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Key Features

  • Many patients with symptoms and signs of a connective tissue disease have features of more than one type of rheumatic disease

  • Special attention has been drawn to a subset of antinuclear antibody–positive patients who have high titers of RNP autoantibodies and overlapping features of SLE, scleroderma, rheumatoid arthritis and inflammatory myositis

Clinical Findings

  • Swollen or puffy hands are a common early feature of disease

  • Raynaud phenomenon, arthralgias and myalgias are common

  • Unlike patients with SLE, renal or central nervous system disease is uncommon

  • A key reason to identify this subset of patients is that pulmonary hypertension and interstitial lung disease are major causes of mortality, and regular screening for these manifestations is required

Diagnosis

  • Clinical

  • Table 20–7

  • Differential diagnosis

    • SLE

    • Scleroderma

    • Polymyositis

    • Sjögren syndrome

    • Rheumatoid arthritis

    • Eosinophilic fasciitis

    • Graft-versus-host disease

Table 20–7.Frequency (%) of autoantibodies in rheumatic diseases.1

Treatment

  • Guided more by the distribution and severity of organ system involvement than by therapies specific to these overlap syndromes

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