Rheumatology最新文献

Objectives: Previous studies have identified unique challenges for rheumatoid arthritis (RA) patients of different genders, which impact disease management. However, the association between disease activity and healthcare-seeking behaviours in male and female RA patients remains underexplored. This study aimed to investigate this association, with a specific focus on the gender-specific effects of follow-up intervals on disease control.

Methods: A nationwide survey (July-September 2023) across 330 rheumatology centres in China included 13 278 female (83.85%) and 2,557 male RA patients (16.15%) aged ≥18 years. Standardized questionnaires captured demographic and healthcare-seeking behaviours. Disease activity was assessed using the Clinical Disease Activity Index (CDAI).

Results: Females had younger disease onset (45.84 vs 51.03 years, p < 0.001), longer disease duration (7.51 vs 5.64 years, p < 0.001), higher low disease activity/clinical remission rates (22.61% vs 15.33%, p < 0.001), and lower glucocorticoid use (39.5% vs 47.73%, p < 0.001). Despite similar self-reported regular follow-up rates (84.73% vs 83.14%), both genders experienced suboptimal visit intervals (>3 months: 61.30% vs 62.65%, p < 0.001). Prolonged follow-up intervals were independently associated with poor disease control (CDAI > 10) in the female subgroup, with longer intervals linked to higher odds of inadequate control at 6-month (OR = 1.22, 95%CI : 1.09-1.37, p < 0.001) and 12-month intervals (OR = 1.43, 95%CI : 1.22-1.60, p < 0.001). Regular monitoring reduced high disease activity risk across genders (OR = 0.64, 95%CI : 0.56-0.74, p < 0.001). A generalized linear model showed no significant follow-up interval-gender interaction on disease activity (all p> 0.20).

Conclusion: This large-scale study reveals gender-dimorphic patterns in RA progression and healthcare engagement. Females tended to exhibit better treatment response, with a more pronounced interval-dependent disease control trend. No significant interval-gender interaction confirms this is a descriptive trend, not a validated gender-specific difference. Our findings emphasize the need for strategies accounting for such gender-dimorphic trends to optimize care continuity and improve RA management.

Objectives: Belimumab (BEL) is an approved biologic therapy for systemic lupus erythematosus (SLE) that, when added to standard care, reduces disease activity, flare rates, and glucocorticoid exposure. In clinical practice, BEL is frequently combined with conventional immunosuppressants (IS), but the added benefit of concomitant IS once disease control is achieved remains uncertain. Observational data suggest that BEL monotherapy may be effective in selected patients, but comparative real-world evidence is limited. Objectives were to evaluate the clinical effectiveness of BEL as monotherapy vs in combination with IS in patients with SLE included in the BEL-Spain Registry.

Methods: We performed a retrospective analysis of SLE patients treated with BEL with or without IS, with at least 12 months of follow-up. The primary outcome was remission according to the 2021 DORIS definition. Secondary outcomes included Lupus Low Disease Activity State (LLDAS), flare rates, and glucocorticoid use. Overlap propensity score weighting was applied to adjust for confounding by indication.

Results: Among 258 patients, 177 (68.6%) received BEL with IS and 81 (31.4%) BEL monotherapy. At 12 months, DORIS remission rates were 48.1% in the IS group and 51.4% in the monotherapy group (p=NS), with comparable LLDAS and flare rates. Propensity score-adjusted analyses confirmed no significant differences in remission rates (OR 0.89, 95% CI 0.37-2.14). Global treatment trajectories were stable, other than progressive glucocorticoid reduction in both groups.

Conclusions: In this real-world cohort, BEL monotherapy achieved similar outcomes to combination therapy with IS in selected SLE patients. Prospective studies are needed to confirm these findings and define optimal treatment strategies.

Objectives: Antineutrophil cytoplasmic antibodies (ANCA) are a key biomarker for ANCA-associated vasculitis (AAV), particularly microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA). Although indirect immunofluorescence (IIF) has traditionally been the reference technique, its diagnostic value in contemporary real-world practice remains uncertain. The aim of this study was to evaluate the diagnostic performance of IIF ANCA testing in routine clinical practice.

Methods: We conducted a retrospective study of all patients with an ANCA request at a tertiary hospital over a four-year period. All IIF-positive sera were subsequently tested for anti-PR3 and anti-MPO antibodies by chemiluminescent immunoassay (CLIA). Clinical data, test indication and final diagnoses were retrieved from electronic medical records.

Results: We included 5,157 patients and IIF was positive in 653 (12.7%): perinuclear ANCA (P-ANCA) in 17.9%, cytoplasmic ANCA (C-ANCA) in 13.3% and atypical ANCA (A-ANCA) in 68.8%. CLIA was negative in 97.3% of A-ANCA. AAV was diagnosed in 47 patients, and 42 (89.4%) had positive IIF. For GPA and MPA, IIF showed a sensitivity of 93% and specificity of 88%, with a very high negative predictive value (NPV) (99.9%) but low positive predictive value (PPV) (6.1%). Specificity improved to 96.8% when restricted to typical patterns (C-ANCA or P-ANCA) and to 99.6% when combined with positive CLIA results >20 IU/ml. Almost all AAV cases were diagnosed in patients with high pre-test probability (such as renal disease, lung infiltrates, or peripheral neuropathies) and interstitial lung disease was the most frequent non-AAV diagnosis in IIF-positive patients.

