Clinical profiles of SS-ILD compared with SS-NILD in a Chinese population: a retrospective analysis of 735 patients.

BACKGROUND: Interstitial lung disease (ILD) is a serious complication in patients with Sjögren's syndrome (SS). Most studies on primary SS (pSS) with ILD are limited in sample size, and studies on secondary SS (sSS) with ILD are rare. This study aimed to elucidate both primary and secondary SS-associated ILD (SS-ILD) based on a large cohort. METHODS: The medical records of hospitalized patients diagnosed with SS at the Second Xiangya Hospital of Central South University from January 2010 to May 2020 were retrospectively reviewed. Clinical manifestations, medical history, biological results and imaging data were collected. RESULTS: Of the 735 SS patients enrolled in this study, 563 (76.6%) were diagnosed with pSS, 172 (23.4%) were diagnosed with sSS. Additionally, 316 (43.0%) were diagnosed with SS-ILD. No significant difference was found between the pSS and sSS groups concerning the incidence of ILD (p = .718). Factors associated with SS-ILD were older age (p < .001), male sex (p = .032), female sex at menopause (p = .002), Raynaud's phenomenon (p < .001), low levels of albumin (p = .010) and respiratory symptoms (p < .001). The SS-ILD group showed higher counts of platelets (p < .001). The three most frequent high-resolution CT (HRCT) findings of SS-ILD were irregular linear opacities (42.7%), grid shadows (30.7%) and pleural thickening (28.5%). NSIP (56.3%) was the most frequent HRCT pattern. Compared with pSS patients with ILD (pSS-ILD) patients, sSS patients with ILD (sSS-ILD) patients had a higher incidence of proteinuria (p < .001) and hypercreatinaemia (p = .013), a higher level of erythrocyte sedimentation rate (ESR) (p = .003), low levels of complement 3 (C3) (p = .013), lymphocytes (p = .009) and leukocytes (p = .024), and worse DLCO (%Pred) (p = .035). CONCLUSIONS: ILD is a common pulmonary involvement in both pSS patients and sSS patients. Older age, male sex, female sex at menopause, Raynaud's phenomenon, low albumin levels and respiratory symptoms are risk factors associated with SS-ILD. NSIP is important HRCT feature of SS-ILD. sSS-ILD patients showed worse laboratory results and pulmonary function.KEY MESSAGEOlder age, male sex, female sex at menopause, Raynaud's phenomenon, low albumin levels and respiratory symptoms are risk factors associated with SS-ILD.SS-ILD patients show higher counts of platelets and less purpura.sSS-ILD patients have worse laboratory results and pulmonary function.

American Indians Have a Higher Risk of Sjögren's Syndrome and More Disease Activity Than European Americans and African Americans.

OBJECTIVE: To describe the clinical and serologic manifestations of Sjögren's syndrome (SS) in ethnic groups of the US. METHODS: This was a cross-sectional study of 648 patients with primary SS: 20 African American (AA), 164 American Indian (AI), 426 European American (EA), and 38 patients of other races evaluated in a multidisciplinary Sjögren's International Collaborative Clinical Alliance research clinic. RESULTS: AA subjects comprised 3.1% of the SS cohort, much lower than the percentage of AA in the state of Oklahoma (P = 3.01 × E - 05), the US (P = 2.24E - 13), or a systemic lupus erythematosus (SLE) cohort at the same institution (P = 4.26 × 10E - 27). In contrast, the percentage of AI in the SS cohort (25.3%) was much higher than expected (P = 3.17E - 09 versus SLE cohort, P = 6.36 - 26 versus Oklahoma, and P = 8.14E - 96 versus US population). The SS classification criteria were similar between AA and EA, but subjects of AI ancestry had lower rates of abnormal tear and salivary flow, as well as anti-Ro/SSA and anti-La/SSB antibodies. Paradoxically, AI had higher levels of disease activity (mean ± SD European League Against Rheumatism Sjögren's Syndrome Disease Activity Index score 3.77 ± 4.78) in comparison to EA (2.90 ± 4.12; P = 0.011) and more extraglandular manifestations affecting mainly the articular and glandular domains. Meanwhile, AA patients were characterized by higher rates of hypergammaglobulinemia (odds ratio [OR] 1.39 [95% confidence interval (95% CI) 1.39-8.65]; P = 0.01), elevated erythrocyte sedimentation rate (ESR) (OR 3.95 [95% CI 1.46-9.95]; P = 0.009), and parotid enlargement (OR 4.40 [95% CI 1.49-13.07]; P = 0.02). CONCLUSION: AI are affected at high rates with SS but present with few classical features, potentially preventing timely diagnosis. In contrast to SLE, SS is infrequent and not more severe among AA, but the triad of hypergammaglobulinemia, increased ESR, and parotid enlargement warrants extra vigilance for lymphomagenesis.

