Recommendations for evaluation and diagnosis of extra-glandular manifestations of primary Sjögren syndrome: results of an epidemiologic systematic review/meta-analysis and a consensus guideline from the Brazilian society of rheumatology (hepatic, gastrointestinal and pancreatic)

Trevisani, Virgínia Fernandes Moça; Pinheiro, Aysa César; Fialho, Sônia Cristina de Magalhães Souza; Fernandes, Marilena Leal Mesquita Silvestre; Pugliesi, A.A.V.; Pasoto, Sandra Gofinet; Lopes, Maria Lúcia Lemos; Guedes, Lissiane Karine Noronha; Miyamoto, Samira Tatiyama; Santos, Luciano Marques dos; Appenzeller, Simone; Fidelix, Tania; Ribeiro, Sandra Lúcia Euzébio; Brito, Danielle Christinne Soares Egypto de; Libório‐Kimura, Tatiana Nayara; Santos, Maria Carmen Lopes Ferreira Silva; Cantali, Diego Ustárroz; Gennari, Juliana D’Agostino; Capobianco, Karina Gatz; Civile, Vinicius Tassoni; Pinto, Ana Carolina Pereira Nunes; Rocha, Aline Pereira da; Rocha-Filho, César Ramos; Oliveira, Fabíola Reis; Valim, Valéria

doi:10.1186/s42358-022-00267-y

Cited by 7 publications

(7 citation statements)

References 92 publications

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“…In order to evaluate the efficacy more comprehensively, we chose both ESSDAI and ESSDAI as the index of subjective symptoms and systemic characteristics that showing to be valid and sensitive measures 37 . Furthermore, it is interesting to underline that, in the present study, the variety immunological indexes were accordant with subjective indexes 38 . The association between them could be explained inflammatory reaction play a leading role in disease mechanism.…”

Section: Discussionsupporting

confidence: 61%

Effect of adipose tissue-derived stem cells therapy on clinical response in patients with primary Sjogren’s syndrome

Li,

Lu,

Shi

et al. 2023

Sci Rep

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The purpose of this trial was to clinically assess the effect and safety of Adipose Tissue-derived Stem Cells (ADSCs) treatment on primary Sjogren’s Syndrome (pSS). In this 6-month randomized, triple-blind, placebo-controlled clinical trial, pSS patients were randomly assigned to two groups. After demographic characteristics and clinical examination were achieved, local injection of ADSCs into bilateral glands was performed with patients in ADSCs group (n = 35) and placebo solution was used for another group (n = 39) at three time points. Patients were followed up at 1-, 3- and 6-month. At each visit, studies of clinical and laboratory outcomes, as well as subjective symptoms, were conducted. A total of 74 subjects who met the including criteria were allocated in two groups and eventually 64 subjects (86.5%) completed the treatments and the follow-up assessments. Secretion of salivary and lachrymal glands were significantly improved in 3-month (P < 0.05). A great improvement of European League Against Rheumatism Sjögren’s Syndrome Disease Activity Index (ESSDAI) was found after ADSCs treatment with intergroup comparison from baseline to follow-up (P < 0.05). There is also a significant difference of European Alliance of Associations for Rheumatology SS Patient Reported Index (ESSPRI) between the two groups in the follow-up (P < 0.05). A significant abatement of IgG, IgM, C3, C4 and ESR between two groups was observed in part of follow-up time points (P < 0.05). The ADSCs therapy can provide relief of oral and eye’s dryness in our trial in a short time and has potential improvement of subjective and systemic syndromes of pSS.

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Section: Discussionsupporting

confidence: 61%

Effect of adipose tissue-derived stem cells therapy on clinical response in patients with primary Sjogren’s syndrome

Li,

Lu,

Shi

et al. 2023

Sci Rep

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“…Patients diagnosed with PBC exhibit a prevalence of pSS of approximately 38%. However, clinical evidence of PBC is observed in fewer than 2% of patients with SS, according to large cohort studies (Trevisani et al, 2022). In instances where SS is accompanied by liver dysfunction, treatment usually involves the isolated use of hepatoprotective drugs such as ursodeoxycholic acid, aimed at symptomatically improving liver function.…”

Section: Liver Involvementmentioning

confidence: 99%

“…Initially, abatacept was found to lessen inflammation and enhance saliva production in SS (Meiners et al, 2014), with subsequent ROSE and ROSE II trials confirming improvements in various outcomes, including glandular, extraglandular, and systemic manifestations (Tsuboi et al, 2023). However, despite these biological effects on biomarkers and immune cells, a phase III trial in active primary SS indicated that abatacept did not achieve significant clinical efficacy compared to placebo (Baer et al, 2021;van N et al, 2020).…”

Section: Application Of Abatacept In Ssmentioning

confidence: 99%

Emerging biologic frontiers for Sjogren’s syndrome: Unveiling novel approaches with emphasis on extra glandular pathology

Li,

Maitiyaer,

Tan

et al. 2024

Front. Pharmacol.

