Beijing Institute of Ophthalmology, Beijing Tongren Hospital, Beijing Tongren Eye Center, Capital Medical University, Beijing, 100730, China.
BMC Ophthalmol. 2023 Jul 27;23(1):336. doi: 10.1186/s12886-023-03090-1.
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.
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.
When the clinical manifestations are atypical, it is necessary to pay attention to the differential diagnosis of SS and MD.
干燥综合征（SS）是一种炎症性自身免疫性疾病，米库利茨病（MD）是一种淋巴增生性疾病。MD 和 SS 均更常见于中年女性，且两者均可出现不同程度的干眼。MD 患者的特征为双侧泪腺肿胀，SS 的早期也可出现这种情况。并且 SS 和 MD 的早期影像学表现缺乏特异性。因此，SS 和 MD 在临床表现、影像学和病理学方面具有相似性，容易混淆诊断。
一名 51 岁女性患者因双侧上眼睑肿胀 2 年来就诊。她还报告有口干和眼干，但可耐受。Schirmer 试验结果阳性，实验室检查提示血清总 IgG 升高。双侧泪腺区可触及柔软肿块。眼眶磁共振成像（MRI）检查显示双侧泪腺脱垂。而组织病理学结果考虑为 MD。免疫组化（IHC）染色结果 IgG 阳性，IgG4 阴性。为明确诊断，进行了与 SS 相关的实验室检查：抗 SSA 抗体（+++），抗 SSB 抗体（+++），抗 Ro-52 抗体（+++）。综合考虑后，最终诊断为 SS。
当临床表现不典型时，需要注意 SS 和 MD 的鉴别诊断。
