Scleroderma Systemic sclerosis

SclerodermaScleroderma
  • Facts:
    Inflammation that leads to progressive tissue fibrosis through excessive deposition of type I and type III collagen
    Commonly manifests as CREST syndrome, but can also be diffuse
  • History / PE:
    Symmetric thickening of skin of face, distal extremities

    CREST syndrome

    C alcinosis (calcium in soft tissue)
    R aynaud's phenomenon
    E sophageal dysmotility
    S clerodactyly
    T elangiectasias

    Diffuse sclerosis
    Pulmoary fibrosis, cor pulmonale , ARF, malignant HTN

  • Diagnosis:
    Positive RF and ANA
    Anticentromere antibodies (CREST)
    Anti-Scl-70 / anti-topoisomerase-1 antibodies (diffuse)
  • Treatment:
    Corticosteroids (acute flares)
    Penicillamine (skin changes)
    Calcium channel blockers (Raynaud's)
    ACEIs (renal disease)
  • Complications:
    Pulmonary HTN
  • Differential Diagnosis:
    Mixed connective tissue disease (anti nRNP)
    Sjogren's syndrome



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August 4th 2010