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Written by | Pippi Shrimp
Source | "Medical Rheumatic Immunization Channel" public number
Idiopathic inflammatory myopathy (IIM) is a group of rare heterogeneous autoimmune diseases characterized by muscle inflammation and a variety of extramuscular manifestations, usually chronic or subacute, mainly containing subtypes of polymyositis (PM), dermatomyositis (DM), and inclusion body myositis (IBM). The incidence is 19 per 1 million person-years in adults and 4 per 1 million person-years in children.
However, there are currently no standardized guidelines for the treatment of IIM. To better address this issue, the British Rheumatological Society officially released IIM management guidelines for children, adolescents and adults on 31 March[1] to make clinicians more informed in their treatment.
Screenshot of the guide
The guidelines cover thirteen sections of the IIM, including the treatment of myositis, the management of skin manifestations, and the use of drugs with interstitial lesions of the lungs. Sounds like a lot of content, but the sentences are dry goods! Let's take a look at it
1. How to treat myositis?
1. High-dose glucocorticoids should be used for the treatment of active myositis (1, B, 100%).
Adults: Oral prednisolone is recommended at a dose of 0.5 to 1 mg/kg/day, usually 40 to 60 mg (1, B, 100%).
Pediatrics: Oral prednisolone 1-2 mg/kg/day or intravenous methylprednisolone 30 mg/kg/day, the maximum intravenous dose per day is 1 g (1, B, 100%).
Intravenous methylprednisolone should be considered, especially when poor absorption in the gastrointestinal tract is concerned. Intravenous methylprednisolone may improve therapeutic efficacy and reduce adverse effects (2, B, 96%) compared with oral glucocorticoids.
2. The amount of prednisolone (1, B, 100%) should be gradually reduced according to the clinical response.
Glucocorticoids are essential for induction and maintenance therapy of myositis remission. When there is a significant improvement in disease activity (usually after about 6 weeks of treatment), glucocorticoids should be discontinued.
3. Myositis should be treated with anti-rheumatic drugs to achieve clinical relief and reduce the burden of steroids (1, C, 100%).
Pediatrics: Adolescent IIM patients should have early and complete control of muscle weakness and inflammation to improve outcomes and reduce disease-related complications (1, B, 100%).
Pediatrics: In most cases, high-dose glucocorticoids plus methotrexate should be used as a first-line regimen (1, B, 100%).
Pediatrics: Prednisolone plus methotrexate is more appropriate for treating adolescent IIM than prednisolone and cyclosporine because it has fewer side effects (1, B, 100%).
Pediatrics: Mycophenolate esters treat skin and muscle disorders (2, C, 100%).
Adults: methotrexate, azathioprine, tacrolimus, cyclosporine, and mycophenolate mofetil treat active myositis and maintain long-term remission (2, C, 96%).
4. Severe and/or refractory myositis may be considered intravenous immunoglobulin (IVIG) (1, B, 100%).
5. IIM management should include a safe and appropriate exercise program led and supervised by a professional physiotherapist and/or professional occupational therapist to improve quality of life and functioning (1, B, 100%).
6. Rituximab is an option for the treatment of refractory myositis, and may be particularly effective in patients with juvenile onset, positive autoantibodies to myositis, or low disease damage (2, A, 100%).
7. Patients with severe and/or refractory IIM can choose cyclophosphamide therapy (1, B, 100%).
Intravenous administration reduces the risk of leukopenia, hemorrhagic cystitis, and gonad toxicity compared with oral cyclophosphamide.
8. Adults: patients with refractory adult IIM can choose abatacept treatment (2, B, 100%).
How to treat IIM-related skin manifestations?
1. Rituximab can be used to treat skin diseases that are ineffective against glucocorticoids/csDMARD (2, B, 100%).
2. IVIG can be used to treat skin diseases (1, B, 100%) that are ineffective against glucocorticoids/csDMARD.
3. Avoiding the sun and regularly using sunscreen with high sunscreen factors can reduce the likelihood of skin or muscle diseases (2, C, 100%).
4. Pediatrics: systemic immunosuppressive drugs can be used to treat active skin diseases, including reducing the density of capillaries in the nail folds (2, C, 100%).
