Objectives To evaluate the prognostic utility of the Systemic Score (SS) in a Spanish cohort of patients with adult-onset Still's disease (AOSD), with a focus on its ability to predict disease complications, need for biologic therapy and mortality. Methods A retrospective study was conducted in two university hospitals. Clinical, laboratory, complication-related data, biologic treatments, and deaths (AOSD-related or not) were recorded. The SS was calculated at disease onset, assigning one point to each of 12 variables. A score ≥7 is considered high risk. The association between SS and clinical course, complications, biologic treatment, and AOSD-related death was analyzed. Results Data from 64 patients were analyzed. SS was <7 in 78.13% and ≥7 in 21.87%. A score ≥7 was significantly associated with macrophage activation syndrome, pericarditis, pleuritis, and renal involvement. Although myocarditis, and amyloidosis were not significantly associated, most individual cases showed SS≥7. Biologic treatment was more frequently required in patients with SS≥7. No association was found with clinical patterns or non-AOSD-related deaths. Both AOSD-related deaths had SS≥7, though the small number of cases limits interpretation. Conclusions SS is a valuable prognostic tool in patients with AOSD, allowing early identification of individuals at higher risk of severe complications and treatment escalation. It may help identify patients at high risk for complications and in need of biologic therapy.

Objective To evaluate the efficacy, safety and predictive factors of belimumab (BEL)-based triple therapy in proliferative lupus nephritis (LN) in real-world settings.Methods We conducted a multicentre, retrospective study including patients with proliferative LN (new-onset or relapsing) who initiated BEL within 6 months of a renal flare, in combination with standard-of-care.Results 49 patients were included (mean age 37 years; 85.7% female; 67.3% Caucasian). The median time from renal flare to BEL initiation was 1 month (IQR 0–3). By 12 months, 67.3% achieved complete renal response (CRR), 75.5% primary efficacy renal response (PERR) and 83.7% at least partial renal response. Median proteinuria declined from 2.7 g/day to 0.49 g/day, with parallel improvement in estimated glomerular filtration rate (71 to 78 mL/min/1.73 m²). Patients with baseline proteinuria <3 g/day achieved significantly higher CRR (78.1% vs 47.1%; p=0.027) and PERR (84.4% vs 58.8%; p=0.048) rates.The mean glucocorticoid (GC) dose decreased from 31.7 mg/day at baseline to 3.5 mg/day at 12 months, and 26.1% of patients achieved complete GC withdrawal. Extrarenal disease activity was present in 81.6% of patients at baseline, predominantly articular and mucocutaneous, with clinically meaningful improvement in 80% during follow-up. At 12 months, 40.8% met remission by Definition Of Remission In Systemic Lupus Erythematosus (DORIS) criteria and 46.9% attained Lupus Low Disease Activity State (LLDAS). Renal treatment failure occurred in 16.3% and renal relapse in 4.1%. Adverse events were mild, and no serious BEL-related events were observed.Conclusion BEL-based triple therapy is effective and safe in proliferative LN, achieving high renal and extrarenal response rates, substantial GC-sparing and treat-to-target outcomes in real-world practice.

To determine incidence of major adverse cardiovascular events (MACE: non-fatal myocardial infarction, non-fatal stroke, or cardiovascular death) in Sjögren’s disease (SjD); identify traditional and disease-specific factors associated with MACE; describe prevalence of cardiovascular risk factors (CVRFs); explore, descriptively, SCORE2-estimated risk by comparing predicted and observed events; estimate cardiovascular mortality; and assess association between MACE and all-cause mortality. Prospective, multicenter cohort study including 314 patients fulfilling 2002 AECG criteria for SjD, followed for median 9.5 years. Clinical, serological, and cardiovascular data were recorded. Factors associated with MACE were evaluated using multivariable logistic regression models. SCORE2 risk was calculated in patients with complete data, and expected events were compared with observed events. Cardiovascular mortality was expressed as crude rates per 1,000 patient-years. Seventeen patients (5.41

