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Clinical Services at Rush Types of Vasculitis

Cryoglobulinemic vasculitis (CRV)

What is the cause of cryoglobulinemic vasculitis?
What are the symptoms of cryoglobulinemic vasculitis?
How is the diagnosis of cryoglobulinemic vasculitis made?
What is the treatment for cryoglobulinemic vasculitis?
What is the prognosis for cryoglobulinemic vasculitis?

Cryoglobulinemic vasculitis (CRV) is a result of the presence of abnormal proteins (antibodies known as cryoglobulins) in the blood. These cryoglobulins become thick or gel-like in cold temperatures, at which point they can block blood vessels throughout the body.

This may lead to complications ranging from skin rashes to kidney failure.

The disorder is grouped into three main types, depending on the type of antibody that is produced:

  • Cryoglobulinemia type I
  • Cryoglobulinemia type II
  • Cryoglobulinemia type III

Types II and III are also referred to as mixed cryoglobulinemia.Type I cryoglobulinemia is most often related to cancer of the blood or immune systems.

Types II and III are most often found in people who have a chronic (long-lasting) inflammatory condition, such as an autoimmune disease or hepatitis C. Many patients with mixed cryoglobulinemia have a chronic hepatitis C infection.

Other conditions that may be related to cryoglobulinemia include:

  • Leukemia
  • Multiple myeloma
  • Mycoplasma pneumonia
  • Sjögren’s disease
  • Primary macroglobulinemia
  • Monoclonal gammopathy of unclear significance
  • Rheumatoid arthritis
  • Systemic lupus erythematosus

What is the cause of cryoglobulinemic vasculitis?

It is not known why cryoglobulins become solid at low temperatures. The hepatitis C virus has been associated with most cases of type II or III cryoglobulinemia. It seems that the virus stimulates the body to make these types of cryoglobulins (antibodies) that become solid at low temperatures.

What are the symptoms of cryoglobulinemic vasculitis?

Symptoms vary depending on the type of CRV and the organs that are affected. In general, symptoms may include:

  • Difficulty breathing
  • Fatigue
  • Kidney disease (glomerulonephritis)
  • Joint pain
  • Muscle pain
  • Skin rash (purpura)
  • Raynaud's phenomenon
  • Skin death
  • Skin ulceration

How is the diagnosis of cryoglobulinemic vasculitis made?

In addition to a physical examination, laboratory tests may include:

  • Complete blood count (CBC)
  • Complement assay (numbers will be low)
  • Cryoglobulin test (may show presence of cryoglobulins)
  • Liver function tests (may be high)
  • Hepatitis C virus test
  • Rheumatoid factor (positive in types II and III)
  • Urinalysis (may show blood in the urine if the kidneys are affected

Other tests may include:

  • Angiogram
  • Chest X-ray
  • Skin biopsy (in case of a rash)
  • Erythrocyte sedimentation rate (ESR)
  • Nerve conduction tests, if there is weakness in the arms or legs
  • Protein electrophoresis - blood

What is the treatment for cryoglobulinemic vasculitis?

Treatment of mild or moderate CRV depends on the underlying cause. Treating the cause will often treat the cryoglobulinemia.

Mild cases can be treated by avoiding cold temperatures and with mild nonsteroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen (Advil, Motrin).

Standard hepatitis C treatments usually work for patients who have hepatitis C and mild or moderate cryoglobulinemia. However, the condition can return when treatment stops.

Severe cryoglobulinemia that involves vital organs or large areas of skin is treated with corticosteroids and possibly also other immunosuppressive medications such as azathioprine (Imuran), chlorambucil or cyclophosphamide (especially if the kidneys are involved). More recently rituximab (Rituxan) has been found to be effective in CRV, especially when the disease is associated with hepatitis C.

Treatment may also involve plasmapheresis. Plasmapheresis is a procedure in which blood plasma is removed from the circulation and replaced by fluid, protein or donated plasma.

What is the prognosis for cryoglobulinemic vasculitis?

Cryoglobulinemic vasculitis is not usually deadly. However, if the kidneys are affected, the outlook is much less favorable.

Medication toxicity remains a major concern in cryoglobulinemic vasculitis. Therefore, it is very important that patients get regular check-ups, be vaccinated and be constantly monitored for any infection or side effects of therapy. All patients affected by cryoglobulinemic vasculitis should be under the supervision of a skilled health team that is familiar with these conditions, their treatment and the side effects of immunosuppressive medications. 





Contact Name
Rush Vasculitis Clinic
Contact Phone
(312) 563-2800
Contact E-mail
contact_rush@rush.edu



Location
Orthopedic Building
1611 W. Harrison St., Suite 510
Chicago, IL 60612



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