重症成人Still病1例报道并文献复习

doi:10.3969/j.issn.1002-1949.2018.08.012

Chinese Journal of Critical Care Medicine

2018, Vol. 38

Issue (8): 700-705 DOI: 10.3969/j.issn.1002-1949.2018.08.012

Severe adult onset Still′s disease: a case report and literature review

Wang Min, Zheng Yao, Yang Min

Department of Intensive Care Medicine, the Second Affiliated Hospital of Anhui Medical University, Hefei 230032, China. Department of Cardiopulmonary Resuscitation and Critical Care Laboratory, the Second Affiliated Hospital of Anhui Medical University, Hefei 230023, China

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Abstract Objective To investigate the clinical characteristic of hemophagocytic syndrome (HPS) secondary to adult onset Still′s disease (AOSD). Methods We searched database including PubMed, CNKI, Wang Fang Data and VIP from inception to 2016, to collect reports for patients with HPS or macrophage activation syndrome or hemophagocytic lymphohistiocytosis secondary to AOSD. Meanwhile, literature was reviewed. Results A female patient, 34 years old, presented with a half-month history of spiking fever, associated with pharyngalgia, arthralgia and an evanescent rash and hospitalized in October 2017. After admission, the patient was diagnosed as fever of unknown origin. The body temperature was not decreased after 8 days of treatment with broad-spectrum antibiotics and nonsteroidal anti-inflammatory drugs (NSAID). The body temperature increased again within 48 hours after administration of glucocorticoid, and her condition suddenly became deteriorated and developed hypotension, progressive dyspnea on 10 days of institution. Bone marrow biopsy revealed hemophagocytosis. She was diagnosed as HPS secondary to AOSD. The patient was treated with dexamethasone and cyclosporine A (CsA). Intravenous immunogloblin (IVIG) and topoisomerase II inhibitor etoposide (VP16) were also applied. The patient was improved. After exclusion those without full medical history and complete data 49 cases in detailed reports were enrolled, including 39 females and 10 males. The mean age was (34.8±13.4) years. Clinical manifestations of HPS secondary to AOSD were recurrently fever, lymphadenopathy and hepatosplenomegaly, and the characteristic of laboratory test are cytopenia, the increasingly serum ferritin (SF), liver injury and phagocytois found in bone marrow biopsy at the early stage of cytopenia. Glucocorticoids were used in 47 patients. The percentage of patients received IVIG and CsA were significantly higher than those who receiving VP16. Eleven patients died, 3 of whom died of central nervous system infection, and 5 deaths from multiple organ dysfunction syndrome (MODS) and the remaining 3 cases died of massive hemorrhage in the intestine, respiratory failure and acute interstitial myocarditis, respectively. Conclusion Intervention of HPS includes early recognition, treatment of glucocorticoid and immunosuppressant. VP16 has good effect on HPS, and it will improve outcome if there is a sustained drop in whole blood. The cases with MODS have poor prognosis.

Key words： Adult onset still′s disease(AOSD) Hemophagocytic syndrome(HPS) Multiple organ dysfunction syndrome(MODS) Etoposide(VP16)

Corresponding Authors: Yang Min, E-mail: 456ym@163.com

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Wang Min,Zheng Yao,Yang Min. Severe adult onset Still′s disease: a case report and literature review[J]. Chinese Journal of Critical Care Medicine, 2018, 38(8): 700-705.

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http://www.cnemergency.org.cn/EN/10.3969/j.issn.1002-1949.2018.08.012 OR http://www.cnemergency.org.cn/EN/Y2018/V38/I8/700

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