Journal of Prevention and Treatment for Stomatological Diseases ›› 2019, Vol. 27 ›› Issue (5): 273-279.doi: 10.12016/j.issn.2096-1456.2019.05.001

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Evolution of diagnostic criteria for Sjögren′s Syndrome

TAO Qian(),LIANG Peisheng   

  1. Hospital of Stomatology, Guanghua School of Stomatology, Sun Yat-sen University, Guangdong Provincial Key Laboratory of Stomatology, Guangzhou 510055, China
  • Received:2018-11-04 Revised:2018-12-04 Online:2019-05-21 Published:2019-05-20

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Abstract:

Sj?gren′s syndrome lacks a single and objective diagnostic index, and its diagnosis often requires comprehensive analysis according to classification criteria. With increasing research and a deepening understanding of the disease, the classification criteria are constantly being adjusted and revised. The sensitivity and specificity of the diagnosis have gradually improved, which has the value of diagnostic criteria largely. Since 1965, more than ten diagnostic criterias for Sj?gren′s syndrome have been published. After debate and experience, international criteria with wide applicability have gradually replaced regional criteria. In the evolution of the diagnostic criteria, the diagnostic rules and evaluation methods have become more concise and unified. Techniques with poor specificity and operative difficulty, which are not easy to popularize or apply in the clinic, have gradually been eliminated. A diagnostic system based on an objective examination and subjective symptoms has gradually formed, and lip gland biopsy and serum autoantibody sampling have demonstrated great diagnostic value. The optimization of diagnostic rules makes the diagnosis of this disease more efficient and scientific. This paper reviews the characteristics of and differences in diagnostic criteria for Sj?gren′s syndrome and analyzes the internal logic of the evolution of different diagnostic criteria in light of development in research on the disease with the hope of benefitting the diagnosis, treatment and related research of Sj?gren′s syndrome.

Key words: Sicca syndrome, Sj?gren′s syndrome, Primary Sj?gren′s syndrome, Secondary Sj?gren′s syndrome, Ocular staining score, Salivary flow rate, Diagnosis criteria, Classification criteria

CLC Number: 

  • R781.7

Common international classification criteria 1)"

项目 欧洲标准,1993[13] AECG标准,2002[14] ACR标准,2012[15] ACR/EULAR标准,2016[16]
Ⅰ眼部症状 有 有 无 无1）
Ⅱ口腔症状 有 有 无 无1）
Ⅲ眼部体征 Schirmer试验Ⅰ ≤ 5 mm/5 min ≤ 5 mm/5 min2） 无 ≤ 5 mm/5 min（1分）
角结膜染色 VBS ≥ 4 VBS ≥ 4 OSS ≥ 53） OSS ≥ 5或VBS ≥ 4（1分）
Ⅳ组织学检查 FS ≥ 1 FS ≥ 1 FS ≥ 1 FS ≥ 1（3分）
Ⅴ唾液腺检查 静息相唾液流率 ≤ 1.5 mL/15 min ≤ 1.5 mL/15 min2） 无 ≤ 0.5 mL/5 min（1分）
腮腺造影 有 有 无 无
核素扫描 有 有 无 无
Ⅵ血清学检查 抗SSA抗体和（或）
抗SSB抗体阳性		 抗SSA抗体和（或）
抗SSB抗体阳性		 [抗SSA抗体和（或）
抗SSB抗体阳性]
或[RF和ANA≥1：320]		 抗SSA抗体阳性（3分）
pSS诊断要求 6项至少符合4项 6项至少符合4项,
必须包括Ⅳ或Ⅵ项		 Ⅲ、Ⅳ、Ⅵ 3项中至少
符合2项		 赋值总分≥4分
sSS诊断要求 Ⅰ或Ⅱ符合1项,Ⅲ、Ⅳ、
Ⅴ3项中至少符合2项		 Ⅰ或Ⅱ符合1项,Ⅲ、Ⅳ、
Ⅴ3项中至少符合2项		 不适用于sSS

Table 2

Evolution of exclusion criteria"

分类（诊断）标准 排除标准
Bloch标准[5] 其他已知原因引起的唾液腺及泪腺疾病
圣地亚哥标准[8] 结节病、已存在的淋巴瘤、获得性免疫缺陷综合征及其他已知原因引起的角膜干燥或唾液腺肿大
欧洲标准[13] 结节病、已存在的淋巴瘤、获得性免疫缺陷综合征、移植物抗宿主病、服用抗乙酰胆碱药、服用抗抑郁药、服用降压药、服用抗精神病药
AECG标准[14] 既往头颈部放疗史、丙肝病毒感染、结节病、已存在的淋巴瘤、获得性免疫缺陷综合征、移植物抗宿主病、服用抗胆碱能药物（未达到药物半衰期4倍）
ACR标准[15] 既往头颈部放疗史、丙肝病毒感染、结节病、获得性免疫缺陷综合征、移植物抗宿主病、淀粉样变、IgG4相关性疾病
ACR/EULAR标准[16] 既往头颈部放疗史、活动性丙肝病毒感染（经PCR检查确认）、结节病、获得性免疫缺陷综合征、移植物抗宿主病、淀粉样变、IgG4相关性疾病

Figure 1

Parotid gland angiography based on three-dimensional CBCT reconstruction"

Figure 2

Saliva collection and flow rate measurement device for large salivary glands"

Table 3

Classification criteria for Sj?gren’s syndrome（AECG,2002）"

项目 内容
Ⅰ.口腔症状 3项中有1项或1项以上：①每日感口干持续3个月以上;②成年后腮腺反复或持续肿大;③吞咽干性食物时需用水帮助
Ⅱ.眼部症状 3项中有1项或1项以上：①每日感到不能忍受的眼干持续3个月以上;②有反复的砂子进眼或砂磨感觉;③每日需用人工泪液 3 次或 3 次以上
Ⅲ.眼部体征 下述检查任1项或1项以上阳性：①Schirmer试验（+）（≤5 mm/5 min）;②角膜染色（+）（≥4 VBS）
Ⅳ.组织学检查 下唇腺病理活检示淋巴细胞灶≥1
Ⅴ.涎腺受损 下述检查任1项或1项以上阳性：①唾液流率≤1.5 mL/15 min;②腮腺造影（+）;③涎腺同位素检查（+）
Ⅵ.自身抗体 抗SSA抗体或抗SSB抗体阳性（双扩散法）
具体分类 pSS 无任何潜在疾病的情况下,符合有下述任1条则可诊断：①符合4条或4条以上,但必须含有条目Ⅳ（组织学检查）和/或条目（自身抗体）;②条目Ⅲ、Ⅳ、Ⅴ、Ⅵ4 条中任3条阳性6项至少符合4项,必须包括Ⅳ或Ⅵ项
sSS 患者有潜在的疾病（如任一结缔组织病）,而符合Ⅰ和Ⅱ中任1条,同时符合条目Ⅲ、Ⅳ、Ⅴ中任2条
必须除外 颈头面部放疗,丙型肝炎病毒感染,获得性免疫缺陷综合征,淋巴瘤,结节病,移植物抗宿主病,抗乙酰胆碱药的应用（如阿托品 、莨菪碱 、溴丙胺太林、颠茄等）
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