ARP Rheumatology最新文献

Aims: Sjögren's disease (SjD) is a complex disease with a wide variety of manifestations and outcomes. We recently created PORTRESS, the Portuguese SjD registry within Reuma.pt. We aim to describe this registry and characterize our national cohort.

Methods: We included patients with a clinical diagnosis of SjD, registered in PORTRESS up to November 2023. Demographic, clinical, treatment, and patient-reported outcomes (PROs) data were collected. Variables were compared according to parametric or non-parametric tests, as applicable.

Results: A total of 1375 patients were included. Patients fulfilled AECG 2002 or ACR/EULAR 2016 classification criteria in 62% and 57% of cases, respectively, although more than half didn't have a complete assessment of all items. Of note, the vast majority (93%) had both SjD manifestations and a positive anti-Ro and/or minor salivary gland biopsy. Most patients (88%) exhibited at least one active ESSDAI domain during the course of their disease. Hydroxychloroquine and corticosteroids were used in 52% and 30% of patients, while other immunosuppressants and pilocarpine in 12% and 18% of cases, respectively. The mean ESSDAI at inclusion was 3.0±4.4 (range 0-42), and, at the last follow-up, 2.1±3.7 (0-31), corresponding to a significant decrease. Dryness, pain and fatigue PROs were scored high, with a significant increase from baseline to follow-up.

Conclusion: PORTRESS is a web-based SjD registry facilitating efficient nationwide data storage. It enables research, trial recruitment, and a comprehensive longitudinal view of patients' evolution. Although systemic activity improved over follow-up, symptom burden worsened when compared to baseline, underlining a major unmet need in SjD.

The Assessment of SpondyloArthritis International Society (ASAS) has recently proposed a consensus-based expert definition for difficult-to-manage (D2M) axial spondyloarthritis (axSpA) and treatment-refractory (TR) axSpA. Our aim is to determine the proportion of D2M and TR axSpA according to the ASAS definition and describe the characteristics of these patients. We conducted an observational cross-sectional single-centre study that included all adult patients with axSpA, meeting the ASAS classification criteria, exposed to biologic or targeted synthetic disease-modifying anti-rheumatic drugs (b/tsDMARDs). D2M axSpA was defined according to the ASAS criteria as 1) treatment according to the ASAS-European alliance of associations for rheumatology recommendations and failure of ≥2 b/tsDMARDs with different mechanisms of action (MoA), 2) insufficient control of signs/symptoms of axSpA (axial spondyloarthritis disease activity score (ASDAS)≥2.1 or C-reactive protein (CRP)>5.0mg/L or active inflammation on magnetic resonance imaging (MRI) or radiographic progression) and 3) the signs/symptoms are perceived as problematic by the patient/physician (patient/physician global assessment ≥4/10). TR axSpA was a subset of D2M axSpA in which 1) the use of ≥2 b/tsDMARDs was due to treatment failure, 2) with high or very high disease activity (ASDAS≥2.1) plus evidence of inflammatory activity (CRP>5.0mg/L or MRI showing active inflammation) and 3) other causes of signs and symptoms excluded. The proportion of D2M and TR axSpA was estimated. Descriptive analysis of axSpA, D2M, and TR was performed, and an exploratory analysis to compare D2M vs non-D2M axSpA. We included 207 patients, of whom 2.9% (n=6) met the criteria for D2M axSpA and 1.4% (n=3) for TR axSpA. Among axSpA patients, 52 (25.1%) had prior treatment with ≥2 b/tsDMARD, but only 12 (5.8%) had different MoA. Additionally, 42.8% (n=86) and 38.3% (n=77) fulfilled the second and third criterion for D2M axSpA, respectively, but only 13.2% (n=26) met the second criterion for TR axSpA. D2M axSpA was associated with a younger age at symptom onset and diagnosis. Applying the ASAS definition, we found a low proportion of D2M and TR axSpA. The first criterion (≥2 b/tsDMARDs with different MoA) limited the classification of patients as D2M or TR. This is among the first studies applying the ASAS definition.

This cross-sectional study analyzed data from the National Health and Nutrition Examination Survey (NHANES) to examine the association between the dietary inflammatory index (DII) and dietary oxidative balance score (DOBS) with osteoporosis risk using multivariable logistic regression models. The results showed that a high DII and low DOBS were significantly associated with increased osteoporosis risk, particularly in women.

Purpose: This cross-sectional study aimed to investigate the association between dietary inflammatory potential, as measured by the dietary inflammatory index (DII), and dietary oxidative balance score (DOBS), with osteoporosis risk in a large, nationally representative sample. Gender-specific analyses were conducted to assess potential differences in these associations.