OBJECTIVE:To evaluate sex-based differences in clinical presentation, vascular phenotype, diagnostic approaches, treatment, adverse events and outcomes among patients with giant cell arteritis (GCA). METHODS:We analysed data from all patients diagnosed with GCA between 1 June 2013 and 29 March 2019 across 26 hospitals in Spain. Eligibility criteria included age ≥50 years and diagnosis confirmed by imaging, the 1990 American College of Rheumatology classification criteria, or expert clinical judgment. RESULTS:A total of 1675 patients were included. The extracranial phenotype was more prevalent in women (14.8% vs 9.9%; P = 0.039), while the mixed phenotype was more common in men (17.7% vs 11.7%; P = 0.002). Temporal artery ultrasound was more frequently positive in men (73.5% vs 65.1%; P = 0.025). Conventional synthetic disease-modifying antirheumatic drugs (csDMARDs) were prescribed more often in women (35.3% vs 28.2%; P = 0.005), while glucocorticoid and tocilizumab use did not differ by sex. Fractures (22.4% vs 10.9%; P = 0.010) and cataracts (6.0% vs 2.8%; P = 0.010) were significantly more frequent in women and men, respectively. Remission rates (23.1% vs 23.8%) and relapse frequencies (19.7% vs 19.3%) were similar between sexes. Mortality was higher in men (13.8% vs 6.6%; P < 0.001). CONCLUSIONS:Women more frequently exhibited the extracranial phenotype, required more csDMARDs, and experienced a higher incidence of fractures. In contrast, men more commonly presented with the mixed phenotype, had more cataracts, and showed a higher mortality rate. These findings highlight the importance of integrating sex as a key biological variable in GCA research and clinical management to support more tailored and effective therapeutic strategies.

To determine the incidence and spectrum of lung involvement in early RA through a structured respiratory assessment and to identify its clinical predictors. A retrospective study was conducted in a cohort of 204 early RA patients screened for lung involvement at RA onset and during follow-up. Cumulative incidence (CI) at four and eight years, incidence rate (IR), and frequency were calculated for the different manifestations identified. Cox regression was used to assess potential risk factors. Pulmonary involvement was identified in 89 of 204 patients (43.6

BACKGROUND/OBJECTIVES:Nutritional risk is increasingly recognized as a relevant but under-assessed dimension of rheumatoid arthritis (RA), particularly in older adults managed in outpatient settings. Simple nutritional indices such as the Prognostic Nutritional Index (PNI) may help identify individuals at increased nutritional risk beyond conventional disease activity measures. This study aimed to characterize nutritional risk in older adults with RA using the Prognostic Nutritional Index, explore sex-specific patterns, and identify clinical associations of PNI variability, with complementary analyses focusing on high nutritional risk. METHODS:We conducted an observational cross-sectional study including 275 consecutive adults aged ≥50 years with RA attending routine follow-up at a tertiary rheumatology clinic. Nutritional risk was assessed using the PNI, calculated from serum albumin and total lymphocyte count, and analyzed primarily as a continuous variable and secondarily using established cut-off values. Clinical characteristics, inflammatory markers, body mass index, laboratory parameters, and patient-reported outcomes were recorded. Analyses were stratified by sex. Multivariable linear regression models were used to identify factors associated with PNI variability, and complementary logistic regression analyses were performed to explore factors independently associated with high nutritional risk (PNI < 40). RESULTS:More than half of the cohort (53.3%) exhibited PNI values compatible with nutritional risk. Men showed lower PNI values than women, with a markedly greater prevalence of high nutritional risk (18.0% vs. 5.0%, p < 0.001). In multivariable linear regression analyses, higher C-reactive protein levels and increasing age were independently associated with lower PNI values, whereas sex was not an independent determinant of PNI. In multivariable logistic regression analyses, increasing age and male sex were independently associated with high nutritional risk. In multivariable linear regression models restricted to men, hemoglobin emerged as the principal independent correlate of PNI. In complementary logistic regression analyses focusing on high nutritional risk (PNI < 40), hemoglobin remained the sole independent predictor (OR = 0.94, 95% CI 0.91-0.98; p < 0.01), supporting a robust association with clinically relevant nutritional risk. CONCLUSIONS:Nutritional risk assessed by the PNI is common among older adults with RA. Although sex does not independently determine PNI as a continuous measure, male sex is associated with severe nutritional risk. The PNI captures a clinically relevant dimension of disease burden that extends beyond joint inflammation and traditional activity indices, supporting its use as a pragmatic nutritional screening tool in routine rheumatology practice.

