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原文題目(出處出處出處出處)::: The role of a labial salivary gland biopsy in the diagnostic procedure for Sjögren’s syndrome; a study of 94 cases 原文作者姓名

原文作者姓名 原文作者姓名

原文作者姓名::: Dewi van Stein-Callenfels , Jonathan Tan , Elisabeth Bloemena , Richard M. van Vugt , Alexandre E. Voskuyl, Nathalie T.Y. Santana , Isaäc van der Waal

通訊作者學校 通訊作者學校 通訊作者學校

通訊作者學校::VU University Medical Center 報告者姓名

報告者姓名 報告者姓名

報告者姓名(組別組別組別組別):::: 簡瑜文簡瑜文簡瑜文簡瑜文 (B 組組組組) 報告日期

報告日期 報告日期

報告日期::103.09.09 內文

內文 內文 內文:::

Introduction

Sjögren’s syndrome:

Sjögren’s syndrome (SS) is a multiorgan, chronic autoimmune disease, primarily directed against exocrine glands(salivary and lacrimal glands)

1. Symptoms & signs:

Dry mouth(Xerostomia),dry eyes(xerophthalimia) 2. Complication:

Dry mouth  angular cheilitis, dental decay (cervical caries) Salivary gland enlargement  retrograde bacterial sialadenitis Fatigue, arthritis, kidney failure, non-Hodgkin lymphoma 3. Population prevalence: 0.5-1%

4. Male-to-Female rate : 1:9 5. Aged: Middle-aged adult

6. Primary & secondary Sjögren’s syndrome

Classification Explanation

Primary no other autoimmune disorder

Secondary other associated autoimmune disease, ex rheumatoid arthritis

(2)

7. Classification criteria: American-European Consensus Group (AECG), at least 4

Symptoms & signs Explanation

Ocular symptoms Dry eyes

Oral symptoms Dry mouth

Ocular signs Schirmer I test, Rose Bengal score

Histopathology Minor salivary glands, lymphocytes foci

Salivary gland involvement

Serologic findings Serum,Anti- Ro(SS-A),Anti-La(SS-B)

8. Labial salivary gland biopsy (LSGB):

a. Site: usually lower lip, normal labial mucosa

b. Dimension: 1.5~2.0 cm incision, ≧5 accessory glands c. Histopathology:

i. Focal chronic inflammatory aggregates

ii. ≧50 lymphocytes and plasma cells within 4-mm2 area iii. Lymphocytes focus adjacent to normal-appearing acini iv. Consistent in most of glands

Not 100% reliable

Purpose of present study:

Examine the role of the outcome of the LSGB in the diagnostic procedure

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Material and Methods

1. In the files of the department of pathology of the VU university medical center, Amsterdam, the Netherlands, 139 labial salivary gland biopsies (LSGBs) could be retrieved in the period between 2000 and 2010.

2. Out of these 139 cases, 45 patients have been excluded because of incomplete data.



94 patients have been included in the study

3. 74 females and 20 males

4. Mean age at the time of biopsy was 50 years (range 21 to 79 years old).

5. The histopathological assessment of the presence of lymphocytic foci in the labial salivary gland biopsies has been performed in a quantitative way

6. Statistical analyses: SPSS 21.0 for Windows, Pierson’s chisquared test

Result

1. Sensitivity of the labial biopsy : 0.92 2. Specificity of the labial biopsy : 0.91

Positive predictive value : 0.80

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1. Primary SS (n=20), secondary SS (n=2), 4 patients no reliable data to allow a distinction between primary and secondary SS.

2. 20 patients diagnosed with primary Sjögren’s syndrome:

a. Positive LSGB (n=19)  80% positive serology (n=16) b. Positive serology (n=16)  1 negative LSGB

c. Negative serology (n=4)  3 positive LSGB

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Discussion and Conclusion

1. The present study has been undertaken for two reasons:

1st : examine in what way labial biopsies taken in our Institution in a 10-year period had contributed to the assessment of a diagnosis of Sjögren’s syndrome

2nd : explore the influence of the recently proposed American College of

Rheumatology(ACR,2012) criteria with regard to the role of the LSGB

2. 2% of patients experience long term

postoperative complaints of numbness or

hyperaesthesia at the site of the biopsy

3. Table 3.It seems advisable to perform LSGBs only after counseling of the patients by the departments of Rheumatology or Internal Medicine.

4. Table 1.

high specificity(0.91) and a high sensitivity(0.92) of the LSGB with

regard to the presence of SS

5. In postmortem studies

older age was associated with high false-positive rates of

LSGB. In view of a mean age of 50 years at the time of the biopsy, age does not

seem to have a major influence on the present results.

6. In the past, it has been suggested that assessment of the various percentages of

immunoglobulinsin the plasma cells can be a criteria for the diagnosis of

Sjögren’s syndrome. However, no other studies have been published and they have not been incorporated in the AECG classification nor in the ACR

classification.

7. American College of Rheumatology(ACR, 2012) criteria, at least 2

Symptoms & signs Explanation

Serologic findings positive serum anti-SSA and/or anti-SSB Ocular signs ocular staining score >3

Histopathology Minor salivary glands, lymphocytes foci

No significant difference between the diagnostic value of AECG and ACR 8. ACR classification

a. Serologic(+) and ocular test(+)  LSGB redundant

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題號 題號 題號

題號 題目 題目 題目 題目

1

何者非何者非何者非何者非 Sjögren’s syndrome 常見的常見的常見的症狀常見的症狀症狀? 症狀

(A) Xerostomia(dry mouth)

(B) Xerophthalimia (dry eyes) (C) Parotid gland enlargement (D) Otitis media

答案答案 答案答案

(D)

出處出處

出處出處::::Oral and maxillofacial pathology, 3rd 2009

Sjögren’s syndrome is a chronic, systemic autoimmune disorder involves salivary and lacrimal glands resulting in xerostomia(dry mouth), xerophthalimia (dry eyes)

Fig. 11-25 Sjögren’s syndrome. Benign lymphoepithelial lesion of the parotid gland.

題號 題號 題號

題號 題目 題目 題目 題目

2

下列何者非下列何者非下列何者非下列何者非 Americam-European Consensus Group (AECG)定義定義定義診斷定義診斷診斷診斷

Sjögren’s syndrome 的

的的的 criteria 的項目的項目的項目的項目?

(A) Oral symptoms

(B) Labial salivary gland biopsy

(C) Immunohistochemical assessment of immunoglobulins in the plasma cells

(D) Ocular signs

答案答案

答案答案

(C)

出處出處

出處出處::::Oral and maxillofacial pathology, 3rd 2009

P468, Box 11-2

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