Methods: Data from the National Health and Nutrition Examination Survey (NHANES) cycles 2007-2008, 2009-2010, 2013-2014, and 2017-2018 were utilized, including 10,709 participants. DII and DOBS scores were calculated based on 24-hour dietary recalls, and participants were stratified into composite dietary risk groups. Osteoporosis was defined based on dual-energy X-ray absorptiometry (DXA) measurements. Multivariable logistic regression models were used to estimate the odds ratios (ORs) for osteoporosis across dietary risk groups, adjusting for demographic, lifestyle, and clinical factors. Subgroup analyses were conducted for male and female participants.

Results: In the overall participants, participants in the high-risk dietary group (high DII, low DOBS) had a significantly higher odds of osteoporosis compared to the low-risk group (Model 3: OR: 2.31, 95% CI: 1.39-3.85, P = 0.002). In gender-stratified analyses, women in the high-risk group had a more than twofold increased odds of osteoporosis compared to the low-risk group (Model 3: OR: 2.71, 95% CI: 1.49-4.93, P = 0.002), whereas in men, the association between dietary risk groups and osteoporosis was not statistically significant (Model 3: OR: 1.61, 95% CI: 0.73-3.57, P = 0.235).

Conclusion: Dietary patterns with high inflammatory potential and low antioxidant intake are associated with an increased risk of osteoporosis, particularly in women. Given the cross-sectional design, causal relationships cannot be established, and prospective studies are warranted to further clarify these associations.

The standard treatment of immune-mediated diffuse alveolar hemorrhage (IM-DAH) encompasses immunosuppression with glucocorticoids (GC) and either cyclophosphamide (CYC) or rituximab (RTX). The role of intravenous immunoglobulin (IVIg) and plasma exchange (PLEX) remains controversial. We conducted a single-centre retrospective observational study on patients admitted with IM-DAH to evaluate treatment approaches and outcomes. Twelve episodes were identified in ten patients. All episodes were treated with GC and nine with CYC. IVIg was administered as first-line and/or bridging therapy in three cases with concomitant infections or high infection risk. PLEX was used in six episodes. IVIg and PLEX were primarily used as add-on therapies or when other immunosuppression was not recommended. After one year, nine patients survived. The combination of GC and CYC was the most common treatment regimen. While the role of PLEX and IVIg is not well established, they may be beneficial as second-line or as add-on therapies in selected cases.

Aims: To provide a comprehensive literature review of the otologic manifestations in RA and discuss potential pathogenesis and risk factors.

Methods: We screened the MEDLINE, Scopus, and Embase databases, and Google Scholar for articles involving patients with RA who presented otological manifestations up to May 2025, including meta-analyses, systematic reviews, observational studies, case series, and case reports published in English and/or Spanish.

Results: Rheumatoid arthritis (RA), besides affecting articulations, may be involved in other systems, including the cardiovascular, neurological, ocular, cutaneous, respiratory, renal, and gastrointestinal systems. Otologic manifestations, such as hearing loss, tinnitus, Meniere's disease, and vertigo, are relatively common in patients with RA. Hearing loss shows a notable prevalence, with an average rate of 16.14% among RA patients, an odds ratio of 3.42, and a relative risk of 2.28 when compared to the general population.

Conclusions: Given the high prevalence and impact of otologic manifestations in RA patients, a multidisciplinary approach involving both rheumatologists and otolaryngologists is essential for proper diagnosis and management. Early recognition of hearing loss and other otologic symptoms by rheumatologists may prevent complications and improve patients' quality of life.

Wunderlich syndrome, characterized by spontaneous perinephric hematoma with subcapsular extension has been scarcely reported in microscopic polyangiitis (MPA). We report the case of a 45-year-old woman, who presented with constitutional symptoms, left-eye episcleritis, and rapidly progressive glomerulonephritis. She developed sudden, severe left flank pain with hemoglobin drop two days after admission. Both computed tomography (CT) and non-contrast magnetic resonance imaging revealed large left-sided perinephric hematoma. CT angiography failed to demonstrate intrarenal aneurysms. A remarkable reduction in size of her perinephric hematoma was observed after three and a half months of treatment with glucocorticoids and intravenous cyclophosphamide (IV CYC) following the international guidelines. A literature review on renal vessel involvement in antineutrophil cytoplasmic antibody-associated vasculitis revealed 26 case reports and one case series with 20 cases of renal aneurysms. Eighteen cases in the case reports (69.2%) and nine in the case series (45%) ruptured their renal arteries. The majority (44.4%) were managed with IV CYC and high-dose glucocorticoids. Angioembolization, renal replacement therapy, and plasma exchange were used as adjuvant measures. Only three patients (16.7%) underwent nephrectomy, while the majority(63.6%) fully recovered.

