Primary Sjögren’s syndrome misdiagnosed as Mikulicz’s disease: a case report
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 chara...
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| Format: | Article |
| Language: | English |
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BMC
2023-07-01
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| Series: | BMC Ophthalmology |
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| Online Access: | https://doi.org/10.1186/s12886-023-03090-1 |
| _version_ | 1797769661493805056 |
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| author | Tingting Ren Rui Liu Jing Li Jianmin Ma |
| author_facet | Tingting Ren Rui Liu Jing Li Jianmin Ma |
| author_sort | Tingting Ren |
| collection | DOAJ |
| description | 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. Case presentation 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. Conclusion When the clinical manifestations are atypical, it is necessary to pay attention to the differential diagnosis of SS and MD. |
| first_indexed | 2024-03-12T21:11:16Z |
| format | Article |
| id | doaj.art-b53d33db1c5743e5b5d3539912b7928b |
| institution | Directory Open Access Journal |
| issn | 1471-2415 |
| language | English |
| last_indexed | 2024-03-12T21:11:16Z |
| publishDate | 2023-07-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Ophthalmology |
| spelling | doaj.art-b53d33db1c5743e5b5d3539912b7928b2023-07-30T11:10:18ZengBMCBMC Ophthalmology1471-24152023-07-012311510.1186/s12886-023-03090-1Primary Sjögren’s syndrome misdiagnosed as Mikulicz’s disease: a case reportTingting Ren0Rui Liu1Jing Li2Jianmin Ma3Beijing Institute of Ophthalmology, Beijing Tongren Hospital, Beijing Tongren Eye Center, Capital Medical UniversityBeijing Institute of Ophthalmology, Beijing Tongren Hospital, Beijing Tongren Eye Center, Capital Medical UniversityBeijing Institute of Ophthalmology, Beijing Tongren Hospital, Beijing Tongren Eye Center, Capital Medical UniversityBeijing Institute of Ophthalmology, Beijing Tongren Hospital, Beijing Tongren Eye Center, Capital Medical UniversityAbstract 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. Case presentation 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. Conclusion When the clinical manifestations are atypical, it is necessary to pay attention to the differential diagnosis of SS and MD.https://doi.org/10.1186/s12886-023-03090-1Sjögren’s SyndromeMikulicz’s diseaseDifferential diagnosis |
| spellingShingle | Tingting Ren Rui Liu Jing Li Jianmin Ma Primary Sjögren’s syndrome misdiagnosed as Mikulicz’s disease: a case report BMC Ophthalmology Sjögren’s Syndrome Mikulicz’s disease Differential diagnosis |
| title | Primary Sjögren’s syndrome misdiagnosed as Mikulicz’s disease: a case report |
| title_full | Primary Sjögren’s syndrome misdiagnosed as Mikulicz’s disease: a case report |
| title_fullStr | Primary Sjögren’s syndrome misdiagnosed as Mikulicz’s disease: a case report |
| title_full_unstemmed | Primary Sjögren’s syndrome misdiagnosed as Mikulicz’s disease: a case report |
| title_short | Primary Sjögren’s syndrome misdiagnosed as Mikulicz’s disease: a case report |
| title_sort | primary sjogren s syndrome misdiagnosed as mikulicz s disease a case report |
| topic | Sjögren’s Syndrome Mikulicz’s disease Differential diagnosis |
| url | https://doi.org/10.1186/s12886-023-03090-1 |
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