Clinical Rheumatology最新文献

Objective: Giant cell arteritis (GCA) is the most common vasculitis and can result in blindness due to anterior ischemic optic neuropathy (AION). Little is known about which patients with GCA are at higher risk of AION.  We did a retrospective chart review to compare demographics, past medical history, labs and imaging of GCA patients with and without AION.

Methods: All patients at the University of Washington who were diagnosed with GCA by a rheumatologist, had a vascular ultrasound and met classification criteria for GCA were included. AION was diagnosed by an ophthalmologist. To compare demographics, symptoms, presentation imaging and lab findings, we used Pearson Chi-square, Fisher's exact test and t-tests to compare GCA patients with and without AION.

Results: 91 patients with GCA without vision loss were compared to 15 patients with GCA and AION. The AION group had significantly more men (p=0.03) and elevated C-reactive protein (CRP) as compared to the non-AION group (p = 0.04). Rates of hypertension, smoking, erythrocyte sedimentation rate (ESR) and classification scores were similar. Vascular ultrasound showed similar rates of small and large vessel vasculitis, but the AION group had higher halo scores, with the AION group having a mean score of 5 and patients with GCA and without AION having a mean score of 3 (p < 0.01).

Conclusions: Older men with GCA and more than three halos on vascular ultrasound imaging of GC may be at higher risk of AION. Key Points • This is the first paper to incorporate the use of vascular ultrasound in comparing patients with anterior ischemic optic neuropathy (AION) to those without giant cell arteritis (GCA). • Male sex is associated with AION despite GCA being more common in women. • Older age and higher CRP are associated with AION. • GCA patients with AION had a higher halo score than GCA patients without AION.

Introduction: Although familial Mediterranean fever (FMF) is a relevant disease in countries surrounding the Mediterranean Sea, there are still few reports from Italy.

Methods: We retrospectively evaluated patients with FMF diagnosed according to the EuroFever/PRINTO classification criteria in three pediatric rheumatology referral centers in central-southern Italy. Logistic regression analysis assessed the associations between age at disease onset and symptoms.

Results: Overall, 48 patients were enrolled (28 females, 20 males), with a median age at onset of 3.3 [3.1] years, and a median follow-up period of 5.1 [10.8] years. The most common MEFV genotype was M694V/- (11 patients, 22.9%), followed by M694V/M694V (6 patients, 12.5%). At onset, recurrent fever was observed in 47 patients (97.9%), with a median time between attacks of 18 [11] days. Overall, recurrent fever was observed in all patients, abdominal pain in 44 (91.7%), and chest pain in 18 (37.5%). At the last follow-up visit, 24 patients were on colchicine (50%), 2 on biologic (4.2%), and 6 on both (12.5%). Canakinumab was the most used biologic drug, in 6 (12.5%) patients. MEFV genotype was associated with disease severity (p = 0.007) and the use of a biological drug (p = 0.01). FMF prevalence in the Abruzzo region was found highly than expected (at least 1:45,000). Differently, we found a relevant gap among FMF patients expected and observed in the Apulia and Sicily regions.

Conclusions: FMF is a relevant issue in central-southern Italy. A large epidemiologic study should be performed to better define its prevalence in the country. Key Points • Italian children with familial Mediterranean fever tend to have an early age of onset, primarily manifesting with recurrent fever and characteristic associated symptoms. • Many MEFV gene variants are present in Italian children with familial Mediterranean fever, and these patients are most often heterozygous, exhibiting a mild to moderate phenotype. • The prevalence of familial Mediterranean fever in Italy is still unknown but recently estimated to be around 1:60,000, probably higher in central and southern Italy. • According to our cohort, the prevalence of FMF in the Abruzzo region is at least 1:45,000, higher than expected. Differently, we found lower prevalence rates of the disease in Apulia and Sicily.

