An ‘n-of-1’ placebo-controlled crossover trial of intravenous immunoglobulin as adjuvant therapy in refractory pemphigus vulgaris

Arnold, David; Burton, Janet; Shine, Brian; Wojnarowska, Fenella; Misbah, Siraj

doi:10.1111/j.1365-2133.2009.09034.x

Cited by 27 publications

(11 citation statements)

References 13 publications

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“…One Japanese study showed a reduction in disease severity and autoantibody titres as measured by enzyme-linked immunoassay (ELISA) in patients who received a single dose of IVIG (11). The second study showed a reduction in autoantibody titres and improvement in subjective disease scores after a single cycle of IVIG (12).…”

mentioning

confidence: 99%

Analysis of High-dose Intravenous Immunoglobulin Therapy in 16 Patients with Refractory Autoimmune Blistering Skin Disease: High Efficacy and No Serious Adverse Events

Seidling¹,

Hoffmann²,

Enk³

et al. 2013

Acta Derm Venerol

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High-dose intravenous immunoglobulin (IVIG) therapy is used in patients with severe autoimmune blistering diseases that are refractory to standard immunosuppressive therapy. To determine the efficacy and frequency of adverse events of IVIG therapy, we retrospectively analysed data for 16 patients with pemphigus vulgaris, pemphigus foliaceus, paraneoplastic pemphigus, bullous pemphigoid and paraneoplastic bullous pemphigoid. Frequency of adverse reactions and efficacy of IVIG were analysed over time with a scoring system for every 6 months of IVIG therapy. Headache (43.8%) and fatigue (43.8%) were the most common side-effects recorded; serious adverse reactions did not occur. There was good overall efficacy, as measured by clinical response rates using a clinical score, as well as indicated by a mean reduction of 75.8% in the starting steroid dose.

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confidence: 99%

Analysis of High-dose Intravenous Immunoglobulin Therapy in 16 Patients with Refractory Autoimmune Blistering Skin Disease: High Efficacy and No Serious Adverse Events

Seidling¹,

Hoffmann²,

Enk³

et al. 2013

Acta Derm Venerol

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“…The patient underwent two phases of treatment consisting of 6 consecutive months of either IVIG or placebo infusion in conjunction with prednisolone and azathioprine. 39 IVIG has an excellent safety profile in comparison with other treatment modalities for pemphigus, with mild side effects that include headache, fever, chills, myalgia, flushing, hypotension, tachycardia, and gastrointestinal symptoms. 11 Additionally, it should be noted that a recent case series of eight pregnant patients with PV who were treated with IVIG showed a good response, without any apparent signs of complications from treatment in the mother or the fetus.…”

Section: Discussionmentioning

confidence: 99%

“…11 The September/October 2014 literature is replete with case reports of refractory PV responding to rituximab and there is growing interest in the use of IVIG. [32][33][34][35][36][37][38][39][40][41] Though no rituximab-dosing protocol is clearly superior, giving 4 weekly 375 mg/ m 2 doses allows for more rapid redosing and would be more suitable for incorporating IVIG therapy if needed. IVIG is an appropriate option when an increased risk of infection cannot be tolerated because IVIG, theoretically, also confers immunoprophylaxis.…”

Section: Discussionmentioning

confidence: 99%

The Management of Pemphigus Vulgaris in a Burn Intensive Care Unit

Miletta

Miller

Lam

et al. 2014

Journal of Burn Care & Research

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Pemphigus vulgaris is a rare, potentially fatal, autoimmune blistering disease of the skin and mucous membranes. Treatment of this disease is problematic because of a lack of high-grade, evidence-based recommendations, the side-effect profiles of the therapies available, and the extensive supportive care that afflicted patients require. The authors present the unfortunate course of a patient with severe pemphigus vulgaris who was admitted to the U.S. Army Institute of Surgical Research Burn Center, to demonstrate the potential complications of therapy. Given the patient's complex course, the authors reviewed the literature and share in this article the most up-to-date treatment recommendations for patients with pemphigus vulgaris. The authors' review of the literature supports using conventional therapy consisting of high-dose corticosteroids and an adjuvant immunosuppressant for mild to moderate cases of pemphigus vulgaris. The immunosuppresants recommended are mycophenolate mofetil, azathioprine, and cyclophosphamide, in order of preference, based on their side-effect profiles and steroid-sparing effects. For severe or recalcitrant cases of pemphigus vulgaris, the authors recommend adding rituximab as early as possible. If increased risk of infection is of particular concern, the use of intravenous immunoglobulin in place of rituximab is advised.

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“…Adverse events were similar between the three groups. Another IVIg study also showed a beneficial effect in pemphigus [19]. The limitation of these studies was their short-term follow-up, so that relapse rates could be compared with other options.…”

Section: Therapies Used In Pemphigus Main Textmentioning

confidence: 99%

Management of pemphigus

Daniel

Murrell

2014

F1000Prime Rep

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Pemphigus is an autoimmune blistering disease characterized by cutaneous and mucosal blisters and erosions. Though systemic corticosteroids have been the mainstay of treatment for pemphigus over the years, more recently research has focused on steroid-sparing agents. This review looks at the commonly used steroid-sparing agents in pemphigus and the evidence from randomized controlled trials (RCTs) supporting their use.

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An ‘n-of-1’ placebo-controlled crossover trial of intravenous immunoglobulin as adjuvant therapy in refractory pemphigus vulgaris

Abstract: The results of this study confirm a beneficial effect of IVIg in the management of refractory PV.

Cited by 27 publications

References 13 publications

Analysis of High-dose Intravenous Immunoglobulin Therapy in 16 Patients with Refractory Autoimmune Blistering Skin Disease: High Efficacy and No Serious Adverse Events

Analysis of High-dose Intravenous Immunoglobulin Therapy in 16 Patients with Refractory Autoimmune Blistering Skin Disease: High Efficacy and No Serious Adverse Events

The Management of Pemphigus Vulgaris in a Burn Intensive Care Unit

Management of pemphigus

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