Morning Report 11/4 – CREST and Dermatomyositis

Teaching Pearls:

  • Clinical Features of CREST syndrome
    • Calcinosis of soft tissue, Raynauds phenomenon, Esophageal dysmotility, Sclerodactyl, Telangiectasia
  • Diffuse Cutaneous Systemic Sclerosis (dcSS)
    • Skin Findings – Extends past elbows and knees from hands and feet, respectively. Can be seen along torso and face/neck
    • Antibody – anti-Scl-70
    • Pulm manifestation – interstitial lung disease
    • Associated with sclerodermal renal crisis
    • Not associated with CREST
  • Limited Cutaneous Systemic Sclerosis (lcSS)
    • Skin Findings – hands/feet, distal to elbows and knees. Face and neck
    • Antibody – ANA centromere pattern
    • Pulm manifestation – pulmonary hypertension
    • Not associated with sclerodermal renal crisis
    • Associated with CREST
  • Dermatomyositis
    • Heliotropic rash, shawls and V sign, gottron’s papules. Mechanic hands
    • Classically associated with proximal muscle weakness
    • Elevated CK and aldolase.
    • Other studies to consider: EMG and muscle biopsy (definitive diagnosis)
    • Associated with anti-Jo-1 antibody (anti-synthetase syndrome)
      • Higher association with ILD and worse prognosis
    • Higher association with malignancy.
      • Colon, breast, ovarian, prostate, etc.
    • Few case reports have found an association with oropharyngeal dysphagia, and seems to suggest a poor prognosis sign.
  • Serologic Studies/Associations
    • Anti-centromere pattern of ANA – lcSS
    • Anti-dsDNA Ab – SLE
    • Anti-smooth muscle Ab – autoimmune hepatitis
    • Anti-La/SSB antibody – Sjogrens, neonatal SLE
    • Anti-RNP antibody – Mixed connective tissue disease
    • Antihistone antibody – drug induced lupus
    • Anti-Scl-70 Ab – dcSS
    • Anti-Ro/SSA antibody – Sjogrens, neonatal heart block
    • c-ANCA – Wegeners granulomatosis
    • p-ANCA – Churg Strauss, microscopic polyangitis
    • Anti-Jo-1 Ab – dermatomyositis/polymyositis/anti-synthetase syndrome
    • Anti-CCP Ab – Rheumatoid arthritis

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