Epidemiological profile and north-south gradient driving baseline systemic involvement of primary Sjögren's syndrome.

OBJECTIVE: To characterize the systemic phenotype of primary Sjögren's syndrome at diagnosis by analysing the EULAR-SS disease activity index (ESSDAI) scores. METHODS: The Sjögren Big Data Consortium is an international, multicentre registry based on worldwide data-sharing cooperative merging of pre-existing databases from leading centres in clinical research in Sjögren's syndrome from the five continents. RESULTS: The cohort included 10 007 patients (9352 female, mean 53 years) with recorded ESSDAI scores available. At diagnosis, the mean total ESSDAI score was 6.1; 81.8% of patients had systemic activity (ESSDAI score ≥1). Males had a higher mean ESSDAI (8.1 vs 6.0, P < 0.001) compared with females, as did patients diagnosed at <35 years (6.7 vs 5.6 in patients diagnosed at >65 years, P < 0.001). The highest global ESSDAI score was reported in Black/African Americans, followed by White, Asian and Hispanic patients (6.7, 6.5, 5.4 and 4.8, respectively; P < 0.001). The frequency of involvement of each systemic organ also differed between ethnic groups, with Black/African American patients showing the highest frequencies in the lymphadenopathy, articular, peripheral nervous system, CNS and biological domains, White patients in the glandular, cutaneous and muscular domains, Asian patients in the pulmonary, renal and haematological domains and Hispanic patients in the constitutional domain. Systemic activity measured by the ESSDAI, clinical ESSDAI (clinESSDAI) and disease activity states was higher in patients from southern countries (P < 0.001). CONCLUSION: The systemic phenotype of primary Sjögren's syndrome is strongly influenced by personal determinants such as age, gender, ethnicity and place of residence, which are key geoepidemiological players in driving the expression of systemic disease at diagnosis.

CXCL13 levels in serum but not in saliva are elevated in Asian Indian patients with primary Sjögren's syndrome.

Human and animal model studies suggest CXCL13 is a potential biomarker in primary Sjögren's syndrome (pSS). CXCL13 has not been studied in Indian patients with pSS. pSS cases classified by American European Consensus Group (AECG) or American college of Rheumatology(ACR) 2012 criteria, attending rheumatology clinic between July 2014 and July 2015 were included. Hospital staff and healthy, non-blood related family members of patients constituted the control group. pSS cases underwent clinical evaluation, laboratory investigations, ESSDAI and ESSPRI scoring. Unstimulated saliva was collected by the spitting method. Salivary and serum CXCL13 were quantified by indirect ELISA. CXCL13 positivity was determined using Receiver Operator Characteristic (ROC) curve. STATA13.1 (StataCorpLP,Texas,USA) software was used for statistical analysis. In this study, 45 pSS cases and 42 healthy controls were recruited. In pSS, median levels of serum CXCL13, but not salivary CXCL13 was significantly higher as compared to the corresponding levels in healthy controls (p < 0.001). Using cutoff of 43.03 pg/ml obtained by ROC, serum CXCL13 positivity was seen in 31/43(72.1%) cases and 10/34 (29.4%) controls, respectively. Serum CXCL13 levels among pSS patients on treatment, treatment naïve patients and healthy controls were statistically different. Serum CXCL13 positivity was associated with oral symptoms (p = 0.02), ocular signs (p = 0.03) and hyperglobulinemia (p = 0.01). There was no association of salivary CXCL13 level with any of the clinical variables. While serum CXCL13 was elevated in pSS, salivary CXCL13 was not. In conclusion, serum CXCL13 positivity was found to be associated with oral symptoms, ocular signs and hyperglobulinemia in pSS.

