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In the first case, the diagnosis of cutaneous lupus was based on the presence of non-specific lupus features such as biopsy-proven vasculitis, leg ulcers, and anonychia. Late-onset lupus is characterized by an onset after 50 years of age. Our initial clinical suspicion was urticarial vasculitis, but the biopsy ruled out leucocytoclastic vasculitis and revealed a classic histology of specific cutaneous lupus lesions. [Extracted from the article]

Objective: The presence of anti-citrullinated protein antibodies (ACPAs) in primary Sjögren's syndrome (pSS) ranges from 3% to 9.9%; however, there is no agreement about their clinical significance. Our aim was to systematically review the literature regarding the association of arthritis and ACPAs in pSS and their role in the development of rheumatoid arthritis (RA).Method: A comprehensive search of MEDLINE, ISI Web of Knowledge, and Cochrane Library from inception until June 2016 was undertaken using the combination of two or three of the keywords: primary Sjögren's syndrome, Sjögren's syndrome, arthritis, synovitis, arthropathy, anti-cyclic citrullinated peptide antibodies, and anti-citrullinated protein antibody - ACPA. No language restriction was used. Studies were included if they: assessed the association of arthritis and ACPAs, had sufficient data to construct a two-by-two table, tested immunoglobulin G ACPA by any method, and included patients with pSS according to a validated set of classification criteria. We used a random effects model and evaluated the heterogeneity and publication bias.Results: Ten studies were included (involving 1322 patients). We found a pooled odds ratio of 4.42 (95% confidence interval 1.15-16.94, p = 0.03). The test for heterogeneity was I2 = 0.87. Publication bias was not observed. Based on data from three studies, 33 of 58 pSS patients with ACPAs (57%) developed RA compared with none of 598 pSS patients with negative ACPA (p < 0.000001).Conclusion: Patients with pSS disclosing ACPAs are prone to arthritis as part of the clinical spectrum of the disease, but are also at risk of developing RA. [ABSTRACT FROM AUTHOR]

The article focuses on the exercise intervention to relieve hip osteoarthritis of the patients in Australia. The exercise involves strengthening the hip parts by having aerobic form of exercise. Patients undergo several sessions which last for 30 minutes each sessions. However, passive mobilization or postoperative exercise therapy was excluded.

Objective: To evaluate the progression of coronary artery calcification (CAC) and associated risk factors in a systemic lupus erythematosus (SLE) cohort., Methods: We reassessed the presence of CAC in patients with SLE who were screened 9 years before, using multidetector computed tomography. Clinical variables (cumulated disease activity and damage accrual), antiphospholipid syndrome and SLE serology, and cardiovascular (CV) risk factors (hypertension, BMI [kg/m 2 ], modified Framingham risk score, lipid profile, menopausal status) were assessed longitudinally., Results: We included 104 patients from the parent study. Most of them were women (94.2%), with a mean age of 41.0 (SD 8.3) years and mean disease duration of 14.8 (SD 2.9) years. We documented CAC in 17 patients (16.3%). Seven cases were from the parent study and 10 were incident cases. The cumulative incidence of CAC was 9% and the incidence density was 1 per 100 person-years. CAC occurred more frequently in the age groups 30-39 years and 40-44 years. All patients with previous CAC had worsening of their calcium indexes, and none developed clinical CV events. When comparing prevalent CAC cases (n = 17) vs patients without calcification (n = 87), both groups were similar in traditional CV risk factors, disease duration, Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) area under the curve (AUC), and Systemic Lupus International Collaborating Clinics (SLICC) score, but were more likely to be postmenopausal and have higher apolipoprotein B (apoB) levels. Patients with previous CAC had higher apoB levels, SLEDAI-2K AUC scores, and anticardiolipin IgG antibodies than incident cases., Conclusion: CAC in patients with SLE progressed over time but was not associated with adverse CV events during the first 9 years of follow-up. ApoB levels and postmenopausal status might be associated with this progression., (Copyright © 2024 by the Journal of Rheumatology.)

Objectives: To evaluate whether the grade of IgG4+ plasma cell infiltration in biopsies is associated with clinical or serologic outcomes in IgG4-RD., Methods: We included 57 patients with biopsy proven IgG4-RD according to the Comprehensive Diagnostic Criteria and/or the 2019 ACR/EULAR Classification Criteria. We collected histological, clinical (disease duration, phenotype, remission and relapses) and serological variables., Results: 29 (50.9%) patients were men, mean age 49.9 years, with a median disease duration of 22 months. The distribution among clinical phenotypes were 14% pancreato-hepato-biliary, 12.3% retroperitoneal/aortic, 29.8% head and neck-limited, 29.8% Mikulicz/systemic and 14% undefined. Thirty-nine patients had a proliferative and 18 a fibrotic phenotype. Most biopsies were from lacrimal gland, lymph node, pancreas, orbit, kidney, retroperitoneum and thyroid gland. Thirty-nine (68.4%) patients had <100 IgG4+ plasma cells/HPF and 18 (31.6%) ≥100 IgG4+ plasma cells/HPF. Patients with ≥100 IgG4+ plasma cells/HPF were more likely to belong to the pancreato-hepato-biliary and the proliferative phenotypes, had fewer relapses and a higher remission rate. On multivariate analysis, the OR for remission at last follow-up was 6.7, 95% CI 1.1-4.42, p=0.03. The log-rank test showed a difference in relapse-free survival between the two groups (HR 2.6, 95% CI 1.2-5.6, p=0.01). According to the ROC analysis, patients with more than 61 IgG4+ plasma cells were less likely to relapse., Conclusions: A count of ≥100 IgG4+ plasma cells/HPF may identify patients with a proliferative phenotype, fewer relapses and a higher remission rate.

