**4.1 DM**

DM is an inflammatory disease, affecting skin and muscle and causing varying degrees of muscle weakness, ranging from mild to severe. Prominent inflammation is observed usually at the periphery of the fascicle, leading to atrophy of the fibres around the fascicle (Hughes et al, 2008).

In a majority of DM patients a favourable response has been reported and therefore IVIg is recommended as a second line treatment in combination with prednisone for those who have not improved with corticosteroids alone. A total dose of 2 g kg given over 2–5 days for adults and over 2 days for children is a safe initial treatment option. In severe, lifethreatening DM, IVIg can be considered as the first line treatment together with other immunosuppressive therapy (Elovaara et al, 2010; EFNS task force, 2008).

Recommendations:


### **4.2 IBM**

IBM is a progressive inflammatory skeletal muscle disease that presents with a distinctive pattern of weakness in the wrist and finger flexors and quadriceps muscles. It is characterized by inflammatory cells surrounding myofibres and rimmed vacuoles.

In IBM, the available evidence based on trials with small to moderate numbers of patients suggests an overall negative outcome even if a small number of patients reported improvement in swallowing difficulties. Therefore, IVIg cannot be recommended for the treatment of sporadic IBM (Hughes et al, 2008).

Recommendation:


#### **4.3 PM**

Polymyositis is an inflammatory myopathy with no rash. It is defined by symmetric proximal muscle weakness, elevated serum muscle enzymes, myopathic changes on electromyography, characteristic muscle biopsy abnormalities and the absence of histopathologic signs of other myopathies. Muscle weakness is indeed the most common presenting feature of polymyositis. The onset is usually insidious and the distribution of weakness is typically symmetric and proximal. Myalgias occur in less than 30% of the patiens (Dalakas & Hohlfeld, 2003).

Only one non-RCT (evidence class III) and two case series (evidence class IV) on IVIg therapy for polymyositis have been published. Only the first one used IVIg exclusively in patients with polymyositis. This study reported clinical improvement in 71% of patients with significant improvement in muscle power, muscle disability scores, and creatinine kinase levels (P < 0.01). Steroid doses could be reduced after IVIg (P < 0.05) (Hughes et al, 2008).

Recommendation:

344 Autoimmune Disorders – Current Concepts and Advances from Bedside to Mechanistic Insights

The inflammatory myopathies are rare autoimmune diseases characterized by muscle weakness, which is usually proximal, painless and of insidious onset. The three groups of autoimmune myopathies are dermatomyositis (DM), polymyositis (PM) and inclusion body myositis (IBM). There are some controlled trials on the use of IVIg in patients with dermatomyositis (DM) and inclusion body myositis (IBM), and only one with polymyositis

DM is an inflammatory disease, affecting skin and muscle and causing varying degrees of muscle weakness, ranging from mild to severe. Prominent inflammation is observed usually at the periphery of the fascicle, leading to atrophy of the fibres around the fascicle (Hughes

In a majority of DM patients a favourable response has been reported and therefore IVIg is recommended as a second line treatment in combination with prednisone for those who have not improved with corticosteroids alone. A total dose of 2 g kg given over 2–5 days for adults and over 2 days for children is a safe initial treatment option. In severe, lifethreatening DM, IVIg can be considered as the first line treatment together with other



IBM is a progressive inflammatory skeletal muscle disease that presents with a distinctive pattern of weakness in the wrist and finger flexors and quadriceps muscles. It is

In IBM, the available evidence based on trials with small to moderate numbers of patients suggests an overall negative outcome even if a small number of patients reported improvement in swallowing difficulties. Therefore, IVIg cannot be recommended for the


Polymyositis is an inflammatory myopathy with no rash. It is defined by symmetric proximal muscle weakness, elevated serum muscle enzymes, myopathic changes on electromyography, characteristic muscle biopsy abnormalities and the absence of histopathologic signs of other myopathies. Muscle weakness is indeed the most common

characterized by inflammatory cells surrounding myofibres and rimmed vacuoles.

immunosuppressive therapy (Elovaara et al, 2010; EFNS task force, 2008).

measure to lower the dose of steroids in patients with DM (level C). - IVIg is not recommended as monotherapy for DM (good practice point).

**4. IVIg in therapy of inflammatory myopathies** 

(PM) (EFNS task force, 2008; Hughes et al, 2008).

treatment of sporadic IBM (Hughes et al, 2008).

**4.1 DM** 

et al, 2008).

Recommendations:

**4.2 IBM** 

**4.3 PM** 

Recommendation:

force, 2008).

