Clearance Efficacy of Autoantibodies in Double Filtration Plasmapheresis for Pemphigus Foliaceus

Abstract

Plasma exchange is a therapeutic option in severe cases of pemphigus. Both centrifugal plasmapheresis and double filtration plasmapheresis (DFPP) are available, but the latter, newer, procedure currently prevails because of its safety advantage (1, 2). In DFPP, immunoglobulins (Igs) are selectively removed, while minimizing the loss of albumin (3). In several studies, the removal rates (RRs) of anti-desmoglein (Dsg) 1 and Dsg3 autoantibodies have been estimated by using serum antibody titres immediately before and after plasmapheresis (3). This simple estimation is designated RRl in the present study. Given the data for anti-Dsg titres and Ig amounts in the exchanged effluents, however, we can calculate the corrected or compensated RR, named RR2 in this study, which reflects the elimination efficacy of antibodies more accurately than RRl. RR2 of pemphigus autoantibodies has been reported only in centrifugal plasmapheresis (4), but not in DFPP. This study examined RR2 of anti-Dsg 1 autoantibody with reference to total immunoglobulins (Igs) in 4 cycles of DFPP performed in 2 patients with pemphigus foliaceus (PF). PATIENTS AND METHODSTwo patients with PF were etirolled in this study.Case 1. Case 1 was an 85-year-old Japanese man with erosions on his trutik and extremities. There was no involvement of the oral mucous membrane. He had had chronic myelomonocytic leukaemia for 6 months without treatment. Immediately before the start of DFPP, the enzyme-linked immunosorbent assay (ELISA) titres for Dsgl and Dsg3 were 2,031 and <5 indices, respectively. Following administration of prednisolone (60 mg daily) and mizoribine (100 mg daily), DFPP was implemented twice in total on 2 consecutive days. Itnmediately after the second DFPP, anti-Dsgl antibody was decreased dramatically to 220 index, with therapeutic success. However, the patient had severe sepsis, with serum IgG lowered from 1,320 to 407 mg/ dl. Blood culture and culture of the tip of the central venous catheter was positive for Staphylococcus aureus. Serum albumin and fibrinogen levels were decreased from 3.5 to 2.5 g/dl, and 87 to >50 mg/dl, respectively. Over a period of 2 months after DFPP, prednisolone was tapered to 30 mg/day without clinical recurrence.Case 2. Case 2 was a 48-year-old Japanese man who had had PF for 2 years, which was controlled with oral prednisolone (20-60 mg daily) plus cyclosporine (150 mg daily). However, flaccid vesicles and erosions continued to recur Because of the adverse effects of corticosteroids (pulmonary embolism and avascular necrosis of the femoral bone), and of intravenous high-dose Ig (thrombocytopaenia, platelet count <50,000 ul), 2 courses of DFPP were implemented on 2 consecutive days. The ELISA titre for anti-Dsgl autoantibody was decreased from 88 to 18 and anti-Dsg3 antibody to <5, with therapeutic success. Serum albumin and fibrinogen levels were decreased from 4.0 to 3.7 g/dl, and 274 to 220 mg/dl, respectively. For 3 months after DFPP, no recurrence of symptoms was observed with prednisolone (20 mg/day) plus cyclo...

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