Autoimmune blistering diseases
Including but not limited to:
- bullous pemphigoid
- epidermolysis bullosa acquisita
- IgA pemphigus
- linear IgA disease and chronic bullous disease of childhood
- mucous membrane pemphigoid / cicatricial pemphigoid
- paraneoplastic autoimmune multiorgan syndrome
- pemphigoid / herpes gestationis
- pemphigus foliaceus
- pemphigus herpetiformis
- pemphigus vulgaris
Recommended Indications in which IG can be used
Order Number: D1-ABD
| Recommendations | IG is recommended for all severe forms of autoimmune blistering diseases when other therapies are ineffective or contraindicated. It is not generally recommended as monotherapy, but this may be justified in isolated cases when other therapies are ineffective or contraindicated. The results are particularly good in pemphigus vulgaris, pemphigus foliaceus, mucous membrane pemphigoid, and epidermolysis bullosa acquisita. However, IG may also be indicated in severe forms of bullous pemphigoid, IgA pemphigus, pemphigus herpetiformis, pemphigoid/herpes gestationis, linear IgA disease and chronic bullous disease of childhood, and paraneoplastic autoimmune multiorgan syndrome. Qualifying Criteria Diagnosis should be made by an appropriate specialist, such as a dermatologist, immunologist, ophthalmologist, otolaryngologist, or oral pathologist. Diagnosis should be confirmed by both routine pathology and appropriate direct or indirect immunofluorescence studies, whenever possible. |
| Dose/Frequency of Administration | 2 g/kg adjusted body weight divided over 2 to 5 days. IVIG should be administered every 4 weeks initially, usually in addition to conventional immunosuppressive therapy. Once the patient’s condition has stabilized, the interval between infusions should be gradually increased to determine whether ongoing treatment is required. IVIG should be administered for 3 to 6 months to assess efficacy. Some patients do not show a definitive sustained response until they have undergone up to 6 treatment cycles. |