Dermatomyositis part II
Treatment:gift:
Non-drug
♀Sun-blocking agents.
♀Physical activity within reason to maintain muscular strength. This may involve consultation with a physiotherapist and occupational therapist.
Evaluation of
♀Swallowing required and a speech and language therapist.
♀Monitor creatine kinase and clinical response but.
Drugs
Immunosuppressive and immunomodulatory therapies are used,
The optimal therapeutic regimen remains unclear.
A lack of high-quality studies that assess the efficacy and toxicity of immunosuppressants in inflammatory myositis.
Early initiation of therapy is essential.
Steroids are the most important drugs.
Mild disease, topical steroids may suffice.
More severe disease, high doses of systemic steroids are used and tapered off. Improvement is usually apparent by the second or third month.
The usual precautions must be exercised when giving high doses of steroids for long periods.
If steroids fail then immunosuppressive drugs such as azathioprine used.
As an alternative, cyclophosphamide is usually better than methotrexate.
Biological agents, including :
Tumour necrosis factor alpha antagonists,
Intravenous immunoglobulin
Rituximab, have been used with varying degrees of success for the treatment of cutaneous dermatomyositis.
Patients with anti-Jo-1 antibodies need long-term immunosuppression.
For lung disease, an aggressive combination regimen including ciclosporin A or tacrolimus with cyclophosphamide is recommended to be added to corticosteroids.
Complications:gift:
Gastrointestinal:circus_tent:
Ulceration can cause melaena or haematemesis.
Infarction of the bowel.
Subcutaneous:circus_tent: calcification that punctures the skin with ulcerations, infection and ugly scars.
Both are associated with an increased risk of🎪 malignancy🎪.
Atrioventricular defects, tachyarrhythmias, dilated cardiomyopathies,
Joint contractures
lung involvement (due to weakness of thoracic muscles, interstitial lung disease).
Complications of steroid therapy:circus_tent:
- eg, osteoporosis, myopathy.
Prognosis:gift:
The prognosis of the idiopathic inflammatory myopathies is very variable.
An associated malignancy indicates a poor prognosis for recovery and increases mortality. Overall,
Drug-free remissions are rare except in juvenile dermatomyositis.
Studies have shown that only 20% to 40% of treated patients will achieve remission,
60% to 80% will experience a polycyclic or chronic continuous course of the disease.
Mortality remains 2-3 times higher than in the general population, with cancer, lung, cardiac complications and infections being the most common causes of deaths.
Poor prognostic factors include older age, male gender, non-Caucasian ethnicity, longer symptom duration, cardiac involvement, dysphagia, malignancy and serum myositis-specific antibodies (including co-existence of anti-Ro52 and anti-Jo1 antibodies,
Presence of antisignal recognition particle antibody, anti-155/140, and anti-CADM-140 antibodies).
Anti-155/140 antibody is associated with malignancy.