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2016 ; 280
(1
): 39-51
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Diagnosis and classification of idiopathic inflammatory myopathies
#MMPMID27320359
Lundberg IE
; Miller FW
; Tjärnlund A
; Bottai M
J Intern Med
2016[Jul]; 280
(1
): 39-51
PMID27320359
show ga
The idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of
diseases, collectively termed myositis, sharing symptoms of muscle weakness,
fatigue and inflammation. Other organs are frequently involved, supporting the
notion that these are systemic inflammatory diseases. The IIMs can be subgrouped
into dermatomyositis, polymyositis and inclusion body myositis. The
myositis-specific autoantibodies (MSAs) identify other and often more distinct
clinical phenotypes, such as the antisynthetase syndrome with antisynthetase
autoantibodies and frequent interstitial lung disease and anti-SRP and anti-HMGCR
autoantibodies that identify necrotizing myopathy. The MSAs are important both to
support myositis diagnosis and to identify subgroups with different patterns of
extramuscular organ involvement such as interstitial lung disease. Another
cornerstone in the diagnostic procedure is muscle biopsy to identify inflammation
and to exclude noninflammatory myopathies. Treatment effect and prognosis vary by
subgroup. To develop new and better therapies, validated classification criteria
that identify distinct subgroups of myositis are critical. The lack of such
criteria was the main rationale for the development of new classification
criteria for IIMs, which are summarized in this review; the historical background
regarding previous diagnostic and classification criteria is also reviewed. As
the IIMs are rare diseases with a prevalence of 10 in 100 000 individuals, an
international collaboration was essential, as was the interdisciplinary effort
including experts in adult and paediatric rheumatology, neurology, dermatology
and epidemiology. The new criteria have been developed based on data from more
than 1500 patients from 47 centres worldwide and are based on clinically easily
available variables.