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Key Features

  • Features of more than one rheumatic disease; overlap connective tissue disease is the preferred designation for patients having features of different rheumatic diseases

  • Special attention has been drawn to antinuclear antibody–positive patients who have overlapping features of SLE, systemic sclerosis (scleroderma), and inflammatory myopathy together with autoantibodies to ribonuclear protein (RNP)

  • Some consider these patients to have a distinct entity ("mixed connective tissue disease"), and others view this as a subset of SLE characterized by a higher prevalence of Raynaud phenomenon, polyarthritis, myositis, and pulmonary hypertension and a lower incidence of renal involvement

  • Other patients have features of more than one connective tissue disease (eg, rheumatoid arthritis and SLE, SLE and systemic sclerosis) in the absence of anti-RNP antibodies and are referred to as having an "overlap syndrome"

Clinical Findings

  • Characteristics of multiple rheumatic diseases


  • Clinical

  • Table 20–7

  • Differential diagnosis

    • SLE

    • Systemic sclerosis

    • Polymyositis

    • Sjögren syndrome

    • Rheumatoid arthritis

    • Eosinophilic fasciitis

    • Graft-versus-host disease

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


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

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