成人斯蒂尔病患者的巨噬细胞活化综合征：意大利临床和实验风湿病学研究组队列研究的生存率和预测因素分析。
Macrophage Activation Syndrome in Patients Affected by Adult-onset Still Disease: Analysis of Survival Rates and Predictive Factors in the Gruppo Italiano di Ricerca in Reumatologia Clinica e Sperimentale Cohort.
机构信息
From the Rheumatology Section, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila; Rheumatology Section, Department of Clinical and Experimental Medicine, Second University of Naples, Naples; Rheumatology Section, Department of Internal Medicine, University of Palermo, Palermo; Department of Experimental and Clinical Medicine, University of Florence, Florence; Department of Health Sciences, University of Catanzaro "Magna Graecia," Catanzaro; Systemic Rheumatic and Autoimmune Diseases Unit, Department of Biomedical Sciences and Human Oncology, University of Bari Medical School, Bari, Italy.
P. Ruscitti, MD, Rheumatology Section, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila; D. Iacono, MD, Rheumatology Section, Department of Clinical and Experimental Medicine, Second University of Naples; F. Ciccia, MD, PhD, Rheumatology Section, Department of Internal Medicine, University of Palermo; G. Emmi, MD, PhD, Department of Experimental and Clinical Medicine, University of Florence; P. Cipriani, MD, PhD, Rheumatology Section, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila; R.D. Grembiale, MD, Department of Health Sciences, University of Catanzaro "Magna Graecia"; F. Perosa, MD, PhD, Systemic Rheumatic and Autoimmune Diseases Unit, Department of Biomedical Sciences and Human Oncology, University of Bari Medical School; L. Emmi, MD, Systemic Rheumatic and Autoimmune Diseases Unit, Department of Biomedical Sciences and Human Oncology, University of Bari Medical School; G. Triolo, MD, Rheumatology Section, Department of Internal Medicine, University of Palermo; R. Giacomelli, MD, PhD, Rheumatology Section, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila; G. Valentini, MD, Rheumatology Section, Department of Clinical and Experimental Medicine, Second University of Naples.
出版信息
J Rheumatol. 2018 Jun;45(6):864-872. doi: 10.3899/jrheum.170955. Epub 2018 Apr 15.
OBJECTIVE
Macrophage activation syndrome (MAS) is a reactive form of hemophagocytic lymphohistiocytosis, which can complicate adult-onset Still disease (AOSD). We investigated AOSD clinical features at the time of diagnosis, to assess predictors of MAS occurrence. Further, we analyzed the outcomes of patients with AOSD who experience MAS.
METHODS
Patients with AOSD admitted to any Gruppo Italiano di Ricerca in Reumatologia Clinica e Sperimentale center were retrospectively analyzed for features typical of AOSD, MAS occurrence, and their survival rate.
RESULTS
Of 119 patients with AOSD, 17 experienced MAS (12 at admission and 5 during followup). Twelve patients with MAS at first admission differed from the remaining 107 in prevalence of lymphadenopathy and liver involvement at the time of diagnosis. In addition, serum ferritin levels and systemic score values were significantly higher in the patients presenting with MAS. At the time of diagnosis, the 5 patients who developed MAS differed from the remaining 102 in the prevalence of abdominal pain, and they showed increased systemic score values. In the multivariate analysis, lymphadenopathy (OR 7.22, 95% CI 1.49-34.97, p = 0.014) and abdominal pain (OR 4.36, 95% CI 1.24-15.39, p = 0.022) were predictive of MAS occurrence. Finally, MAS occurrence significantly reduced the survival rate of patients with AOSD (p < 0.0001).
CONCLUSION
MAS occurrence significantly reduced the survival rate in patients with AOSD. Patients with MAS at baseline presented an increased prevalence of lymphadenopathy and liver involvement, as well as high serum ferritin levels and systemic score values. The presence of lymphadenopathy and abdominal pain was associated with MAS occurrence.
目的
巨噬细胞活化综合征（MAS）是噬血细胞性淋巴组织细胞增生症的一种反应性形式，可并发成人Still 病（AOSD）。我们研究了 AOSD 诊断时的临床特征，以评估 MAS 发生的预测因素。此外，我们分析了发生 MAS 的 AOSD 患者的结局。
方法
回顾性分析了任何 Gruppo Italiano di Ricerca in Reumatologia Clinica e Sperimentale 中心收治的 AOSD 患者的特征，包括 AOSD 、MAS 发生及其生存率。
结果
119 例 AOSD 患者中，17 例发生 MAS（12 例为初诊时，5 例为随访时）。初诊时发生 MAS 的 12 例患者与其余 107 例患者相比，淋巴结病和肝脏受累的患病率较高。此外，MAS 患者的血清铁蛋白水平和全身评分值显著较高。初诊时发生 MAS 的 5 例患者与其余 102 例患者相比，腹痛的患病率较高，且全身评分值较高。多变量分析显示，淋巴结病（OR 7.22，95%CI 1.49-34.97，p = 0.014）和腹痛（OR 4.36，95%CI 1.24-15.39，p = 0.022）是 MAS 发生的预测因素。最后，MAS 的发生显著降低了 AOSD 患者的生存率（p < 0.0001）。
结论
MAS 的发生显著降低了 AOSD 患者的生存率。基线时发生 MAS 的患者淋巴结病和肝脏受累的患病率较高，血清铁蛋白水平和全身评分值较高。存在淋巴结病和腹痛与 MAS 发生相关。
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