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不同年齡段得狼瘡的差異

目前文獻報導示不同年齡發病的系統性紅斑狼瘡患者的的臨床表現及嚴重程度顯著不同。 大部分文獻均提示幼年發病的狼瘡臨床表現更重。 而報導老年患者的檔非常有限, 且沒有大的研究觀察所有的年齡組。

我們通過分析英國2個大的佇列資料, 評估發病的年齡在臨床表現上發揮的作用。

總共比較的人數為924人, 413例幼年發病的狼瘡患者, 511例成人發病的狼瘡患者。 女性患者數量優勢明顯。 隨著年齡的增長, 關節炎發病率更普遍(93%vs72%)。 而腎病(44%vs33%)、脫髮(47%vs23%)、口腔潰瘍(39%vs26%)等更常見於青少年狼瘡患者。 神經精神狼瘡在成人發病的狼瘡患者中發病率低。

幼年發病的狼瘡患者更容易出現血小板減少(21%vs15%)、溶血性貧血(20%vs3%)、抗ds-DNA抗體(71%vs63%)、抗Sm抗體(22%vs16%)、抗RNP抗體(36%vs29%)。 白細胞減少症隨著發病年齡增大逐漸增多。 近幾十年來, 狼瘡死亡率一直在下降。 然而, 標準化死亡比:幼年發病狼瘡18.3:成人發病狼瘡3.1。

結論:

這些大的資料對幼年及成人發病狼瘡患者的直接比對提示:幼年發病的狼瘡患者更容易出現預後不良的、進展性的臨床表現, 強調對這一人群密切隨訪的必要性。

Differencesin disease phenotype and severity in SLE across age groups

Ambrose N1, Morgan TA2, Galloway J3 Lupus. 2016May 4. pii: 0961203316644333

Abstract

OBJECTIVES:

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.

METHODS:

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).

RESULTS:

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.

CONCLUSION:

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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