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患者女,31岁。躯干及四肢红斑1个月,加重伴发热1周。查体:双侧颈部可扪及肿大淋巴结。前胸皮损组织病理示:表皮角化过度,棘层增厚,真皮血管周淋巴细胞、组织细胞及少量嗜酸性粒细胞浸润。直接免疫荧光IgM(+)。乳酸脱氢酶1 249U/L,铁蛋白>2 000ng/mL;ANA(+)1∶80;抗ds-DNA抗体(+);抗Sm抗体(+);入院诊断SLE?经过糖皮质激素治疗,患者临床症状好转。但是10d后患者突发高热,并出现血小板降低,血清纤维蛋白原降低,甘油三酯升高。经过改变糖皮质激素剂型,患者的白细胞与血小板数目回升。最终诊断为系统性红斑狼疮(SLE),巨噬细胞活化综合征(MAS)。
Female patient, 31 years old. Trunk and limbs erythema 1 month, increased with fever for 1 week. Physical examination: bilateral neck palpable and enlarged lymph nodes. Pathological chest lesions showed: epidermal hyperkeratosis, stratum spinosum, dermal perivascular lymphocytes, tissue cells and a small amount of eosinophil infiltration. Direct immunofluorescence of IgM (+). Anti-ds-DNA antibody (+); Anti-Sm antibody (+); Admission diagnosis SLE? Glucocorticoid therapy , The patient’s clinical symptoms improved. However, 10 days after the sudden onset of fever in patients with thrombocytopenia, decreased serum fibrinogen, triglycerides increased. After changing glucocorticoid dosage form, the number of patients with white blood cells and platelets rose. The final diagnosis of systemic lupus erythematosus (SLE), macrophage activation syndrome (MAS).