Treatment of pemphigus vulgaris with protein A immunoadsorption: case report of long-term history showing favorable outcome.
A pemphigus vulgaris (PV) patient with a 14-year history of severe and painful blistering of skin and mucous membranes as well as side effects from corticosteroids and concomitant immunosuppressive drug treatment was managed successfully by protein A immunoadsorption (IA). After 19 sessions of protein A IA, the patient showed remission of PV and healing of mucocutaneous lesions and the skin. The level of the pathogenic autoantibodies to the adhesion proteins desmoglein 1 (Dsg-1) and desmoglein 3 (Dsg-3) measured by ELISA and immunofluorescence microscopy revealed a significant removal of autoantibodies after each IA therapy. There was a weak rebound of anti-Dsg-1 and anti-Dsg-3 antibodies between IA sessions but an overall decrease over the period of IA therapy. This case demonstrates the effective use of protein A IA as an adjuvant and corticosteroid-sparing therapy in severe pemphigus refractory to standard immunosuppressive therapy and underscores the need for careful monitoring of autoantibodies.
Frost
Nina
N
Universitätsklinikum Tübingen, Abteilung Innere Medizin IV, Sektion Nieren und Hochdruckkrankheiten, 72076 Tübingen, Germany.
Messer
Gerald
G
Fierlbeck
Gerhard
G
Risler
Teut
T
Lytton
Simon D
SD
D001323:Autoantibodies / Q000097:blood; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D006801:Humans; D007163:Immunosorbent Techniques; D008875:Middle Aged; D010392:Pemphigus / Q000276:immunology / Q000628:therapy*; D013205:Staphylococcal Protein A / Q000627:therapeutic use*
D001323:Autoantibodies; D013205:Staphylococcal Protein A