Management overview for idiopathic inflammatory myopathies

Note: Managing idiopathic inflammatory myopathies requires specialist supervision, and patients usually need lifelong treatment and monitoring.

Management for idiopathic inflammatory myopathies requires specialist supervision. The management for most inflammatory myopathies (excluding inclusion body myositis) is similar.

Inclusion body myositis (IBM) is different because IBM is not very responsive to pharmacological treatmentsNeedham, 2016; see the separate section on Specific management for IBM. Management for IBM is mainly supportive.

Refer patients with severe disease, including severe weakness, dysphagia and risk of aspiration, urgently to a specialist centre.

Note: Refer patients with severe disease, including severe weakness, dysphagia and risk of aspiration, urgently to a specialist centre.

Key points in management for idiopathic inflammatory myopathies outlines some key points in the management for these conditions.

Figure 1. Key points in management for idiopathic inflammatory myopathies

Management for all forms of idiopathic inflammatory myopathy (excluding inclusion body myositis) is similar.
Inclusion body myositis (IBM) is usually not very responsive to pharmacological treatments; see Specific management for IBM Needham, 2016.
Refer patients with severe disease, including severe weakness, dysphagia and risk of aspiration, urgently to a specialist centre.
First-line therapy for severe disease involves high-dose systemic (oral or pulse intravenous) corticosteroids often in combination with:
- conventional synthetic DMARDs (eg azathioprine, ciclosporin, methotrexate, mycophenolate)Pipitone, 2020
- intravenous immunoglobulin (IVIg)Aggarwal, 2022
- a biological DMARD—usually rituximab.
Regular screening is required for complications of disease and therapy, and coexistent malignancies, which can occur before or after diagnosis; see Nonpharmacological management for idiopathic inflammatory myopathies
Exercise may improve strength, quality of life, and interstitial lung disease.

Note: DMARD = disease-modifying antirheumatic drug