Objective: To assess the prevalence of systemic and organ-specific autoimmunity among individuals with human inborn errors of immunity (IEI)., Methods: Retrospective study. We recorded demographic variables, type of immunodeficiency, and systemic and organ specific autoimmunity., Results: We included 48 patients (54.1% men) with mean age of 32.1 years. The most common IEIs included combined immunodeficiency with syndromic features (31.2%) and predominantly antibody deficiency (20.1%). We observed autoimmunity in 15 patients (31.2%): 12 organ-specific autoimmunity and 5 systemic autoimmunity, not mutually exclusive groups. Organ-specific autoimmunity preceded the onset of IEI in 5 patients, was concurrent in one patient, and developed after the diagnosis of IEI in 6 cases. From the systemic autoimmunity group, we observed polyarteritis nodosa (n = 2), antiphospholipid syndrome (APS) (n = 2), and overlap of limited systemic sclerosis/APS/Sjögren's syndrome (n = 1), and in all cases, this occurred after the IEI diagnosis., Conclusion: Our findings confirm the coexistence of autoimmunity and IEI. This overlap may be attributed to B and T cell disorders, as well as potential alterations in the microbiota in these patients., (Copyright © 2024 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.)

Abstract: A 65-year-old woman presented with unexplained weight loss, recurrent fever, and a dermatosis with painful nodules on the extremities. Biopsies showed focal lobular panniculitis with neutrophilic microgranulomas. Comprehensive investigations ruled out infection and hematologic and solid organ neoplasms. Laboratory results showed anti-Ro/SSA and anti-La/SSB antibody positivity, and elevated inflammatory markers. Dry mouth and eye were confirmed. The diagnosis of Sjögren syndrome with cutaneous panniculitis was established. Prednisone treatment with 30 mg/d resulted in remission of fever and pain improvement. This case emphasizes Sjögren syndrome as an autoimmune disease with multiple cutaneous manifestations and highlights its association with granulomatous panniculitis., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Objective: The objective is to assess the performance of the 2023 American College of Rheumatology/EULAR classification criteria (2023 AECC) for antiphospholipid syndrome (APS) in a Mexican cohort., Methods: We enrolled patients with primary APS (PAPS) and secondary APS (SAPS) and a control group of nonautoimmune thrombophilia. We evaluated the fulfillment of the 2023 AECC and the 2006 revised Sapporo classification criteria (2006 RSCC) and their performance against the clinical diagnosis as the gold standard. The baseline Global APS Score (GAPSS) and the Damage Index for APS (DIAPS) at last follow-up were calculated., Results: We included 85 patients with PAPS, 54 with SAPS, and 50 with thrombophilia. According to the 2023 AECC criteria, 69 patients (81.2%) with PAPS, 28 patients (51.9%) with SAPS, and none of the patients with thrombophilia met the criteria. When comparing true positive (TP) (n = 69) versus false negative (n = 16) cases within the PAPS group, TP cases exhibited a higher frequency of thrombotic manifestations and IgM anti-cardiolipin and IgG anti-β2-glycoprotein-I positivity. For PAPS, there was a correlation between the 2023 AECC score and both GAPSS (rho = 0.621, P < 0.0001) and DIAPS scores (rho = 0.433, P < 0.0001). When comparing the 2023 AECC with the 2006 RSCC, a lower sensitivity (81.2% vs 88.2%) but a higher specificity (100.0% vs 92.0%) was observed for PAPS. Similar findings were observed in SAPS., Conclusion: In both PAPS and SAPS, the 2023 AECC have higher specificity than the 2006 RSCC. The main feature of patients with PAPS according to the 2023 AECC was thrombosis. These criteria might identify patients at higher risk of thrombosis and damage accrual., (© 2024 American College of Rheumatology.)

Objectives: We aimed to evaluate the prevalence of non-criteria clinical features in patients with primary antiphospholipid syndrome (APS), and to assess their relationship to thrombosis and damage., Methods: We retrospectively included 177 primary APS patients, and/or patients who only achieved the serological Sydney criteria but had thrombocytopenia and/or haemolytic anaemia. We registered demographics, serology, treatment, thrombotic/obstetric manifestations and non-criteria clinical manifestations (cutaneous, haematologic, renal, heart valve disease, and neurological). We scored the DIAPS and a modified SLICC index. We used logistic regression and reported OR with 95% CI., Results: 78% were women with a median follow-up of 6.7 years. Thrombosis was found in 74% of patients, 29.3% had obstetric features, and 64% had non-criteria clinical manifestations. The frequency of the non-criteria clinical manifestation was: haematologic 40.1%, cutaneous 20.9%, neurologic 18%, cardiac 5% and renal 4.5%. Non-criteria features were associated with LA (OR 2.3, 95% 1.03-5.1) and prednisone use (OR 8.2, 95% CI 1.7-39.3). A DIAPS score ≥1 was associated with thrombosis (OR 53.1, 95% CI 17.8-15.2), prednisone use (OR 0.27, CI 95% 0.09-0.83) and neurological involvement (OR 6.4, 95% CI 1.05-39.8); whereas a modified SLICC ≥ 1 with thrombosis (OR 10.2; IC 95% 4.43-26.1), neurological involvement (OR 6.4, 95%CI 1.05-39.8), obstetric features (OR 0.32 CI 95% 0.12-0,81) and cutaneous features (OR 5.3, CI 95% 1.4-19), especially livedo reticularis (OR 5.45; IC 95% 1.49-19.8)., Conclusions: Non-criteria clinical manifestations are common and associated with LA. Among them, neurologic involvement and the presence of livedo were associated with damage accrual.

