Plasma exchange in pemphigus. Uncontrolled study of ten patients

Abstract

Plasma exchange, together with oral immunosuppressive drugs and various doses of adrenal steroids, were used in the treatment of ten patients with pemphigus. Initially plasma exchange decreased the intercellular antibody (IC Ab) level in all patients and induced a clinical improvement in the conditions of eight of ten patients. This improvement was sustained for the six patients who received simultaneously 0.5 mg/kg/day or more of prednisone. Among the eight patients, four previously corticoresistant patients had perfect control of their disease. Conversely the other four patients, treated with low doses of prednisone, experienced a posttreatment rebound in IC Ab level and showed no or only transient clinical improvement. Plasma exchange may be useful in bringing corticoresistant pemphigus under control but it is not to be used without accompanying high doses of adrenal steroids.

The Management of Pemphigus Vulgaris in a Burn Intensive Care Unit

“…[42][43][44][45] Several small retrospective case series have found clinical improvement in anywhere from 57 to 80% of patients with refractory pemphigus when plasmapheresis has been added to a regimen of steroids and immunosuppressants. [45][46][47] Although it lacked the power to address clinical benefit, one randomized controlled trial of 40 patients with PV evaluated the steroid-sparing benefit of 10 largevolume plasmapheresis procedures when added to prednisolone (n = 19) in comparison with prednisolone alone (n = 15). 48 The group receiving plasmapheresis did not enjoy a significant steroid-sparing effect and, in addition, had four deaths caused by sepsis, suggesting that the intervention promoted an increased risk of infection.…”

Immunoapheresis in Paraneoplastic Pemphigus

“…This poor response to systemic corticosteroids is in keeping with reports from the literature indicating that various immunosuppressive regimens (high-dose glucocorticosteroids, azathioprine, and/or cyclosporine) are of no or little value in patients with PNP. 4 Despite conflicting reports on its efficacy in pemphigus, [18][19][20][21] we originally considered plasmapheresis as a possible treatment strategy but ultimately decided to use a more specific technique to deplete our patient's serum of the pathogenic IgG1 autoantibodies, ie, immunoadsorption on anti-human IgG bead-formed agarose gel. 9 Immunoapheresis is an established treatment modality in various autoantibody-mediated diseases, eg, idiopathic thrombocytopenic purpura, cryoglobulinemia, Goodpasture syndrome, lupus erythematosus, and myasthenia gravis.…”

Plasmapheresis as an Alternative or Adjunctive Therapy in Problem Cases of Pemphigus

“…From then on several pa tients with pemphigus have been successful ly treated by this procedure [Auerbach and Bystryn, 1979;Blaszczyk et al, 1979;Meurer and Braun-Falco, 1979 ;Neppert et al, 1980;Neumann et al, 1980;Hunziker et al, 1981 ;Swanson and Dahl, 1981 ;7b-rok et al, 1981 ;Coverton and Armstrong, 1982;Roujeau et al, 1982;1983], and new evidence, both clinical [Terpstra et al, 1979;Moncada et al, 1982;Storer et a!., 1982;Green and Maize, 1982] and experimental [Buschard et al, 1981;Anhalt et al, 1982], has confirmed the pathogenic role of pem phigus antibodies (Pab). This has prompted us to reapply plasmapheresis to pemphigus, reserving it for carefully selected patients.…”