李红霞, 王鹏宇, 周雅馨, 王亚飞, 吴振彪. 195例干燥综合征患者临床特点及唇腺病理特征分析[J]. 解放军医学院学报, 2021, 42(9): 900-905. DOI: 10.3969/j.issn.2095-5227.2021.09.002
引用本文: 李红霞, 王鹏宇, 周雅馨, 王亚飞, 吴振彪. 195例干燥综合征患者临床特点及唇腺病理特征分析[J]. 解放军医学院学报, 2021, 42(9): 900-905. DOI: 10.3969/j.issn.2095-5227.2021.09.002
LI Hongxia, WANG Pengyu, ZHOU Yaxin, WANG Yafei, WU Zhenbiao. Clinical and labial gland pathologic features of Sjogren’s syndrome: An analysis of 195 cases[J]. ACADEMIC JOURNAL OF CHINESE PLA MEDICAL SCHOOL, 2021, 42(9): 900-905. DOI: 10.3969/j.issn.2095-5227.2021.09.002
Citation: LI Hongxia, WANG Pengyu, ZHOU Yaxin, WANG Yafei, WU Zhenbiao. Clinical and labial gland pathologic features of Sjogren’s syndrome: An analysis of 195 cases[J]. ACADEMIC JOURNAL OF CHINESE PLA MEDICAL SCHOOL, 2021, 42(9): 900-905. DOI: 10.3969/j.issn.2095-5227.2021.09.002

195例干燥综合征患者临床特点及唇腺病理特征分析

Clinical and labial gland pathologic features of Sjogren’s syndrome: An analysis of 195 cases

  • 摘要:
      背景  干燥综合征(Sjogren’s syndrome,SS)以淋巴细胞增殖和进行性外分泌腺体损伤为特征,可伴有多系统损伤,现阶段缺乏针对门诊SS患者临床表现、自身抗体和唇腺病理特点相结合的研究。
      目的  分析干燥综合征患者的临床表现、自身抗体及唇腺病理特点,为了解门诊SS患者的疾病特点提供临床资料。
      方法  收集2020年6月- 2021年4月在空军军医大学第一附属医院门诊行唇腺活检的195例原发性干燥综合征(primary Sjogren’s syndrome,pSS)和继发性干燥综合征(secondary Sjogren’s syndrome,sSS)患者的临床及唇腺病理资料并进行分析。
      结果  195例中女183例,男12例,年龄20~85(49.7 ± 14.0)岁。1)166例pSS患者,血常规示白细胞减低和(或)血小板减低占30.3%,肺、皮肤、神经系统和肾受累分别占23%、16.3%、7.8%和6.6%。抗核抗体、抗SSA抗体、抗Ro-52抗体、抗SSB抗体阳性分别为98.2%、83.1%、83.1%和34.9%,所有抗SSB抗体阳性的患者抗SSA抗体均阳性。抗AMA-M2、抗nRNP/Sm、抗CENP-B和抗甲状腺相关抗体阳性分别为11.4%、10.2%、8.4%和39.5%。唇腺病理,灶性指数(focus score,FS) ≥ 1占44%,正常占13.9%,SSⅠ度/Ⅰ~ Ⅱ度占29.5%,12.7%为慢性涎腺炎。2)sSS患者29例,合并类风湿关节炎22例(75.9%),合并系统性红斑狼疮、系统性硬化症、混合性结缔组织病和大动脉炎分别为2例、2例、2例和1例。抗SSA抗体阳性占58.6%。3)pSS与sSS比较,口干、眼干、猖獗齿、腮腺肿大、Schirmer试验和未刺激唾液流率阳性无统计学差异(P均 > 0.05)。pSS患者抗SSA抗体阳性率高于sSS患者(83.1% vs 58.6%,P=0.002),唇腺病理,pSS患者FS ≥ 1较sSS患者增多(44.0% vs 24.1%, P=0.045),而SSⅠ度/Ⅰ~Ⅱ度较sSS减少(29.5% vs 51.7%,P=0.019),正常与慢性涎腺炎在两组间的差异均无统计学意义(P 均> 0.05)。
      结论  抗SSA抗体阳性和FS ≥ 1在pSS组中占比较高,抗SSB抗体阳性一般伴有抗SSA抗体阳性。在SS患者中抗AMA-M2、抗nRNP/Sm和抗CENP-B抗体阳性常见。

     

    Abstract:
      Background  Sjogren’s syndrome (SS) is characterized by lymphocyte proliferation and progressive exocrine gland damage, which can be accompanied by multi-system damage, but there is a lack of research on the combination of clinical manifestations, autoantibodies and labial gland pathological characteristics of outpatients with SS.
      Objective  To investigate the clinical manifestations, autoantibodies and labial gland pathology of patients with SS and provide clinical data for outpatients with SS.
      Methods  Clinical and pathological data of 195 patients with SS including primary Sjogren’s syndrome (pSS) and secondary Sjogren’s Syndrome (sSS) who all underwent labial biopsy were collected from outpatient clinic of the First Affiliated Hospital of Air Force Medical University from June 2020 to April 2021.
      Results  There were 183 females and 12 males aged 20-85 (49.7 ± 14.0) years. Of the 166 patients with pSS, 30.3% of patients had involvement of hematological system including leukopenia and/or thrombocytopenia. Involvements of lung, skin, nervous system and kidney were seen in 23%, 16.3%, 7.8% and 6.6% of the patients, respectively. Of these patients, 98.2% had positive ANA, and the antibody positivity rates of anti-SSA, anti-Ro52 and anti-SSB antibodies were 83.1%, 83.1%, 34.9%, respectively. All patients with positive anti-SSB antibody were positive for anti-SSA antibody. Anti-AMA-M2, anti-nRNP/Sm, anti-CENP-B and anti-thyroid-related antibodies were positive in 11.4%, 10.2%, 8.4% and 39.5% of the pSS patients, respectively. In labial gland pathology, FS ≥ 1, degree of SSⅠ/Ⅰ~Ⅱ, chronic sialadenitis and normal were seen in 44%, 29.5%, 13.9% and 12.7% of pSS patients, respectively. Of the 29 sSS patients, there were 22 (75.9%) cases with RA. The other cases were SLE (2 cases), SSc (2 cases), MCTD (2 cases) or Takayasu arteritis (1 case). The positive rate of anti-SSA was 58.6% in sSS. There were no differences in incidence rates of dry mouth, dry eye, rampant caries, parotid swelling, positive Schirmer test or abnormal unstimulated salivary flow rate between the pSS group and the sSS group (all P > 0.05). Compared with the sSS group, patients in the pSS group had higher rate of anti-SSA antibodies (83.1% vs 58.6%, P=0.002). In labial gland pathology examination, compared with the sSS group, the number of patients with FS ≥ 1 was significantly greater in the pSS group(44.0% vs 24.1%, P=0.045), while patients with degree of SSⅠ/Ⅰ~Ⅱ was significantly less (29.5% vs 51.7%, P=0.019), and there were no significant differences in the prevalence of chronic sialadenitis between the two groups (P > 0.05).
      Conclusion  The proportion of patients with positive anti-SSA antibody and FS ≥ 1 is higher in pSS as compared with sSS. Positive anti-SSB antibody generally accompanies positive anti-SSA antibody. Positive anti-AMA-M2, anti-nRNP/Sm and anti-CENP-B antibodies are common in SS patients.

     

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