自己免疫性水疱症とアフェレシス : Paraneoplastic pemphigusに対する血漿交換療法の経験から

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Cases of paraneoplastic pemphigus have been accumulated since Anhalt et al. documented this rare autoimmune bullous disease. Therapeutic protocol, however, has not been established, and poor responsiveness to corticosteroids, azathioprine, and cyclosporine has been reported. We have experienced a patient with paraneoplastic pemphigus who responded to repeated plasmapheresis. The patient, a 56-year-old man in remission from chronic lymphocytic leukemia, developed erosive and ulcerative lessions on the oral mucosa with vesicles on the body. He was first diagnosed with Stevens-Johnson syndrome (erythema multiforme major) in the first hospital and treated with pulsed methyl predniscolone (1,000 mg/day for 3 days), but with poor response. He was transferred to our hospital, and examination revealed suprabasal acantholytic separation in histology ; antibasememt membrane deposit of complement (C) 3 in direct immunohistochemistry, besides antiintracellular deposition of IgG and C 3 ; and serum reactivity to transitional epithelium of rat urinary bladder. Autoantibodies in the serum of patients to human epidermal 210 kD and 190 kD proteins were shown in a Western blotting technique, ancl 250 kD, 210 kD, and 190 kD proteins were shown in immunoprecipitation study, leading to our final diagnosis. Pulsed corticosteroid therapy was tried resulting in minimal effect. The plasmapheresis technique was applied, since recalcitrant pemphigus vulgaris and pemphigus foliaceus have been successfully treated. A single filtration procedure 5 times within 2 weeks followed by a double filtration technique 6 times within the next 3 weeks resulted in a reduction of autoantibody titer measured with indirect immunofluorescence technique from the level of X 1280 to the level of X 40-20. This was associated with mucosal and cutaneous re-epithelialization. The double filt ration method was continued further once every two weeks and maintained the antibody level at low titer, associated with controlled mucosa and skin condition. The patient suffered from respiratory infection and septicaemia, however, and died 6 months after onset of the disease. This case suggests that plasmapheresis may be beneficial in the reduction of autoantibody in IgG fraction of the patient's serum and in clinical improvement. Since no established therapeutic method is available for the treatment of paraneoplastic pemphigus, we suggest that the use of plasmapheresis may be a therapeutic option for the paraneoplastic pemphigus.
日本アフェレシス学会の論文
2000-10-31

自己免疫性水疱症とアフェレシス : Paraneoplastic pemphigusに対する血漿交換療法の経験から

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