Mirouse Adrien, Saadoun David, Cacoub Patrice
Département de médecine interne et immunologie clinique, groupe hospitalier La Pitié-Salpêtrière, AP-HP, Paris, France. INSERM UMRS 959, I3 Immunologie-immunopathologie-immunothérapie, Sorbonne Université, Paris.
Département de médecine interne et immunologie clinique, groupe hospitalier La Pitié-Salpêtrière, AP-HP, Paris, France. Sorbonne Université.
Rev Prat. 2022 Dec;72(10):1122-1128.
Cryoglobulins are immunoglobulins that precipitate in vitro when serum is incubated at temperatures lower than 37°C. Cryoglobulins are classified under three subgroups according to their components. Cryoglobulinemic vasculitis corresponds to manifestations associated with vascular occlusion by cryoglobulins or inflammatory manifestations induced by the deposition of cryoglobulins containing immune complex. Main manifestations are skin lesions, including vascular purpura, necrosis, kidney involvement, and peripheral nerve involvement. Initial work-up aims at identifying the underlying disease, which may be a B-lineage hematological malignancy, a connective tissue disorder, or a chronic viral infection like hepatitis C. Treatment and prognosis are closely dependent of the underlying disease.
冷球蛋白是在血清于低于37°C的温度下孵育时会在体外沉淀的免疫球蛋白。根据其成分，冷球蛋白可分为三个亚组。冷球蛋白血症性血管炎对应于与冷球蛋白引起的血管闭塞或由含有免疫复合物的冷球蛋白沉积诱导的炎症表现相关的症状。主要表现为皮肤病变，包括血管性紫癜、坏死、肾脏受累和周围神经受累。初步检查旨在确定潜在疾病，其可能是B淋巴细胞系血液系统恶性肿瘤、结缔组织病或慢性病毒感染，如丙型肝炎。治疗和预后与潜在疾病密切相关。