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Primary Sjögren’s Syndrome (pSS) is a complex autoimmune disorder characterized by exocrine gland dysfunction, leading to dry eyes and mouth. Despite growing interest in biologic therapies for pSS, FDA approval has proven challenging due to trial complications. This review addresses the absence of a molecular-target-based approach to biologic therapy development and highlights novel research on drug targets and clinical trials. A literature search identified potential pSS treatment targets and recent advances in molecular understanding. Overlooking extraglandular symptoms like fatigue and depression is a notable gap in trials. Emerging biologic agents targeting cytokines, signal pathways, and immune responses have proven efficacy. These novel therapies could complement existing methods for symptom alleviation. Improved grading systems accounting for extraglandular symptoms are needed. The future of pSS treatment may involve gene, stem-cell, and tissue-engineering therapies. This narrative review offers insights into advancing pSS management through innovative biologic interventions.

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“…Primary Sjögren’s syndrome (pSS) is a systemic autoimmune disease that affects the exocrine glands, such as the lacrimal and the salivary glands ( 1 – 3 ). In addition, various extraglandular lesions may occur in the lung, the kidney, the joints, or the lymph nodes in many patients with this syndrome ( 4 , 5 ). Systemic symptoms in pSS patients are well known and include arthralgia, fatigue, general discomfort, and cardiovascular events ( 6 , 7 ).…”

Section: Introductionmentioning

confidence: 99%

CD4+ T-cell-dependent differentiation of CD23+ follicular B cells contributes to the pulmonary pathology in a primary Sjögren’s syndrome mouse model

Sato-Fukuba,

Arakaki,

Ushio

et al. 2023

Front. Immunol.

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IntroductionPrimary Sjögren’s syndrome (pSS) is a systemic autoimmune disease that affects the function of exocrine glands, such as the lacrimal and the salivary glands. Extraglandular lesions and malignant lymphoma also occur during the progressive stage of pSS. We have, herein, focused on the pulmonary lesions of pSS and have aimed clarifying their pathophysiological mechanism by comparing the glandular with the extraglandular lesions observed in a mouse model of pSS.ResultsThe histopathological analysis of lung tissues obtained from NFS/sld mice that have undergone neonatal thymectomy was performed. Moreover, in vivo and in vitro experiments were conducted along with immunological analyses in order to characterize the unique phenotypes of the pulmonary lesions identified in these pSS model mice. Inflammatory lesions with a bronchus-associated lymphoid tissue-like structure were identified in the lungs of pSS model mice. In addition, relative to salivary gland lesions, pulmonary lesions showed increased CD23+ follicular B (FB) cells. In vitro and pulmonary B cells were more readily driven to CD23+ FB cell phenotype than salivary gland B cells in pSS model mice. Furthermore, the CD23+ FB cell differentiation was found to be enhanced in a CD4+ T-cell-dependent manner under a Th2-type condition in the lungs of herein examined pSS model mice.DiscussionA Th2-type response in the pSS lung may promote the progression of autoimmune lesions through an enhanced abnormal differentiation of B cells.

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Recommendations for evaluation and diagnosis of extra-glandular manifestations of primary Sjögren syndrome: results of an epidemiologic systematic review/meta-analysis and a consensus guideline from the Brazilian society of rheumatology (hepatic, gastrointestinal and pancreatic)

Cited by 7 publications

References 92 publications

Effect of adipose tissue-derived stem cells therapy on clinical response in patients with primary Sjogren’s syndrome

Effect of adipose tissue-derived stem cells therapy on clinical response in patients with primary Sjogren’s syndrome

Emerging biologic frontiers for Sjogren’s syndrome: Unveiling novel approaches with emphasis on extra glandular pathology

CD4+ T-cell-dependent differentiation of CD23+ follicular B cells contributes to the pulmonary pathology in a primary Sjögren’s syndrome mouse model

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