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KEY FEATURES

  • A subset of patients with overlapping features of the following autoimmune diseases

    • Systemic lupus erythematosus (SLE)

    • Systemic sclerosis

    • Rheumatoid arthritis (RA)

    • Inflammatory myositis

  • Serologies

    • Positive antinuclear antibodies

    • High titers of ribonuclear protein (RNP) autoantibodies

  • 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

  • “Overlap syndrome”

    • Patients with features of more than one connective tissue disease (eg, RA and SLE, SLE and systemic sclerosis)

    • However, there are no high-titer anti-RNP antibodies

  • “Undifferentiated connective tissue disease”: patients have only a few features of autoimmunity and cannot yet be classified

CLINICAL FINDINGS

  • Swollen or puffy hands are a common early feature of the disease, referred to as mixed connective tissue disease

  • Raynaud phenomenon, arthralgias, and myalgias are common

  • Renal or central nervous system disease is uncommon (unlike in patients with SLE)

DIAGNOSIS

  • Clinical

  • See Table 22–6

  • Differential diagnosis

    • SLE

    • Systemic sclerosis

    • Polymyositis

    • Sjögren syndrome

    • RA

    • Eosinophilic fasciitis

    • Graft-versus-host disease

Table 22–6.Frequency (%) of autoantibodies in rheumatic diseases.1

TREATMENT

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

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