• Scleroderma = systemic sclerosis vs. localized scleroderma (morphea)
  
  Systemic sclerosis
  Limited cutaneous systemic sclerosis (lcSSc): skin involvement restricted to face and distal extremities
  • CREST: calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia) --> subset of limited cutaneous SS
  • High risk of pulmonary HTN in absence of ILD
  Diffuse cutaneous systemic sclerosis (dcSSc): skin involvement proximal to distal forearms and knees
  • Higher risk of ILD, serositis, renal crisis
  
  Localized scleroderma (morphea)
  Typically circumscribed, generalized, linear skin lesions without internal organ involvement
  
  

  Posted 06/22/16 10:10:23 AM by Matthew Cummings

• Palpable tendon friction rubs: pathogenesis unclear, likely due to fibrin deposition in tendon’s synovial sheath
  • Found in 20-40% of patients with dcSSc, associated with disease activity and predictor of continued diffuse skin involvement
  
  

  Posted 06/22/16 10:17:16 AM by Matthew Cummings

• Nailbed capillaroscopy: presence of megacapillaries and decreased capillary density present in 60-80% of patients with lcSSc and dcSSc
  
  

  Posted 06/22/16 10:42:29 AM by Matthew Cummings

• Extractable nuclear antigen (ENA) panel
  • Anti-SSA (Ro), Anti-SSB (La): Sjögren's (and associated with congenital heart block in neonatal lupus)
  • Anti-Smith: SLE
  • Anti-U1-RNP: Mixed connective tissue disease
  • Anti-Jo-1 (synthetase): Polymyositis, anti-synthetase syndrome (ILD, symmetric small joint arthritis, myositis, mechanic's hands)
  • Anti-Scl-70 (anti-topoisomerase I): diffuse systemic sclerosis (higher risk for ILD)
  
  Anti-centromere: limited cutaneous systemic sclerosis
  
  Anti-RNA polymerase 3:  associated with rapidly progressive skin disease in diffuse cutaneous systemic sclerosis, increased risk for renal crisis, GAVE, and malignancy
  
  

  Posted 06/22/16 10:53:49 AM by Matthew Cummings

• Scleroderma renal crisis
  Risk factors: +anti-RNA polymerase, diffuse cutaneous or rapidly progressive SSc, recent treatment w/ high dose corticosteroids
  
  Physiology: narrowing of arterial vessels --> renal hypoperfusion --> hyperplasia of JGM apparatus --> increased renin release
  
  Treatment: ACEI
  
  
  
  

  Posted 06/22/16 11:07:57 AM by Matthew Cummings

• Raynoud's phenomenon treatment: dihydropyridine CCBs (nifedipine XL, amlodipine), ASA
  
  
  
  

  Posted 06/22/16 11:16:56 AM by Matthew Cummings

• For further reading...

  Tendon friction rubs in SSc
  Nailbed capillaroscopy in SSc
  Scleroderma renal crisis
  
  

  Posted 06/22/16 11:18:55 AM by Matthew Cummings

Created by Christopher Kelly

Know a hypochondriac? Get them the best-selling book 'AM I DYING?!: A Complete Guide to Your Symptoms, and What to Do Next'
The information on the website does not constitute official guidelines except where explicitly stated.
It is not meant to replace the advice of a health professional.