Coexistent Sjogren’s syndrome and Birt-Hogg-Dube´ syndrome: a case report
Abstract We report a rare case of Sjogren’s syndrome complicated with Birt-Hogg-Dubé syndrome (BHDS) not previously mentioned in the literature. Further, there is insufficient evidence linking the two diseases. Here, we review existing diagnostic algorithms for diagnosing diffuse cystic lung disease...
| Main Authors: | , , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
BMC
2023-11-01
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| Series: | BMC Pulmonary Medicine |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s12890-023-02680-5 |
| _version_ | 1797454115686580224 |
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| author | Yongkang Lin Ting Guo Cheng Lei Binyi Yang Danhui Yang Hong Luo Hong Peng |
| author_facet | Yongkang Lin Ting Guo Cheng Lei Binyi Yang Danhui Yang Hong Luo Hong Peng |
| author_sort | Yongkang Lin |
| collection | DOAJ |
| description | Abstract We report a rare case of Sjogren’s syndrome complicated with Birt-Hogg-Dubé syndrome (BHDS) not previously mentioned in the literature. Further, there is insufficient evidence linking the two diseases. Here, we review existing diagnostic algorithms for diagnosing diffuse cystic lung disease and provide new insights. The patient initially complained of thirst and dry eyes for ten years, and gradually developed shortness of breath. After admission, physical examination showed five missing teeth, decreased respiratory sounds in both lower lungs, and Velcro rales. Computed tomography showed multiple thin-walled cystic lesions in both lungs. Initial xerophthalmia and labial gland biopsy seemed to reveal a pulmonary cystic change associated with Sjogren’s syndrome. Before discharge, a rash suspected to indicate a fibrofollicular tumor in the neck was observed, and then FLCN variant has been found. The challenges how to clarify the diagnosis of DCLD causes are discussed. |
| first_indexed | 2024-03-09T15:32:36Z |
| format | Article |
| id | doaj.art-dc854bbec09945b68ca6af5e0037f846 |
| institution | Directory Open Access Journal |
| issn | 1471-2466 |
| language | English |
| last_indexed | 2024-03-09T15:32:36Z |
| publishDate | 2023-11-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Pulmonary Medicine |
| spelling | doaj.art-dc854bbec09945b68ca6af5e0037f8462023-11-26T12:12:41ZengBMCBMC Pulmonary Medicine1471-24662023-11-012311610.1186/s12890-023-02680-5Coexistent Sjogren’s syndrome and Birt-Hogg-Dube´ syndrome: a case reportYongkang Lin0Ting Guo1Cheng Lei2Binyi Yang3Danhui Yang4Hong Luo5Hong Peng6Department of Pulmonary and Critical Care Medicine, the Second Xiangya Hospital, Central South UniversityDepartment of Pulmonary and Critical Care Medicine, the Second Xiangya Hospital, Central South UniversityDepartment of Pulmonary and Critical Care Medicine, the Second Xiangya Hospital, Central South UniversityDepartment of Pulmonary and Critical Care Medicine, the Second Xiangya Hospital, Central South UniversityDepartment of Pulmonary and Critical Care Medicine, the Second Xiangya Hospital, Central South UniversityDepartment of Pulmonary and Critical Care Medicine, the Second Xiangya Hospital, Central South UniversityDepartment of Pulmonary and Critical Care Medicine, the Second Xiangya Hospital, Central South UniversityAbstract We report a rare case of Sjogren’s syndrome complicated with Birt-Hogg-Dubé syndrome (BHDS) not previously mentioned in the literature. Further, there is insufficient evidence linking the two diseases. Here, we review existing diagnostic algorithms for diagnosing diffuse cystic lung disease and provide new insights. The patient initially complained of thirst and dry eyes for ten years, and gradually developed shortness of breath. After admission, physical examination showed five missing teeth, decreased respiratory sounds in both lower lungs, and Velcro rales. Computed tomography showed multiple thin-walled cystic lesions in both lungs. Initial xerophthalmia and labial gland biopsy seemed to reveal a pulmonary cystic change associated with Sjogren’s syndrome. Before discharge, a rash suspected to indicate a fibrofollicular tumor in the neck was observed, and then FLCN variant has been found. The challenges how to clarify the diagnosis of DCLD causes are discussed.https://doi.org/10.1186/s12890-023-02680-5Sjogren’s syndromeBirt-Hogg-Dubé syndromeDCLDFLCNGeneDiagnostic algorithm |
| spellingShingle | Yongkang Lin Ting Guo Cheng Lei Binyi Yang Danhui Yang Hong Luo Hong Peng Coexistent Sjogren’s syndrome and Birt-Hogg-Dube´ syndrome: a case report BMC Pulmonary Medicine Sjogren’s syndrome Birt-Hogg-Dubé syndrome DCLD FLCN Gene Diagnostic algorithm |
| title | Coexistent Sjogren’s syndrome and Birt-Hogg-Dube´ syndrome: a case report |
| title_full | Coexistent Sjogren’s syndrome and Birt-Hogg-Dube´ syndrome: a case report |
| title_fullStr | Coexistent Sjogren’s syndrome and Birt-Hogg-Dube´ syndrome: a case report |
| title_full_unstemmed | Coexistent Sjogren’s syndrome and Birt-Hogg-Dube´ syndrome: a case report |
| title_short | Coexistent Sjogren’s syndrome and Birt-Hogg-Dube´ syndrome: a case report |
| title_sort | coexistent sjogren s syndrome and birt hogg dube´ syndrome a case report |
| topic | Sjogren’s syndrome Birt-Hogg-Dubé syndrome DCLD FLCN Gene Diagnostic algorithm |
| url | https://doi.org/10.1186/s12890-023-02680-5 |
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