Background/objective: The 2019 American College of Rheumatology/European League Against Rheumatism Classification Criteria (2019 AECC) for IgG4-related disease (IgG4-RD) is considered a significant advancement in the study of this condition. Most studies evaluating their performance have focused on White and Asian patients, leaving a knowledge gap regarding Latin American populations. Therefore, this study aimed to assess the performance of the 2019 AECC for IgG4-RD in a cohort of Latin American patients., Methods: A multicenter medical records review study was conducted, involving centers from Argentina, Chile, Mexico, Peru, and Uruguay. Data on IgG4-RD patients and mimicker conditions were collected through a standardized online form. The criterion standard for diagnosing IgG4-RD was based on the fulfillment of the Comprehensive Diagnostic Criteria for IgG4-RD and/or the Consensus Statement on Pathology. The 2019 AECC was retrospectively applied., Results: We included 300 patients, with 180 (60%) having IgG4-RD and 120 (40%) having mimicker conditions. The 2019 AECC had a sensitivity of 66.7% and a specificity of 100%. Sensitivity increased to 73.3% when disease-specific autoantibody items were removed, without affecting specificity. The true-positive cases had more involved organs, a higher availability of biopsy results, and were more likely to belong to the Mikulicz/systemic and proliferative phenotypes., Conclusions: The use of the 2019 AECC for IgG4-RD in a Latin American population confirms its high specificity in excluding those without the disease. The presence of concomitant autoimmune diseases and clinically nonsignificant disease-specific autoantibodies excludes a significant number of patients from fulfilling the criteria., Competing Interests: The authors declare no conflict of interest., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Objectives: The aim of our study was to investigate whether TNFAIP3, PTPN22, and TRAF1-5 single nucleotide polymorphisms (SNPs) are associated with susceptibility, severity, or serological markers in primary Sjögren's syndrome (pSS)., Patients and Methods: The cases and controls study was conducted between December 2021 and June 2022. TNFAIP3 rs10499194C/T, rs6920220G/A, and rs2230926T/G, PTPN22 rs2476601C/T and rs33996649G/A, and TRAF1-C5 rs10818488G/A polymorphisms were genotyped in 154 female pSS patients (mean age: 45.2±6.8 years) and 313 female control subjects (mean age: 50.3±7.5 years) using the TaqMan® SNP genotyping assay. An association analysis between TNFAIP3, PTPN22, and TRAF1-C5 SNPs and susceptibility, clinical characteristics, and serological markers of pSS was performed. Interactions between TNFAIP3, PTPN22, and TRAF1-C5 SNPs were also evaluated in patients and controls., Results: The genotype and allele frequencies showed no association with susceptibility, severity, or serological markers of pSS. Nevertheless, several interactions between TNFAIP3 and TRAF1-C5 or TNFAIP3, PTPN22, and TRAF1-C5 genotypes were associated with susceptibility to pSS (p<0.01)., Conclusion: Individual TNFAIP3, PTPN22, and TRAF1-C5 SNPs are not associated with susceptibility, severity, or serological markers of pSS. However, genetic interactions between TRAF1-C5 and TNFAIP3 or TNFAIP3, PTPN22, and TRAF1-C5 SNPs are risk factors for pSS., Competing Interests: Conflict of Interest: The authors declared no conflicts of interest with respect to the authorship and/or publication of this article., (Copyright © 2024, Turkish League Against Rheumatism.)

Objectives: To describe the taxonomy of the microbiota in crevicular fluid of primary Sjögren's syndrome (pSS) patients, and evaluate its association with clinical/serological variables, and oral quality of life., Methods: Observational study that included 48 pSS without diabetes mellitus, no active neoplasia, no antibiotic use in the previous two weeks, and no current active infection. We registered demographics, oral/ocular sicca symptoms, parotid enlargement and anti-Ro/La serology. We assessed the non-stimulated whole salivary flow (NSWSF), the EULAR Sjögren's Syndrome Patient Reported Index (ESSPRI), and the Xerostomia-related Quality of Life Scale (XeQoLS). Two periodontists determined the presence of periodontal disease and collected crevicular fluid from 6 teeth using filter paper. Samples were frozen at -86°C until processing. We included 17 sex- and age-matched control subjects. Bacterial DNA was extracted from the crevicular fluid sample using a commercial kit. 16SrRNA V3-V4 region was sequenced using reversible adaptor technology. Sequences were pre-processed and analysed using QIIME2 and phyloseq software programs. Functionality profiles were predicted using the Tax4Fun2 package., Results: PSS patients had more bacteria of the genera Prevotella, Streptococcus, Veillonella, Fusobacterium, and Leptotrichia and fewer bacteria of the genus Selenomonas than controls. The pSS microbiota contained more genes encoding accessory secretory proteins. Microbiota also differed between patients with anti-Ro/La status, parotid gland enlargement, and periodontal disease severity, but did not correlate with NSWSF and XeQoLS., Conclusions: The crevicular fluid microbiota of pSS patients and controls differed significantly, even in SSP patients depending on their serology, parotid gland enlargement, and periodontal disease status.

