Recommendations for evaluation and diagnosis of extra-glandular manifestations of primary sjogren syndrome: results of an epidemiologic systematic review/meta-analysis and a consensus guideline from the Brazilian Society of Rheumatology (articular, pulmonary and renal)

Trevisani, Virgínia Fernandes Moça; Pugliesi, A.A.V.; Pasoto, Sandra Gofinet; Lopes, Maria Lúcia Lemos; Guedes, Lissiane Karine Noronha; Miyamoto, Samira Tatiyama; Fernandes, Marilena Leal Mesquita Silvestre; Fialho, Sônia Cristina de Magalhães Souza; Pinheiro, Aysa César; 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; Civile, Vinicius Tassoni; Pinto, Ana Carolina Pereira Nunes; Rocha-Filho, César Ramos; Oliveira, Fabíola Reis; Rocha, Aline Pereira da; Valim, Valéria

doi:10.1186/s42358-022-00248-1

Cited by 13 publications

(4 citation statements)

References 172 publications

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“…They not only significantly impact a person's overall health, but also increase the burden of the disease, in addition to causing dryness [ 4 , 5 ]. EGM in patients with pSS affects the prognosis of patients [ 4 , 6 , 7 ].…”

Section: Introductionmentioning

confidence: 99%

CXCL9 may serve as a potential biomarker for primary Sjögren’s syndrome with extra-glandular manifestations

Hong,

Cheng,

Wang

et al. 2024

Arthritis Res Ther

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Background Primary Sjögren's syndrome (pSS) is an autoimmune condition that causes harm to exocrine glands and also has extra-glandular manifestations (EGM). pSS patients with EGM have a worse prognosis than those with only sicca symptoms. Previous studies have shown that the minor salivary glands (MSG) of pSS patients exhibit a unique profile of cytokines and chemokines compared to healthy controls. However, there is a lack of research comparing pSS with EGM (pSS-EGM) and pSS without EGM (pSS-non-EGM). This study aims to explore potential biomarkers associated with pSS, particularly pSS with EGM. Methods By utilizing RNA sequencing, we conducted an analysis on the gene expression profiles of MSG in 63 patients diagnosed with pSS, as well as 12 non-pSS individuals. Furthermore, we also investigated the MSG of pSS patients, both with and without EGM. Through bioinformatics analysis, we identified genes with differential expression (DEGs) and determined the core hub genes using PPI network. We then analyzed the top 20 DEGs and their correlation with the patients' clinical characteristics, and validated our findings using peripheral blood plasma. Results A total of 725 differentially expressed genes (DEGs) were identified in the comparison between pSS and non-pSS groups, and 727 DEGs were observed between pSS-EGM and pSS-non-EGM. It is noteworthy that the expression levels of CXCL9 were higher in both pSS patients and pSS-EGM when compared to the control group. Taking into consideration the significance of the top 20 DEGs in relation to clinical parameters and the central hub genes, we ultimately chose CXCL9. In comparison to the non-pSS group, pSS patients exhibited notably greater expression of the CXCL9 gene in the MSG, as well as higher levels of CXCL9 protein in their plasma (p < 0.001). Furthermore, the expression of the CXCL9 gene and levels of CXCL9 protein were notably higher in pSS patients accompanied by EGM and those with SSA antibodies. Additionally, a correlation was found between the expression of the CXCL9 gene and the EULAR Sjogren’s Syndrome Disease Activity Index (ESSDAI), as well as with immunoglobulin G (IgG) levels and erythrocyte sedimentation rate (ESR). Meanwhile, the protein levels of CXCL9 were found to be correlated with IgG levels and ESSDAI. Conclusion CXCL9 proves to be a valuable biomarker in pSS, specifically due to its strong ability to differentiate between pSS patients with EGM and those without EGM. There is a significant correlation between CXCL9 and various clinical parameters both at the gene and protein level. Therefore, CXCL9 could be a potential target for future treatment of pSS.

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Section: Introductionmentioning

confidence: 99%

CXCL9 may serve as a potential biomarker for primary Sjögren’s syndrome with extra-glandular manifestations

Hong,

Cheng,

Wang

et al. 2024

Arthritis Res Ther

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“…All patients should also undergo radiological evaluation of the kidneys. Patients with a persistent urinary pH above 5.5 should be evaluated for RTA, which can be defined as complete, when the blood pH is less than 7.35, or incomplete, when the blood pH is greater than or equal to 7.35 and there is inability to acidify the urine when evaluated by some urine acidification test [10][11][12][13][14][15][16].…”

Section: Introductionmentioning

confidence: 99%

Hypocitraturia as a biomarker of renal tubular acidosis in patients with Sjögren’s disease

