Overview of cryoglobulinaemia and cryoglobulinaemic vasculitis

Bhandari, 2021Davuluri, 2021Kolopp-Sarda, 2021

Cryoglobulins are serum immunoglobulins that reversibly precipitate in the cold. Cryoglobulins most commonly develop in response to chronic infections with lymphotropic viruses such as human immunodeficiency virus (HIV) or hepatitis C. Other causes are lymphoproliferative disorders (eg chronic lymphocytic leukaemia, multiple myeloma) and inflammatory connective tissue diseases (eg systemic lupus erythematosus [SLE], Sjögren syndrome).

Cryoglobulinaemia is a term for cryoglobulins existing in the serum that may be asymptomatic. Sometimes cryoglobulinaemia can cause symptoms, the most common being rash and arthralgias.

Cryoglobulinaemic vasculitis (also known as mixed cryoglobulinaemia syndrome) is an inflammatory syndrome associated with cryoglobulinaemia that can cause a spectrum of disease ranging from minor skin manifestations to life-threatening vasculitis and kidney disease; see Clinical features of cryoglobulinaemia for details. The vasculitis is an immune complex–mediated vasculitis of small- and medium-sized vesselsGalli, 2019.

Management for more severe vasculitis and organ-related disease may require immunomodulatory therapy as the disease escalates.