Advances in Rheumatology最新文献

Background: Anti-PM/Scl autoantibody has been associated with an overlap between polymyositis (PM) and systemic sclerosis (SSc). However, due to limited studies, the relevance of this autoantibody in patients with idiopathic inflammatory myopathies (IIMs) without SSc was analyzed.

Methods: This single-center retrospective cohort study was conducted between 2004 and 2024. A total of 93 adult patients with IIMs (66 with dermatomyositis and 27 with PM - EULAR/ACR 2017) without SSc were included: 16 anti-PM/Scl(+) and 77 anti-PM/Scl(-). Patients with other types of IIMs, cancer-associated myositis, or overlap myositis, including SSc, as well as those with other myositis-specific and/or myositis-associated autoantibodies were excluded.

Results: The median age, sex distribution, and median follow-up duration were comparable between the anti-PM/Scl(+) and anti-PM/Scl(-) groups. There were no differences in clinical and laboratory characteristics, except for a higher frequency of lung involvement, joint involvement, "mechanics' hand," "hiker's feet," and Raynaud's phenomenon, in contrast to a lower frequency of facial rash and "V"-neck sign in patients with anti-PM/Scl(+) than in those with anti-PM/Scl(-) (all P < 0.05). Furthermore, patients with anti-PM/Scl(+) exhibited a higher frequency of disease relapse (68.8% vs. 33.8%), disease activity (50.0% vs. 24.7%), and immunosuppressant use (methotrexate or azathioprine) at the last medical evaluation (all P < 0.05). Severe infection and death rates were comparable between the groups.

Conclusions: Anti-PM/Scl positivity was observed in 17.2% of the sample analyzed in the present study. Patients with this autoantibody present clinical manifestations resembling anti-synthetase syndrome, with increased disease relapse and activity rates.

Background: There is no consensus on the classification of lupus arthritis (LA). In this study, we aimed to investigate patients with LA who were clinically and serologically very similar to those patients with rheumatoid arthritis (RA).

Methods: The electronic medical files of systemic lupus erythematosus (SLE) patients from a single tertiary rheumatology department between 2017 and 2022 were reviewed. The inclusion criteria were being age ≥ 18 years, having nonerosive peripheral arthritis lasting longer than six months, and having dual seropositive (rheumatoid factor (RF) and anti-citrullinated protein antibody (anti-CCP)) serology. A nonerosive course of arthritis was demonstrated by both conventional radiography and joint ultrasound. Images were assessed by two blinded rheumatologists. Patients with drug-induced lupus and those with other rheumatologic diseases were excluded.

Results: The cases of 528 patients were reviewed, and eight patients were included in the study. All patients were female, and the median age was 48.5 years. The median SLE and arthritis durations were 12 and seven years, respectively. The most common SLE symptom was photosensitivity (n = 8). Only one patient had life-threatening involvement (LTI), which was a seizure and autoimmune haemolytic anaemia. All patients had arthritis affecting the wrist and hand. Anti-dsDNA was the most common anti-ENA antibody (n = 7), followed by anti-SSA (n = 5). The median RF and anti-CCP titres were 82.5 IU/ml and 81.5 U/ml, respectively. Five patients had high titres of autoantibodies, and only one patient had slight hypocomplementemia. Three patients needed biologic agents, and remission was achieved after treatment with rituximab.

Conclusion: Despite a long arthritis duration and dual seropositive serology with high titres of RF and anti-CCP, our patients had SLE rather than rhupus syndrome. The low frequency of LTIs, such as lupus nephritis, was a remarkable feature of our patients. Lupus arthritis may be clinically and serologically indistinguishable from RA. Prospective studies are needed to better define LA.

Background: Neurological and psychiatric manifestations occur in patients with primary Sjogren's disease (SjD) with a wide-ranging clinical presentation, affecting quality of life, social participation, and prognosis. Despite this, neither central nor peripheral neurological symptoms are systematically evaluated in the context of autoimmunity or identified as manifestations of SjD. The EULAR Sjogren's Syndrome Disease Activity Index (ESSDAI) covers only part of them in the neurological domain.

