Morán Álvarez Patricia, Bracaglia Claudia, Nicolai Rebecca, Giovannelli Luigi, Caiello Ivan, Boni Alessandra, Matteo Valentina, Moneta Gian Marco, Messia Virginia, De Benedetti Fabrizio, Marasco Emiliano
Division of Rheumatology, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.
Laboratory of Autoimmunity, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.
Lupus. 2024 Dec;33(14):1594-1604. doi: 10.1177/09612033241298729. Epub 2024 Nov 4.
to investigate the trend of autoantibody titers during a 2-year follow-up in pediatric systemic lupus erythematosus (pSLE) and pediatric Sjögren's syndrome (pSS).
Autoantibodies testing was performed every 3-4 months during 2 years from disease onset in a cohort of children with pSLE and pSS.
We enrolled 21 children with pSLE and 22 children with pSS. All pSLE patients at 2 years showed ANA titers significantly lower compared to disease onset. Eleven patients (73%) were still ANA positive at 2 years, while 4 (26%) became ANA negative. At diagnosis, 12 (80%) patients showed a homogeneous pattern, while 3 (20%) patients showed a speckled pattern. The latter remained ANA positive with the same pattern; only 2 patients with a homogenous pattern converted to speckled, 4 patients with a homogeneous pattern became ANA negative. ANA negative pSLE patients showed lower levels of interferon score compared to ANA positive patients. Anti-dsDNA titers declined equally in the two groups. All patients with pSS, at disease onset, were ANA and anti-Ro positive and 14 (66%) were anti-La positive. After 2 years of follow-up, 100% remained ANA positive but showed significant lower titers. During follow-up anti-Ro and anti-La titers remained stable.
different patterns in changes of ANA and ENA titers in pSLE and pSS were shown. At 2 years of follow-up, all pSLE patients had a lower ANA titer and 26% became negative; however, all pSS patients remained both ANA and ENA positive. This evidence may be due to different pathogenetic pathways in SLE and pSS.
研究儿童系统性红斑狼疮(pSLE)和儿童干燥综合征(pSS)患者在2年随访期间自身抗体滴度的变化趋势。
对一组pSLE和pSS患儿自疾病发作起2年内每3 - 4个月进行一次自身抗体检测。
我们纳入了21例pSLE患儿和22例pSS患儿。所有pSLE患者在2年时的抗核抗体(ANA)滴度均显著低于疾病发作时。11例患者(73%)在2年时ANA仍为阳性,而4例(26%)ANA转为阴性。诊断时,12例(80%)患者表现为均质型,3例(20%)患者表现为斑点型。后者ANA仍为阳性且模式相同;仅2例均质型患者转为斑点型,4例均质型患者ANA转为阴性。与ANA阳性患者相比,ANA阴性的pSLE患者干扰素评分较低。两组抗双链DNA(dsDNA)滴度均同样下降。所有pSS患者在疾病发作时ANA和抗Ro均为阳性,14例(66%)抗La为阳性。经过2年随访,100%患者ANA仍为阳性,但滴度显著降低。随访期间抗Ro和抗La滴度保持稳定。
pSLE和pSS患者ANA和可提取核抗原(ENA)滴度变化模式不同。随访2年时,所有pSLE患者ANA滴度降低,26%转为阴性;然而,所有pSS患者ANA和ENA均仍为阳性。这一证据可能归因于SLE和pSS不同的发病机制。