Gadolinium-Associated Nephrogenic Systemic Fibrosis

Aka: Gadolinium-Associated Nephrogenic Systemic Fibrosis, Nephrogenic Fibrosing Dermopathy, Scleromyxedema-like Cutaneous Disease

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II. Epidemiology

  1. Incidence:
    1. Rare, overall Incidence
    2. Severe Renal Failure and exposure to gadalinium: 4%

III. Pathophysiology

  1. Triad of Gadolinium exposure, End-stage Renal disease (ESRD), and Proinflammatory state
  2. Nephrogenic multi-systemic fibrosis involves the heart, lung and diaphragm, skeletal Muscle, liver, CNS and genitourinary tract

IV. Risk Factors

  1. Gadolinium Exposure
    1. Gadodiamide (Omniscan)
    2. Gadopentetate dimeglumine (Magnevist)
    3. Gadoversetamide (Optimark)
  2. Renal dysfunction (Stage 4-5)
    1. Severe renal dysfuntion (GFR<30 ml/min)
    2. Hemodialysis or Peritoneal Dialysis
    3. Acute Renal Failure
  3. Proinflammatory state
    1. Recent major surgery
    2. Thrombosis history
    3. Malignancy
    4. High dose Erythropoietin

V. Prevention

  1. Avoid gadolinium-based Contrast Material when GFR <30 ml/min or Hepatorenal-mediated Acute Renal Failure

VI. Course

  1. Onset within 2-3 months of gadolinium exposure (median 11 days)

VII. Symptoms

  1. Pruritic rash involving symmetric extremities and trunk

VIII. Signs

  1. Characteristics
    1. Erythematous Plaques with induration and swelling
    2. Peau d'orange appearance
    3. Lesions are tender and may be painful
  2. Distribution
    1. Symmetrically distributed lesions on extremities and trunk
    2. Lower extremities are most involved (but may also affect the arms and torso)
    3. Spares the face

IX. Differential Diagnosis

  1. Pretibial Myxedema (Hypothyroidism)
  2. Panniculitis
  3. Lipodermatosclerosis
  4. Morphea

X. Diagnosis

  1. Gadolinium or Gadodiamide may be found in lesions
  2. Skin biopsy of lesion (include depth to subcutaneous fat or fascia)
    1. CD34 spindle-shaped fibrocytes with thickened Collagen bundles

XI. Complications

  1. Debilitating joint contractures
  2. Multi-system fibrosis and resulting multi-system organ failure
  3. Respiratory Failure (due to diaphragmatic involvement)

XII. Prognosis

  1. Chronic unremitting course
  2. Mortality: 31%

XIII. References

  1. Wolff (2017) Fitzpatrick's Clinical Dermatology, 8th ed, McGraw Hill, p. 428
  2. Cowper (2003) Curr Opin Rheumatol 15(6): 785-90 [PubMed]
  3. Sadowski (2007) Radiology 243(1): 148-57 [PubMed]
  4. Schlaudecker (2009) Am Fam Physician 80(7): 711-4 [PubMed]

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