临床病例评析——突发胸痛左下肢肿痛颧部红斑

Sudden chest pain,painful swelling left lower limb and malar erythema

目的提高临床对APS与SLE之间关系的认识。方法报道1例以原发APS为首发临床表现,随诊过程中新发皮肤损害、抗dsDNA抗体阳性,转为患者的临床特点及诊治经过,并进行分析讨论。结果15岁女性患者,病程初期表现为急性肺栓塞、下肢深静脉血栓,抗磷脂抗体高滴度阳性,其他自身抗体阴性,诊断原发性APS,予溶栓抗凝治疗后好转。随访期间患者新发面部红斑,伴淋巴细胞减少、大量蛋白尿,ANA、抗dsDNA抗体阳性,补体降低,诊断SLE,予激素冲击治疗及免疫抑制剂治疗后症状缓解。结论部分原发性APS患者病程中可进展为SLE,青年起病、ANA阳性、抗人球蛋白抗体阳性等危险因素患者更应密切随访监测。

Objective To improve the understanding of the relationship between antiphospholipid syndrome(APS)and systemic lupus erythematosus(SLE).Methods The clinical characteristics and process of diagnosis and treatment of a case was reported and analyzed.This patient was initially diagnosed as antiphospholipid syndrome and later developed new skin lesion and positive anti-dsDNA antibody,which made the diagnosis of systemic lupus erythematosus.Results A 15-year-old girl suffered acute pulmonary embolism,lower extremity deep vein thrombosis,and high titer of anti-phospholipid antibody,but negative for other autoantibodies.So primary antiphospholipid syndrome was diagnosed.Symptoms were improved after thrombolysis and anticoa-gulation treatment.During the follow-up period,the patient developed malar erythema,lymphocytopenia,proteinuria,positive ANA,anti-dsDNA antibody,and reduced complement level.So she was diagnosed with systemic lupus erythematosus.After glucocorticoid pulse therapy and immunosuppressants treatment,the symptoms were relieved and lupus disease activity was decreased.Conclusion A few primary APS patients can progress into SLE.Patients with risk factors such as a younger age of onset,positive ANA and positive Coomb's test results should be closely followed up.