Rav Reuma Mixed Connective Tissue Disease

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clinical

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combination of multiply reumatologic disease without fitting one diagnose-- combination of SLE finding + muscle weakness ( dermatomyositis0

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labs

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may have combination of + RF
anti RNP( anti ribonucleoprotein) is specific for DS

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Mixed connective tissue disease typically manifests

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with arthralgia or arthritis, Raynaud phenomenon, serositis, and an inflammatory muscle diseas

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ffected patients may have difficulty with activities that involve

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ffected patients may have difficulty with activities that involve raising their hands above their head and have difficulty rising from a chair and climbing stairs. Neck flexor muscles may be affected, and involvement of the pharyngeal muscles predisposes patients to aspiration. Muscle pain in patients with an inflammatory myopathy is atypical and, if present, is generally mild. Significant myalgia suggests an alternative diagnosis, such as arthritis, polymyalgia rheumatica, or fibromyalgia.

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Rashes Associated with Dermatomyositis

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Gottron papules Violaceous to pink plaques with scaling overlying the extensor surface of the hand joints, knees, and elbows
Heliotrope rash Violaceous discoloration of the eyelids accompanied by periorbital edema
Macular erythema Photosensitive rash involving the face and neck
Shawl sign: rash involves the posterior neck, upper back, and shoulders
V sign: rash involves the anterior neck and chest
Mechanic’s hands Rough, scaly lesions with fissuring of the skin over the lateral margins and pads of the fingers

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Cardiopulmonary Involvement

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nterstitial lung disease (ILD) with progressive pulmonary fibrosis and secondary pulmonary arterial hypertension is one of the leading causes of death in patients with polymyositis and dermatomyositis. ILD may be prominent at the onset of myopathy or develop over the course of the disease. The presence of anti–Jo-1 antibodies is associated with an increased risk for ILD.