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