Methods: We performed a systematic review of the diagnosis and prevalence of central, peripheral, and autonomic nervous system manifestations in primary SjD, following the recommendations proposed by the Cochrane Collaboration Handbook. Observational studies were included when their main issue was the diagnosis and the prevalence of the manifestations individually. We employed a generalized linear mixed model (GLMM) method with a random-effects model, and the results were computed using logit transformation, implemented through the 'meta' and 'metafor' packages in the R software (version 3.6.1). To present these recommendations, agreement among experts was investigated using the Delphi method in in-person meetings.

Results: We propose ten recommendations regarding the investigation and management of neurological involvement in SjD that had 100% agreement among participants.

Conclusion: These recommendations add to the literature on the clinical care of patients with SjD.

Background: To date, there has been limited exploration, particularly on a national scale, of the prevalence patterns of comorbidities and complications associated with rheumatoid arthritis (RA) in Colombia. We aimed to analyze the prevalence patterns of comorbidities and disease-related complications of RA patients enrolled in Colombia's contributory healthcare regime.

Methods: We performed a nationwide observational descriptive cross-sectional study using administrative claims data. We used a set of sensitive and specific electronic algorithms (i.e., a set of rules) applied to linked data based on ICD-10 codes and unique medication use codes. We compared all those algorithms with several sources, including governmental agencies and scientific literature, to identify all the known adults treated for RA.

Results: A total of 123,080 RA cases for 2018 were identified, corresponding to a point prevalence of 0.86 (95% CI 0.86-0.87) per 100. Compared to a non-RA reference population, hypertension (68.2 vs. 20.0%), osteoarthritis (43.6 vs. 6.1%), and osteoporosis (18.6 vs. 1.1%) provided larger standardized mean differences. Lupus (30.04; 95%CI 29.3-30.8), multiple sclerosis (7.18; 95%CI 6.6-7.8), and osteoporosis (5.57; 95%CI 5.5-5.6) provided higher age- and sex-adjusted prevalence ratios. Disease-related complications were found in 62.2% of cases.

Conclusions: We describe the first comprehensive assessment of the prevalence patterns of disease-related complications and comorbidities that define the RA burden of disease within a multimorbidity profile. Also, our study provides a narrower and more reliable point prevalence estimate for RA in Colombia.

Background: The clinical manifestations and course of rheumatoid arthritis-associated interstitial lung disease (RA-ILD) exhibits considerable heterogeneity. In this study, we aimed to explore radiographic progression over a defined period, employing the Warrick score as a semi-quantitative measure in early RA-ILD, and to assess the associated risk factors for progression.

Methods: RA-ILD patients underwent consecutive Warrick scoring based on initial high-resolution computed tomography (HRCT) at diagnosis and the first follow-up. Associations between Warrick scores, pulmonary function tests, and patient characteristics were analyzed. The ROC curve assessed the predictive performance of the Warrick score change rate for ILD progression, while multivariable logistic regression analysis identified risk factors for progression.

Results: Significant correlations were found between Warrick scores and age at RA-ILD diagnosis, age at ILD diagnosis, and baseline DAS28-ESR. For the severity score, correlations were r = 0.359, r = 0.372, and r = 0.298 (p = 0.001, p < 0.001, p = 0.014, respectively); for the extent score, r = 0.364, r = 0.318, and r = 0.255 (p = 0.001, p = 0.005, p = 0.038, respectively); and for the total score, r = 0.376, r = 0.367, and r = 0.280 (p < 0.001, p = 0.001, p = 0.022, respectively). Annual changes in severity, extent, and total Warrick scores showed sensitivities of 91-97% and specificities of 98% for predicting progression over a 5-year follow-up. Cut-off values were 0.0278 for the severity score (AUC 0.954), 0.0227 for extent score (AUC 0.976), and 0.0694 for total score (AUC 0.946). Warrick severity, extent, and total scores increased significantly during follow-up. Age > 50 years (OR 7.7; p = 0.028) and baseline usual interstitial pneumonia (UIP) pattern (OR 3.1, p = 0.041) were identified as risk factors for progression.

