Scleromyositis is an autoimmune overlap syndrome involving scleroderma and dermato/polymyositis.
Synonym: PDM/SSc or SSc/PDM
Clinical features:
(1) scleroderma type skin involvement
(2) myositis
(3) variable interstitial lung disease (ILD)
(4) variable Raynaud's syndrome
(5) variable presence of mechanic's hand
(6) variable arthritis or synovitis
(7) variable digital ulcers
(6) variable dropped head or bent spine syndrome
(7) vasculopathy
(8) variable myocarditis
(9) variable pulmonary arterial hypertension
(10) variable gastrointestinal manifestations (dysphagia, reflux, other)
(11) scleroderma renal crisis
Laboratory findings:
(1) one or more antibodies associated with scleroderma (anti-PM/Scl, anti-Ku, anti-I1RNP, anti-U3-RNP, anti-SMN, anti-RuvBL1/2, other).
(2) one or more antibodies associated with dermatomyositis/polymyositis.
(3) elevated serum levels of CK and other muscle markers
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Condition
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Autoantibody
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dermatomyositis
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anti-Mi-2
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anti-TIFI-gamma
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anti-NXP-2
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anti-MDA5
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anti-SAE
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polymyositis
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anti-ARS (anti-Jo1, anti-Ha)
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Seronegative forms of scleromyositis may also occur.