Validation of the ACR-EULAR criteria for primary Sjögren's syndrome in a Dutch prospective diagnostic cohort.

Objectives: To validate the ACR-EULAR classification criteria for primary SS (pSS), and compare them to the American-European Consensus Group (AECG) and ACR criteria in a Dutch prospective diagnostic cohort. Methods: Consecutive patients (n = 129) referred for suspicion of pSS underwent a multidisciplinary evaluation, including a labial and/or parotid gland biopsy. Patients with an incomplete work-up (n = 8) or associated systemic auto-immune disease (n = 7) were excluded. The ACR-EULAR classification was compared with expert classification, AECG and ACR classification. Additionally, the accuracy of individual ACR-EULAR items in discriminating pSS from non-pSS was evaluated. The validity of criteria sets was described separately using parotid or labial gland biopsy results for classification. Results: Of the 114 evaluated patients, the expert panel classified 34 (30%) as pSS and 80 (70%) as non-pSS. Using labial gland biopsy results, ACR-EULAR classification showed 87% absolute agreement (κ = 0.73) with expert classification, with a sensitivity of 97% and specificity of 83%. Using the parotid gland biopsy results, the ACR-EULAR criteria performed excellently as well. Focus score, anti-SSA titre and ocular staining score showed good to excellent accuracy, whereas unstimulated whole saliva and Schirmer's test had poor accuracy. The ACR-EULAR and AECG criteria had equal validity. Compared with ACR classification, ACR-EULAR classification showed higher sensitivity but lower specificity. Conclusion: The ACR-EULAR criteria showed good agreement with expert classification, but some patients may be misclassified as pSS. Unstimulated whole saliva and Schirmer's test showed poor discriminative value. The ACR-EULAR criteria performed equally to the AECG criteria, and had higher sensitivity but lower specificity than the ACR criteria.

Investigation of autoantibodies to SP-1 in Chinese patients with primary Sjögren's syndrome.

In order to evaluate autoantibody to SP-1 as an early biomarker in pSS, we investigated autoantibody to SP-1 in Chinese patients with primary Sjögren's syndrome (pSS). Autoantibodies to SP-1 are significantly increased in pSS patients compared to RA patients, SLE patients, and healthy people without secondary SS. The presence of anti SP-1 antibodies was negatively correlated with the focus score (FS), RF, and salivary gland function. It was positively correlated with FS=0, RF≤20, and normal salivary gland function. In further studies, the autoantigen SP-1 was identified in ductal epithelia of salivary glands in il14α TG mice by IIF. SP-1 mRNAs expression increased with growing age in il14α TG mice. SP-1 mRNA was also identified in labial biopsies of patients with pSS. In conclusion, autoantibody to SP-1 is an early marker in pSS. It is useful to diagnose pSS patients who lack RF or antibodies to Ro/La.

Influence of geolocation and ethnicity on the phenotypic expression of primary Sjögren's syndrome at diagnosis in 8310 patients: a cross-sectional study from the Big Data Sjögren Project Consortium.

