Clinical Manifestations but not Cytokine Profiles Differentiate Adult-onset Still's Disease and Sepsis
Clinical Manifestations but not Cytokine Profiles Differentiate Adult-onset Still's Disease and Sepsis
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DOI：
10.3899/jrheum.100247
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发表时间：
2010-11-01
影响因子：
3.9
通讯作者：
Blank, Norbert
中科院分区：
文献类型：
--
作者：
Rau, Monika;Schiller, Martin;Blank, Norbert
目标。分析成人斯蒂尔病(AOSD)和细菌性脓毒症患者的临床表现、血清铁蛋白和血清细胞因子水平，评价其在鉴别诊断中的潜在价值。对22例首次发病的AOSD患者和6例免疫抑制治疗确诊的AOSD患者与14例细菌性脓毒症患者进行比较。临床表现采用Pouchot AOSD活动度评分，包括血清铁蛋白水平升高，以获得修正的Pouchot评分。对每个患者的血清细胞因子谱进行分析。未经治疗的活动期AOSD患者的临床表现评分(平均5.6±1.93)显著高于慢性AOSD患者(平均1.16±-0.98p<；0.001)和脓毒症患者(平均2.38±1.19；p<；0.001)。改良Pouchot评分=4对活动期AOSD的敏感性为92%，特异性为93%。急性AOSD和脓毒症患者血清IL-1β、IL-6、IL-8、IL-10、IL-12、IL-18、干扰素-γ、肿瘤坏死因子-α和钙保护素水平升高。仅在IL-6和IL-8水平较高的脓毒症患者中检测到显著差异。两组的重叠限制了细胞因子在个体患者鉴别诊断中的使用。与脓毒症患者相比，包括血清铁蛋白水平升高的改良Pouchot AOSD活动评分更有助于确认AOSD的诊断。血清细胞因子升高与炎症相关，但在区分活动性AOSD和细菌性脓毒症方面作用有限。(2010年9月1日首次发布；J Rheumatol 2010；37：2369-76；DOI：10.3899/jhurum.100247)
Objective. To analyze clinical manifestations, serum ferritin, and serum cytokine levels in patients with adult-onset Still's disease (AOSD) or bacterial sepsis and to evaluate their potential use for differential diagnosis.Methods. Twenty-two consecutive patients with the first flare of AOSD and 6 patients with an established diagnosis of AOSD under immunosuppressive therapy were compared with 14 patients with bacterial sepsis. Clinical manifestations were scored in a Pouchot AOSD activity score including elevated serum ferritin levels to obtain a modified Pouchot score. Serum cytokine profiles were analyzed from each patient.Results. The scores of clinical manifestations using a modified Pouchot activity score were significantly higher in patients with active untreated AOSD (mean 5.60 +/- 1.93) compared with patients with chronic AOSD (mean 1.16 +/- 0.98; p < 0.001) and patients with sepsis (mean 2.38 +/- 1.19; p < 0.001). A modified Pouchot score >= 4 shows a sensitivity of 92% and a specificity of 93% for active AOSD. Serum cytokine levels of interleuk in 1 beta (IL-1 beta), IL-6, IL-8, IL-10, IL-12, IL-18, interferon-gamma, tumor necrosis factor-alpha, and calprotectin were elevated in acute AOSD and sepsis. Significant differences were detected only in patients with sepsis who had higher levels of IL-6 and IL-8. The overlap of the 2 groups limits the use of cytokines for differential diagnosis in individual patients.Conclusion. A modified Pouchot AOSD activity score including elevated serum ferritin levels was more useful to confirm the diagnosis of AOSD compared to patients with sepsis. Elevated serum cytokines correlate with inflammation but are of limited use to differentiate between active AOSD and bacterial sepsis. (First Release September 1 2010; J Rheumatol 2010;37:2369-76; doi:10.3899/jrheum.100247)