Scleroderma, maybe!

Thanks to Paige for presenting the case of a middle aged homeless man who presented with heart failure exacerbation, found to have digital ischemia and subsequently diagnosed with scleroderma and likely contribution from cocaine-induced Raynaud’s!


Clinical Pearls

  • Break digital ischemia down to its culprit vessels to help you formulate a DDx: large, medium, and small (see below)
  • Raynaud’s phenomenon can be primary (idiopathic) or secondary to autoimmune conditions, hematologic disorders, drugs/toxins, environmental factors, or hypothyroidism.
  • Treatment of Raynaud’s involves avoiding known triggers, calcium channel blockers, and phosphodieterase inhibitors.
  • Three antibodies are associated with scleroderma: anti-centromere (limited cutaneous systemic sclerosis), anti-Scl70 (diffuse cutaneous systemic sclerosis), and anti-RNA polymerase III (diffuse cutaneous systemic sclerosis).  Patients with the latter antibody are more likely to develop scleroderma renal crisis.
  • Avoid steroids in patients with scleroderma because they can precipitate renal crisis!

Digital Ischemia

Large vessel

  • Atherosclerosis
  • Arterial dissection
  • Takayasu
  • Thoracic outlet

Medium vessel

  • Thromboangiitis obliterans (Buerger’s disease)
  • PAN
  • Hypercoagulability (APLS, DIC)
  • Scleroderma
  • Endocarditis
  • Trauma
  • Post-surgical

Small Vessel

  • Lupus
  • Scleroderma
  • Cryoglobulinemia
  • Other connective tissue diseases
  • Hypercoagulability
  • Endocarditis
  • Paraproteinemia
  • Polycythemia
  • Disfibrinogenemia
  • Trauma
  • Frostbite
  • Post-surgical

Raynaud’s Phenomenon:

  • Exaggerated vascular response (vasospasm) to cold temperature or emotional stress, manifested by discomfort and sharply demarcated color changes of the skin of the digits.
  • Primary Raynaud’s: Those without a definable cause, idiopathic
    • Onset is 15-30 years
    • More common in women
    • Occurs in multiple family members
    • Higher prevalence in people with fibromyalgia and migraines though unclear linkage.
  • Secondary Raynaud’s: There is a cause
    • Autoimmune rheumatic diseases
      • Systemic sclerosis, SLE, MCTD, sjogren’s, DM/PM
    • Drugs/toxins: cocaine, amphetamines, chemo (cisplatin, bleomycin)
    • Hematologic abnormalities: cryoglobulinemia, cold agglutinin disease, paraproteinemia, POEMS, cryofibrinogenemia
    • Occupational/environmental: vascular trauma, use of vibrating tools, frostbite, CTS
    • Hypothyroidism

Scleroderma 

  • Localized
    • Morphea
    • Linear scleroderma
  • Systemic cutaneous
    • Limited:
      • Face, hands, feet
      • 10-15% of patients may develop pHTN, ILD, or CREST
      • >70% survival at 10 years
      • Anti-centromere antibody
    • Diffuse
      • Early and significant renal, ILD, GI, and myocardial disease
      • Survival 50-60% at 10 years
      • Anti-Scl70
      • Anti-RNA polymerase III

Screen for malignancy in patients with new diagnosis of scleroderma!

Extracutaneous disease:

  • Pulm:
    • ILD (75%)
    • Pulmonary HTN (10-40%)
    • Lung cancer
  • GI (90%):
    • Esophageal dysmotility (GERD-like symptoms)
  • Renal:
    • Scleroderma renal crisis (15%)
  • MSK:
    • Inflammatory arthritis (rare)
  • Vascular:
    • Digital ischemia,
    • Pulmonary HTN,
    • Scleroderma renal crisis
    • MI
    • GAVE

Management:

  • Avoid steroids (it can precipitate scleroderma renal crisis)
  • Biologics: modest benefit to slow progression/severity of complications if started early in disease course
  • Otherwise, treatment is organ based

BONUS Thromboangiitis obliterans (Buerger’s disease)

  • Nonatherosclerotic, segmental, inflammatory disease that affects the small to medium-sized arteries and veins of the extremities
  • Usually young smokers
  • Associated primarily with tobacco products but cannabis arteritis has also been reported and is clinically indistinguishable.
  • Can present with superficial thrombophlebitis, Raynaud’s, digital ischemia, other organ ischemia (cerebral, coronary, internal thoracic, renal, and mesenteric arteries), or joint complaints.
  • Work up
    • Labs to rule out other similar disorders:
      • Acute phase reactants
      • Immunologic panel – ANA, RF, complements, anticentromere antibody, anti-scl70
      • Complete hypergoabulability screen
      • Tox panel for cocaine, amphetamines, and cannabis
    • ABI: a normal ABI does not rule out this disease because vessel occlusion could be limited to distal vasculature. Could perform digit plethysmography or toe pressures to confirm.  Abnormal test is not specific to TAO.
    • Vascular imaging:
      • MRA or CTA may not provide sufficient spatial resolution for the distal digits so it’s best to image the entire aorta and upper and lower extremities for evidence of disease that has not yet clinically manifested itself.
      • Angiographic findings would be similar to patients with cocaine, amphetamine, or cannabis ingestion related digital ischemia.
    • DDx
      • PAD: usually only in lower extremities as opposed to both upper and lower.
      • Thromboembolic disease
      • Vasculitis
      • Repetitive trauma
    • Treatment:
      • Smoking cessation
      • Intermittent pneumatic compression for lower extremities.
      • Vasodilators
        • IV prostaglandins (iloprost) à best studied but not very practical because it’s a 6 hour daily infusion
        • Phosphodiesterase inhibitors (cilostazol, sildenafil, tadalafil)
        • CCB (nifedipine)
      • Outcomes:
        • Largest series of 224 patients who stopped smoking had a vascular event-free survival of 41% at 5 years and 23% at 10 years.
          • Amputation free survival was 85, 74, and 66% at 5, 10, and 15 years

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