Objectives: To analyse how the key components at the time of diagnosis of the Sjögren's phenotype (epidemiological profile, sicca symptoms, and systemic disease) can be influenced by the potential exposure to climate-related natural hazards., Methods: For the present study, the following variables were selected for harmonisation and refinement: age, sex, country, fulfilment of 2002/2016 criteria items, dry eyes, dry mouth, and overall ESSDAI score. Climate-related hazards per country were defined according to the OECD and included seven climate-related hazard types: extreme temperature, extreme precipitation, drought, wildfire, wind threats, river flooding, and coastal flooding. Climatic variables were defined as dichotomous variables according to whether each country is ranked among the ten countries with the most significant exposure., Results: After applying data-cleaning techniques and excluding people from countries not included in the OECD climate rankings, the database study analysed 16,042 patients from 23 countries. The disease was diagnosed between 1 and 3 years earlier in people living in countries included among the top 10 worst exposed to extreme precipitation, wildfire, wind threats, river flooding, and coastal flooding. A lower frequency of dry eyes was observed in people living in countries exposed to wind threats, river flooding, and coastal flooding, with a level of statistical association being classified as strong (p<0.0001 for the three variables). The frequency of dry mouth was significantly lower in people living in countries exposed to river flooding (p<0.0001) and coastal flooding (p<0.0001). People living in countries included in the worse climate scenarios for extreme temperature (p<0.0001) and river flooding (p<0.0001) showed a higher mean ESSDAI score in comparison with people living in no-risk countries. In contrast, those living in countries exposed to worse climate scenarios for wind threats (p<0.0001) and coastal flooding (p<0.0001) showed a lower mean ESSDAI score in comparison with people living in no-risk countries., Conclusions: Local exposure to extreme climate-related hazards plays a role in modulating the presentation of Sjögren across countries concerning the age at which the disease is diagnosed, the frequency of dryness, and the degree of systemic activity.

We aim to assess and compare a cytokine and chemokine profile in tears from patients with IgG4-related disease (IgG4-RD) and Sjögren's syndrome (SS), and to see if this profile could aid in differentiating these two diseases. We included 10 patients with IgG4-RD who met the Comprehensive Diagnostic Criteria for IgG4-RD and 17 patients who met the AECG criteria for primary SS. The Schirmer-I test was carried out using two standardized sterile tear strips, which were then immediately frozen at - 86 °C until assayed. The tears were extracted from the strips after they had been defrosted using a buffer containing 0.5 M NaCl and 0.5% Tween-20. The amounts (pg/ml) of the following cytokines and chemokines were then measured using luminometry: IFN-γ, TNF-α, G-CSF, IL-1-α, IL-1β, IL-4, IL-7, IL-12p40, IL-12p70, IL-13, IL-17A, CCL2, CCL3, CCL4, CCL11, and CXCL10. In the IgG4-RD group, seven patients had lacrimal gland involvement, five had dry eye symptoms, and six had a positive Schirmer-I test. In the SS group, 16 (94.1%) had dry eyes and all had a positive Schirmer-I test. We were able to differentiate between both diseases using levels of IL-7, IL-1α, and IL-1β; in particular, the IL-7/IL-1α and IL-7/IL-1β ratios had the best discriminatory potential, with cut-off values of 0.32 (AUC: 0.93, sensitivity: 94%, specificity: 80%, p = 0.0003) and 12.55 (AUC: 0.96, sensitivity: 94%, specificity: 90%, p = 0.0001), respectively. Our results suggest that IL-7, IL-1α, and IL-1β tear levels could help differentiate IgG4-RD from SS. Key Points • The lacrimal gland is frequently involved in IgG4-RD and SS. This characteristic makes both diseases mimics of one another. • Patients with IgG4-RD and SS have different profiles of tear cytokines and chemokines. • Tear IL-7, IL-1α, and IL-1β levels may serve as helpful biomarkers in separating IgG4-RD from SS., (© 2023. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR).)

Rosai-Dorfman disease is characterized by dilated lymph node sinuses filled with lymphocytes, plasma cells, and histiocytes. Many of these histiocytes classically exhibit emperipolesis of lymphocytes and plasma cells. Abundant immunoglobulin G4+ plasma cells occur in some cases, and a potential relationship with immunoglobulin G4-related disease has been suggested. Here, we report 3 cases of immunoglobulin G4-associated Rosai-Dorfman disease. Immunoglobulin G4-related disease was suspected based on immunoglobulin G4+ plasma cell infiltration, but the final diagnosis was immunoglobulin G4-associated Rosai-Dorfman disease. At present, the evidence does not support a link between immunoglobulin G4-associated Rosai-Dorfman disease and immunoglobulin G4-related disease, and one condition should not be considered part of the spectrum of the other. We believe it is of paramount importance to increase the awareness of immunoglobulin G4-associated Rosai-Dorfman disease for pathologists who interpret the biopsies and clinicians who integrate the diagnosis and treat such patients to not overdiagnose immunoglobulin G4-related disease.