The reported prevalence of hypermobility spectrum disorders (HSD) and hypermobile Ehlers-Danlos Syndrome (hEDS) is unclear due to complex presentations and lack of awareness amongst professionals, contributing to diagnostic and management uncertainties. Standardising an outcome measure to assess symptoms most important to patients should help determine the impact of interventions. This Delphi study aims to reach a consensus with stakeholders on the core outcome set for children and adults with HSD/hEDS. A three-round modified Delphi consensus study with a follow-up consensus meeting was used. Stakeholder groups consisted of (1) individuals with HSD/hEDS; (2) family/friends/carers and (3) healthcare professionals. Participants could belong to more than one stakeholder group. They rated 74 symptoms using a 9-point Likert scale: 1 "not important to 9 "critically important". Symptoms achieving a consensus rating of ≥ 70.0% critical importance across all groups were included. In Round 1, 766 responses were received from 600 participants, reducing to 566 responses from 438 participants by Round 3, with 53 participating in the consensus meeting. Overall, 30 symptoms met the ≥ 70.0% critically important threshold to be included in the final core outcome set. These were categorised under the specialties of musculoskeletal and orthopaedics, social, pain, gynaecology and urology, negative affect, neurological, gastrointestinal and "other". This study is the first to identify by consensus the core outcome set to be measured for patients with HSD/hEDS. The importance of these outcomes was confirmed by individuals living with the condition, their family, friends, carers and relevant healthcare professionals. Trial registration: IRAS ID: 326,855; East Midlands - Leicester South REC (reference: 23/EM/0143); Protocol registered with the COMET Initiative. Key Points • There is a lack of standardised outcome measure for HSD/hEDS research studies due to the heterogeneity of symptom presentations. • Symptoms across musculoskeletal and orthopaedics, social, pain, gynaecology and urology, negative affect, neurological, gastrointestinal and "other" specialties were identified as the core outcome set defined as ≥ 70.0% critically important to measure for individuals with HSD/hEDS. • There were a significant number of symptoms, widely recognised in the literature to be comorbid to HSD/hEDS, that reached a critical importance threshold of 50.0-69.9%, restricting the core outcome set to only those that met 70% or above may be limiting.

To evaluate the efficacy of baricitinib compared to TNF-α Inhibitors and conventional DMARDs (cDMARDs) in patients with RA. Our study included 334 RA patients classified into 3 groups: the first receiving baricitinib, the second receiving TNF-α Inhibitors, and the third receiving cDMARDs. Patients were evaluated at baseline, week 12, and week 24 using TJC, SJC, VAS, DAS28, CDAI, and HAQ-DI. Larsen score was measured at baseline and 24 weeks. The response to therapy was assessed at weeks 12 and 24 using ACR 20, ACR 50, and ACR 70 response criteria. Emerging treatment side effects were monitored. Patients receiving baricitinib showed significant improvement regarding all outcome measures at weeks 12 and 24. In addition, baricitinib was comparable to TNF Inhibitors in all outcome measures except the ACR 70 at week 12, which was higher in the baricitinib group. Furthermore, baricitinib group showed significantly better outcome measures and response to therapy in comparison to cDMARDs group. The most common side effects in the baricitinib group were infection, GIT, and CVS complications. The most common side effects in the TNF inhibitors group were infection and skin complications. The cDMARDs had the least side effects, mostly GIT complications. Baricitinib is an effective drug for treating RA refractory to cDMARDs, improving disease activity measures and functional status and reducing the progression of structural joint damage. It has a comparable efficacy and safety profile to TNF Inhibitors. Multicenter studies are recommended to support our results. Key Points • Baricitinib is an effective therapeutic choice for rheumatoid arthritis refractory to cDMARDs. • Patients treated with baricitinib showed improvement in all outcome measures and functional status. • Bricitinib delayed the progression of radiographic joint damage more effectively than cDMARDs. • The efficacy and safety of baricitinib for treating rheumatoid arthritis is comparable to that of TNF inhibitors.

Introduction/objectives: Overuse of antinuclear antibody (ANA) tests leads to increased costs, false positives, and unnecessary treatments. This study evaluated ANA overuse in internal medicine and neurology departments and assessed the impact of an educational intervention.

Method: This quality improvement educational intervention study examined ANA test overuse in five internal medicine departments and one neurology department at a university-affiliated medical center. The educational intervention included a session focusing on ANA testing appropriateness. Outcome measures comprised the ANA/new patient ratio (APR) and the percentage of positive ANA test results. Outcomes were compared between the pre- and post-intervention periods (both 6 months).

Results: The intervention took place in December 2021. The APR decreased from 43% in the pre-educational intervention period to 27% in the post-intervention period in the neurology department (odds ratio [OR] 0.49, confidence interval [95% CI] 0.37-0.63, P < 0.0001) and from 2.6% to 2.2% in the internal medicine departments (OR 0.89, 95% CI 0.73-1.10, P = 0.28). The percentage of positive ANA tests increased from 43% pre-intervention to 53% in the post-intervention period (OR 1.49, 95% CI 0.90-2.46, P = 0.12) in the neurology department and from 48% to 59% (OR 1.56, 95% CI 0.99-2.44, P = 0.0543) in the internal medicine departments.

