Ivig

For the indications listed here, there are guideline recommendations with varying degrees of evidence.

• Dermatomyositis (polymyositis) Level of evidence Ib
• Severe forms of systemic lupus erythematosus
• ANCA negative vasculitis
• Severe forms of blistering autoimmune diseases (level of evidence Ib)
• Pyoderma gangraenosum
• Toxic epidermal necrolysis (TEN)
• livedo racemosa
• Livedovasculopathy
• autoimmunological urticaria
• Kawasaki Syndrome
• Casuistic articles have been published on pemphigus vulgaris, Arndt-Gottron scleromyxedema and CREST syndrome.

0,2-1,0 (up to 2,0 g) g/kg bw i.v. over several days (guideline standard dosage: 2g/kg bw over 2-5 days).

1. Ahmed AR et al (2007) Treatment of pemphigus vulgaris with rituximab and intravenous immune globulin. N Engl J Med 355: 1772-1779
2. Lemm G (2002) Composition and properties of IVIg preparations that affect tolerability and therapeutic efficacy. Neurology 59(12 Suppl 6): S28-32
3. Sami N et al (2002) Influence of IVIg therapy on autoantibody titers to desmoglein 1 in patients with pemphigus foliaceus. Clin Immunol 105: 192-198
4. Samuelsson A et al (2001) Anti-inflammatory activity of IVIG mediated through the inhibitory Fc receptor. Science 291: 484-486
5. Simon HU, Spath PJ (2003) IVIG - mechanisms of action. Allergy 58: 543-552
6. Schanz S et al (2008) Response of dystrophic calicification to intravenous immunoglobulin. Arch Dermatol 144: 585-587
7. Schwörer CV(2015) Guidelines for the use of IVIG in dermatology. derm 21:233
8. Topf S et al. (2007) Significant improvement of a scleromyxedema Arndt-Gottron by high-dose intravenous immunoglobulins. Dermatologist 58: 525-528