5. Pediatrics: For patients with persistent skin diseases, consider increasing treatment as soon as possible to help alleviate and reduce the development of calcinosis (2, C, 100%).
How to manage IIM-related pulmonary interstitial lesions (ILDs)?
1. Pediatrics: because lung function abnormalities are common and may be asymptomatic, routine lung function assessment should be performed, including measurement of lung carbon monoxide diffusion or transfer factor (DLCO or TLCO) (1, B, 100%) in adolescent IIM.
2. Adults: high-risk patients should be screened for ILD (1, B, 100%).
3. Adults: in the treatment of rapidly progressive ILD (RP-ILD):
Induction therapy with high-dose steroids (2, C, 96%)should be considered.
Patients with RP-ILD should consider cyclosporine, tacrolimus, and steroids (2, C, 96%).
Induction therapy (2, C, 96%) should be considered as early as possible for cyclophosphamide or rituximab.
4. Adults: treatment of chronic IIM-related ILD:
Treatment with glucocorticoids alone or in combination with DMARD (azathioprine, cyclosporine, tacrolimus, mycophenolates) (2, C, 100%)) should be considered.
Rituximab or cyclophosphamide (2, C, 100%) should be considered in refractory patients.
note:
IM-associated ILD management should be performed in conjunction with a respiratory physician.
ILD risk is increased by antisynthetase antibody syndrome, positive antisynthetase-associated autoantibodies, positive antimelanoma differentiation-related protein 5 (MDA5) antibodies, and overlapping scleroderma.
ILD screening methods include plain chest x-rays, lung function tests (including DLCO), and high-resolution chest CT.
There is insufficient evidence to form recommendations for the administration of IIM-related ILD drugs in adolescents.
What measures can be taken to reduce the risk of fractures in IIM patients?
Adults: regardless of glucocorticoid therapy, a skeletal evaluation should be performed and appropriate management (1, B, 100%).
What are the key prognosis and management factors that adolescent IMMs should consider?
1. Adolescent IIM should be managed by a pediatrician because it differs from adult IIM in many ways, including more subcutaneous calcification, lower disease damage, not associated with cancer, an increased risk of vasculitis, and different autoantibodies (1, C, 95%).
2. The time of diagnosis is related to the improvement of disease outcomes, so it should be referred to specialist treatment as soon as possible (2, C, 100%).
3. When using tools that measure muscle strength, function and quality of life, age-specific factors (1, B, 100%) should be considered.
4) Healthcare professionals should look for overlap with other connective tissue diseases, as this increases the risk of death (1, C, 89%).
5. Adolescent IIM patients should be evaluated for calcinosis (1, C, 100%).
Factors associated with an increased risk of calcinosis include a younger age of onset, particularly in infancy, delayed diagnosis or treatment, more severe disease, prolonged disease duration, and positive antinuclear matrix protein 2 (NXP-2) antibodies. Clinical examination and plain x-rays may be used to differentiate calcitonia.
Is autoantibody testing useful for IIM patients?
1. Patients should be tested for myositis autoantibodies (1, B, 100%).
Myositis-specific antibodies and myositis-associated autoantibodies are associated with diagnosis, recognition of disease phenotypes, and treatment. Autoantibody titers should not be used to monitor disease activity.
How should patients with IIM be screened for cancer?
1. Pediatrics: Adolescent IM does not require routine cancer screening (1, B, 100%).
Compared to adult IIM, adolescent IIM is not associated with cancer, with only a few cases reported. Routine cancer screening for adolescent IIM is not recommended unless the underlying cancer is suspected.
2. Adults: all patients should consider the risk of cancer, especially patients with the following risk factors should pay special attention to screening (1, B, 100%):
Older age, male, dysphagia, skin necrosis, resistance to immunosuppressive therapy, rapid onset of disease, positive anti-transcriptional mediator-1γ (anti-TIF1-γ), positive anti-NXP-2 antibody, negative known myositis-specific autoantibodies.
VIII. How should IIM treatment during pregnancy and lactation be adjusted?
1. It is recommended to try to conceive when the disease is well controlled (1, B, 100%).
2. Pregnancy should be managed with obstetrics and gynecology specialists (1, B, 96%).
3. Postpartum vigilance is needed, because patients may have a risk of disease attack (1, C, 96%).
How to evaluate and treat IIM-related cardiovascular diseases?
