干燥综合症评分最终版

ESSPRI的实验设计内容
4个主要症状：干燥症状、疼痛、躯体疲劳、精神疲倦。每项分值 范围0——10分 患者认为最需要改善的一项症状
疲劳与不适问卷(PROFAD)
Sicca症状调查表(SSI) 金标准：患者整体评价（PGA） 0——10分
EULAR Sjogren's Syndrome Patient Reported Index (ESSPRI):development of a consensus patient index for primary Sjogren's syndrome.
现有pSS疾病评价标准分类：
1疾病活动度评分（ SSDAI 、 SCAI、 ESSDAI）
2患者主观评分(PROFAD、SICCA、ESSPRI)
3疾病损伤评分（SSDDI、SSDI）
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ESSPRI的产生
EULAR Sjogren's Syndrome Patient Reported Index
ESSPRI的实验结果
1.多变量回归分析证明，干燥症状、肢体痛、疲劳与PGA符合度好 ，但是精神疲倦与PGA符合度较差。 2.患者认为最需要改善的症状：干燥症状（ 38.7%）、疲劳（ 32.2% ）、肢体痛（19.6%），最不重要的为精神疲倦
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3.在干燥症状中，口干、眼干被认为最重要
ESSPRI的特性
• 1.完全由患者主观填表所得，与ESSDAI良好互补 • 2. 与PGA、PROFAD、SSI符合度好（r=0.70、 0.73、0.66)，用以评价患者症状的准确性较高。
• 3.大样本、多中心
• 4.简单易行，适用于临床
EULAR Sjogren's Syndrome Patient Reported Index (ESSPRI):development of a consensus patient index for primary Sjogren's syndrome. Ann Rheum Dis. 2011 Jun;70(6):968-72. Epub 2011 Feb 22.
SSI评分
(sicca symptoms inventory)
Validation of the Sicca Symptoms Inventory for clinical studies of Sjögren's syndrome. Bowman SJ, Booth DA, Platts RG, Field A, Rostron J; UK Sjögren's Interest Group. J Rheumatol. 2003 Jun;30(6):1259-66.
enlarged parotid (≤ 3cm), or limited submandibular or lachrymal swelling1 enlarged parotid (>3cm ) or important submandibular or lachrymal swelling1
Moderate activity Major glandular swelling with:
4.ESSPRI最终评分为干燥症状、疲乏、肢体痛三个积分的平均 值，范围为0——10分
EULAR Sjogren's syndrome disease activity index: development of a consensus systemic disease activity index for primary Sjogren's syndrome. Ann Rheum Dis. 2010 Jun;69(6):1103-9. Epub 2009 Jun 28. Erratum in: Ann Rheum Dis. 2011 May;70(5):880.
EULAR的疾病活动度评分标准 ESSDAI
EULAR Sjogren's syndrome disease activity index
Constitutional domain [3] Please be careful of not rating constitutional symptoms not related to the disease (such as fever of infectious origin, voluntary weight loss) No activity Low activity Absence of the following symptoms Mild or intermittent fever (37.5° -38.5° C) / night sweats Involuntary weight loss of 5 to 10% of body weight Moderate activity Severe Fever (>38.5° C) / night sweats Involuntary weight loss of >10% of body weight Lymphadenopathy domain [4] No activity Low activity Absence of the following features - Lymphadenopathy ≥ 1cm in any nodal region or ≥ 2cm in inguinal region
Distinction between limited and important submandibular or lachrymal swelling is left to the physician judgment
Articular domain [2] Please be careful of not rating articular involvement not related to the disease, such as osteoarthritis No activity Low activity Moderate activity High activity Absence of currently active articular involvement Arthralgias in hands, wrists, ankles and feet accompanied by morning stiffness (>30 min) 1 to 5 synovitis among a 28 count ≥ 6 synovitis among a 28 count
Cutaneous domain [3] Please be careful of rating as “No activity” stable long lasting features that are related to damage rather than disease activity, or cutaneous involvement not related to the disease No activity Low activity Moderate activity Absence of currently active cutaneous involvement Erythema multiforme Limited cutaneous vasculitis, including urticarial vasculitis 2, or purpura limited to feet and ankle, or subacute cutaneous lupus High activity Diffuse cutaneous vasculitis, including urticarial vasculitis2, or diffuse purpura or ulcers related to vasculitis
2008.12.4——2009.11.30 入组230名SS患者，来自欧洲、南北美洲12个国家 21名SS专家参与EULAR的国际合作项目
2011年2月研究结果online版首发发表于ARD
EULAR Sjogren's Syndrome Patient Reported Index (ESSPRI):development of a consensus patient index for primary Sjogren's syndrome. Ann Rheum Dis. 2011 Jun;70(6):968-72. Epub 2011 Feb 22.
干燥综合征的疾病评价
北京积水潭医院-伍沪生
SS以淋巴细胞增殖和进行性外分泌腺体损伤为特性的系统性疾病
临床特点： 温和但可能影响功能的症状：干燥症状、疼痛、疲乏，见于大多 患者 潜在系统性表现：见于20-40%的患者
核心指标： 症状（sicca特点、疲乏、健康相关的生活质量） 系统损害的活动性（复合的活动指标和实验室检测）
Pulmonary domain [5] Please be careful of rating as “No activity” stable long lasting features that are related to damage rather than disease activity, or respiratory involvement not related to the disease, (tobacco…) No activity Low activity Absence of currently active pulmonary involvement Persistent cough or bronchial involvement with no radiographic abnormalities on X-ray Or radiological or HRCT evidence of interstitial lung disease3 with: No breathlessness, And normal lung function test.
Glandular domain [2] Please be careful of not rating glandular swelling not related to the disease (such as stone or infection) No activity Low activity Absence of glandular swelling Small glandular swelling with: 1
2
Limited cutaneous vasculitis involve <18% body surface area; Diffuse Cutaneous vasculitis involve >18% body
surfaБайду номын сангаасe area
Body surface area (BSA) is defined using the rules of nines (used to assess extent of burns) as follows: Palm (excluding fingers) = 1% BSA; each lower limb = 18% BSA; each upper limb = 9% BSA; torso (front) = 18% BSA; torso (back) = 18% BSA
PROFAD评分
Measurement of fatigue and discomfort in primary Sjogren's syndrome using a new questionnaire tool. Rheumatology (Oxford). 2004 Jun;43(6):758-64. Epub 2004 Mar 23.
Moderate activity - Lymphadenopathy ≥ 2cm in any nodal region or ≥ 3cm in inguinal region, - and/or splenomegaly (clinically palpable or assessed by imaging) High activity - Current malignant B-cell proliferative disorder