Conclusions: Advanced age and UIP pattern were significant risk factors for progression. Warrick scoring may may help predict progression in RA-ILD, particularly through changes in severity, extent, and total scores. Due to the retrospective design and small sample size, further prospective studies with larger cohorts are needed to confirm these findings and validate Warrick scoring as a reliable marker for RA-ILD progression.

Objectives: To compare the impact of COVID-19 on the clinical status and psychological distress of patients with immune-mediated rheumatic disease (IMRD) caused by SARS-CoV-2 infection with that of noninfected IMRD controls during a 6-month follow-up period.

Methods: The ReumaCoV Brazil is a longitudinal study designed to follow IMRD patients for 6 months after COVID-19 (patients) compared with IMRD patients without COVID-19 (controls). Clinical data, disease activity measurements and current treatments regarding IMRD and COVID-19 outcomes were evaluated in all patients. Disease activity was assessed through validated tools at inclusion and at 3 and 6 months post-COVID-19. Fatigue, using FACIT-F (Functional Assessment of Chronic Illness Therapy) and psychological distress, using DASS 21 (Depression, Anxiety and Stress Scale - 21 Items), used to evaluated psychological distress, were evaluated at 6 months after COVID-19 in both groups. The significance level was set as p < 0.05, with a 95% confidence interval.

Results: A total of 601 patients were evaluated-321 patients (IMRD COVID-19 + patients) and 280 controls (IMRD COVID-19- patients)-who were predominantly female with similar median ages. Disease activity assessment over a 6-month follow-up showed no significant difference between cases and controls. Although the mean activity scores did not differ significantly, some patients reported worsened disease activity post-COVID-19, particularly in rheumatoid arthritis (RA) (32.2%) and systemic lupus erythematosus (SLE) patients (23.3%). Post-COVID-19 worsening in RA patients correlated with medical global assessment (MGA) and CDAI scores, with a moderate to large effect size. Diabetes mellitus showed a positive association (OR = 7.15), while TNF inhibitors had a protective effect (OR = 0.51). Fatigue, depression, anxiety, and stress were significantly greater in patients than in controls. Worse disease activity post-COVID-19 correlated with worse FACIT-F and DASS-21 scores in RA patients. No significant associations were found between COVID-19 outcomes and post-COVID-19 disease activity, FACIT-F or DASS-21.

Conclusions: Post-COVID-19 IMRD patients exhibited significant fatigue, depression, anxiety, and stress, which can be mistaken for disease activity, despite having similar disease activity scores. The variability in reports on IMRD flares and the potential triggering of SARS-CoV-2 for autoimmune manifestations underscore the need for detailed clinical assessment and a comprehensive approach to managing them.

Background: Endoplasmic reticulum stress (ERS) and the unfolded protein response (UPR) are adaptive mechanisms for conditions of high protein demand, marked by an accumulation of misfolded proteins in the endoplasmic reticulum (ER). Rheumatic autoimmune diseases (RAD) are known to be associated with chronic inflammation and an ERS state. However, the activation of UPR signaling pathways is not completely understood in Sjögren's disease (SD). This study evaluated the expression of ERS-related genes in glandular tissue of patients with primary SD (pSD), associated SD (aSD) with other autoimmune diseases, and non-Sjögren sicca syndrome (NSS).

Methods: In a cross-sectional study, minor salivary gland biopsies were obtained from 44 patients with suspected SD and 13 healthy controls (HC). Patients were classified as pSD, aSD, or NSS based on clinical, serological, and histological assessment. Histopathological analysis and mRNA expression analysis of genes associated with ERS and UPR (PERK, XBP1, ATF-6, ATF-4, CANX, CALR, CHOP, and BIP) were performed on the samples. Differences between groups (pSD, aSD, NSS, and HC) were assessed. The influence of chloroquine (CQ) on the ER was also investigated.

