Houghton Kristin, Malleson Peter, Cabral David, Petty Ross, Tucker Lori
Division of Rheumatology, Department of Pediatrics, University of British Columbia, British Columbia's Children's Hospital, Vancouver, Canada.
J Rheumatol. 2005 Nov;32(11):2225-32.
To compare the proposed criteria for the diagnosis of primary Sjögren's syndrome (pSS) in childhood to the validated American-European Consensus Group (AECG) classification criteria for pSS in adults.
Charts of 7 children with pSS seen at British Columbia's Children's Hospital (BCCH) and data on 128 children identified through Medline in the English language literature between 1963 and 2003 were reviewed for pediatric and AECG criteria for pSS. The presence of > or = 4 criteria was required to satisfy the respective classification criteria. The expert clinical opinion of pediatric rheumatologists was considered the gold standard for diagnosis.
A total of 24/62 (39%) cases satisfied the AECG criteria; 47/62 (76%) satisfied the proposed pediatric criteria. Inclusion of recurrent parotitis increased the sensitivity of the pediatric clinical criteria. From the cases, 78/133 (59%) satisfied the pediatric oral symptom criteria; only 6/78 (8%) had xerostomia in the absence of recurrent parotitis. There was no reported case of recurrent conjunctivitis in the absence of keratoconjunctivitis sicca. We found 101/130 (78%) cases had at least one positive autoantibody test result [antinuclear antibodies (ANA), rheumatoid factor (RF), SSA, SSB]; 78/123 (63%) had autoantibodies to SSA or SSB.
The AECG adult criteria for pSS should not be applied to children as the sensitivity is unacceptably low. The inclusion of recurrent parotitis increases the sensitivity of the pediatric criteria, and recurrent parotitis should alert the clinician to the possibility of pSS. The inclusion of recurrent conjunctivitis did not improve the sensitivity over the AECG ocular criteria. The addition of ANA and RF to the AECG criteria did not change the number of patients satisfying the criteria for pediatric pSS.
比较所提出的儿童原发性干燥综合征（pSS）诊断标准与已验证的成人pSS美国-欧洲共识小组（AECG）分类标准。
回顾了不列颠哥伦比亚儿童医院（BCCH）诊治的7例pSS患儿的病历，以及1963年至2003年间通过Medline在英文文献中确定的128例患儿的数据，以了解pSS的儿科标准和AECG标准。满足相应分类标准需要存在≥4条标准。儿科风湿病学家的专家临床意见被视为诊断的金标准。
共有24/62（39%）例符合AECG标准；47/62（76%）例符合所提出的儿科标准。纳入复发性腮腺炎提高了儿科临床标准的敏感性。在这些病例中，78/133（59%）例符合儿科口腔症状标准；在无复发性腮腺炎的情况下，只有6/78（8%）例有口干。在无干燥性角结膜炎的情况下，未报告复发性结膜炎病例。我们发现101/130（78%）例至少有一项自身抗体检测结果呈阳性[抗核抗体（ANA）、类风湿因子（RF）、SSA、SSB]；78/123（63%）例有抗SSA或抗SSB自身抗体。
pSS的AECG成人标准不适用于儿童，因为其敏感性低得令人无法接受。纳入复发性腮腺炎提高了儿科标准的敏感性，复发性腮腺炎应提醒临床医生注意pSS的可能性。纳入复发性结膜炎并未比AECG眼部标准提高敏感性。在AECG标准中增加ANA和RF并没有改变符合儿科pSS标准的患者数量。