OBJECTIVES: To analyse the influence of geolocation and ethnicity on the clinical presentation of primary Sjögren's syndrome (SjS) at diagnosis. METHODS: The Big Data Sjögren Project Consortium is an international, multicentre registry designed in 2014. By January 2016, 20 centres from five continents were participating. Multivariable logistic regression analyses were performed. RESULTS: We included 7748 women (93%) and 562 men (7%), with a mean age at diagnosis of primary SjS of 53 years. Ethnicity data were available for 7884 patients (95%): 6174 patients (78%) were white, 1066 patients (14%) were Asian, 393 patients (5%) were Hispanic, 104 patients (1%) were black/African-American and 147 patients (2%) were of other ethnicities. SjS was diagnosed a mean of 7 years earlier in black/African-American compared with white patients; the female-to-male ratio was highest in Asian patients (27:1) and lowest in black/African-American patients (7:1); the prevalence of sicca symptoms was lowest in Asian patients; a higher frequency of positive salivary biopsy was found in Hispanic and white patients. A north-south gradient was found with respect to a lower frequency of ocular involvement in northern countries for dry eyes and abnormal ocular tests in Europe (OR 0.46 and 0.44, respectively) and Asia (OR 0.18 and 0.49, respectively) compared with southern countries. Higher frequencies of antinuclear antibodies (ANAs) were reported in northern countries in America (OR=1.48) and Asia (OR=3.80) while, in Europe, northern countries had lowest frequencies of ANAs (OR=0.67) and Ro/La (OR=0.69). CONCLUSIONS: This study provides the first evidence of a strong influence of geolocation and ethnicity on the phenotype of primary SjS at diagnosis.

Association of Sjögrens Syndrome in Patients with Chronic Hepatitis Virus Infection: A Population-Based Analysis.

OBJECTIVE: The association between Sjögren's syndrome (SS) and chronic hepatitis virus infection is inconclusive. Hepatitis B (HBV) and hepatitis C virus (HCV) infections are highly prevalent in Taiwan. We used a population-based case-control study to evaluate the associations between SS and HBV and HCV infections. MATERIALS AND METHODS: We identified 9,629 SS patients without other concomitant autoimmune diseases and 38,516 sex- and age-matched controls without SS from the Taiwan National Health Insurance claims data between 2000 and 2011. We utilized multivariate logistic regression to estimate the odds ratios (ORs) and 95% confidence intervals (CIs) of the associations between SS and HBV and HCV infections. Sex- and age-specific (<55 and ≥55 years) risks of SS were evaluated. RESULTS: The risk of SS was higher in patients with HCV than in those without chronic viral hepatitis (OR = 2.49, 95% CI = 2.16-2.86). Conversely, HBV infection was not associated with SS (OR = 1.10, 95% CI = 0.98-1.24). Younger HCV patients were at a higher risk for SS (<55 years: OR = 3.37, 95% CI = 2.62-4.35; ≥55 years: OR = 2.20, 95% CI = 1.84-2.62). Men with HCV were at a greater risk for SS (women: OR = 2.26, 95% CI = 1.94-2.63; men: OR = 4.22, 95% CI = 2.90-6.16). Only men with chronic HBV exhibited a higher risk of SS (OR = 1.61, 95% CI = 1.21-2.14). CONCLUSION: HCV infection was associated with SS; however, HBV only associated with SS in men.

The EULAR Sjögren's Syndrome Patient-Reported Index is an independent determinant of health-related utility values of Korean patients with primary Sjögren's syndrome.

OBJECTIVES: This study was undertaken to evaluate the health-related quality of life (HRQoL) in patients with primary Sjögren's syndrome (pSS) and to identify its predictors among various clinical parameters. METHODS: The EuroQoL-5 dimension (EQ-5D) was used to measure the patients' HRQoL. The utility values of 178 patients with pSS enrolled in a prospective pSS cohort in Korea were analysed and compared with the Korean normative data. The associations among the clinical parameters and utility values were evaluated. RESULTS: The mean utility value of the pSS patients was significantly lower than that of the Korean general population (0.773±0.138 vs. 0.944±0.095, p<0.001). The proportion of patients with problems in the 4 dimensions was significantly higher in the pSS patients than in the general population (anxiety/depression 70.2 vs. 24.2%, pain 78.7 vs. 28.8%, usual activities 37.6 vs. 9.8%, and mobility 40.4 vs. 12.5%, p<0.001). Bivariate correlation analyses revealed that the degree of pain, fatigue, and patient global assessment of the disease was positively correlated with the utility value. The xerostomia inventory score, ocular surface disease index, and the EULAR Sjögren's syndrome patient-reported index (ESSPRI) also correlated with the utility value. On multiple regression analysis, only the ESSPRI remained in the model after stepwise selection adjusted for age and sex (coefficient ß =-0.053, p<0.001). CONCLUSIONS: The HRQoL of pSS patients is significantly lower than that of the general population, and the ESSPRI is an independent predictor of the HRQoL in pSS patients.

