Childhood Sjögren syndrome: features of an international cohort and application of the 2016 ACR/EULAR classification criteria

Basiaga, Matthew L.; Stern, Sara; Mehta, Jay; Edens, Cuoghi; Randell, Rachel L.; Pomorska, Anna; Irga‐Jaworska, Ninela; Ibarra, Maria; Bracaglia, Claudia; Nicolai, Rebecca; Sušić, Gordana; Boneparth, Alexis; Srinivasalu, Hemalatha; Dizon, Brian; Kamdar, Ankur A; Goldberg, Baruch; Knupp-Oliveira, Sheila; Antón, Jordi; Mosquera, Juan Manuel; Appenzeller, Simone; O’Neil, Kathleen M.; Protopapas, Stella; Saad‐Magalhães, Claudia; Akikusa, Jonathan; Thatayatikom, Akaluck; Cha, Seunghee; Nieto‐González, Juan Carlos; Lo, Mindy S.; Treemarcki, Erin Brennan; Yokogawa, Naoto; Lieberman, Scott M.

doi:10.1093/rheumatology/keaa757

Cited by 55 publications

(55 citation statements)

References 26 publications

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“…There is no gold standard diagnostic tool for SS with childhood onset and therefore diagnosis is based on expert clinical opinion, which is dependent on findings from the clinical history and examination, functional exocrine gland tests, as well as serological and histological evidence [ 8 ]. Although historically the diagnostic label of ‘primary SS’ has been used for children [ 9 ], recent reports have highlighted the large spectrum of clinical symptoms children present with, which are difficult to map against the classification criteria for adults with primary SS [ 5 ].…”

Section: Introductionmentioning

confidence: 99%

Treatment strategies for Sjögren’s syndrome with childhood onset: a systematic review of the literature

et al. 2021

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Objectives Sjögren's Syndrome (SS) with childhood onset is a rare autoimmune disease characterised by heterogeneous presentation. The lack of validated classification criteria makes it challenging to diagnose. Evidence-based guidelines for treatment of juvenile SS are not available due to the rarity of disease and the paucity of research in this patient population. This systematic review aims to summarise and appraise the current literature focused on pharmacological strategies for management of SS with childhood onset. Methods PubMed and MEDLINE/Scopus databases up to December 2020 have been screened for suitable reports highlighting pharmacological treatment of SS with childhood onset using the PRISMA 2009 reporting checklist. Animal studies have been excluded. Results 43 studies (34 case reports, 8 mini case series and one pilot study) were eligible for analysis. The studies retrieved included girls in 88% (120/137) of cases and had very low confidence level. Hydroxychloroquine (HCQ) was prescribed for parotid swelling, as well as in association with methotrexate (MTX) and non-steroidal anti-inflammatory drugs (NSAIDS) in patients with arthritis and arthralgia. Corticosteroids such as long courses of oral prednisone and IV methylprednisolone were commonly prescribed for children with severe disease presentations. Rituximab was mainly indicated for MALT lymphoma, and renal and nervous system complications. Other conventional DMARDs were prescribed in selected cases with extra-glandular manifestations. Conclusion Various therapies are used for the management of juvenile SS and are prescribed based on expert clinician’s opinion. There are currently no good quality studies that allow clinical recommendations for treatment in SS with childhood onset.

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Section: Introductionmentioning

confidence: 99%

Treatment strategies for Sjögren’s syndrome with childhood onset: a systematic review of the literature

et al. 2021

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“…In our cohort, patient reported recurrent parotid gland swelling was present in 91% of the paediatric patients, compared to 49% of adult patients. In the scarce literature available on paediatric onset pSS, recurrent parotid gland swelling is seen as an important feature, with a prevalence ranging from 50% to 75% (5,8,10,23). A possible explanation for the high prevalence of recurrent parotid swelling in our cohort might be that it was scored as subjective symptom (reported by the patients), while other studies only scored parotid gland swelling when parotid enlargement was observed by a physician.…”

Section: Discussionmentioning

confidence: 78%

“…Paediatric patients present more often with non-specific extra-glandular manifestations like fever and arthralgias (8). Furthermore, paediatric patients present less often with sicca complaints (5), which is one of the patient-reported entry criteria for the ACR/EULAR classification, and more often with recurrent parotid gland swelling (9,10). The latter is currently only included in the ACR/EULAR criteria as an entry criterium embedded in the glandular domain of the EULAR SS disease activity index (ESSDAI), when parotid, submandibular or lacrimal gland swelling is assessed by clinical examination (6,7).…”

