Hospital Santa Izabel, Praça Almeida Couto 500, Salvador, Bahia, Brazil.
Serviços Especializados em Reumatologia da Bahia, Rua Conde Filho, Salvador, Bahia, Brazil.
Curr Rheumatol Rev. 2022;18(3):186-194. doi: 10.2174/1573397118666220325110737.
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 thrombocytopenic 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 validated classification criteria. Management includes corticosteroids, immunosuppressive therapy, anticoagulants, and fibrinolytic agents. This narrative review aims to make physicians, particularly rheumatologists, aware of the existence of this underdiagnosed condition. There are no epidemiological studies evaluating the prevalence of cryofibrinogenemia in different rheumatological disorders. Studies are also required to investigate if certain features of rheumatological diseases are related to the presence of cryofibrinogenemia.
冷纤维蛋白原血症是指血浆中存在冷纤维蛋白原。这种蛋白质具有在较低温度下沉淀的特性。冷纤维蛋白原血症是一种罕见的疾病，临床上以皮肤病变为特征，如溃疡、坏死、网状青斑、关节炎、血栓形成和肢体缺血。这些特征在风湿病学实践中最为常见，包括抗磷脂综合征、原发性血管炎、血栓性血小板减少性紫癜和冷球蛋白血症的鉴别诊断。经典的组织病理学发现包括血管内腔存在血栓，而无血管炎。迄今为止，尚无经过验证的分类标准。治疗包括皮质类固醇、免疫抑制疗法、抗凝剂和纤维蛋白溶解剂。本叙述性综述旨在使医生，特别是风湿病学家，意识到这种诊断不足的疾病的存在。目前尚无评估不同风湿病中冷纤维蛋白原血症患病率的流行病学研究。还需要研究某些风湿病的特征是否与冷纤维蛋白原血症的存在有关。