Primary Sjögren syndrome in Han Chinese: clinical and immunological characteristics of 483 patients.

The epidemiological characteristics of Sjögren syndrome (SS) are significantly varied in different countries. We conducted the present study to survey the epidemiological characteristics of primary SS in China. We recruited 483 primary SS patients from 16 Chinese medical centers nationwide from January 2009 to November 2011 and assessed salivary and lacrimal gland dysfunction, organ involvement, and autoimmunity in these patients. The cohort included 456 women and 27 men (ratio, 17:1; mean age at onset, 42 ±â€Š11 years; median age at diagnosis, 49 years; range, 41-56 years). Male patients showed a lower frequency of xerophthalmia (37.0% vs 60.7%) and a higher frequency of arthritis (40.7% vs 16.4%). Young-onset patients showed a higher frequency of low C3 levels (57.7% vs 36.3%) and pancytopenia (22.2% vs 8.8%). Patients with systemic involvement had a higher frequency of immunoglobulin A (IgA) (39.4% vs 22.5%) and immunoglobulin M (IgM) (12.4% vs 37.9%). Patients with pulmonary involvement had a higher parotid enlargement (21.4% vs 10.2%), purpura (12.1% vs 5.7%) and higher anti-La/SS-B (61.7% vs 41.8%), immunoglobulin G (IgG) (80.7% vs 64.6%) and IgA (48.9% vs 30.6%) levels. Patients with anti-Ro/SSA antibodies had more frequent exocrine gland symptoms and some extraglandular symptoms and immunological alterations. Compared with previous studies performed in other countries, SS patients in China showed particular clinical manifestation, systemic involvement, and immunological alterations.

Hepatitis C virus infection and the risk of Sjögren or sicca syndrome: a meta-analysis.

Previous studies have suggested an association between hepatitis C virus (HCV) infection and the development of Sjögren's syndrome (SS), also known as sicca syndrome. The main objective of this study was to summarize the existing evidence and quantitatively evaluate the association between hepatitis C virus infection and SS/sicca syndrome by performing a meta-analysis of observational studies. MEDLINE and PubMed (January 1980-August 2013) were searched to identify relevant studies in English. Outcomes were calculated and are reported as odds risk (OR) and 95% CIs based on a random-effects model. Heterogeneity was assessed with I(2) statistics. Quality assessment was performed with the Newcastle-Ottawa scale. Based on meta-analysis of five cross-sectional and five cohort studies, a significant positive relationship between HCV infection and development of SS/sicca syndrome was found, the pooled random effects OR being 3.31 (95% CI, 1.46-7.48; P < 0.001). In subset analyses, the studies that used European diagnostic criteria showed a higher summary OR than did studies that adopted other diagnostic criteria. When the data were stratified by source of controls, significant associations were also observed when healthy people (OR = 9.44; 95% CI = 2.67-33.40; P = 0.204) or subjects with hepatitis B virus infection (OR = 6.57; 95% CI = 1.21-35.57; P = 0.5) were used as controls, but not when the controls were hospital-based (OR = 0.99; 95% CI = 0.61-1.61; P = 0.169). In summary, the findings suggest that HCV infection is associated with SS/sicca syndrome. The observed increased risk in studies in which European diagnostic criteria and healthy controls were used and the decreased risk in studies with hospital-based controls may be attributable to selection bias or other unknown factors.

Epidemiology of primary Sjögren's syndrome in a French multiracial/multiethnic area.

