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).

See below Immunoglobulins, non-specific.

Severe ADRs are rare. These include anphylactic reactions, aseptic meningitis, acute renal failure and thromboembolic complications. Neutropenia and mild thrombocytopenia are common but have received little attention (Stoevesand J et al. 2020).

1. Ahmed AR et al. (2007) Treatment of pemphigus vulgaris with rituximab and intravenous immune globulin. N Engl J Med 355: 1772-1779
2. Hadaschik E et al. (2020) European guidelines (S1) for the use of high-dose intravenous immunoglobulins in dermatology. Dermatology 71:542-552.
3. Lemm G (2002) Composition and properties of IVIg preparations that affect tolerability and therapeutic efficacy. Neurology 59(12 Suppl 6): S28-32
4. 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
5. Samuelsson A et al. (2001) Anti-inflammatory activity of IVIG mediated through the inhibitory Fc receptor. Science 291: 484-486
6. Simon HU, Spath PJ (2003) IVIG - mechanisms of action. Allergy 58: 543-552
7. Schanz S et al. (2008) Response of dystrophic calicification to intravenous immunoglobulin. Arch Dermatol 144: 585-587
8. Schwörer CV(2015) Guideline for the use of IVIG in dermatology. derm 21:233
9. Topf S et al. (2007) Significant improvement of Arndt-Gottron scleromyxedema with high-dose intravenous immunoglobulins. Dermatologist 58: 525-528