Diverse immune cell subsets have been described in IgG4-related disease (IgG4-RD). If there is a different immunophenotype according to clinical phenotype and activity status is not known. Levels of IL-4-, IL-13-, IL-5-, and IL-21-producing CD4 + T cells (Th2 subsets), CD4 + cytotoxic T lymphocytes (CD4 + CTLs), T helper 9 cells, T follicular helper cells (Tfh; Tfh1/Tfh2/Tfh17/Tf regulatory [Tfr]), Foxp3 + regulatory T cells, Type 1 regulatory T cells (Tr1), T helper 3 regulatory cells (Th3), IL-10-producing regulatory B cells (Bregs), IL-10-expressing regulatory plasmacytoid dendritic (pDC IL-10 + ) cells, and M1 and M2 monocytes were determined by flow cytometry in 43 IgG4-RD patients and 12 controls. All immune subsets were higher in patients vs. controls. CD4 + /IL-4 + , CD4 + /IL-5 + , CD4 + CTLs, Tfh2, Tfh17, Tfr, and M1 monocyte cell number was different among IgG4-RD clinical phenotypes. The pancreato-hepato-biliary phenotype was characterized by a higher CD4 + CTLs, Tfh17, Tfh2, and Tfr and lower M1 cell number. An increased CD4 + CTLs and Th3 cell number distinguished the head and neck-limited phenotype, while the retroperitoneal/aortic and Mikulicz/systemic phenotypes were characterized by increased Th2 subsets. Tfh17, Tr1, Th3, pDC, M1, and M2 monocytes were augmented in active patients. In summary, the clinical heterogeneity of IgG4-RD might be driven by the participation of different immunophenotypes and, consequently, by a different fibroinflammatory process.

Background: Rheumatoid arthritis (RA) patients commonly report medicinal cannabis use (MCU). Personality has been independently associated with both RA-related outcomes and MCU, but there is no information available on how they interact in RA patients. This study aimed to investigate a potential association between personality traits and MCU in RA outpatients, as well as to identify additional factors associated with its use., Methods: This cross-sectional study was performed between June 2020 and August 2021. Consecutive RA outpatients had standardized evaluations using an interview format to collect sociodemographic information, comorbidities, risk of recreational substance use, RA-related disease activity/severity, health-related quality of life, depressive and anxiety symptoms, five personality traits, and MCU in the 12 months before the interview. Multivariable logistic regression estimated adjusted odds ratios (aOR). The study was IRB-approved., Results: 180 patients were included; 160 (88.9%) were women with a mean age of 53.4 ± 13 years. Fifty-three (29.4%) patients reported MCU. Among them, 52 (98.1%) used topical formulations. Neuroticism had the highest overall score ([Formula: see text] = 3.47 ± 0.34). Openness to experience trait was higher in MCU patients in the comparative analysis (p = 0.007). In the multivariable regression, higher openness trait (aOR: 2.81, 95%CI: 1.11-7.10) along with moderate risk in tobacco use (aOR: 3.36, 95%CI: 1.04-10.7) and higher RA disease activity/severity (aOR: 1.10, 95%CI: 1.01-1.19) were independently associated with MCU., Conclusions: In the current study, personality influenced the seeking of MCU for pain relief, associating dynamically with higher disease activity/severity and tobacco use. Contrary to other available information, it did not relate to psychopathology or the recreational use of cannabis. Proactive interdisciplinary clinical evaluations around MCU in RA outpatients should include personality, besides standard clinical assessments, to understand patients' motivations for its use as they may reveal important clinical information., Competing Interests: The authors have declared that no competing interests exist., (Copyright: © 2023 Galindo-Donaire et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.)

