Dermatomyositis part II

Dermatomyositis part II :volcano:
:gift:Treatment​:gift:
:circus_tent:Non-drug
:weight_lifting_man:‍♀Sun-blocking agents.
:weight_lifting_man:‍♀Physical activity within reason to maintain muscular strength. This may involve consultation with a physiotherapist and occupational therapist.
Evaluation of
:weight_lifting_man:‍♀Swallowing required and a speech and language therapist.
:weight_lifting_man:‍♀Monitor creatine kinase and clinical response but.
:circus_tent:Drugs
:warning:Immunosuppressive and immunomodulatory therapies are used,
:motorcycle:The optimal therapeutic regimen remains unclear.
:motorcycle:A lack of high-quality studies that assess the efficacy and toxicity of immunosuppressants in inflammatory myositis.
:motorcycle:Early initiation of therapy is essential.
:dna:Steroids are the most important drugs.
:dna:Mild disease, topical steroids may suffice.
:dna:More severe disease, high doses of systemic steroids are used and tapered off. :dna:Improvement is usually apparent by the second or third month.
:dna:The usual precautions must be exercised when giving high doses of steroids for long periods.
:dna:If steroids fail then immunosuppressive drugs such as azathioprine used.
:dna:As an alternative, cyclophosphamide is usually better than methotrexate.
:dna:Biological agents, including :
:firecracker:Tumour necrosis factor alpha antagonists,
:firecracker:Intravenous immunoglobulin
:firecracker:Rituximab, have been used with varying degrees of success for the treatment of cutaneous dermatomyositis.
:dna:Patients with anti-Jo-1 antibodies need long-term immunosuppression.
:dna:For lung disease, an aggressive combination regimen including ciclosporin A or tacrolimus with cyclophosphamide is recommended to be added to corticosteroids.
:gift:Complications​:gift:
:circus_tent:Gastrointestinal​:circus_tent:
:firecracker:Ulceration can cause melaena or haematemesis.
:firecracker:Infarction of the bowel.
:circus_tent:Subcutaneous​:circus_tent: calcification that punctures the skin with ulcerations, infection and ugly scars.
Both are associated with an increased risk of🎪 malignancy🎪.
:circus_tent:Atrioventricular defects, tachyarrhythmias, dilated cardiomyopathies,
:circus_tent:Joint contractures
:circus_tent:lung involvement (due to weakness of thoracic muscles, interstitial lung disease).
:circus_tent:Complications of steroid therapy​:circus_tent:

  • eg, osteoporosis, myopathy.
    :gift:Prognosis​:gift:
    :honeybee:The prognosis of the idiopathic inflammatory myopathies is very variable.
    :honeybee:An associated malignancy indicates a poor prognosis for recovery and increases mortality. Overall,
    :honeybee:Drug-free remissions are rare except in juvenile dermatomyositis.
    Studies have shown that only 20% to 40% of treated patients will achieve remission,
    :honeybee:60% to 80% will experience a polycyclic or chronic continuous course of the disease.
    :honeybee: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.
    :honeybee: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,
    :honeybee:Presence of antisignal recognition particle antibody, anti-155/140, and anti-CADM-140 antibodies).
    Anti-155/140 antibody is associated with malignancy.