Conclusions: ANCA IIF retains good diagnostic efficiency and a very high NPV for GPA and MPA, but has low PPV, particularly when tested for nonspecific symptoms.

Objective: To evaluate the clinical utility of serial fetal echocardiographic surveillance, specifically atrioventricular interval (AVI) monitoring, in managing Anti-SSA/Ro-SSB/La-Positive systemic lupus erythematosus (SLE) pregnancies.

Methods: SLE pregnancies with positive anti-SSA/Ro-SSB/La antibodies were retrospectively included (n = 50). Pregnancies were stratified into two groups based on based on whether optimal pre-gestational conditions were met. All pregnancies underwent serial fetal echocardiography, including structural assessment and precise AVI measurement.

Results: The overall incidence of fetal cardiac conduction abnormalities was 12% (6/50). The incidence was significantly higher in the suboptimal-conditions group (group 2, 26% [5/19]) compared with the optimal-conditions group (group 1, 3% [1/31]) (p = 0.024). Through close monitoring, one fetus with persistent AVI prolongation received dexamethasone and intravenous immunoglobulin, successfully preventing progression to advanced heart block; the other five cases normalized spontaneously upon short-term follow-up, avoiding overtreatment.

Conclusion: SLE pregnancies with anti-SSA/Ro-SSB/La antibodies and suboptimal gestational conditions constitute a distinct high-risk subgroup. A management strategy through risk-stratified serial fetal AVI monitoring allows for precise treatment identification, significantly improving neonatal outcomes while avoiding unnecessary interventions.

Objectives: A core set of patient-reported outcomes (PROs) for systemic lupus erythematosus (SLE) has not been established, and no studies have compared predictive validity of disease-specific and generic quality of life (QOL) instruments. We aimed to compare the predictive validity of the Lupus PRO and Medical Outcomes Study Short-Form-12 (SF-12) for damage accrual in patients with SLE.

Methods: The Lupus PRO questionnaire contains both health-related (HR) and non-HR-QOL measures, whereas the SF-12 indices are the physical component summary (PCS), mental component summary (MCS), and role-social component summary (RCS). Damage accrual was evaluated using an increase of one unit in the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI). We examined the association and predictive accuracy of the Lupus PRO and SF-12 scores at baseline for damage accrual using survival models for recurrent events.

Results: Among 1326 patients, those with a higher HR-QOL on Lupus PRO at baseline showed significantly lower damage accrual (hazard ratio: 0.94, 95% confidence interval [CI]: 0.89-0.99), whereas higher PCS and RCS of SF-12 were associated with lower damage accrual (0.92, 95% CI: 0.86-0.98; 0.92, 95% CI: 0.86-0.99). The Akaike Information Criterion, Bayesian Information Criterion, C-index, and area under the curve were comparable between the Lupus PRO and SF-12. Only higher PCS of the SF-12 was associated with glucocorticoid-independent SDI.

Conclusion: Predictive metrics and discriminatory performance were comparable between the Lupus PRO and SF-12. Our findings highlight both disease-specific and generic QOL measurements can be valuable options for core set outcomes.

Objectives: To determine the prevalence of objectively defined gastric dysmotility in systemic sclerosis (SSc) and to evaluate its associations with SSc clinical and immunological features and adverse outcomes (SSc-related death and/or lung transplantation).

Methods: We conducted a retrospective cohort study. All participants underwent a standardized 4-h solid gastric emptying scintigraphy. Gastric dysmotility was defined as ≥ 20% gastric retention at 4-h. We examined associations between gastric dysmotility and demographic, clinical, and immunologic characteristics of SSc, as well as relevant clinical outcomes.

Results: Ninety-four patients were included (mean age 48 ± 12 years; 81% female). Gastric dysmotility was identified in 33 patients (35%). Patients with gastric dysmotility had a higher prevalence of interstitial lung disease (63.6%.vs.30.3%, p= 0.033) and absent esophageal contractility (75.8%.vs.45.9%, p< 0.001) compared with those without. Gastric dysmotility was less frequent in patients with anti-centromere antibodies (27.3%.vs.55.0%, p= 0.009) but more common in those with anti-U1RNP antibodies (15.6%.vs.1.7%, p= 0.011). During follow-up (mean duration 2.8 ± 1.7 years; 260 person-years), 16 patients (17.0%) experienced the composite end point of SSc-related death and/or lung transplantation. In unadjusted Cox regression, gastric dysmotility was strongly associated with adverse outcomes (HR = 6.47, 95%CI: 2.09-20.10, p= 0.001), which remained significant after adjustment for confounders (HR = 4.23, 95%CI: 1.25-14.33, p= 0.020).

Conclusions: In SSc, we have confirmed that gastric dysmotility is associated with a distinct clinical and serological phenotype and that it independently predicts serious adverse outcomes. These findings underscore the importance of objective assessment of gastric function and its potential role in risk stratification.