PDF(Pubmed)
Abstract：
BACKGROUND: Sjögren\'s Syndrome (SS) is an inflammatory autoimmune disease, and Mikulicz\'s disease (MD) is a lymphoproliferative disorder. Both MD and SS are more common in middle-aged female, and the dry eyes could be presented in both of them with different degree. The MD patients are characterized by symmetrical swelling of the lacrimal glands which also can occur in the early stage of SS. And the imaging findings between early stage of SS and MD are lack of specificity. Therefore, SS and MD have similarities in the clinical manifestations, imaging and pathological findings and are confused in diagnosis.
METHODS: A 51-year-old female patient presented with bilateral swelling of the upper eyelids for 2 years. She also reported having dry mouth and dry eyes which could be tolerated. The Schirmer\'s test result is positive and the laboratory tests indicate serum total IgG increased. In the bilateral lacrimal gland area could palpate soft masses. The orbital magnetic resonance imaging (MRI) examination showed bilateral lacrimal gland prolapse. While the histopathological result was considered as MD. The immunohistochemical (IHC) staining results were positive for IgG and negative for IgG4. To clarify the diagnosis, SS-related laboratory tests were performed: anti-SSA antibody (+++), anti-SSB antibody (+++), anti-Ro-52 antibody (+++). With a comprehensive consideration, the final diagnosis was SS.
CONCLUSIONS: When the clinical manifestations are atypical, it is necessary to pay attention to the differential diagnosis of SS and MD.
METHODS: A 51-year-old female patient presented with bilateral swelling of the upper eyelids for 2 years. She also reported having dry mouth and dry eyes which could be tolerated. The Schirmer\'s test result is positive and the laboratory tests indicate serum total IgG increased. In the bilateral lacrimal gland area could palpate soft masses. The orbital magnetic resonance imaging (MRI) examination showed bilateral lacrimal gland prolapse. While the histopathological result was considered as MD. The immunohistochemical (IHC) staining results were positive for IgG and negative for IgG4. To clarify the diagnosis, SS-related laboratory tests were performed: anti-SSA antibody (+++), anti-SSB antibody (+++), anti-Ro-52 antibody (+++). With a comprehensive consideration, the final diagnosis was SS.
CONCLUSIONS: When the clinical manifestations are atypical, it is necessary to pay attention to the differential diagnosis of SS and MD.
摘要：
背景：干燥综合征（SS）是一种炎症性自身免疫性疾病，Mikulicz病（MD）是一种淋巴增生性疾病。MD和SS在中年女性中更常见，干眼症在两者中都有不同程度的表现。MD患者的特征是泪腺对称肿胀，这也可能发生在SS的早期。而早期SS与MD之间的影像学表现缺乏特异性。因此,SS和MD在临床表现上有相似之处，影像学和病理结果，诊断混淆。
方法：一名51岁女性患者表现为上眼睑双侧肿胀2年。她还报告了口干和眼睛干，这是可以忍受的。Schirmer的检测结果为阳性，实验室检测表明血清总IgG升高。在双侧泪腺区可触诊软肿块。眼眶磁共振成像（MRI）检查显示双侧泪腺脱垂。而组织病理学结果被认为是MD。免疫组织化学(IHC)染色结果IgG阳性,IgG4阴性。为了明确诊断，进行了SS相关的实验室测试：抗SSA抗体（+++），抗SSB抗体（+++），抗Ro-52抗体(+++)。综合考虑,最终诊断为SS。
结论：当临床表现不典型时，有必要注意SS和MD的鉴别诊断。
方法：一名51岁女性患者表现为上眼睑双侧肿胀2年。她还报告了口干和眼睛干，这是可以忍受的。Schirmer的检测结果为阳性，实验室检测表明血清总IgG升高。在双侧泪腺区可触诊软肿块。眼眶磁共振成像（MRI）检查显示双侧泪腺脱垂。而组织病理学结果被认为是MD。免疫组织化学(IHC)染色结果IgG阳性,IgG4阴性。为了明确诊断，进行了SS相关的实验室测试：抗SSA抗体（+++），抗SSB抗体（+++），抗Ro-52抗体(+++)。综合考虑,最终诊断为SS。
结论：当临床表现不典型时，有必要注意SS和MD的鉴别诊断。