Background: Systemic lupus erythematosus (SLE) is an autoimmune disease that affects multiple organs, particularly the kidneys. Interleukin (IL) biomarkers including IL-10 and IL17/23 axis play an important role in SLE pathogenesis.

Objectives: To investigate the predictive value of IL-17, IL-23, and IL-10 biomarkers in detecting lupus nephritis (LN) class in SLE cases.

Methods: This is a case-control study involving 160 individuals: 100 patients with SLE (80 LN patients who had a recent report of kidney biopsy in the two months prior to the study +20 non renal SLE patients), and 60 age- and sex-matched healthy volunteers. All participants were subjected to clinical and laboratory studies, as well as the evaluation of their IL-17, IL-23, and IL-10 biomarkers.

Results: IL-17, IL-23, and IL-10 were significantly elevated in SLE patients (p-value < 0.001), especially in cases with high disease activity (p-value < 0.001). Moreover, these biomarkers were considerably higher in LN patients (p-value < 0.001), particularly among class III and IV LN (p-value < 0.001) and in cases with high nephritis activity index (p-value < 0.001). ROC curve analysis revealed precise cutoff points of IL-17, IL-23, and IL-10 levels in each renal histopathological class with high sensitivity and specificity.

Conclusion: IL-17, IL-23, and IL-10 biomarkers are higher in SLE patients and are correlated with SLE Disease Activity Index (SLEDAI). They are more prevalent in individuals with LN, particularly in cases with high activity index and with more aggressive classes (in renal classes III and IV). These biomarkers might function as indicators for detecting LN activity and as predictors of LN class.

Objectives: To investigate the diagnostic prevalence of juvenile fibromyalgia syndrome (JFMS) causing widespread pain in patients with a diagnosis of juvenile idiopathic arthritis (JIA).

Methods: Patients with JIA from seven pediatric rheumatology centers in Türkiye were included. 2010 American College of Rheumatology criteria for fibromyalgia was utilized throughout a face-to-face interview. The Pain and Symptom Assessment Tool was used, and data were analyzed using the Widespread Pain Index and the Symptom Severity Scale. Patients were stratified into two groups: Group 1 (JIA with concomitant juvenile fibromyalgia) and Group 2 (JIA without juvenile fibromyalgia).

Results: A total of 313 patients with JIA were included, of whom 21 (6.7%) were found to have concomitant JFMS. In group 1, 71% (15 patients) were female and 29% (6 patients) were male, with a median age at JFMS evaluation of 16 years (range: 12.8-19). Among patients with JFMS, 62% (13 patients) were classified as having spondyloarthropathy (enthesitis-related arthritis or juvenile psoriatic arthritis), 28.5% (6 patients) as having oligoarticular JIA, and 9.5% (2 patients) as having polyarticular JIA. Seventeen patients (81%) were on medication, including five (24%) on biologics. The most common symptoms in the JFMS group were muscle pain and fatigue, followed by headache, nervousness, numbness, dizziness, acne, abdominal pain, and anorexia.

Conclusion: In JIA patients with chronic musculoskeletal pain, fatigue, headache, and irritability lasting more than three months, the possible diagnosis of JFMS should be considered in the clinical evaluation.

Introduction: Still's Disease (SD), encompassing both systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still's disease (AOSD) is a rare condition, therefore posing unique challenges in diagnosis and management, which can lead to delayed treatment and increased morbidity. The current state of practice regarding SD management varies widely across different countries. Very recently, a EULAR/PReS taskforce published new recommendations for the diagnosis and management of SD, providing tools to accelerate its diagnosis, including new biomarkers such as IL-18 and S100 proteins and recommending early initiation of IL-1 or IL-6 inhibition to avoid prolonged systemic glucocorticoid exposure.

Methods: Cross-sectional, descriptive, and observational study utilizing a structured questionnaire to collect data from healthcare professionals involved in the diagnosis and treatment of SD in Portugal.

Results: We obtained 52 responses from Portuguese clinicians. Only 10% use IL-18 levels and 25% use S100 proteins to aid in the diagnosis of SD. Half of the responders expect to achieve clinically inactive disease (CID) with low-dose glucocorticoids after 3 months, but only 39% aim to achieve CID without glucocorticoids at month 6. For 95% of responders the use of glucocorticoids is part of the first line of therapy. Less than half (37%) did not include IL-1 or IL-6 inhibitors in their first line of treatment.

Conclusion: The results of the questionnaire applied show that there is still a gap between clinical practice and the recent recommendations, as demonstrated by the underuse of recent biomarkers and biologic therapies, which should be bridged in order to improve health outcomes for individuals affected by SD.