Vasculitis refers to a group of conditions that are characterized by inflammation of the walls of blood vessels; these include the veins, arteries, and capillaries. Essential mixed cryoglobulinemia is a blood disorder that is caused by abnormal proteins in the blood called cryoglobulins that precipitate or clump together when blood is chilled and then dissolve when re-warmed. These proteins can be deposited in small and medium-sized blood vessels, which can lead to restricted blood flow to joints, muscles, and organs.
This article will discuss cryoglobulinemic vasculitis (C. vasculitis). C. vasculitis has a strong association to hepatitis C infection. It should be pointed out the C. vasculitis is an uncommon disease in people with hepatitis C. It is also a disease that progresses slowly and requires many years to cause damage. But it is a devastating disease for those who have it.
Symptoms: vasculitis can affect almost every organ in the body. The more common symptoms and conditions produced by vasculitis include:
- Skin – rashes characterized by purplish red spots that are usually found on the legs
- Joints – joint aches, and arthritis that include swelling
- Lungs – shortness of breath, cough, and lung infiltrates
- Kidneys – clumps of red blood cells in the kidneys, and loss of protein through the urine
- Gastrointestinal tract – abdominal pain, and bloody diarrhea
- Blood – anemia and/or elevated white blood cell counts
- Sinuses and nose – chronic sinus congestion and infections, hearing problems, and inflammation of nasal tissues
- Eyes – damage to the blood vessels of the eye
- Brain – headaches, difficulty with coordination, changes in mental status, and strokes (rare)
- Nerves – shooting pains in the arms and legs, as well as numbness and weakness
- Other symptoms include fever, itching welts, fatigue, weight loss, muscle ache and pain, enlarged lymph nodes, and peripheral neuropathy
Diagnosis: There are no standardized diagnostic tests or criteria for vasculitis. Vasculitis is usually diagnosed by a variety of tests that includes testing for cryoglobulins, antinuclear antibody test (that can detect underlying connective tissue disorders), skin or tissue biopsy, electromyography (detecting electrical signals in muscle cells), arteriography (pictures of blood vessels), and by the symptoms listed above.
Treatment: Treatment of C. vasculitis is an approach depending on the severity of disease progression. Since C. vasculitis is caused by hepatitis C it is generally recommended to treat the underlying cause (HCV). Unfortunately, HCV therapy does not always result in long-term resolution of vasculitis.
Treatment with direct-acting antivirals (DAAs) was found to be safe and effective in treating hepatitis C but DAA treatment did not improve C. vasculitis in the majority of symptomatic C. vasculitis patients requiring other treatment strategies.
Plasmapheresis- a treatment that removes the blood, separates the blood cells from plasma and returns the blood cells to the body. This therapy is usually combined with other immunosuppressant medications.
Cryofiltration- an addition to plasmapheresis, this method may be used to remove the blood by plasmapheresis then chilled in a refrigeration unit and the cryoglobulins are filtered and removed. The filtered plasma is re-warmed and combined with the blood cells and returned to the body. This procedure is rarely performed since it may also filter out other components of blood.
Rituximab- Rituximab is an immunosuppressant drug that is FDA approved to treat some forms of non-Hodgkin’s lymphoma. Treatment with rituximab has been found to be the most effective treatment with long-term remission in some patients. There is some evidence that it may be helpful when combined with HCV therapy.
Steroids- In severe cases, prednisone and cytotoxic drugs (such as cyclophosphamide and chlorambucil) may be used, however, the use of these drugs in people with hepatitis C is generally discouraged.
Conclusion: In summary, C. vasculitis is an uncommon disease condition in people infected with hepatitis C. It is a difficult condition to diagnose because there are no established diagnostic criteria, and current treatment strategies have had limited success. Fortunately, there is much research into the cause and treatment of vasculitis as well as new drugs to treat hepatitis C. Imagine a future free of hepatitis C and hepatitis C-related extrahepatic manifestations including c. vasculitis.
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