Updated: 4/22/2019

Mixed Connective Tissue Disease

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Snapshot
  • A 31-year-old woman presents to her primary care physician's office with general malaise, myalgias, and low-grade fever. She notices that during the winter months the tips of her fingers turn blue and then returns to her baseline color when in a warm environment. On physical exam, there is a malar rash, sclerodactyly, and joint tenderness. Laboratory testing is positive for anti-nuclear antibodies (ANA) and anti-U1 RNP antibodies.
Introduction
  • Clinical definition
    • generalized connective tissue disorder with clinical features of systemic lupus erythematosus, systemic sclerodermia, and polymyositis
      • along with high levels of anti-U1-RNP antibodies
  • Epidemiology
    • demographics
      • more common in women
      • 15-25 years of age but can occur at any age
  • Pathogenesis
    • autoimmune process
  • Prognosis
    • generally a favorable outcome
Presentation
  • Symptoms
    • Raynaud phenomenon
    • arthralgia
    • arthritis
    • myositis
    • sclerodactyly
    • hand swelling
Imaging
  • Echocardiography
    • indication
      • for early diagnosis of pulmonary arterial hypertension and for the evaluation of valvular disease
Studies
  • Labs
    • positive for anti-U1-RNP antibodies 
  • Diagnostic criteria
    • clinical diagnosis supported by anti-U1-RNP antibodies on serology
Differential
  • Systemic lupus erythematosus (SLE)
  • Scleroderma
  • Polymyositis
  • Rheumatoid arthritis
  • Idiopathic pulmonary arterial hypertension
Treatment
  • Management approach
    • treatment is aimed at controlling symptoms and is targeted to organ involvement
  • Medical
    • prednisone
      • indications
        • responsive in patients presenting with symptoms consistent with SLE
        • in patients with fatigue, myositis, myalgias, arthralgias, pleuritis, pericarditis, autoimmune anemia, and thrombocytopenia
    • calcium channel blockers
      • indications
        • pulmonary arterial hypertension
        • Raynaud phenomenon
Complications
  • The major cause of death is pulmonary hypertension in MCTD
 

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