Clinical Outcome of Severe, Recalcitrant or Steroid Contraindicated Pemphigus Treated with Intravenous Immunoglobulin : A Case Series
Keywords:Intravenous immunoglobulin, IVIG, Severe pemphigus, Rrecalcitrant pemphigus, Steroid contraindicated, Treatment
Background : Intravenous immunoglobulin (IVIG) has been used as an adjuvant treatment in severe, recalcitrant or steroid contraindicated pemphigus patients. Rapidly disease controlled without immunosuppression was a significant advantage different from other treatment modalities.
Objectives : This study aimed to evaluate the clinical response, and side effects of IVIG treatment in severe, recalcitrant, or steroid contraindicated pemphigus patients.
Methods : A retrospective review was conducted at the Institute of dermatology over seven years. Pemphigus patients who received at least six cycles of IVIG as adjuvant treatment were recorded demographic data, treatment before IVIG, indication for IVIG, dose, type, and the number of IVIG cycles. Clinical remission was evaluated at one month after the 6th cycle of IVIG. A dose of prednisolone before and after IVIG treatment was recorded. An adverse event was monitored.
Results : Eleven pemphigus vulgaris patients, three with active extensive involvements, six with treatment recalcitrant and two with side effects or contraindication to systemic steroids and immunosuppressive agents were reviewed. Ten of eleven patients (90.9%) showed a response to six cycles of IVIG, five (45.5%) having complete remission. All five patients remained in remission during the 4-28 months follow up period. Mean prednisolone dose reduction was 57.3% in IVIG response patients. The therapy was well tolerated; however, congestive heart failure occurred in one patient with an underlying left ventricular hypertrophy. One patient developed cutaneous side effect which response to topical steroid. One extensive pemphigus patient did not respond to IVIG and died of sepsis and multi-organ failure.
Conclusions : Adjuvant six cycles of IVIG treatment in severe, recalcitrant, or steroid contraindicated pemphigus patients increased disease control, induced remission, and demonstrated steroid-sparing effect. The results encourage the use of IVIG due to the safety and effectiveness.
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