A Case of Pemphigus Vulgaris Successfully Treated with Single Filtration Plasmapheresis: A Correlation of Clinical Disease Activity with Serum Antibody Levels

Abstract

We report a patient with pemphigus vulgaris (PV) successfully treated with single filtration plasmapheresis. A 40-year-old man with PV was started on therapy with prednisolone (PSL). Although the dosage of PSL was doubled, and both cyclosporin A (CyA) and pulse therapy were added, the disease was not controlled. After single filtration plasmapheresis began, most of the eroded lesions on the trunk reepithelialized. A switch to double filtration was followed by recurrence. Finally, additional treatments with single filtration plasmapheresis were required to obtain remission. To evaluate the efficacy of the treatment, circulating antibodies were measured by immunofluorescence (IIF) and enzyme-linked immunosorbent assays (ELISAs) using recombinant desmoglein (Dsg) 3. IIF titer and the ELISA scores correlated with the clinical disease activity. It is suggested that ELISA was more sensitive than IIF.

Anti‐desmoglein IgG autoantibodies in patients with pemphigus in remission

“…In contrast, when modified ELISA was performed with 1 : 6400 serum dilution, a clear difference between active and remission time‐point antibody titres emerged (4032 vs. 1536). Our results corroborate other studies that show ELISA scores fluctuate in parallel with disease activity 4–13 …”

“…Ability to measure Dsg1 and Dsg3 antibody levels separately has allowed the examination of their relationship with disease severity in detail. ELISA scores have been shown to fluctuate in parallel with the time course of disease activity 4–13 …”

Follicular Non-Hodgkin's Lymphoma with Refractory Paraneoplastic Pemphigus: Case Report with Review of Novel Treatment Modalities

“…Plasmapheresis or plasma exchange in combination with corticosteroids and/or cyclophosphamide or azathioprine for prevention of rebound flare of disease showed similar rapid and beneficial results in association with decreasing auto-antibody levels in this group of refractory pemphigus, leading to reduced dosage of these drugs in time [11,13,14,23,24]. Synchronization of PE with subsequently IVIg therapy is theoretically even more powerful and has been used successfully in patients refractory to PE and immunosuppressants [14].…”

Autoantibody Levels and Clinical Disease Severity in Patients with Pemphigus: Comparison of Aggregated Anti-desmoglein ELISA Values and Indirect Immunofluorescence Titres

“…A correlation between ELISA scores and disease activity has been assessed in several studies and it was proposed that ELISA values tend to correlate with the severity of the disease (2,10,13,14,(16)(17)(18)(19)(20)(21)(22)(23)(24). More precisely, skin disease severity was initially suggested to be related to Dsg1 antibody levels, whereas Dsg3 antibody levels correlate directly with the involvement of mucous membranes (4,21).…”