摘要
干燥综合征是以外分泌腺受累、出现高度淋巴细胞浸润为特征的自身免疫病,临床表现为口干、眼干,还可累及关节、皮肤及肝、肾、肺等内脏器官。冷凝集素病是低温时冷凝集素引起阻塞微循环、溶血性贫血的自身免疫病。当患者血液在抗凝采血管中出现凝固时,需警惕合并冷凝集素病,如不及时治疗,可能会出现严重的溶血性贫血,甚至危及生命。现将北京大学首钢医院2022年收治的1例患者报告如下。
SUMMARY Sjogren’s syndrome(SS)is a chronic autoimmune disease that affects exocrine glands,especially salivary and lacrimal glands.The main clinical manifestations are dry mouth and dry eyes,but also multi-organ and multi-system can be involved.Cold agglutinin disease(CAD)is an autoimmune disease characterized by red blood cell agglutination in the blood vessels of extremities caused by cold agglutinin at low temperature,resulting in skin microcirculation disturbance,or hemolytic anemia.Cold agglutinin disease is divided into two categories,primary cold agglutinin disease and secondary cold agglutinin disease.Primary cold agglutinin disease is characterized with cold agglutinin titer of 1∶4000 or more and positive Coomb’s test.However,the Coomb’s test is not necessarily positive and the cold agglutinin titer is between 1∶32 and 1∶4000 in secondary cold agglutinin disease.Here,we reported an elderly patient admitted to hospital due to fever.He was diagnosed with respiratory infection,but he showed incompletely response to the anti-infection treatment.Further laboratory tests showed the patient with positive ANA and anti-SSA antibodies.Additionally,the patient complained that he had dry mouth and dry eyes for 1 year.Schirmer test and salivate gland imaging finally confirmed the diagnosis Sjogren’s syndrome.During the hospital stay,the blood clots were found in the anticoagulant tubes.Hemolytic anemia was considered as the patient had anemia with elevated reticulocytes and indirect bilirubin.In addition,further examination showed positive cold agglutination test with a titer of 1∶1024,and cold agglutinin disease was an important type of cold-resistant autoimmune hemolytic anemia.Furthermore,the patient developed cyanosis after ice incubating at the tip of the nose.Hence,the patient was diagnosed as CAD and he was successfully treated with glucocorticoids instead of anti-infection treatments.Hence,the patient was diagnosed with SS combined with secondary CAD.SS combined CAD are rarely reported,and they are both autoimmune diseases.The abnormal function of B lymphocytes and the production of autoantibodies might be the common pathogenesis of them.Cold agglutinin disease can lead to severe hemolytic anemia,even life-threatening.In clinical practice,timely recognizing and dealing with CAD might promote the prognosis of the patient.
作者
王丽芳
石连杰
宁武
高乃姝
王宽婷
WANG Li-fang;SHI Lian-jie;NING Wu;GAO Nai-shu;WANG Kuan-ting(Department of Rheumatology and Immunology,Peking University Shougang Hospital,Beijing 100144,China;Departmem of Infectious Disease,Peking University Shougang Hospital,Beijing 100144,China;Depanment of Clinical Laboratory,Peking University Shougang Hospital,Beijing 100144,China)
出处
《北京大学学报（医学版）》
CAS
CSCD
北大核心
2023年第6期1130-1134,共5页
Journal of Peking University:Health Sciences
关键词
干燥综合征
冷凝集
感染
糖皮质激素
Sjogren’s syndrome
Cold agglutinin
Infection
Glucocorticoid
作者简介
Corresponding author’s:王宽婷,e-mail,kuantingwang@163.com。
