Journal of Rheumatology最新文献

Objective: To evaluate the association between the daily GC dose and various patient-reported outcomes (PROs) in patients who have achieved lupus low disease activity state (LLDAS).

Methods: Patients from a single-center cohort were included. PROs included were the FACIT-F (Functional Assessment of Chronic Illness Therapy-Fatigue), the LupusQoL (Lupus Quality of Life), the physical and mental component summary measures of the SF-36 (36-Item Short Form Health Survey) and the LFA-REAL PRO (Lupus Foundation of America Rapid Evaluation of Activity in Lupus PRO). Univariable and multivariable generalized estimating equations (GEE) were performed; the multivariable models were adjusted for possible confounders: age at diagnosis, sex, socioeconomic status, educational level, ethnicity, disease duration, disease activity and damage, antimalarial and immunosuppressant use. In an alternative analysis, GEE were also performed with patients categorized on the basis of 4 prednisone dose categories: 0mg, >0mg & ≤2.5mg, >2.5mg & ≤5mg and >5mg & ≤7.5mg.

Results: Three-hundred and twenty-four patients and 1338 LLDAS visits were included. In the adjusted analysis, the daily GC dose was associated with worse FACIT-F, LFA-REAL PRO and four out of the eight domains of the LupusQoL scores. In the alternative analysis, after adjustment, the patients in the high GC dose category had a trend of worse PRO scores in the adjusted analysis and the ones in the lower GC dose category showed a trend of better PRO scores.

Conclusion: The daily GC dose is associated with worse PROs in SLE patients on LLDAS, even after adjusting for possible confounders.

Objective: To investigate respiratory syncytial virus (RSV) vaccine uptake, associations, and breakthrough infection among patients with systemic autoimmune rheumatic diseases (SARDs).

Methods: We performed a retrospective cohort study investigating RSV vaccination among patients with SARDs at Mass General Brigham (Boston, Massachusetts, USA). We identified all patients with SARDs who were aged ≥ 60 years and thus eligible to receive the RSV vaccine between May 2023 and February 2025. We used multivariable logistic regression to identify factors associated with RSV vaccination. Among the vaccinated, we described documented cases of laboratory-confirmed breakthrough RSV infection.

Results: Among 10,587 patients with SARDs (median age 71.7 years, 72.4% female) eligible for RSV vaccination, 1075 (10.2%) received RSV vaccination. Factors associated with higher odds of RSV vaccination included higher median census-tract household income and comorbidities, such as cancer and interstitial lung disease. Associations with lower odds of RSV vaccination included Black race, lack of previous influenza or COVID-19 vaccinations, and glucocorticoid use. RSV vaccination was not associated with specific SARD types or disease-modifying antirheumatic drugs (DMARDs), including CD20 inhibitors. Among the 1075 who were vaccinated, there were 9 (0.8%) documented cases of RSV breakthrough infection (2 hospitalizations and no deaths).  CONCLUSION: Only 10.2% of eligible patients with SARDs received RSV vaccination. Glucocorticoid users were less likely to receive RSV vaccination, whereas specific SARD types and DMARDs were not associated. Although some predictors of vaccine uptake were observed in this dataset, there are many unmeasured factors that may play a role in vaccine uptake. There were few documented breakthrough infections and no deaths. Future studies are needed to optimize RSV vaccine use and establish safety and efficacy in this vulnerable population.

Objective: To compare five Health Assessment Questionnaire (HAQ) scoring methods to measure functional disability among people with systemic sclerosis (SSc, scleroderma).

Methods: Scleroderma Patient-centered Intervention Network Cohort participants completed the Health Assessment Questionnaire (20 items, 8 domains) at enrolment. We calculated HAQ Disability Index (HAQ-DI) scores, which sum the highest item score for each domain and account for the use of aids, devices, or assistance; Alternative Disability Index (HAQ-ADI) scores, which are calculated similarly but do not account for aids, devices, or assistance; Modified HAQ (MHAQ) scores, which are based on one administered item from each domain, as well as a simple summed score of all 20 items. We then compared these scores and those generated from an Item Response Tree (IRTree) on convergent validity with physical function, pain interference, and hand function using Pearson's correlations.