Objective: To characterize 414 patients with primary SS who developed haematological malignancies and to analyse how the main SS- and lymphoma-related features can modify the presentation patterns and outcomes., Methods: By January 2021, the Big Data Sjögren Project Consortium database included 11 966 patients fulfilling the 2002/2016 classification criteria. Haematological malignancies diagnosed according to the World Health Organization (WHO) classification were retrospectively identified., Results: There were 414 patients (355 women, mean age 57 years) with haematological malignancies (in 43, malignancy preceded at least one year the SS diagnosis). A total of 376 (91%) patients had mature B-cell malignancy, nearly half had extranodal marginal zone lymphoma (MZL) of mucosa-associated lymphoid tissue (MALT lymphoma) (n = 197), followed by diffuse large B-cell lymphoma (DLBCL) (n = 67), nodal MZL lymphoma (n = 29), chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) (n = 19) and follicular lymphoma (FL) (n = 17). Rates of complete response, relapses and death were 80%, 34% and 13%, respectively, with a 5-year survival rate of 86.5% after a mean follow-up of 8 years. There were significant differences in age at diagnosis (younger in MALT, older in CLL/SLL), predominant clinical presentation (glandular enlargement in MALT lymphoma, peripheral lymphadenopathy in nodal MZL and FL, constitutional symptoms in DLBCL, incidental diagnosis in CLL/SLL), therapeutic response (higher in MALT lymphoma, lower in DLBCL) and survival (better in MALT, nodal MZL and FL, worse in DLBCL)., Conclusion: In the largest reported study of haematological malignancies complicating primary SS, we confirm the overwhelming predominance of B-cell lymphomas, especially MALT, with the salivary glands being the primary site of involvement. This highly-specific histopathological scenario is linked with the overall good prognosis with a 5-year survival rate of nearly 90%., (© The Author(s) 2022. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Objectives: To evaluated the total body water (TBW) among patients with primary Sjögren's syndrome (pSS) and assess its correlation with the severity of oral and ocular sicca symptoms, and some objective sicca variables., Methods: We included 85 patients and 85 controls matched by sex, age, and body mass index (BMI). We assessed the Schirmer-I test and the non-stimulated whole salivary flow (NSWSF). We evaluated ocular and oral symptoms during the past 15 days using a 0-10 visual analogue scale (VAS) (highest score=worst symptoms). We obtained the TBW by bioelectric impedance analysis., Results: 80% were women (mean age 54.8 years and mean disease duration 11.5 years). TBW was similar in pSS and controls (46.8±4.6 vs. 46.9±4.5, p=0.88). TBW correlated with age (ρ=-0.25, p=0.02), disease duration (ρ=-0.30, p=0.005), BMI (ρ=-0.78, p=0.001) and ocular VAS scale (ρ=-0.28, p=0.01); but not with NSWSF, Schirmer test or oral VAS scale. When comparing patients in the lowest TBW percentile (≤25%) with the remaining patients, the former group was older, had longer disease duration, higher BMI, lower frequency of anti-Ro/SSA and antinuclear antibodies, and higher ocular VAS scores. In the multivariate analysis, the ocular VAS score (OR 1.88, 95% CI 1.08-3.2, p=0.02) and the BMI 1.92 (OR 1.4, 95% CI 1.4-2.66, p=0.0001) remained associated with a lower TBW percentage., Conclusions: Patients with pSS had similar TBW percentages to control subjects. However, lower TBW percentages in the pSS were associated with higher BMI and also with the most severe ocular symptoms.

Objectives: To investigate the safety and efficacy of SARS-Cov-2 vaccination in patients with primary Sjögren syndrome (pSS) due to scarcity of data in this population., Methods: By the first week of May 2021, all Big Data SS Consortium centres patients who had received at least one dose of any SARS-CoV-2 vaccine were included in the study. The in-charge physician asked patients about local and systemic reactogenicity to collect SARS-CoV-2 vaccination data., Results: The vaccination data of 1237 patients were received. A total of 835 patients (67%) reported any adverse events (AEs), including local (53%) and systemic (50%) AEs. Subjective symptoms (63%) were the most common local AEs, followed by objective signs at the injection site (16%), and general symptoms were the most commonly reported systemic AEs (46%), followed by musculoskeletal (25%), gastrointestinal (9%), cardiopulmonary (3%), and neurological (2%). In addition, 141 (11%) patients reported a significant worsening/exacerbation of their pre-vaccination sicca symptoms and fifteen (1.2%) patients reported active involvement in the glandular (n=7), articular (n=7), cutaneous (n=6), pulmonary (n=2), and peripheral nervous system (n=1) domains due to post-vaccination SS flares. In terms of vaccination efficacy, breakthrough SARS-CoV-2 infection was confirmed after vaccination in three (0.24 %) patients, and positive anti-SARS-Cov-2 antibodies were detected in approximately 95% of vaccinated SS patients, according to data available., Conclusions: Our data suggest that patients with pSS develop adequate humoral response and no severe AEs after SARS-CoV-2 vaccination and therefore raise no concerns about the vaccine's efficacy or safety profile in this population.

Introduction: The objectives of this study were to compare the quality-of-care and compliance with medical record regulations between in-person consultations (QIP and CIP) and telephone consultations (QTP and CTP), from rheumatoid arthritis (RA) outpatients, during the COVID-19 pandemic, and to explore the impact of the consultation modality on the treatment. Methods: Data from 324 medical notes corresponding to rheumatic consultations between July and December 2020 were abstracted. Notes were selected considering a stratified (in-person and telephone consultations) random sampling strategy. QIP, CIP, QTP, and CTP were scored based on prespecified criteria as percentages, where higher numbers translated into better standards. Logistic regression analysis investigated the association between the consultation modality and the treatment recommendation (dependent variable). Results: There were 208 (64.2%) medical notes related to in-person consultations and 114 (35.2%) to telephone consultations. Overall, medical notes corresponded to middle-aged women with long-standing disease. QIP was superior to QTP (median, interquartile range): 60% (60-75%) versus 50% (25-60%), p ≤ 0.001, and differences were related to disease activity and prognosis documentation (81.3% vs. 34.5% and 55.8% vs. 33.6%, respectively, p ≤ 0.001) and the prolonged prescription of glucocorticoids with a documented management plan (58.5% vs. 30.4%, p = 0.045). Meanwhile, CIP and CTP were similar. Telephone consultation was a significant risk factor for no changes in the treatment recommendation (odds ratio: 2.113, 95% confidence interval: 1.284-3.479, p = 0.003), and results were consistent in the 142 medical notes with documented absence of disease activity. Conclusions: In the clinical context of RA, the quality-of-care provided by telephone consultations is below the standard of care and impacts the treatment.

