Juvenile Primary Sjögren Syndrome in a 15-Year-Old Boy with Renal Involvement: A Case Report and Review of the Literature
Juvenile primary Sjögren syndrome (pSS) with renal involvement is extremely rare, reported approximately in 50 children, predominantly girls. Here, we present the first reported case of a male child with juvenile pSS with ocular surface disease (previously keratoconjunctivitis sicca), submandibular...
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| Language: | English |
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MDPI AG
2024-01-01
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| Series: | Diagnostics |
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| Online Access: | https://www.mdpi.com/2075-4418/14/3/258 |
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| author | Katerina Bouchalova Hana Flögelova Pavel Horak Jakub Civrny Petr Mlcak Richard Pink Jaroslav Michalek Petra Camborova Zuzana Mikulkova Eva Kriegova |
| author_facet | Katerina Bouchalova Hana Flögelova Pavel Horak Jakub Civrny Petr Mlcak Richard Pink Jaroslav Michalek Petra Camborova Zuzana Mikulkova Eva Kriegova |
| author_sort | Katerina Bouchalova |
| collection | DOAJ |
| description | Juvenile primary Sjögren syndrome (pSS) with renal involvement is extremely rare, reported approximately in 50 children, predominantly girls. Here, we present the first reported case of a male child with juvenile pSS with ocular surface disease (previously keratoconjunctivitis sicca), submandibular salivary gland involvement, and tubulointerstitial nephritis. First, two symptoms were clinically apparent at presentation. We illustrate here that kidney involvement in pSS should be actively looked for, as juvenile pSS may be associated with asymptomatic renal involvement. Immunophenotyping of peripheral blood cells using multicolor flow cytometry revealed at the time of diagnosis changes in both adaptive (T memory cells and B memory cells), and innate immunity (an increased activation of natural killer cells, as well as monocytes and neutrophils, and an increased representation of intermediate monocytes). Our case report points to the importance of kidney examination, early diagnosis and therapy in juvenile pSS, as well as highlights international collaboration to obtain more data for this rare disease. |
| first_indexed | 2024-03-08T03:58:55Z |
| format | Article |
| id | doaj.art-bc5672b24e864d5a94606114b718797b |
| institution | Directory Open Access Journal |
| issn | 2075-4418 |
| language | English |
| last_indexed | 2024-03-08T03:58:55Z |
| publishDate | 2024-01-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Diagnostics |
| spelling | doaj.art-bc5672b24e864d5a94606114b718797b2024-02-09T15:10:00ZengMDPI AGDiagnostics2075-44182024-01-0114325810.3390/diagnostics14030258Juvenile Primary Sjögren Syndrome in a 15-Year-Old Boy with Renal Involvement: A Case Report and Review of the LiteratureKaterina Bouchalova0Hana Flögelova1Pavel Horak2Jakub Civrny3Petr Mlcak4Richard Pink5Jaroslav Michalek6Petra Camborova7Zuzana Mikulkova8Eva Kriegova9Department of Pediatrics, Faculty of Medicine and Dentistry, Palacky University and University Hospital, 779 00 Olomouc, Czech RepublicDepartment of Pediatrics, Faculty of Medicine and Dentistry, Palacky University and University Hospital, 779 00 Olomouc, Czech RepublicDepartment of Internal Medicine III-Nephrology, Rheumatology and Endocrinology, Faculty of Medicine and Dentistry, Palacky University and University Hospital, 779 00 Olomouc, Czech RepublicDepartment of Radiology, Faculty of Medicine and Dentistry, Palacky University and University Hospital, 779 00 Olomouc, Czech RepublicDepartment of Ophthalmology, Faculty of Medicine and Dentistry, Palacky University and University Hospital, 779 00 Olomouc, Czech RepublicDepartment of Oral and Maxillofacial Surgery, Faculty of Medicine and Dentistry, Palacky University and University Hospital, 779 00 Olomouc, Czech RepublicDepartment of Clinical and Molecular Pathology, Faculty of Medicine and Dentistry, Palacky University and University Hospital, 779 00 Olomouc, Czech RepublicDepartment of Pediatrics, Tomas Bata Regional Hospital, 762 75 Zlin, Czech RepublicDepartment of Immunology, Faculty of Medicine and Dentistry, Palacky University and University Hospital, 779 00 Olomouc, Czech RepublicDepartment of Immunology, Faculty of Medicine and Dentistry, Palacky University and University Hospital, 779 00 Olomouc, Czech RepublicJuvenile primary Sjögren syndrome (pSS) with renal involvement is extremely rare, reported approximately in 50 children, predominantly girls. Here, we present the first reported case of a male child with juvenile pSS with ocular surface disease (previously keratoconjunctivitis sicca), submandibular salivary gland involvement, and tubulointerstitial nephritis. First, two symptoms were clinically apparent at presentation. We illustrate here that kidney involvement in pSS should be actively looked for, as juvenile pSS may be associated with asymptomatic renal involvement. Immunophenotyping of peripheral blood cells using multicolor flow cytometry revealed at the time of diagnosis changes in both adaptive (T memory cells and B memory cells), and innate immunity (an increased activation of natural killer cells, as well as monocytes and neutrophils, and an increased representation of intermediate monocytes). Our case report points to the importance of kidney examination, early diagnosis and therapy in juvenile pSS, as well as highlights international collaboration to obtain more data for this rare disease.https://www.mdpi.com/2075-4418/14/3/258juvenile primary Sjögren syndromerenal involvementmale child patientimmunophenotypingdiagnostic criteriatherapy |
| spellingShingle | Katerina Bouchalova Hana Flögelova Pavel Horak Jakub Civrny Petr Mlcak Richard Pink Jaroslav Michalek Petra Camborova Zuzana Mikulkova Eva Kriegova Juvenile Primary Sjögren Syndrome in a 15-Year-Old Boy with Renal Involvement: A Case Report and Review of the Literature Diagnostics juvenile primary Sjögren syndrome renal involvement male child patient immunophenotyping diagnostic criteria therapy |
| title | Juvenile Primary Sjögren Syndrome in a 15-Year-Old Boy with Renal Involvement: A Case Report and Review of the Literature |
| title_full | Juvenile Primary Sjögren Syndrome in a 15-Year-Old Boy with Renal Involvement: A Case Report and Review of the Literature |
| title_fullStr | Juvenile Primary Sjögren Syndrome in a 15-Year-Old Boy with Renal Involvement: A Case Report and Review of the Literature |
| title_full_unstemmed | Juvenile Primary Sjögren Syndrome in a 15-Year-Old Boy with Renal Involvement: A Case Report and Review of the Literature |
| title_short | Juvenile Primary Sjögren Syndrome in a 15-Year-Old Boy with Renal Involvement: A Case Report and Review of the Literature |
| title_sort | juvenile primary sjogren syndrome in a 15 year old boy with renal involvement a case report and review of the literature |
| topic | juvenile primary Sjögren syndrome renal involvement male child patient immunophenotyping diagnostic criteria therapy |
| url | https://www.mdpi.com/2075-4418/14/3/258 |
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