Results: IRTree-based scores were highly correlated (r = 0.90 to 0.95) with other scoring procedures and showed moderate-to-strong correlations with all external measures (r = 0.68 to 0.80). There was no evidence of a difference between IRTree-based and HAQ-DI correlations with external measures. IRTree-based scores performed better than HAQ-ADI, MHAQ, and summed scores for physical function and pain interference but worse for hand function.

Conclusion: IRTree-based scoring is a novel approach that incorporates information from all HAQ items and whether participants use aids, devices, or assistance. Its association with external measures, however, did not differ from the standard HAQ-DI. HAQ-DI scoring is easy to implement, and extensive comparative data are available, making it the preferred scoring method.

Objective: To assess the effect of calcium pyrophosphate crystal deposition disease (CPPD, chondrocalcinosis) on the axial skeleton and compare it with degenerative disc disease (DDD).

Methods: Conventional radiographs (CR) and Magnetic Resonance Imaging (MRI) of patients with CPPD or DDD were retrospectively assessed by two independent readers for vacuum phenomena, disc calcification, endplate erosion, osteophytes, disc height changes and spondylolisthesis. Available follow-up CR were assessed.

Results: CR from 140 CPPD (1.171 discovertebral units, DVU) and 99 DDD (803 DVU) were evaluated (mean age 74.4±9.9 vs. 71±6.2, 20% vs. 20.2% males, respectively). Disc calcification, osteophytes and erosions were noted significantly more frequently in CPPD vs. DDD (16.5% vs 5.2%, 73.5% vs 59.8% and 13.6% vs 3.2%, respectively, all p≤0.002). Follow-up CR from 29 CPPD and 46 DDD patients were available. Significant progression of endplate erosions and osteophytes was seen (p≤0.02) in both groups. CR follow-up in the CPPD group was compared to DDD (median (IQR) 1.9 (2.4) vs 3.0 (3.1) years, P=0.03, respectively), shorter, but radiographic thoracic erosive changes were more frequent in CPPD vs. DDD for (6.8% vs. 0.6%, P=0.02) and lumbar disc calcification (5.8% vs. 0.6%, P=0.01).

Conclusion: CPPD is associated with more severe and progressive structural changes in the lower spine compared to DDD. Differentiating CPPD from DDD has clinical implications, influencing both appropriate management strategies and clinical course estimation.

Objective: Idiopathic pulmonary fibrosis (IPF) and systemic autoimmune rheumatic disease (SARD)-associated interstitial lung disease (ILD) are lung disorders with distinct clinical trajectories. This study aimed to compare survival and pulmonary function trends between IPF and SARD-ILD.

Methods: We retrospectively analyzed 410 patients with ILD (154 IPF, 256 SARD-ILD) from 6 Italian centers. SARD-ILD subtypes included antisynthetase syndrome (ASyS; n = 58), dermatomyositis (DM; n = 55), systemic sclerosis (SSc; n = 106), and Sjögren disease (SjD, n = 37). Outcomes included 5-year survival and pulmonary function test (PFT) changes.

Results: Five-year survival was lower in patients with IPF (mean 33.6 months) than in those with SARD-ILD (mean 56.0 months; P < 0.001). SARD subtypes showed comparable survival: 58.2 months in patients with ASyS, 52.9 months in DM, 55.2 months in SSc, and 58.6 months in SjD. Patients with ASyS and DM demonstrated significant functional improvement, with forced vital capacity (FVC) increasing from 71% to 81% in ASyS (+14.1% relative) and from 69% to 78% in DM (+13%). IPF FVC declined from 78% to 72% (-7.7%). Usual interstitial pneumonia pattern was universal in IPF but seen in < 20% of patients with SARD-ILD. ILD pattern did not significantly influence functional trajectory in patients with SARD-ILD; instead, diagnosis was the primary determinant (multivariable ANOVA P < 0.001). Multivariable analysis confirmed SARD-ILD as a favorable prognostic factor (adjusted hazard ratio [aHR] 0.21), with age (aHR 1.06) and male sex (aHR 1.98) linked to poorer outcomes.

Conclusion: SARD-ILD is associated with higher survival than IPF. Functional trajectories improved in patients with ASyS and DM, in contrast to the decline observed in those with IPF. Prognosis is more strongly influenced by the underlying diagnosis, supporting a diagnosis-centered approach to disease management.