Background/objective: Data on IgG4-related disease (IgG4-RD) come almost exclusively from cohorts from Asia, Europe, and North America. We conducted this study to describe the clinical presentation, phenotype distribution, and association with sex, ethnicity, and serological markers in a large cohort of Latin American patients with IgG4-RD., Methods: We performed a multicenter medical records review study including 184 Latin American IgG4-RD patients. We assigned patients to clinical phenotypes: group 1 (pancreato-hepato-biliary), group 2 (retroperitoneal/aortic), group 3 (head and neck-limited), group 4 (Mikulicz/systemic), and group 5 (undefined). We focused the analysis on how sex, ethnicity, and clinical phenotype may influence the clinical and serological presentation., Results: The mean age was 50.8 ± 15 years. Men and women were equally affected (52.2% vs 48.8%). Fifty-four patients (29.3%) were assigned to group 1, 21 (11.4%) to group 2, 57 (30.9%) to group 3, 32 (17.4%) to group 4, and 20 (10.8%) to group 5. Male sex was associated with biliary tract (odds ratio [OR], 3.4; 95% confidence interval [CI], 1.36-8.26), kidney (OR, 3.4; 95% CI, 1.28-9.25), and retroperitoneal involvement (OR, 5.3; 95% CI, 1.45-20). Amerindian patients presented more frequently with atopy history and gallbladder involvement. Group 3 had a female predominance., Conclusions: Latin American patients with IgG4-RD were younger, and men and women were equally affected compared with White and Asian cohorts. They belonged more commonly to group 1 and group 3. Retroperitoneal and aortic involvement was infrequent. Clinical and serological features differed according to sex, ethnicity, and clinical phenotype., Competing Interests: The authors declare no conflict of interest., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Purpose: To evaluate CCL2, CXCL8, and CXCL10 in the tears of patients with Primary Sjögren's syndrome (PSS) and correlate them with ocular symptoms/discomfort and objective ocular tests., Methods: We studied 21 patients with PSS. A single ophthalmologist, expert in dry eye, examined the patients and assessed tear film breakup time, Schirmer I test, tear meniscus height, Van Bijsterveld staining score and SICCA Ocular Staining Score. We also assessed the ESSPRI and ocular dryness VAS and the Ocular Surface Disease Index (OSDI), a 12-item scale assessing symptoms associated with dry eye disease and their impact on vision (ocular symptoms/discomfort). Tear samples collected with sterile tear flow strips were frozen at -86 °C until testing. After thawing, tears were extracted from the strips. We tested CCL2, CXCL8, and CXCL10 by luminometry. We also included 21 healthy control subjects without a dry eye., Results: CXCL8 levels were similar in patients and controls. PSS patients had lower levels of CXCL10 (472.8 vs. 1652 pg/μL, p = 0.009) and CCL2 (1.08 vs. 9 pg/μL, p = 0.0001) than controls. Patients with worse ocular sicca symptoms/discomfort had the lowest CXCL10 levels (239.3 vs. 646.2 pg/μL, p = 0.02). CCL2 correlated with tear meniscus height (τ = 0.37, p = 0.02) and with OSS (τ = -0.3, p = 0.05)., Conclusions: We found lower levels of CXCL10 and CCL2 in the tears of patients with PSS, associating the former with worse ocular symptoms and the latter with positive ocular target tests., (© 2022. The Author(s), under exclusive licence to Springer Nature B.V.)

Objective: To assess the prevalence of parotid gland swelling (PGS) and its association with features of SS and other causes of sialadenosis in a Latin-American cohort of primary SS., Methods: We included 668 patients from Argentina, Brazil, Mexico and Paraguay. We retrospectively registered demographics, disease duration, oral/ocular symptoms, serology and scored the basal ESSDAI. We defined PGS as a recurrent or persistent increase of volume of any parotid glands during adulthood (self-reported and/or physical examination). We registered the presence of diabetes mellitus, dyslipidaemia, body mass index and alcohol consumption. We used logistic regression analysis reporting odds ratio (OR) and 95% CI., Results: PGS was present in 242 patients (36.2%): 78 previous to SS diagnosis, 86 concomitantly, 73 during follow-up and five unknown. At the multivariate analysis, PGS was associated with RF (OR 2.47, 95% CI: 1.1, 6.5, P = 0.0001), basal articular ESSDAI domain (OR 1.63, 95% CI: 1.01, 2.6, P = 0.04) and alcohol consumption (OR 2.42, 95% CI: 1.41, 4-15). Patients with PGS during the follow-up had a higher prevalence of alcohol consumption (45.3%) compared with the remaining PGS cases (26.8%; OR 2.41 95% CI: 1.2, 4.7), or patients without parotid gland swelling (15.6%; OR 3.8 95% CI: 1.7, 8.2) in all the adjusted models., Conclusion: PGS generally precedes or presents concomitantly with SS diagnosis, and is related to RF and articular activity. Alcohol consumption is an additional factor in PGS, especially during follow-up. The meaning of this last finding as well as its prognostic implications remains to be elucidated and deserves further evaluation in prospective studies., (© The Author(s) 2021. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Purpose: Rheumatoid arthritis (RA) and primary Sjögren's syndrome (pSS) are autoimmune diseases (ADs) characterized by joint damage and involvement of the salivary glands, respectively. ADs share some susceptibility loci, such as TNFSF4, which is a classical susceptibility gene associated with systemic lupus erythematosus, but its role in RA and pSS is not yet clear. Thus, the aim of this study was to determine whether three TNFSFS4 polymorphisms are associated with RA and pSS., Methods: Our case-control study included 500 controls, 459 patients with RA, and 210 patients with pSS from Mexico. TNFSF4 single nucleotide polymorphisms (SNPs) rs1234315C/T, rs2205960G/T, and rs704840T/G were genotyped using TaqMan probes and discrimination allelic assay., Results: The three TNFSF4 SNPs were associated with susceptibility to RA (rs1234315C/T: odds ratio [OR] 1.4, p = 0.01; rs2205960G/T: OR 1.23, p = 0.03; rs704840T/G: OR 1.24, p = 0.02). An association between TNFSF4 rs1234315C/T and pSS was also observed (OR 1.28, p = 0.04), however, after Bonferroni correction, this association was lost., Conclusion: Our data suggest that TNFSF4 could be a risk factor in RA but not pSS in a Mexican population., (Published by Elsevier GmbH.)

