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Key Features

  • Type I cryoglobulins (monoclonal proteins that lack rheumatoid factor activity)

    • More commonly seen in lymphoproliferative disease

    • Usually cause hyperviscosity syndromes rather than vasculitis

  • Type II (monoclonal antibody with rheumatoid factor activity) and type III (polyclonal antibody with rheumatoid factor activity) cryoglobulins

    • Cause vasculitis

    • Associated with hepatitis C and connective tissue diseases (eg, Sjögren syndrome)

Clinical Findings

  • Palpable purpura

  • Peripheral neuropathy

  • Proliferative glomerulonephritis can develop and can manifest as rapidly progressive glomerulonephritis

  • Abnormal liver function tests

  • Abdominal pain

  • Digital gangrene

  • Pulmonary disease

Diagnosis

  • Compatible clinical picture and a positive serum test for cryoglobulins

  • Presence of a disproportionately low C4 level can be a diagnostic clue to the presence of cryoglobulinemia

Treatment

  • Antiviral regimens that do not include interferon are first-line therapy for hepatitis C–associated cryoglobulinemic vasculitis that is neither life- nor organ-threatening

  • Patients with severe cryoglobulinemic vasculitis (eg, extensive digital gangrene, extensive neuropathy, and rapidly progressive glomerulonephritis) and hepatitis C should receive immunosuppressive therapy with corticosteroids and either rituximab or cyclophosphamide as well as interferon-free antiviral therapy

  • Plasma exchange may provide additional benefit in selected cases

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