Cryofibrinogenemia: What Rheumatologists Should Know

Abstract

Abstract: Cryofibrinogenemia refers to the presence of cryofibrinogen in plasma. This protein has the property of precipitating at lower temperatures. Cryofibrinogenemia is a rare disorder, clinically characterized by skin lesions, such as ulcers, necrosis, livedo reticularis, arthralgia, thrombosis, and limb ischemia. These features are most often observed in rheumatological practice and consist of differential diagnoses of antiphospholipid syndrome, primary vasculitis, thrombotic thrombocyto-penic purpura, and cryoglobulinemia. Classical histopathological findings include the presence of thrombi within the lumen of blood vessels of the skin without vasculitis. To date, there are no vali-dated classification criteria. Management includes corticosteroids, immunosuppressive therapy, an-ticoagulants, and fibrinolytic agents. This narrative review aims to make physicians, particularly rheumatologists, aware of the existence of this underdiagnosed condition. There are no epidemio-logical studies evaluating the prevalence of cryofibrinogenemia in different rheumatological disor-ders. Studies are also required to investigate if certain features of rheumatological diseases are relat-ed to the presence of cryofibrinogenemia.