OBJECTIVE:To determine the prevalence, characteristics and clinical significance of subclinical polymyalgia rheumatica (PMR) in patients with apparently isolated giant cell arteritis (GCA). METHODS:Single-centre retrospective cohort study including 168 patients with newly diagnosed GCA who underwent 18F-FDG PET-CT (18F-fluorodeoxyglucose positron emission tomography-computed tomography) at diagnosis. Patients were classified into clinical PMR, subclinical PMR or pure GCA based on PET findings and clinical symptoms. FDG uptake was assessed at predefined typical PMR sites, and only sites with grade ≥2 uptake were considered positive. Subclinical PMR was defined as FDG uptake involving either bilateral periarticular shoulders or bilateral trochanteric bursae or ischial regions, plus uptake at one additional typical PMR site. RESULTS:PET findings consistent with subclinical PMR were observed in 56% (57/102) of patients with apparently isolated GCA at diagnosis. A substantial proportion of these patients fulfilled different proposed PET-based diagnostic criteria for PMR. Patients with GCA and subclinical PMR showed less extensive musculoskeletal FDG uptake than those with GCA and clinical PMR but greater large-vessel involvement than pure GCA, particularly in the thoracic aorta (P = 0.023), abdominal aorta (P = 0.0001) and supra-aortic trunks (P = 0.007). Compared with pure PMR, they displayed a milder and more localized inflammatory pattern, mainly affecting the shoulder and pelvic girdles. No significant differences were observed across groups in cranial manifestations, severe ischaemic events, systemic inflammation or relapse rates. CONCLUSION:Subclinical PMR findings are frequently detected by PET-CT in patients with apparently isolated GCA, supporting the concept of a GCA-PMR spectrum disease.

To assess the prevalence and persistence of self-reported depression and its associated factors in patients with systemic lupus erythematosus (SLE), using a patient-reported outcome measure in a large, multicentre, prospective cohort. We conducted a longitudinal analysis of patients enrolled in the RELESSER-PROS registry who responded to item 7 (“I was depressed”) of the Lupus Impact Tracker questionnaire (LITQ7) over five annual visits. Self-reported depression was defined as any response other than “none of the time.” Covariates assessed at each visit included: age, disease duration, SELENA-SLEDAI (S-SLEDAI), glucocorticoid (GC) use, SLICC/ACR Damage Index (SDI), fibromyalgia, Charlson index, BMI, smoking status, menopause, sedentary lifestyle, marital and employment status. Generalized estimating equation (GEE) models were used to examine longitudinal associations. Of 1463 patients (mean age 55 years; 90

OBJECTIVE:SSc is characterized by micro- and macrovascular damage, increasing cardiovascular (CV) risk. The SCORE2 algorithm underestimates CV risk in systemic autoimmune diseases. Nailfold capillaroscopy (NFC) is used to assess SSc-related microvascular damage. We aimed to evaluate whether incorporating NFC findings into SCORE2/SCORE2-OP improves CV risk stratification in SSc. METHODS:Retrospective multicentre study including 276 patients with SSc. Baseline 10-year CV risk was estimated using SCORE2 or SCORE2-OP. NFC at diagnosis was assessed. Cox regression identified NFC variables associated with major CV events (MCEs). Their adjusted hazard ratios (HRs) were applied to derive NFC-modified SCORE2 models. Discrimination, calibration and reclassification of original vs modified models were compared, with bootstrap internal validation. RESULTS:Over a median follow-up of 9.5 years, 45 (16.3%) patients experienced an MCE. In multivariable models, late NFC pattern (HR 4.056, P = 0.002) and avascular areas (HR 2.631, P = 0.039) were independently associated with MCEs, whereas microhaemorrhages were associated with reduced risk (HR 0.345, P = 0.017). Original SCORE2 showed modest discrimination for incident MCEs (AUC 0.687, 95% CI 0.574-0.801). NFC-modified models improved discrimination (AUC 0.784, 95% CI 0.674-0.894 for the NFC-findings model and 0.764, 95% CI 0.654-0.875 for the NFC-pattern model; both P < 0.05 vs SCORE2), with consistent gains in Harrell's C-index. Risk reclassification analyses showed classification of patients with MCEs, with categorical net reclassification indices of 0.31 and 0.36, respectively. CONCLUSIONS:Incorporating baseline NFC into SCORE2 improves CV stratification in patients with SSc, and may support more individualized CV prevention strategies in SSc.

