29th January 2024

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.