Table 2:

Treatment options for inflammatory myopathies other than sporadic inclusion-body myositis

	Doses and treatment suggestions	Treatment for	Side-effects
Immunosuppressive or immunomodulatory drugs
Corticosteroids3	Prednisone 0·5–1 mg/kg per day. Consider adding 500 mg to 1 g intravenous methylprednisolone pulses once per day for 3 days for severe cases	All patients and all manifestations	Hypertension, hyperglycaemia, hyperlipidaemia, osteoporosis, infections, and cataracts
Azathioprine3	2–3 mg/kg per day	Predominantly myositis	Gastrointestinal symptoms, myelosuppression, leukaemia pancreatitis, infections, and liver toxicity
Methotrexate3,77	Up to 25 mg per week	Arthritis; use with caution in interstitial lung disease	Stomatitis, gastrointestinal symptoms, leucopoenia, liver toxicity, infections, and lung toxicity
Ciclosporin78	Up to 5 mg/kg per day	Skin involvement (panniculitis and dermatomyositis skin rashes) and interstitial lung disease	Renal insufficiency, anaemia, infections, and hypertension
Tacrolimus79	0·06 mg/kg per day	Interstitial lung disease	Hypertension, renal insufficiency, gastrointestinal symptoms, infections, and tremor
Mycophenolate mofetil80	2–3 g per day	Interstitial lung disease	Gastrointestinal symptoms, myelosuppression, infections, and hypertension
Cyclophosphamide81	Intravenous 0·5–1 g/m2 per month or 10–15 mg/kg per month for 6–12 months	Interstitial lung disease	Myelosuppression, myeloproliferative disorders, haemorrhagic cystitis, bladder cancer, infections, and infertility
Intravenous immunoglobulins82,83	2 g per kg every 4–6 weeks	Dysphagia and severe disease refractory to other treatments	Hypotension, anaphylaxis, headache, aseptic meningitis, blood clots, infections, and renal toxicity
Biological agents
Rituximab21,40,84,85	1 g given twice within a 2-week interval; maintenance with either one or two doses of 0·5–1 g rituximab on the basis of the patient’s clinical situation and their CD19 and CD20 counts (usually given every 6–9 months)	Rapidly progressive interstitial lung disease and severe cases of inflammatory myopathies	Infusion-related reaction, infections, and progressive multifocal leukoencephalopathy
Abatacept86	750 mg intravenously every 4 weeks (if patient’s weight <60 kg, 500 mg; if patient’s weight >100 kg, 1000 mg)	Consider for refractory disease	Infusion reactions and infections
Tocilizumab87	8 mg per kg intravenously every 4 weeks or 162 mg per week subcutaneously	Consider for refractory disease	Liver toxicity, neutropaenia, thrombocytopaenia, infections, hyperlipidaemia, and intestinal perforation
Physical exercise73,74	Aerobic and resistance-tailored programmes (about 4 weeks after starting medical treatment or as soon as the patient can cope with exercise)	All patients, as a coadjuvant therapy	Not described