Coradin,

Lopes,

Goldani

et al. 2024

Adv Rheumatol

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Introduction Sjögren’s disease (SD) is an immune-mediated chronic inflammatory disease that affects epithelial tissues, mainly salivary and lacrimal glands. It also presents extraglandular manifestations. The main renal manifestation is tubulointerstitial nephritis (TIN), which can manifest as renal tubular acidosis (RTA). Urinary citrate may be a biomarker of RTA in these patients. The objective of this study was to evaluate whether hypocitraturia is a predictive biomarker of RTA in a sample of patients with SD in a tertiary hospital in southern Brazil. Methods All patients with SD who met the inclusion criteria and who participated in the rheumatology outpatient clinic of the Irmandade Santa Casa de Misericórdia de Porto Alegre were included. Demographic, SD, serological and urinary data were obtained. RTA was considered in those patients who persistently presented urinary pH above 5.5 and serum pH below 7.35. Patients who persistently had urinary pH above 5.5 underwent a urinary acidification test with furosemide and fludrocortisone. These patients received 1 mg of fludrocortisone and 40 mg of furosemide and had their urine samples tested 2, 4 and 6 h after taking the medications. The test was stopped at any urine sample with pH 5.5 or less. The variables were expressed as mean and standard deviation or interquartile range. The association between hypocitraturia and RTA was assessed using the chi-square. Results Forty-two patients were included, 95.2% female with a median age of 61.73 years. The prevalence of complete distal RTA was 4.88%. Twenty-eight patients underwent urine acidification testing. Five patients had hypocitraturia, and two of them had complete distal RTA. The association between hypocitraturia and RTA was statistically significant (p < 0.012), with a sensitivity of 100%, specificity of 91.2% and accuracy of 91.7%. The negative predictive value was 100%. The global renal assessment of the population demonstrated two patients with RTA, one patient with decreased renal function and six patients with proteinuria greater than 0.5 g/24 h. Conclusion The prevalence of RTA in the studied population was 4.88%. Hypocitraturia had high sensitivity and accuracy for the diagnosis of RTA.

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“…Patients with a persistent urinary pH above 5.5 should be evaluated for RTA, which can be de ned as complete, when the blood pH is less than 7.35, or incomplete, when the blood pH is greater than or equal to 7.35 and there is inability to acidify the urine when evaluated by some urine acidi cation test. [10][11][12][13][14][15][16] . Some TIN biomarkers have been evaluated to obtain an early marker of renal impairment in patients with SD, with emphasis on b-2 microglobulin, a1 microglobulin, and urinary citrate; however, there is a lack of robust scienti c evidence to prove its e cacy as predictors of nephritis [17][18][19][20][21] .…”

Section: Introductionmentioning

confidence: 99%

Hypocitraturia as a biomarker of renal tubular acidosis in patients with Sjögrens disease

Coradin,

Lopes,

Goldani

et al. 2024

Preprint

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Introduction: Sjögren's disease (SD) is an immune-mediated chronic inflammatory disease that affects epithelial tissues, especially salivary and lacrimal glands, as well as extra glandular manifestations. The main renal manifestation is tubulointerstitial nephritis (TIN), which may present clinically as renal tubular acidosis (RTA). Urinary citrate may be an early biomarker of RTA in these patients. The aim of this study was to evaluate whether hypocitraturia is a predictive biomarker of ATR in a sample of patients with SD in a tertiary hospital in southern Brazil. Methods: All patients with SD who met the inclusion and exclusion criteria and participated in the rheumatology outpatient clinic at Irmandade Santa Casa de Misericórdia of Porto Alegre were included. Demographic data, SD data, and serological and urinary data were obtained. Patients who persistently had urinary pH above 5.5 underwent a urine acidification test with furosemide and fludrocortisone. Continuous variables with a normal distribution were expressed as the mean and standard deviation. The others were expressed as medians and interquartile ranges. Categorical variables are expressed as numbers and frequencies. The association between hypocitraturia and ATR was assessed using the chi-square test. Results: Forty-two patients were included, 95.2% female with a median age of 61.73 years. The prevalence of complete distal RTA was 4.88%. Twenty-eight patients underwent urine acidification testing. Five patients had hypocitraturia, and two of them had complete distal RTA. The association between hypocitraturia and ATR was statistically significant (p< 0.012), with a sensitivity of 100%, specificity of 91.2% and accuracy of 91.7%. The negative predictive value was 100%. The renal evaluation of the population demonstrated, in addition to two patients with ATR, one patient with impaired renal function and six patients with proteinuria greater than 0.5 g/24 hours. Conclusion: The prevalence of ATR in the studied population was 4.88%. Hypocitraturia had high sensitivity and accuracy for the diagnosis of ATR.

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Recommendations for evaluation and diagnosis of extra-glandular manifestations of primary sjogren syndrome: results of an epidemiologic systematic review/meta-analysis and a consensus guideline from the Brazilian Society of Rheumatology (articular, pulmonary and renal)

Cited by 13 publications

References 172 publications

CXCL9 may serve as a potential biomarker for primary Sjögren’s syndrome with extra-glandular manifestations

CXCL9 may serve as a potential biomarker for primary Sjögren’s syndrome with extra-glandular manifestations

Hypocitraturia as a biomarker of renal tubular acidosis in patients with Sjögren’s disease

Hypocitraturia as a biomarker of renal tubular acidosis in patients with Sjögrens disease

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