Protein A immunoadsorption: a novel and effective adjuvant treatment of severe pemphigus.
BACKGROUND
Pemphigus foliaceus (PF) and pemphigus vulgaris (PV) are autoimmune blistering skin diseases usually treated with high-dose systemic corticosteroids and other immunosuppressants that may cause severe side-effects. Plasmapheresis also has been demonstrated to be of benefit in the treatment of pemphigus. In contrast to plasmapheresis, staphylococcal protein A immunoadsorption (PA-IA) specifically removes immunoglobulin from the circulation, allows treatment of larger plasma volumes, and does not require the substitution of plasma components.


OBJECTIVE
To determine the effectiveness and side-effects of PA-IA in patients with severe pemphigus.


METHODS
Five patients with severe pemphigus (PV, n = 4; PF, n = 1) were treated by PA-IA. Three of these patients had been refractory to various treatment regimens. In addition to PA-IA, methylprednisolone, 0.5 mg x kg-1 body weight day-1 was given initially and subsequently tapered.


RESULTS
In all patients, a dramatic clinical improvement was seen within 2 weeks after initiation of therapy. Patients were free of lesions after 3, 4, 4, 10 and 21 weeks of treatment, respectively. Concurrently, autoantibody levels decreased rapidly.


CONCLUSIONS
PA-IA is a rational, effective, and safe adjuvant therapy for severe pemphigus and warrants wider use for this indication. A controlled study should compare side-effects and effectiveness of PA-IA with other treatment options for pemphigus.
Schmidt
E
E
Departments of Dermatology and Transfusion Medicine and Immunohematology, University of Würzburg, Josef-Schneider-Str 2, 97080 Würzburg, Germany.
Klinker
E
E
Opitz
A
A
Herzog
S
S
Sitaru
C
C
Goebeler
M
M
Mansouri Taleghoni
B
B
Bröcker
E-B
EB
Zillikens
D
D
D000276:Adjuvants, Immunologic / Q000627:therapeutic use; D000328:Adult; D000368:Aged; D002648:Child; D002908:Chronic Disease; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D019084:Fluorescent Antibody Technique, Indirect; D006801:Humans; D008297:Male; D008875:Middle Aged; D010392:Pemphigus / Q000628:therapy*; D013205:Staphylococcal Protein A / Q000627:therapeutic use*
D000276:Adjuvants, Immunologic; D013205:Staphylococcal Protein A