Background: The tendency of IgG4-related disease (IgG4-RD) to form pseudotumors, as well as its multisystemic nature, makes it the perfect mimicker of many conditions. Moreover, some clinical, serological, radiological, or histological features of the disease might be shared with some mimickers.Recently, 4 clinical phenotypes have been identified, and patients grouped in each phenotype have distinctive demographic, clinical, and serological features and outcomes, and, as expected, for each phenotype, a set of differential diagnoses should be considered., Summary of the Literature: The main differential diagnoses for the pancreato-hepato-biliary phenotype are pancreatic adenocarcinoma and cholangiocarcinoma. Other differential diagnoses include type 2 autoimmune pancreatitis and primary sclerosing cholangitis. In patients with retroperitoneal/aortic phenotype, inflammatory conditions such as idiopathic retroperitoneal fibrosis and large vessel vasculitides should be ruled out, and most of the time, a biopsy will be needed to exclude malignancies. In head and neck limited phenotype, autoimmune conditions (eg, granulomatosis with polyangiitis, Graves orbitopathy, sarcoidosis), malignancies, and histiocytosis should be ruled out, whereas the main differential diagnoses of the Mikulicz/systemic phenotype are Sjögren syndrome, granulomatosis with polyangiitis, and multicentric Castleman disease., Conclusions: Approaching a patient with probable IgG4-RD through a clinical phenotype framework will ease the diagnostic algorithm and facilitate the prompt recognition of the disease. There are certain clinical, serological, radiological, and histological features in each clinical phenotype that, if present, increase the likelihood that a patient may have IgG4-RD instead of the mimicker condition. Those clues that point toward IgG4-RD diagnosis should be actively sought in the workup of patients., Competing Interests: The authors declare no conflict of interest., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Previous models that assess quality-of-Life (QoL) in patients with rheumatic diseases have a strong biomedical focus. We evaluated the impact of COVID-19 related-health care interruption (HCI) on the physical, psychological, social relationships and environment QoL-dimensions, and explored factors associated with QoL when patients were reincorporated to the outpatient clinic, and after six-month follow-up., Patients and Methods: Study phase-1 consisted of a COVID-19 survey administered from June 24th-October 31st 2020, to outpatients with rheumatic diseases who had face-to-face consultation at outpatient clinic reopening. Study phase-2 consisted of 3 consecutive assessments of patient´s QoL (WHOQOL-BREF), disease activity/severity (RAPID-3), and psychological comorbidity/trauma (DASS-21 and IES-R) to patients from phase-1 randomly selected. Sociodemographic, disease and treatment-related information, and comorbidities were obtained. Multiple linear regression analysis identified factors associated with the score assigned to each WHOQOL-BREF dimension., Results: Patients included (670 for phase-1 and 276 for phase-2), had primarily SLE and RA (44.2% and 34.1%, respectively), and all the dimensions of their WHOQOL-BREF were affected. There were 145 patients (52.5%) who referred HCI, and they had significantly lower dimensions scores (but the environment dimension score). Psycho-emotional factors (primarily feeling confused, depression and anxiety), sociodemographic factors (age, COVID-19 negative economic impact, years of scholarship, HCI and having a job), and biomedical factors (RAPID-3 score and corticosteroid use) were associated with baseline QoL dimensions scores. Psycho-emotional factors showed the strongest magnitude on dimensions scores. Most consistent predictor of six-month follow-up QoL dimensions scores was each corresponding baseline dimension score, while social determinants (years of scholarship and having a job), emotional factors (feeling bored), and biomedical aspects (RAPID 3) had an additional impact., Conclusions: HCI impacted the majority of patient´s QoL dimensions. Psycho-emotional, sociodemographic and biomedical factors were consistently associated with QoL dimensions scores, and these consistently predicted the QoL trajectory., Competing Interests: The authors have declared that no competing interests exist.