Section: Introductionmentioning

confidence: 99%

Differences in presentation between paediatric- and adult-onset primary Sjögren's syndrome patients

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et al. 2021

Clinical and Experimental Rheumatology

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Objective. Primary Sjögren's syndrome (pSS) is a rare disease in paediatric patients. Presenting symptoms differ from those in adult patients. The aim of this study was to evaluate presenting symptoms, classification criteria and clinical assessments, including salivary gland ultrasonography (SGUS), at disease onset in paediatric and adult patients with pSS. Methods. Data of 23 paediatric-and 33 adult-onset patients with pSS were obtained from our standardised multidisciplinary REpSULT and RESULT cohorts, respectively. Clinical, patientreported, serological, functional, biopsy and SGUS parameters were compared. Results. In paediatric-onset pSS (pedSS) patients, recurrent parotid gland swelling (91% vs. 49%, p<0.001) and fever (30% vs. 3%, p=0.006) were more often present than in adult-onset patients. In contrast, sicca symptoms of mouth (52% vs. 79%, p=0.046) and eyes (26% vs. 73%, p<0.001) were less common in pedSS patients. In paediatric patients, the entry criteria of the ACR/EULAR classification were most often met due to activity in the glandular domain of the ESSDAI. When applying the ACR/EULAR classification criteria, only 78% of pedSS fulfilled these criteria compared to 100% of adult patients. Abnormal glandular function tests had a greater contribution to fulfilling the criteria in adults, while the biopsy had a greater contribution in paediatric patients. Anti-SSA/Ro serology had similar contribution for both cohorts. SGUS Hocevar score was significantly higher in paediatric compared to adult patients (median 25 vs. 18, p=0.004). Conclusion. PedSS has a different presentation than adult-onset pSS. Recurrent parotid gland swelling in paediatric patients should alert clinicians to the potential presence of pSS.

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“…There are currently no universally accepted classification criteria for sSS and some experts argue that making a distinction between pSS and sSS is not adequate anymore, as both phenotypes represent the same disease [ 4 ]. Moreover, the classification criteria validated in adults have minimal utility in SS with childhood-onset (defined as disease onset before the age of 18 years), as the disease presentation in children and young people, although rare, is different [ 5 ]. This, in addition to the lack of validated classification criteria for childhood-onset SS, further limits the research opportunities for younger people affected by this disease [ 6 ].…”

Section: Introductionmentioning

confidence: 99%

Multi-Omic Biomarkers for Patient Stratification in Sjogren’s Syndrome—A Review of the Literature

et al. 2022

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Sjögren’s syndrome (SS) is a heterogeneous autoimmune rheumatic disease (ARD) characterised by dryness due to the chronic lymphocytic infiltration of the exocrine glands. Patients can also present other extra glandular manifestations, such as arthritis, anaemia and fatigue or various types of organ involvement. Due to its heterogenicity, along with the lack of effective treatments, the diagnosis and management of this disease is challenging. The objective of this review is to summarize recent multi-omic publications aiming to identify biomarkers in tears, saliva and peripheral blood from SS patients that could be relevant for their better stratification aiming at improved treatment selection and hopefully better outcomes. We highlight the relevance of pro-inflammatory cytokines and interferon (IFN) as biomarkers identified in higher concentrations in serum, saliva and tears. Transcriptomic studies confirmed the upregulation of IFN and interleukin signalling in patients with SS, whereas immunophenotyping studies have shown dysregulation in the immune cell population frequencies, specifically CD4+and C8+T activated cells, and their correlations with clinical parameters, such as disease activity scores. Lastly, we discussed emerging findings derived from different omic technologies which can provide integrated knowledge about SS pathogenesis and facilitate personalised medicine approaches leading to better patient outcomes in the future.

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Childhood Sjögren syndrome: features of an international cohort and application of the 2016 ACR/EULAR classification criteria

Cited by 55 publications

References 26 publications

Treatment strategies for Sjögren’s syndrome with childhood onset: a systematic review of the literature

Treatment strategies for Sjögren’s syndrome with childhood onset: a systematic review of the literature

Differences in presentation between paediatric- and adult-onset primary Sjögren's syndrome patients

Multi-Omic Biomarkers for Patient Stratification in Sjogren’s Syndrome—A Review of the Literature

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