Abstract
Myopathies are a rare type of acquired, chronic autoimmune diseases of the skeletal muscles and affect both children and adults. The hallmark symptoms of idiopathic inflammatory myopathies (IIM) are muscle inflammation, proximal muscle weakness and disability, arthritis, cutaneous rashes, calcinosis, ulceration, malignancy and interstitial lung disease (ILD). Subforms of IIM include polymyositis, dermatomyositis, cancer-related myositis and sporadic inclusion body myositis. Autoantibodies function as biomarkers for diagnosis of IIM and can be used to delimit clinically distinguishable IIM subforms. To maximise the diagnostic information it is essential to perform comprehensive multiparametric serological testing including both screening and confirmation tests.
Highlights
Myopathies are a rare type of acquired, chronic autoimmune diseases of the skeletal muscles and affect both children and adults
Sporadic inclusion body myositis is a rare subform of idiopathic inflammatory myopathies (IIM) and difficult to distinguish from other subforms.[17]
A comprehensive strategy for serological testing comprises parallel determination of both myositis-specific autoantibodies (MSA) and myositis-associated autoantibodies (MAA) to reduce the time to diagnosis as well as including as many parameters as possible to ensure the highest detection rate
Summary
Polymyositis (PM) is a systemic inflammatory disease of the skeletal musculature with perivascular lymphocytic infiltration. Clinical symptoms of PM are recurring bouts of fever, muscle weakness, arthralgia, Raynaud syndrome and trouble with swallowing. When the skin is involved, the disease is known as dermatomyositis (DM). In DM, skin symptoms appear as purple-coloured exanthema on the eyelids, nose and cheeks, periorbital oedema, local erythe-
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