How to use "gamma globulin" for these diseases in rheumatology?

It is only for medical professionals to read for reference.
Which rheumatology diseases does IVIg apply to? how to use? Intravenous human immunoglobulin (IVIg) has been used to treat primary and secondary immunodeficiency related diseases for more than 50 years.

In the field of rheumatism and dermatology, IVIg has been used to treat diseases such as autoimmune diseases and toxic epidermal necrolysis (Table 1).

Table 1.
Indications of IVIg This article will mainly discuss the following questions: What diseases does IVIg apply to? Use IVIg as first-line or second-line treatment? Duration of initial treatment? What is the interval between IVIg infusion cycles? Dosage of immunoglobulin? What is the duration of treatment for each IVIg cycle? What methods can be used to assess efficacy? What conditions need long-term treatment? The following table is the recommended dosage regimen in the literature (Table 2) for communication and learning.

Table 2.
The recommended IVIg dosing regimen in the literature is 3 g/kg for toxic epidermal necrolysis.
Kawasaki disease and toxic epidermal necrolysis require only one cycle.
The figure below shows the decision to use IVIg to treat severe autoimmune diseases.
Tree (Figure 1).

Figure 1.
The use of IVIg in the treatment of severe autoimmune diseases in dermatology.
Barkomyositis Dermatomyositis is the most effective disease except pemphigus vulgaris.

Autoimmune bullous disease Autoimmune bullous disease is a disease mediated by autoantibodies, and its autoantigens are known to a large extent and have been molecularly characterized.

Vasculitis syndrome Vasculitis syndrome is a systemic inflammatory disease that affects the blood vessels of one or more organ systems.

Since the skin is often involved as an indicator organ and the disease is often highly refractory, immunoglobulin can be used as an alternative treatment.

Lupus erythematosus and other collagen vascular diseases Almost all autoimmune connective tissue diseases have been reported to be treated with IVIg, but systemic lupus erythematosus is the most effective.

Sclerosing mucinous edema Sclerosing mucinous edema is a serious multi-organ disease characterized by the proliferation of fibroblasts and mucin deposition in the skin and internal organs, most of which are related to monoclonal gammopathy.

Skin thickening and fibrosis usually lead to weakness, and internal organ involvement can be life-threatening.

Sclerosing mucinous edema does not respond to most immunosuppressive treatments, but IVIg has a rapid onset.

References: 1.
Hadaschik E,Eming R,French LE,et al.
Europäische Leitlinien(S1)für die Verwendung von hoch dosierten intravenösen Immunglobulinen in der Dermatologie.
Hautarzt.
2020;71(7):542-552.
2.
Enk AH, Hadaschik EN, Eming R, et al.
European Guidelines(S1) on the use of high-dose intravenous immunoglobulin in dermatology.
J Eur Acad Dermatol Venereol.
2016;30(10):1657-1669.