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The Myopathy Committee of the Brazilian Society of Rheumatology (BSR) has put forward recommendations for the management of patients with systemic autoimmune myopathies (SAM).
The challenge has been that there is a paucity of effective controlled clinical trials, and hence, glucocorticoids (GC) have played a first-line and prominent role in the treatment of SAM.
This review of the literature considers many of the agents currently and use and provides a evidence grading of such evidence. In addition to the appropriate use of GC in SAM, this report also addresses intravenous immunoglobulin (IVIG) along iwth other immunosuppressive drugs and when these may be contra-indicated.
Recommendations for the treatment of systemic autoimmune myopathies
“PICO” questions -- followed by the Recommendations
1. What are the general and educational recommendations for SAM?
Patients families and/or caregivers, should be offered environmental adaptations, rehabilitation programs as a prominent role in the rehabilitation and therapeutic process of patients with SAM (degree of recommendation B).
2. What are some precautions before immunosuppression in patients with SAM?
Immunosuppressive drugs may be associated with an increased risk for infections. The risk of these infections is related to the total dose and duration of immunosuppressive drug. Vaccination status should be evaluated. If possible, vaccine should be given prior to the start of the immunosuppressive drug. Vaccines composed of live attenuated viruses should be administered at least four weeks before the start of the immunosuppressive drug. (degree of recommendation B, C, D)
3. What treatment is recommended in the initial phase of SAM?
GC via oral route is the first-line treatment in cases of SAM (degree of recommendation C). Immunosuppressive drugs with methotrexate, azathioprine and cyclosporine may be associated with a reduction in GC doses. (degree of recommendation B)
4. Which drug treatments are recommended for refractory SAM cases?
Refractory cases of SAM may be treated with intravenous immunoglobulin, tacrolimus, cyclosporine, cyclophosphamide, azathioprine, methotrexate, abatacept, tocilizumab and rituximab, as monotherapy or in combination. There is a need for more controlled studies with greater numbers of patients for evaluation (degree of recommendation B) of efficacy and tolerability are needed. Anti-TNFα agents are not recommended. (degree of recommendation C)
5. What initial dose of glucocorticoids should be used and for how long in patients with SAM?
First-line treatment should be the administration of GC, starting doses of prednisone or its equivalent potency range from 0.5 to 1.0 mg/kg/day given on a fractional basis, daily or on alternate days. In severe cases, MP pulse therapy should be considered (1 g/day for three consecutive days followed by a regimen with oral GC). (degree of recommendation B)
6. How long should SAM patients receive immunosuppressive / immunomodulatory drugs after discontinuation of GC?
There is no established timeframe for how long immunosuppressive/immunomodulatory drugs should be maintained after GC are discontinued. Follow-up evaluations of these individuals should be scheduled according to clinical evolution and changes observed during treatment monitoring. (degree of recommendation B)
7. What is the evidence on the benefit of immunosuppressive / immunomodulatory drugs association (association versus exchange) in SAM?
Agents such as intravenous immunoglobulin, mycophenolate mofetil, cyclosporine, azathioprine and methotrexate, used alone or in combination appear to contribute to improvement in muscle strength, CK levels and lung function, with no significant difference in efficacy between the treatment schemes (azathioprine with methotrexate, cyclosporine with methotrexate and intramuscular methotrexate with oral methotrexate and azathioprine). (degree of recommendation A)
8. What is the role of rehabilitation, physical exercise and physiotherapy in the treatment of SAM?
The implementation of a physical exercise program (resistance or aerobic physical training or the combination of these two) seems to be safe and beneficial in adult patients with SAM and should be used as a complement to pharmacological treatments in all stages of the disease. (degree of recommendation B)
9. How to monitor disease activity (biomarkers) in patients with SAM?
Evidence has pointed to the possibility of using objective measures, in the form of identification and dosage of molecules that present the potential to discriminate the activity of the disease and predict its damage. (degree of recommendation B)
10 How to define activity versus remission of SAM in clinical practice?
Other than the clinical assessment of disease activity or remission, there are some instruments that have not yet been fully validated and are largely based on a subjective assessment conducted by both patient and physician on the disease status. There is no single gold standard measure to assess disease activity. (degree of recommendation D)