Syndrome de Goujerot-Sjögren primitif de l’enfant : à propos d’un cas

Majdoub, I.; Kallel, Souha; Hsaïri, Mohamed; Snoussi, M.; Charfi, Manel; Halima, Afef Ben; Frikha, A.; Wèli, Manel; Safi, Fatemeh; Gargouri, L.; Halima, N. Ben; Mahfoudh, A.; Ghorbel, A.; Bahloul, Z.

doi:10.1016/j.arcped.2017.09.009

Cited by 13 publications

(32 citation statements)

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“…The most frequent clinical indications for HCQ were unilateral or bilateral parotid swelling in 15% (7/46) of children [ 28 , 41 , 43 , 51 ], arthralgia [4.3% (2/46)] [ 18 , 44 ], renal involvement [4.3% (2/46)] [ 27 ] and a combination of arthralgia and fatigue [2% (1/46)] [ 42 ] ( Table 2 ). HCQ was also prescribed as maintenance therapy for SS presenting with psychiatric symptoms [2% (1/46)] ( Table 3 ).…”

Section: Resultsmentioning

confidence: 99%

“…Data about the patients’ ages were retrievable in 81% (21/26) of children: the mean age at SS diagnosis was 13 years (range 7–17) and 77% (20/26) were females. The most used therapeutic interventions for eye dryness were humidifier eye drops [ 17 ], artificial tears [ 18 , 22 , 27 , 28 , 32 , 42 , 51 ] and topical lubricants [ 25 , 28 ], however, the response to treatment was not specified in the majority of cases.…”

Section: Resultsmentioning

confidence: 99%

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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: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

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

et al. 2021

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“…Diagnosis research is difficult in children because of the often-atypical clinical presentation. The clinical manifestations listed in this study are the same commonly cited in the literature [ 10 , 11 , 13 ]. The American–European consensus criteria and that of Vitali allowed us to establish the diagnosis in the patients studied in this work.…”

Section: Discussionmentioning

confidence: 99%

“…Despite the many diagnostic criteria for Sjögren’s syndrome, controversy persists as to the choice of a precise diagnostic criterion, especially in children. By adapting the American–European consensus group (CETA) classification, Bartounkova et al have proposed a new scheme for the diagnosis of Sjögren’s syndrome in children [ 10 – 12 ]. We report 15 cases of childhood Sjögren’s syndrome.…”

Section: Introductionmentioning

confidence: 99%

Sjögren’s syndrome in children: about 15 cases in Guinea Conakry

et al. 2021

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Objectives Sjögren’s syndrome is rare in children and most often secondary. It frequently affects girls and is characterized by dry eye syndrome, mouth and sometimes systemic involvement. Its diagnosis is difficult to establish in children. We report a series of 15 cases of Sjögren’s syndrome in order to clarify the peculiarities of this condition in children. Patients and methods This retrospective study was carried out over a 2-year period focused on children under 16 years of age who had been followed for Sjögren’s syndrome in the rheumatology and pediatric departments. Patient data were collected and then analyzed by STATA/SE version 11.2 software. Anonymity and respect for ethical rules were the norm. There was no connection between the patients and the researchers. Description of cases The mean age of the patients was 11 years with extremes of 5–15 years. History reveals that a dry mouth was found in more than half of the cases, or in 10 (66.7%) patients. Clinical examination found oral ulceration and periodontitis in equal proportions, 6 (40%). The immunological workup and the biopsy of the accessory salivary glands served as diagnostic evidence in the 15 patients according to the US–European criteria of 2002. Conclusion Sjögren’s syndrome is a rare entity in pediatrics. Its diagnosis is difficult to establish in pediatrics and its severity is linked to the occurrence of late visceral and lymphomatous sicca syndrome. Rapid diagnosis and initiation of a synthetic antimalarial (hydroxychloroquine) increases the hope of a cure.

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“…Prompt diagnosis is necessary to prevent early complications, especially functional ones related to dry and late syndrome such as visceral and lymphomatous [5].…”

Section: Introductionmentioning

confidence: 99%

Sjögren's Syndrome in Children: About 15 Cases in Guinea Conakry

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Barry

et al. 2021

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Objectives- Sjögren's syndrome is difficult to diagnose in the African context. It is rarely reported in children in Black Africa. We report a series of 15 cases of Sjögren’s syndrome in order to clarify the particularities of this condition in children.Methods- This 2-year retrospective study focused on children under 16 years of age with a male predominance who were followed for AS in the rheumatology and pediatrics departments. Patient data were collected and analyzed using STATA/SE version 11.2 software. Anonymity and respect for ethical rules were the norm. There was no link between patients and researchers.Results- The average age of the patients was 11 years with extremes of 5 to 15 years. An anamnesis revealing dry mouth was found in more than half of the cases, i.e. in 10 (66.7%) patients. The clinical examination found oral ulceration and periodontitis in equal proportions, i.e. 6 (40%). The immunological assessment and the biopsy of the accessory salivary glands were used as diagnostic evidence in the 15 patients according to the American-European criteria of 2002.Conclusion- Sjögren's syndrome is a rare entity in pediatrics. It is difficult to diagnose in pediatrics and its severity is linked to the occurrence of visceral and lymphomatous late dry syndrome. Rapid diagnosis and the use of a synthetic antimalarial drug (Hydroxychloroquine) increases the hope of a cure.

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Syndrome de Goujerot-Sjögren primitif de l’enfant : à propos d’un cas

Cited by 13 publications

References 10 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

Sjögren’s syndrome in children: about 15 cases in Guinea Conakry

Sjögren's Syndrome in Children: About 15 Cases in Guinea Conakry

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