Results: Twenty-eight SD patients showed increased expression of PERK (p = 0.0117) and XBP1 (p = 0.0346), and reduced expression of ATF-6 (p = 0.0003) and CHOP (p = 0.0003), compared to the HC group. Increased expression of BIP (p < 0.0001), PERK (p = 0.0003), CALR (p < 0.0001), and CANX (p = 0.0111) was also observed in the SD group compared to the NSS group (n = 16). Patients receiving CQ (n = 16) showed a significant increase in ATF-6 (p = 0.0317) compared to patients not taking the medication (n = 29).

Conclusions: Altogether, the results suggest a greater activation of the ERS and UPR genes in patients with SD, especially in the pSD group. Antimalarial drugs, like CQ, used to treat RAD, may affect the ER function in exocrine glands.

Introduction: Transition clinics are conceived as programs dedicated to the active, multidimensional development of a process that addresses the medical, psychosocial, educational, and vocational needs of pediatric patients suffering from a chronic disease that will persist into adulthood. Their understanding is justified in physiological, psychological, and sociocultural terms on the basis of the differential morbidity and mortality associated with a chronic disease that begins in childhood and prevails into adulthood.

Materials and methods: Here, we reflect on the history, structure, and impact of transition clinics in pediatrics, with an emphasis on pediatric rheumatologic diseases. Additionally, we propose comprehensive reflection as an alternative for the patient, their family, and the medical team, outlining guidelines for development, implementation, and evaluation.

Results: The transition of care should commence in early adolescence, considering each patient's cognitive ability as a condition for the initiation of an educational process involving introspection into the disease. Interdisciplinarity is defined as a team that addresses the clinical, physical, emotional, and social dimensions of each patient and their interaction with the environment within the framework of individualized care and family support. Despite this, the lack of evidence supporting standardized guidelines for the implementation and overall effectiveness evaluation of these interventions was highlighted.

Conclusions: The transition process is considered successful when the patient is adherent and has a positive and informed perception of their health‒disease journey. We urge the generation of evidence documenting the comprehensiveness of processes inherent to transition clinics as the foundation of necessity.

Background: Osteoarthritis (OA) is a common degenerative joint disease. Circular RNA Phosphodiesterase 1 C (circ-PDE1C, hsa_circ_0134111) has participated in the IL-1β-induced chondrocyte damages. The objective of our study was to explore the molecular mechanism of circ-PDE1C.

Methods: Circ-PDE1C, microRNA-766-3p (miR-766-3p) or Small Glutamine Rich Tetratricopeptide Repeat Co-Chaperone Beta (SGTB) expression was determined using reverse transcription-quantitative polymerase chain reaction (RT-qPCR). Cell counting kit-8 (CCK-8) assay and flow cytometry were used to analyze proliferation and apoptosis, respectively. Western blotting assay was performed for protein detection. The inflammatory cytokines were measured by Enzyme-linked immunosorbent assay (ELISA). Oxidative stress was assessed by commercial kits. Target analysis was conducted by dual-luciferase reporter assay and RNA immunoprecipitation (RIP) assay.

Results: Circ-PDE1C was abnormally overexpressed in OA tissues and IL-1β-exposed chondrocytes. Downregulation of circ-PDE1C alleviated the IL-1β-induced cell apoptosis, inflammation, extracellular matrix degradation and oxidative stress. Circ-PDE1C could interact with miR-766-3p to serve as miRNA sponge. The function of si-circ-PDE1C was attributed to the inhibition of miR-766-3p. Additionally, miR-766-3p directly targeted the 3'UTR of SGTB. The miR-766-3p upregulation impeded the IL-1β-triggered cell damages through reducing the level of SGTB. Moreover, SGTB expression was regulated by circ-PDE1C via binding to miR-766-3p in IL-1β-induced chondrocytes.

Conclusion: Altogether, circ-PDE1C enhanced the IL-1β-induced dysfunction in chondrocytes via upregulating SGTB by targeting miR-766-3p.