SJOGRENS-SYNDROME - PROPOSED CRITERIA FOR CLASSIFICATION
SJOGRENS-SYNDROME - PROPOSED CRITERIA FOR CLASSIFICATION
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DOI：
10.1002/art.1780290501
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发表时间：
1986-05-01
影响因子：
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通讯作者：
HOWELL, FV
中科院分区：
文献类型：
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作者：
FOX, RI;ROBINSON, CA;HOWELL, FV
干燥综合征（SS）常用于描述与自身免疫性疾病相关的角结膜干燥症和口干症。然而，尚未建立明确的SS分类标准，这种诊断适用于广泛的疾病，从一些患者的明确的“自身免疫性”疾病，到老年患者无全身免疫过程证据的干燥症。在这里，我们回顾了临床和实验室特征的病人提到评估干燥症状。特别是，我们强调在选择小涎腺活检部位时需要注意，我们描述了有助于评估这些活检标本的组织学特征。在试图确定一个患者群体的条件可能有一个共同的发病机制，因此，一个共同的治疗方案，我们提出了以下标准的诊断SS：1）客观证据的角膜结膜干燥，如记录的玫瑰红或荧光素染料染色; 2）客观证据的唾液腺流量减少; 3）小涎腺活检，通过正常粘膜获得，标本至少含有4个可评价的唾液腺小叶，平均至少2个病灶/4 mm 2; 4）系统性自身免疫过程的证据，如自身抗体如类风湿因子和/或抗核抗体的存在所表现的。当满足所有4项标准时，将诊断为“明确SS”;当存在3项标准时，将诊断为“可能SS”。此诊断的特定排除是既存淋巴瘤、移植物抗宿主病、结节病和获得性免疫缺陷病。这些建议的标准比目前用于诊断SS的标准更具限制性。未来的多中心试验将需要确定这些标准是否普遍有用。最后，我们强调流变学专家在选择安全、廉价的方法客观监测干燥症（即干燥性角结膜炎和腮腺唾液流）和评估小唾液腺活检结果方面的作用。
The term “Sjögren's syndrome”(SS) is frequently used to describe the occurrence of keratocon-junctivis sicca and xerostomia in association with an autoimmune disorder. However, well-defined criteria for the classification of SS have not been established, and this diagnosis is being applied to a wide spectrum of conditions, ranging from clear “autoimmune” disease in some patients, to sicca complaints without evidence of a systemic immune process in elderly patients. Here, we review the clinical and laboratory features of patients referred for evaluation of sicca symptoms. In particular, we emphasize the need for care in choosing the site for minor salivary gland biopsy, and we describe the histologic features that aid in the evaluation of these biopsy specimens. In an attempt to identify a population of patients whose conditions might have a common etiopathogenesis and, thus, a common treatment program, we propose the following criteria for a diagnosis of SS: 1) objective evidence of keratoconjunctivis sicca, as documented by rose bengal or fluorescein dye staining; 2) objective evidence of diminished salivary gland flow; 3) minor salivary gland biopsy, obtained through normal mucosa, with the specimen containing at least 4 evaluable salivary gland lobules, and having an average of at least 2 foci/4 mm 2; 4) evidence of a systemic autoimmune process, as manifested by the presence of autoantibodies, such as rheumatoid factor and/or anti-nuclear antibody. The diagnosis of “definite SS” would be made when all 4 criteria are met; the diagnosis of “possible SS” would be made when 3 criteria are present. Specific exclusions for this diagnosis are preexisting lymphoma, graft-versus-host disease, sarcoidosis, and acquired immunodeficiency disease. These proposed criteria are more restrictive than are those currently used for diagnosing SS. Future multicenter trials will be required to determine whether these criteria are generally useful. Finally, we emphasize the rheumatologist's role in selecting safe, inexpensive methods of objectively monitoring sicca complaints (ie, kerato-conjunctivitis sicca and parotid salivary flow) and in evaluating the results of minor salivary gland biopsy.