29th January 2024

Cryoglobulinemic vasculitis

Background

Epidemiology 

Cryoglobulinemia is a rare disease which affects approximately 1 per 100,000 population with significant geographic variations. The disease is more common over the age of 50 and affects females more than males at a ratio of 3:1. 

Aetiology

Cryoglobulinemic vasculitis is the most common extra-hepatic manifestation of hepatitis C. Other associations include multiple myeloma, lymphoproliferative disorders and autoimmune diseases such as systemic lupus erythematosus. 

Pathophysiology 

Cryoglobulins can be transiently detected in asymptomatic individuals without leading to disease. Three types of cryoglobulins are distinguished based on whether the cryoglobulin is monoclonal and has rheumatoid factor activity. Temperature-dependent formation of immune complexes (IgG and IgM) in small and medium-sized blood vessel walls leads to complement activation and inflammation of the involved vessels and surrounding tissue. 

Diagnosis 

Cold-precipitable immunoglobulins or immune complexes and raised rheumatoid factor are characteristic of the condition. The majority of the cases have hypocomplementemia. It is crucial to screen for hepatitis C with associated liver function derangements. Skin or renal biopsy can identify inflammatory vascular changes and renal damage assessed with cryoglobulin deposits of IgG and IgM deposits in the glomeruli. Further tests for underlying causes include WBC, autoimmune antibodies and imaging such as angiography, CT and CXR. 

Treatment 

Treatment of the underlying disease such as hepatitis C, hepatitis B and HIV are crucial. Immunosuppression with systemic corticosteroids and rituximab is used in mild disease with organ involvement. Plasmapheresis combined with immunosuppressive therapy is indicated in severe or life-threatening cases. 

References

• About vasculitis, Vasculitis UK. Available at: https://www.vasculitis.org.uk/about-vasculitis [Accessed 28th January 2022].
• Cryoglobulinemia, Vasculitis Foundation. Available at: https://www.vasculitisfoundation.org/education/forms/cryoglobulinemia/ [Available 28th January 2022].
• Davuluri, S. and Bansal, P., 2020. Cryglobulinemic Vasculitis. Stat Pearls Publishing, Treasure Island (FL)
• Vasculitides, Amboss. Available at: https://www.amboss.com/us/knowledge/Vasculitides/ [Accessed 17th January 2022].