OBJECTIVE:Aortitis associated with giant cell arteritis (GCA) is a severe manifestation, potentially leading to aneurysms and aortic dissection. Tocilizumab (TCZ) has demonstrated efficacy in the treatment of GCA, both intravenously or subcutaneously administered. However, pivotal studies did not specifically evaluate aortic involvement, and no comparison of intravenous (IV) versus subcutaneous (SC) TCZ has been performed in patients with GCA-related aortitis. The objective of this study was to compare the effectiveness of TCZ according to the administration route in patients with GCA-associated aortitis under clinical practice conditions. METHODS:This was a multicenter observational study including 196 patients diagnosed with GCA-associated aortitis by imaging and treated with TCZ. Patients were grouped by administration route: IV or SC. GCA was diagnosed following the 1990 American College of Rheumatology criteria, temporal artery biopsy, and/or vascular imaging. Aortitis was identified using 18F-fluorodeoxyglucose positron emission tomography/computed tomography scan. Main outcomes included EULAR remission, clinical and imaging remission, absence of systemic inflammation, and glucocorticoid-sparing effect. RESULTS:Of 196 patients (148 women; mean age 69.8 ± SD 9.4 years), 110 received IV TCZ and 86 SC TCZ. Baseline clinical characteristics and markers of inflammation were comparable between groups. The glucocorticoid-sparing effect was similar. At 24-month follow-up, EULAR-defined remission was significantly more frequent in the SC group (83.3% vs 80.6%; P < 0.05). However, rates of imaging remission and absence of systemic inflammation were comparable between treatment arms. CONCLUSIONS:In this real-world cohort of GCA-associated aortitis, SC TCZ showed slightly greater effectiveness than IV TCZ in achieving EULAR-defined remission, whereas no significant differences were observed between both routes regarding imaging remission.

OBJECTIVE:To summarise the clinical characteristics, radiological and histopathological features, treatment approaches, and outcomes of isolated antineutrophil cytoplasmic antibody-associated interstitial lung disease (ANCA-ILD). METHODS:A systematic literature review conducted according to the PRISMA statement. RESULTS:Twenty-four studies comprising 794 patients were included. Isolated ANCA-ILD predominantly affected older adults, between 50 and 70 years of age, with a slight male predominance (57%). MPO-ANCA positivity was observed in approximately 70% of patients, whereas PR3-ANCA positivity accounted for 10-15%. Fibrotic patterns predominated, with usual interstitial pneumonia (UIP) or UIP-like changes reported in 45-65% of cases, followed by NSIP in 15-30%. Glucocorticoids were prescribed in 58% of patients, immunosuppressive therapies in 31%, and antifibrotic treatment in approximately 26% of those reported in more recent cohorts. Functional decline occurred in approximately 30-50% of patients, and radiological progression in 30-45%. Acute exacerbations were reported in 19-25%, and up to 40% fulfilled criteria for progressive pulmonary fibrosis during follow-up. Progression to systemic ANCA-associated vasculitis (AAV) occurred in approximately 10-40% of cases, with timing ranging from the first year to several years later. This evolution occurred mainly toward microscopic polyangiitis (MPA), whereas granulomatosis with polyangiitis was infrequent, representing ≤10-20% of cases. Survival was generally comparable to ANCA-negative idiopathic interstitial pneumonia and AAV-ILD. CONCLUSION:Isolated ANCA-ILD is an emerging, increasingly recognised fibrotic interstitial lung disease phenotype characterised by frequent MPO-ANCA positivity and two partly independent trajectories: (1) an IPF-like fibrotic course and (2) progression to systemic AAV, most commonly MPA.

To evaluate the prevalence, associated factors, and prognostic performance of NIH activity index–defined interstitial inflammation (NIH-TII) and interstitial fibrosis and tubular atrophy (IFTA) in lupus nephritis (LN). This retrospective cohort study analyzed 195 renal biopsy episodes from 135 LN patients. Tubulointerstitial lesions were graded according to histopathological severity. NIH-TII was assessed semiquantitatively using the interstitial inflammation component of the modified NIH activity index. Among 195 renal biopsies, class IV LN was most frequent (49.2

The objective of SjögrenSER Prospective (SjD-PROS) was to evaluate the improvement, stability or progression of SjD in clinical practice. SjD-PROS is an observational, longitudinal, multicenter study of SjD in Spain. Participants from the prior transversal phase were invited to a follow-up visit after 9.5 years. Data were collected via interviews and medical records. Variables were analyzed using means, medians and frequencies. Statistical associations were assessed using T student test, Kruskal–Wallis and the Chi-square test. We included 314 patients, 95