Differences in disease phenotype and severity in SLE across age groups.
Differences in disease phenotype and severity in SLE across age groups.
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DOI：
10.1177/0961203316644333
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发表时间：
2016-12
期刊：
影响因子：
2.6
通讯作者：
UK JSLE Study Group
中科院分区：
文献类型：
--
作者：
Ambrose N;Morgan TA;Galloway J;Ionnoau Y;Beresford MW;Isenberg DA;UK JSLE Study Group
据报道，不同年龄段的系统性红斑狼疮(SLE)的疾病表型和严重程度存在显著差异。多数提示幼年型系统性红斑狼疮(JSLE)有更严重的表型。在老年患者中进行的研究有限，而且没有针对所有年龄段的SLE的大型研究。我们通过分析两个大型英国队列(英国JSLE队列和UCLH SLE队列)的数据，评估了SLE发病年龄对临床表型的影响。共有924名个体进行了比较(413名JSLE，511名成人发病的SLE)。女性占多数，但在年龄谱的两端都不太明显。随着年龄的增长，关节炎更常见(93%比72%，p <； 0.001)，而肾脏疾病(44%比33%，p = 0.001)、脱发(47%比23%，p &l； 0.001)和口疮(39%比26%，p = 0.001)在年轻人中更常见。神经精神性狼疮在成熟起病的系统性红斑狼疮中较少见(p <； 0.01)。JSLE与血小板减少(21%比15%，p = 0.01)、溶血性贫血(20%比3%，p <； 0.001)、高抗ds DNA(71%比63%，p = 0.009)、Sm(22%比16%，p = 0.02)和RNP(36%比29%，p <； 0.04)自身抗体有关。白细胞减少症随年龄增长而增加(p <； 0.001)。近几十年来，死亡率一直在下降。然而，死亡率大大高于一般人口。标化死亡率JSLE为18.3，成人型SLE为3.1。这些数据来自有史以来对JSLE和成人起病的SLE进行的最大规模的直接比较，表明JSLE患者具有侵袭性的疾病表型，结果更差，并强调了在这一人群中仔细随访的重要性。
Significant differences have been reported in disease phenotype and severity of systemic lupus erythematosus (SLE) presenting in different age groups. Most indicate a more severe phenotype in juvenile-onset SLE (JSLE). There have been limited studies in older patients and no large studies looking at SLE across all age groups. We assessed the effect of age of onset of SLE on the clinical phenotype by analysing data from two large UK cohorts (the UK JSLE Cohort and the UCLH SLE cohort). A total of 924 individuals were compared (413 JSLE, 511 adult-onset SLE). A female preponderance was present, but less pronounced at either end of the age spectrum. Arthritis was more common with advancing age (93% vs 72%, p < 0.001), whereas renal disease (44% vs 33%, p = 0.001), alopecia (47% vs 23%, p < 0.001) and aphthous ulcerations (39% vs 26%, p = 0.001) were more common in the young. Neuropsychiatric lupus was less common in mature-onset SLE (p < 0.01). JSLE was associated more commonly with thrombocytopenia (21% vs 15%, p = 0.01), haemolytic anaemia (20% vs 3%, p < 0.001), high anti-dsDNA (71% vs 63%, p = 0.009), Sm (22% vs 16%, p = 0.02) and RNP (36% vs 29%, p < 0.04) auto-antibodies. Leucopenia increased with advancing age (p < 0.001). Mortality has been declining over recent decades. However, death rates were substantially higher than the general population. The standardized mortality ratio was 18.3 in JSLE and 3.1 in adult-onset SLE. These data from the largest-ever direct comparison of JSLE with adult-onset SLE suggest an aggressive phenotype of disease with a worse outcome in patients with JSLE and emphasizes the importance of careful follow-up in this population.
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影响因子：
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作者：
Petri, Michelle;Orbai, Ana-Maria;Alarcon, Graciela S.;Gordon, Caroline;Merrill, Joan T.;Fortin, Paul R.;Bruce, Ian N.;Isenberg, David;Wallace, Daniel J.;Nived, Ola;Sturfelt, Gunnar;Ramsey-Goldman, Rosalind;Bae, Sang-Cheol;Hanly, John G.;Sanchez-Guerrero, Jorge;Clarke, Ann;Aranow, Cynthia;Manzi, Susan;Urowitz, Murray;Gladman, Dafna;Kalunian, Kenneth;Costner, Melissa;Werth, Victoria P.;Zoma, Asad;Bernatsky, Sasha;Ruiz-Irastorza, Guillermo;Khamashta, Munther A.;Jacobsen, Soren;Buyon, Jill P.;Maddison, Peter;Dooley, Mary Anne;van vollenhoven, Ronald F.;Ginzler, Ellen;Stoll, Thomas;Peschken, Christine;Jorizzo, Joseph L.;Callen, Jeffrey P.;Lim, S. Sam;Fessler, Barri J.;Inanc, Murat;Kamen, Diane L.;Rahman, Anisur;Steinsson, Kristjan;Franks, Andrew G., Jr.;Sigler, Lisa;Hameed, Suhail;Fang, Hong;Ngoc Pham;Brey, Robin;Weisman, Michael H.;McGwin, Gerald, Jr.;Magder, Laurence S.
通讯作者：
Magder, Laurence S.
影响因子：
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作者：
Brunner, Hermine I.;Gladman, Dafna D.;Silverman, Earl D.
通讯作者：
Silverman, Earl D.
影响因子：
27.4
作者：
Richer, O.;Ulinski, T.;Bader-Meunier, B.
通讯作者：
Bader-Meunier, B.
影响因子：
2.6
作者：
Morgan, T. A.;Watson, L.;Beresford, M. W.
通讯作者：
Beresford, M. W.
影响因子：
27.4
作者：
Webb R;Kelly JA;Somers EC;Hughes T;Kaufman KM;Sanchez E;Nath SK;Bruner G;Alarcón-Riquelme ME;Gilkeson GS;Kamen DL;Richardson BC;Harley JB;Sawalha AH
通讯作者：
Sawalha AH