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Corticosteroid-Sparing Effect of Intravenous Immunoglobulin Therapy in Patients With Pemphigus Vulgaris
Naveed Sami, MD;
Ansa Qureshi, MD;
Eleonora Ruocco, MD;
A. Razzaque Ahmed, MD, DMSc
Arch Dermatol. 2002;138:1158-1162.
Background Pemphigus vulgaris (PV) is a rare, potentially fatal autoimmune mucocutaneous
blistering disease. The prolonged use of systemic corticosteroids, though
clinically effective in high doses, can result in multiple debilitating adverse
effects. Immunosuppressive agents, used as adjuvants and as corticosteroid-sparing
agents, are not effective in all patients and are contraindicated in some.
Therefore, alternative treatment modalities are needed to provide effective
control of PV in such patients.
Objective To demonstrate the corticosteroid-sparing effect of intravenous immunoglobulin
(IVIg) therapy in patients with moderate to severe PV.
Design A retrospective analysis in a cohort of 15 patients with moderate to
severe PV who were treated with IVIg therapy. All 15 patients were corticosteroid
dependent, and the use of other systemic conventional immunosuppressive agents
was contraindicated. The patients were followed up over a mean period of 6.2
years.
Setting Ambulatory tertiary medical care facility of a university-affiliated
hospital.
Intervention All 15 patients received an IVIg dose of 1 to 2 mg/kg per cycle.
Main Outcome Measures The following information was documented in each of the 15 patients
before and after the initiation of IVIg therapy: total dosage and total duration
of prednisone therapy and number of relapses. Also, the highest dosage and
adverse effects of prednisone therapy, as well as the total duration of observation,
were recorded.
Results All 15 patients had a satisfactory clinical response to IVIg therapy.
The use of systemic prednisone was gradually discontinued over a mean period
of 4.3 months. A statistically significant difference was noted in the total
dose of prednisone (P = .004), total duration of
prednisone therapy (P = .003), and number of relapses
(P<.001) before and after the initiation of IVIg
therapy.
Conclusions Intravenous immunoglobulin therapy has a demonstrable corticosteroid-sparing
effect. It is a safe and effective alternative treatment modality in patients
with PV who are dependent on systemic corticosteroids or who develop significant
adverse effects as a result of their use.
From the Department of Oral Medicine, Harvard School of Dental Medicine
(Drs Sami, Qureshi, Ruocco, and Ahmed), the Department of Dermatology, Harvard
Medical School (Dr Ahmed), and the Center for Blistering Diseases, Department
of Medicine, New England Baptist Hospital (Dr Ahmed), Boston, Mass.
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