OBJECTIVE: To describe the epidemiology of primary Sjögren's syndrome (SS) in a multiracial/multiethnic population. METHODS: A cross-sectional study with 5 case-retrieval sources identified adults with primary SS living in the Greater Paris area (population 1,172,482 adults) in 2007. Diagnoses were verified by the American-European Consensus Group (AECG) criteria and study-specific enlarged criteria based on the presence of ≥3 of 4 AECG items among subjective oral or ocular dryness, anti-SSA/SSB positivity, and positive minor salivary gland biopsy results. Prevalence estimates were standardized to those for the world population and a 5-source capture-recapture analysis (CRA) was used. Racial/ethnic differences in primary SS features were evaluated. RESULTS: In all, 133 subjects met the AECG criteria and 203 met the enlarged criteria. The 2007 prevalence of primary SS was 1.02 cases per 10,000 adults (95% confidence interval [95% CI] 0.85-1.22) for the AECG criteria and 1.52 cases per 10,000 adults (95% CI 1.30-1.76) for the enlarged criteria. The CRA indicated completeness of case findings of ∼90%. Compared to subjects with European backgrounds, those with non-European backgrounds had 2.1-2.3 times higher primary SS prevalence and were younger (P < 0.0001) and were more likely to have polyclonal hypergammaglobulinemia (P < 0.0001) and anti-SSA/SSB antibodies (P = 0.0005 and P < 0.0001 for the AECG and enlarged criteria, respectively). CONCLUSION: The figure of 1.02­1.52 cases per 10,000 adults we found and estimates from the few other population-based census surveys support that the prevalence of diagnosed primary SS is between 1 and 9 cases per 10,000 (0.01-0.09%) [corrected] in the general population. Non-European race/ethnicity may be associated with increased primary SS risk and a distinct disease profile.

Polymorphisms in the FAM167A-BLK, but not BANK1, are associated with primary Sjögren's syndrome in a Han Chinese population.

OBJECTIVES: Primary Sjögren's syndrome (pSS) is an autoimmune disease with a complex genetic background. Single nucleotide polymorphisms (SNPs) in the BANK1 and FAM167A-BLK genes have been associated with multiple autoimmune diseases. In this study, we investigated whether SNPs in the BANK1 (rs4522865, rs17266594, and rs10516487) and in the FAM167A-BLK region (rs2736340, rs13277113) could be associated with pSS in Chinese Han. METHODS: Blood DNA was extracted from 540 patients with pSS and 577 healthy controls, and genotyped using the Sequenom MassArray system. RESULTS: There was no significant association between the polymorphisms of BANK1 and pSS. However, the frequency of Pss patients with the T allele (rs2736340) and A allele (rs13277113) of the FAM167A-BLK region was higher than that in the controls (p=0.034; p=0.026 respectively). Genotype and haplotype frequencies of these two SNPs (rs2736340 and rs13277113) between the patients and healthy controls were also significantly different. In addition, associations were observed between the two SNPs and the patients negative for anti-LA/SSB antibodies (p=0.036 and p=0.031 respectively). There was no epistatic interaction between the SNPs in the BANK1 and FAM167A-BLK region. CONCLUSIONS: Our results indicated that the SNPs (rs2736340, rs13277113) of the FAM167A-BLK region, but not the BANK1 SNPs (rs4522865, rs17266594, and rs10516487), were associated with the development of pSS in Han Chinese.

Association of TNFSF4 polymorphisms with susceptibility to primary Sjögren's syndrome and primary biliary cirrhosis in a Chinese Han population.

OBJECTIVES: We aimed to evaluate the association between polymorphisms of TNFSF4 and primary Sjögren's syndrome (pSS) and primary biliary cirrhosis (PBC) in a Chinese Han population. METHODS: A total of 250 pSS patients, 221 PBC patients, and 393 healthy controls were enrolled. All individuals were ethnic Chinese Han, and each group was matched for gender ratio and age. We identified single nucleotide polymorphisms (SNPs) via the HapMap Han Chinese Beijing databank for a genetic region containing TNFSF4, and then identified haplotype tagging SNPs with the Tagger programme of Haploview. DNA samples were amplified through polymerase chain reaction (PCR) and extension products were differentiated via mass spectrometry. Association analyses were performed using PLINK software. RESULTS: In TNFSF4, T allele and TT genotype of rs2205960, and G allele of rs1234313, were associated with pSS (p<0.05); T allele of rs2205960 was correlated with PBC (p<0.05) as a risk factor. In the haplotype analysis, TAGG and TGGT were correlated with pSS (p<0.05). In genetic additive, dominant, and recessive models analysis, rs2205960 had a significant association with both pSS and PBC, and rs1234313 presented a significant association with pSS (p<0.05). However, no statistically significant difference was found after Bonferroni corrections. CONCLUSIONS: Overall, no association between the allele, or genotype, or haplotype frequencies of TNFSF4 and the risk of pSS or PBC was found. TNFSF4 may have little significance as a common genetic component of pSS and PBC in the Chinese Han population.

