Cryoglobulinemias

 

 

Uploaded: 2008-02-08, Updated: 2008-02-08

  • A cryoglobulin is an immunoglobulin (Ig) that precipitates when the serum is incubated at a temperature lower than 37 C.

  • The term `cryoglobulinemic syndrome' is used when patients with cryoglobulinemia have clinical manifestations.

  • Main clinical hallmark of the cryoglobulinemic syndrome:

    • Palpable purpura, usually confined to the lower extremities.

    • Arthralgias (50-80%): most frequently proximal interphalangeal joints, metacarpophalangeal joints, knees and ankles.

    • Renal involvement (25%): proteinuria with progression to kidney failure. Histologically, membranoproliferative glomerulonephritis.

    • Peripheral neuropathy (7-60%): sensory nerves, paresthesia, pain.

 

Type I

Type II

Type III

Immunoglobulin Monoclonal IgM >IgG>IgA Monoclonal IgM forms complex with polyclonal IgG Polyclonal IgM forms complex with polyclonal IgG

Associated diseases

Lymphoproliferative disorders: Waldenström macroglobulinemia, multiple myeloma, lymphoma/leukemia.

Chronic infections, autoimmune disease, malignant disorders

HCV (most common causes), chronic infections, autoimmune disease, malignant disorders

Rheumatoid factor

None

Monoclonal IgM

Polyclonal IgM

Symptoms/signs

   Purpura

+

+++

+++

   Gangrene

+++

+/++

±

   Arthralgias

+

++

+++

   Renal

+

++

+

   Neurologic

+

++

++

   Liver

±

++

+++

 

Lupus (2000) 9, 83-91