Systemic Sclerosis

Evaluation Guide

• Concern for organ involvement other than skin?
  
• Pattern of skin disease? Diffuse or limited to extremities? (Skin tightening proximal or distal to elbow/wrist, knee/ankle? Does it involve the trunk (diffuse SSc)?
  
• What internal organs involved?
  • Raynaud’s (view image)
  • Gastrointestinal tract
  • Lung: ILD: PFTs, high-res CT w/o contrast (view image), chest x-ray
  • Pulmonary artery (pHTN): DLCO, TTE
  • Renal: scleroderma renal crisis (SRC)
  • Heart: cardiac fibrosis
  • Musculoskeletal: myositis, arthritis

Basic Labs

• CBC with differential (anemia of chronic disease, microangiopathic hemolytic anemia if scleroderma renal crisis)
• CMP
• ESR/CRP
• Urinalysis

SSc Serologies

• ANA (80-90%)
• SCL-70/topoisomerase: diffuse SSc
• Centromere: limited SSc
• RNA polymerase III: SRC

• Scleredema (DM2)
  
• Scleromyxedema (MGUS)
  
• Paraneoplastic
  
• POEMS
  
• Chronic graft vs. host disease
  
• Nephrogenic systemic fibrosis
  
• Eosinophilic fasciitis
  
• Amyloidosis
  
• Drug exposure (e.g., bleomycin)
  
• Environmental exposure (organic solvents, etc.)

• Highly recommend rheumatology referral before initiating treatment, as many components of SSc management require multi-disciplinary care and collaboration
  
• Treatment is based on extent of skin disease, organs involved, severity of disease
• Avoid prednisone ≥15 mg/day (associated with scleroderma renal crisis [SRC])
• Skin fibrosis:
  • Mycophenolate; can slow progression
  • Methotrexate
  • Collaboration with dermatology
    
• Raynaud’s:
  • Avoid triggers (cold, smoking, stress/anxiety)
  • Calcium channel blockers, topical nitroglycerin, PDE-5 inhibitors (do not combine with nitroglycerin), SSRI (fluoxetine). If refractory or severe digital ischemia, IV prostaglandin (hospitalization required)
    
• Arthritis:
  • DMARDS: hydroxychloroquine, methotrexate
    
• ILD:
  • Mycophenolate: less toxic than cyclophosphamide
  • Cyclophosphamide
  • Anti-fibrotics (nintedanib)
  • Tocilizumab
• SRC: ACEi and collaboration with nephrology
• pHTN treatment in collaboration with pulmonology
• GERD/dysphagia: H2 blockers, PPI and collaboration with gastroenterology

• Manage BP to decrease risk of renal crisis
• SRC is an emergency! Give ACEi (captopril) without delay. However, prophylactic ACE inhibitor is not suggested as it can lead to harder-to-detect SRC; recommend avoiding ACEi for antihypertensive therapy if alternate options available.
• Every visit, monitor for symptoms of extra-derm disease:
  • ILD (view image)
  • pHTN
  • Renal crisis (20% of dSSc, 5% of lSSc)
  • Myopericarditis
• CBC, CMP, U/A: monitored for drug toxicity and disease activity
• Follow-up testing for organs of concern: PFTs with DLCO, TTE, CT chest, GI scope
• Cancer screening
•  

General

• If there is no Raynaud’s and/or ANA is negative, SSc is rarely the diagnosis!  
• Scleroderma renal crisis (SRC): usually with diffuse SSc and early on in disease (first 5 yrs) – presents with sudden increase of BP

Evaluation

• Lab abnormalities in SRC: increase in serum creatinine, microangiopathic hemolytic anemia (MAHA)
• Antibodies to RNA polymerase III important to identify risk for SRC

Treatment & Monitoring

• ACEi should not be given prophylactically to prevent scleroderma renal crisis (SRC), but it is the gold standard treatment for SRC.
  • Do not delay giving ACEi (captopril)
  • Needs to be treated in hospital
• Avoid high dose glucocorticoids due to their associated increased risk of renal crisis
  • Steroids do not decrease skin tightening
• Interstitial lung disease (ILD) carries high mortality and is seen more with anti-Scl-70
• Pulmonary HTN is significant cause of mortality for limited SSc
• Close monitoring of BPs and serial TTEs and PFTs are key