The prevalence and the clinical relevance of anti-Ro52 in Korean patients with primary Sjögren's syndrome.

So far, there was no report on the prevalence and clinical relevance of anti-Ro52 in primary Sjögren's syndrome (pSS) patients in Korea. In this study, we investigated the prevalence and the clinical relevance of anti-Ro52 in Korean patients with pSS. We retrospectively reviewed the medical records of 96 patients with pSS. On the first visit clinical manifestations, laboratory features and autoantibodies were assessed. We divided subjects into 4 groups according to the presence of anti-Ro60 or anti-Ro52 and investigated the association between those autoantibodies and clinical manifestations. Anti-Ro52 (66.7%) was the most frequently detected autoantibody, followed by anti-Ro60 (52.1%) and anti-La (49.0%). Patients with anti-Ro52 had higher frequency of liver and muscle involvements than those without, while anti-Ro60 exhibited negative association with liver involvement. Anti-Ro52 showed significant relative risk for liver involvement (OR = 5.987, P = 0.038, 95% CI = 1.109-32.326), while anti-Ro60 showed inverse relative risk for liver involvement (OR = 0.122, P = 0.003, 95% CI = 0.031-0.479). Anti-Ro52 also showed significant OR for muscle involvement (OR = 9.533, P = 0.044, 95% CI = 1.059-85.793). In conclusion, anti-Ro52 was the most frequently detected autoantibody except ANA in patients with pSS in Korea. Anti-Ro52 was significantly associated with liver and muscle involvements, while anti-Ro60 was inversely associated with liver involvement in Korean patients with pSS.

Primary Sjögren's syndrome in Moroccan patients: characteristics, fatigue and quality of life.

Our aim was to evaluate fatigue and quality of life (QoL) in Moroccan patients with primary Sjögren's syndrome (PSS) and determine their correlates with disease-related parameters. Fifty-seven consecutive patients with PSS according to the American-European Consensus group (AEGG) criteria were included. Demographic, clinical, biological and immunological characteristics for all patients were collected. Xerostomia was demonstrated by histological grading of lower lip glandular biopsy. A Schirmer test was performed to measure lachrymal flow. Oral, ocular, skin, vaginal and tracheal dryness were evaluated by using a visual analogue scale (VAS). Fatigue was assessed by the Multidimensional assessment of fatigue (MAF) and the QoL by using the generic instrument: SF-36. 90% of our patients were women. The mean age of patients was 53.73 ± 7.69 years, and the mean disease duration was 5.38 ± 4.11 years. The mean oral dryness was 68.38 ± 20.29, and the mean ocular dryness was 51.91 ± 14.03. The mean total score of the MAF was 26.73 ± 8.33, and 87.5% of our patients experienced severe fatigue. Also, physical and mental domains of QoL were altered in a significant way, and the severity of fatigue had a negative impact on SF-36 scores. MAF and SF-36 scores were correlated with the delay of diagnosis, the intensity of xerostomia and the activity of joint involvement. A low socioeconomic and educational level had a negative impact on fatigue scores and QoL. Histological grading of lower lip glandular biopsy, immunological status and the severity of systemic involvement had no correlations with fatigue scores or the alteration of QoL. Patients receiving antidepressant have lesser fatigue and those receiving Methotrexate have better SF-36 scores. In our data, there was a high prevalence of fatigue in Moroccan patients with PSS associated with altered QoL. Severe fatigue and reduced QoL seem to be related to the severity of joint involvement, xerostomia and both educational and socioeconomic levels. Also, treatment with methotrexate and antidepressant seems to improve patients' living and QoL. An appropriate therapeutic intervention for depression and articular manifestations in PSS should be applied to improve patients' living.