1. Adults: Patients should undergo cardiovascular risk assessment (1, C, 100%) regularly.
IIM is associated with an increased incidence of hypertension, diabetes mellitus, dyslipidemia, obesity, and coronary artery disease (specific to adults).
2. Pediatrics: consideration should be given to assessing and managing cardiovascular risk factors, including hypertension, obesity, or metabolic abnormalities (lipid/insulin resistance) (2, C, 100%).
The cause of hypertension in 25% to 50% of patients with JDM is related to microangiopathy and glucocorticoid therapy. Studies have found that changes in cardiovascular risk factors in patients with juvenile dermatomyositis may lead to an increased risk of early atherosclerosis in late adulthood.
How to screen for cardiac involvement in IIM?
1. Adults: patients should be screened for cardiac involvement, including serum heart injury markers, electrocardiogram, cardiac ultrasound, and cardiac MRI (2, B, 100%).
2. Adults: myocardial troponin I (not myocardial troponin T) should be used as the preferred serum marker for screening and monitoring cardiac involvement (1, B, 100%).
3. Pediatrics: ECG and cardiac ultrasound should be considered to screen adolescent IIM patients for cardiac involvement (2, C, 100%).
How to screen and manage IIM-related dysphagia?
1. All patients should be routinely evaluated for dysphagia (2, C, 92%).
2. Patients with dysphagia should be considered for swallowing evaluation, and require the participation of speech-speech therapist/gastroenterology team (2, C, 100%).
3. Active disease and dysphagia resistant to other treatments should be considered IVIG therapy (2, C, 100%).
Dysphagia is common and has been linked to weight loss and aspiration pneumonia, which can affect quality of life and, in severe cases, can be life-threatening. Swallowing dysfunction is not always predicted by systemic muscle weakness, and positive anti-NXP-2 antibodies or malignant tumors also increase the risk of developing them.
Some scholars believe that dysphagia is an indication for IVIG treatment. IVIG and other immunomodulatory therapies such as glucocorticoids, csDMARDs (methotrexate, azathioprine, cyclosporine, tacrolimus, mycophenolate mofetil, hydroxychloroquine), cyclophosphamide, and rituximab have been reported to improve symptoms of dysphagia.
How is the quality of life and mental health of IIM patients assessed and treated?
Mental health and psychiatric comorbidities (1, C, 92%) should be evaluated.
2. Mental health and health-related quality of life (HRQoL) should be regularly assessed using age-appropriate tools (1, B, 100%).
3. Factors that have a negative impact on HRQoL (e.g., skin involvement, itching, and adverse steroid reactions) (1, C, 96%) should be addressed.
4. Pediatrics: The negative factors of HRQoL produced by children include pain, muscle weakness and poor sleep, and should be treated appropriately (1, C, 95%).
5. Patients within the range of disease activities should be encouraged to undergo personalized exercise and/or rehabilitation to improve mental health (1, B, 96%).
6. Due to the impact of poor grip on daily life and quality of life, targeted exercises (2, C, 96%) should be considered by professional physiotherapists and/or professional occupational therapists.
How should the IIM be administered for certain ethnic groups?
Ethnicity is considered in evaluating patients, and clinical presentation, associated autoantibodies, and potential risk factors may vary by race (2, C, 96%).
Minorities appear to be more susceptible to anti-signal recognition granule (anti-SRP) autoantibody-related disease, increased cardiovascular risk, and more likely to develop juvenile polymyositis/adolescent connective myopathy.
brief summary
The limited high-quality evidence presented in this guideline for IIM is primarily based on observational studies with a relative lack of randomized controlled trials (RCTs) or head-to-head comparative studies. Therefore, controlled trials are essential to further evaluate promising treatments. And the guidelines do not address the diagnosis, classification, and inclusion of IBM. In addition, the impact of the IIM on mental health and quality of life should not be underestimated.
bibliography:
[1] Alexander GS,Oldroyd,et al. British Society for Rheumatology guideline on management of paediatric,adolescent and adult patients with idiopathic inflammatory myopathy[J]. Rheumatology，2022;00:1–9.
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