inducing remission. Moreover, the possible effect of therapeutic plasma exchange on the complement protein system and immunomodulation of the immune system should not be ignored.…”

“…Considering the patient's underlying clinical status (age, diabetes mellitus, chronic HD) and associated high risk of systemic immunosuppressive side effects, the decision was made to perform therapeutic plasmapheresis, rather than give high-dose systemic steroids. [10][11][12] Contrary to the United States, DFPP is approved in Europe and Japan. In DFPP, the plasma is first separated by a plasma filter, and then the separated plasma is filtered through a second filter (plasma fractionator).…”