Improved protocol for treatment of pemphigus vulgaris with protein A immunoadsorption.
BACKGROUND
Pemphigus vulgaris is a life-threatening autoimmune blistering skin disease, usually treated with high-dose corticosteroids in combination with other immunosuppressants. However, this regimen may prove inadequate in severe cases and can cause dangerous side-effects. We have recently reported protein A immunoadsorption (PAIA) to be an effective adjuvant treatment for induction of remission in severe pemphigus. However, in a significant number of cases, the disease rapidly recurred once PAIA and immunosuppressive medication were tapered.


OBJECTIVE
The aim of the present study was to develop a PAIA-based therapeutic regimen that would result in a more prolonged remission of pemphigus.


METHODS
Nine patients with pemphigus vulgaris were treated with a modified protocol characterized by a combination of PAIA with a higher initial dose of systemic methylprednisolone (2 mg/kg). In addition, azathioprine or mycophenolate mofetil was administered as a steroid-sparing agent.


RESULTS
In all nine patients treated with this regimen, we observed a sharp decline of circulating autoantibody levels and dramatic improvement of cutaneous and mucosal lesions within 4 weeks of therapy. The patients remained free of clinical disease for up to 26 months after PAIA treatment was discontinued.


CONCLUSIONS
The improved treatment protocol appears to combine highly effective induction of clinical remission in severe or treatment-resistant pemphigus with a prolonged subsequent symptom-free interval.
Shimanovich
I
I
Department of Dermatology, University of Lübeck, Lübeck, Germany.
Herzog
S
S
Schmidt
E
E
Opitz
A
A
Klinker
E
E
Bröcker
E-B
EB
Goebeler
M
M
Zillikens
D
D
D000368:Aged; D001323:Autoantibodies / Q000097:blood; D002985:Clinical Protocols; D003131:Combined Modality Therapy; D051184:Desmoglein 3 / Q000276:immunology; D005260:Female; D006801:Humans; D007163:Immunosorbent Techniques; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D008875:Middle Aged; D010392:Pemphigus / Q000188:drug therapy / Q000276:immunology / Q000473:pathology / Q000628:therapy*; D012074:Remission Induction; D012720:Severity of Illness Index; D016060:Sorption Detoxification / Q000379:methods*; D013205:Staphylococcal Protein A; D016896:Treatment Outcome
D001323:Autoantibodies; C071894:DSG3 protein, human; D051184:Desmoglein 3; D007166:Immunosuppressive Agents; D013205:Staphylococcal Protein A; D008775:Methylprednisolone