Conclusion: A simple educational intervention reduced unnecessary ANA testing in the neurology department but not in internal medicine departments, improving patient selection and potential cost savings. The results underscore the importance of targeted education to promote evidence-based behavior among healthcare professionals. Further research with longer follow-up is needed to assess the sustainability of these improvements.

Objectives: To determine the association between blood metal mixture and HUA risk.

Methods: A cross-sectional study was performed based on the populations from Kailuan cohort trial in China. Inductively coupled plasma mass spectrometry was utilized to detect the blood concentrations of iron (Fe), lead (Pb), calcium (Ca), copper (Cu), magnesium (Mg), zinc (Zn), selenium (Se), and manganese (Mn). Multivariate logistic regression (MLR) models and weighted quantile sum (WQS) regression models were employed to assess the relationships.

Results: There were 3706 participants included in this study, and 464 (12.52%) were with HUA. The mean level of uric acid was 485.60 μmol/L in the subjects with HUA, which was remarkably increased compared to those without HUA (293.10 μmol/L). The multivariate-adjusted odds ratios (95% confidence intervals) of HUA were 2.15 (1.40, 3.29) for Pb, 3.42 (2.24, 5.23) for Fe, 1.61 (1.15, 2.25) for Ca, 3.06 (2.04, 4.59) for Mg, and 1.69 (1.26, 2.28) for Zn by comparing the highest and lowest metal quartiles using the single-metal logistic regression model. Meanwhile, the WQS regression models demonstrated a positive association between metal mixtures and HUA risk, to which, Fe, Mg, Pb, and Zn were the major contributors. Pb, Fe, and Zn were still robust in multiple-metal models. Age, gender, weight, smoking, and drinking status could modify these relationships with significant interactions.

Conclusions: Co-exposure to Fe, Pb, Ca, Cu, Zn, Mg, Se, and Mn were related to increased HUA risk in Chinese adults, of with, Pb, Fe, and Zn appeared to have greater impacts. These relationships were more obvious in adults aged < 60 years, or women, or overweight, or non-smoking, or drinking with significant interactions. Key Points • Increased Fe, Pb, Mg, Ca, and Zn were related to increased HUA risk in dose-response fashion among Chinese adults. • The metal mixture dominated by Fe, Mg, Pb, and Zn was positively associated with HUA risk. Fe, Pb, and Zn were still robust in MM model. • Such association was stronger showed in adults aged < 60 years, or women, or overweight, or non-smoking, or drinking with significant interactions.

Objective: To analyze the HRQoL loss associated with self-perceived anxiety/depression in patients with seropositive rheumatoid arthritis (RA).

Method: This secondary data analysis is based on a registry-based retrospective follow-up study of patients with seropositive RA treated between August 2014 and January 2023 in ARTMEDICA, Colombia. HRQoL loss and self-perceived anxiety/depression were defined as outcomes. Disease activity (DAS-28) and other patient data were also gathered. Statistical analyses were performed using the ordinal logistic and generalized linear regression models.

Results: A total of 3579 patients with a mean follow-up of 2.9 (SD 2.4) years, 85.6% women with a median age at diagnosis of 48.1 (IQR 37.8-57.5) years, and a median of 6.5 (IQR 1.9-14.7) years living with RA were included. At program admission, the median DAS-28 score was 2.8 (IQR 2.1-4.2), and 6.6% of patients reported extreme anxiety/depression. The average HRQoL loss was 3.4 months per year lived with seropositive AR. Among patients with no pain or discomfort, moderate and extreme anxiety/depression were associated with mean HRQoL losses of 2.2 (95% CI - 2.3 to - 2.2) and 4.1 (95% CI - 4.3 to - 3.8) months. In patients with extreme pain/discomfort, these estimations were 0.8 (95% CI - 0.9 to - 0.7) and 1.9 (95% CI - 2.1 to - 1.7) months, respectively.

Conclusion: Our study adds to the available body of evidence by clarifying the differential impact of anxiety/depression on HRQoL, depending on the severity of pain. These findings highlight the importance of strengthening mental health care and psychological well-being interventions for patients with RA, regardless of pain or disease activity. Key Points • The average HRQoL loss was 3.4 months per year lived with seropositive AR. • Pain/discomfort rather than disease activity explained the severity of anxiety/depression as well as its associated HRQoL loss. • For patients with extreme pain/discomfort and anxiety/depression, the average HRQoL loss was 8.1 months per year lived with the disease compared to 0.4 months for patients without those impacts.