Marrani, Edoardo;
Al-Obaidi, Muthana;
Ciurtin, Coziana;
(2024)
Childhood onset Sjogren Disease in Adolescence and Young Adulthood.
In: Ciurtin, Coziana and Hackett, Janine, (eds.)
Adolescent and Young Adult Rheumatology In Clinical Practice.
Springer Nature: Cham, Switzerland.
(In press).
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Abstract
Childhood-onset Sjögren's disease (cSjD) is a rare and complex autoimmune disease with heterogeneous clinical features ranging from mild glandular symptoms to severe systemic involvement. Previously neglected due to its low prevalence—a diagnosis before 18 years of age is reported in only 1% of all SjD cases —cSjD has recently garnered significant attention from researchers as a clinically relevant condition affecting adolescents and young adults (AYA), with a potential negative impact on quality of life (QoL) and a high risk of serious complications. Compared with adult-onset disease, children and AYA have a high prevalence of recurrent parotitis and systemic or extra-glandular symptoms. Early diagnosis is critical, but the classification criteria used for adults may not be sensitive enough for AYA. In addition, a large percentage of patients have not undergone all the necessary diagnostic tests according to adult criteria. Therefore, most cases are diagnosed based on expert opinion. A multidisciplinary approach is the cornerstone for managing these AYA with cSjD, addressing both the glandular and extra-glandular components of the disease. Treatment combines topical and systemic therapies to manage symptoms and prevent long-term damage. Regular dental and ophthalmologic follow-up is essential. Vaccination and patient education in self-management and lifestyle changes are critical, along with psychological support. Transitioning to adult care requires careful planning to ensure continuity and comprehensive management. Despite challenges in disease recognition and lack of evidence-based treatments, early and effective management can potentially exert a significant positive impact on the outcomes and QoL of AYAs with cSjD
| Type: | Book chapter |
|---|---|
| Title: | Childhood onset Sjogren Disease in Adolescence and Young Adulthood |
| Open access status: | An open access version is available from UCL Discovery |
| Language: | English |
| Additional information: | This version is the author-accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions. |
| Keywords: | Childhood-onset Sjögren's disease |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences |
| URI: | https://discovery-pp.ucl.ac.uk/id/eprint/10196379 |
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