OBJECTIVE:This study aimed to explore evidence related to long-term health outcomes for patients with connective tissue disease (CTD)-associated pulmonary arterial hypertension (PAH) and any clinical practices associated with screening, early detection, and initiation of treatments. METHODS:We conducted a systematic literature review (SLR) of observational studies and randomised controlled trials of adult patients with confirmed CTD-PAH, including systemic lupus erythematosus (SLE)-PAH, systemic sclerosis (SSc)-PAH, and mixed CTD (MCTD)-PAH. Based on the findings from our SLR, we conducted a Delphi panel to develop recommendations and consensus around early detection and initiation of appropriate treatment for patients with CTD-PAH. RESULTS:Sixty-three publications (54 studies and 9 guidelines) were included in the SLR, with 22 involving Asia-Pacific populations. Combination therapy reduced clinical worsening and mortality versus monotherapy. After three Delphi rounds, consensus was reached on the importance of early PAH screening in CTD, with disease-specific recommendations for SLE, SSc, and MCTD. Consensus was also achieved on appropriate treatments, including endothelin receptor antagonists (ERA), phosphodiesterase-5 (PDE5) inhibitors, and pulmonary vasodilators. However, long-term outcomes remain uncertain due to limited evidence. CONCLUSION:This study highlights the need for early screening and timely treatment initiation in CTD-PAH to potentially reduce adverse long-term outcomes.
In this review, we summarized the current evidence on the risks of incident malignancy and cancer recurrence in patients with rheumatoid arthritis (RA) treated with methotrexate (MTX). Before considering the effect of MTX, it is important to recognize the malignancy risk associated with RA itself. The overall risk of malignancy in patients with RA modestly increased, as per two meta-analyses, although Japanese studies report inconsistent findings, with one showing a slightly decrease and another a slight increase. The risk of malignant lymphoma has increased, whereas the risk of colorectal cancer appears reduced. Additionally, there are concerns regarding the increased risk of lung and skin cancers. However, there is limited evidence specific to patients with RA treated with MTX. According to an Australian report, patients have a higher risk of overall malignancy, with an increased risk of malignant lymphoma, lung cancer, and melanoma. Studies comparing MTX with tumor necrosis factor inhibitors have reported no significant difference in the overall malignancy risk or increased malignancy risk. In White patients, MTX has been associated with an increased risk of recurrence in patients with RA and a history of non-melanoma skin cancer. There is insufficient evidence regarding the risks of MTX use in patients with RA and malignancy. Therefore, decisions regarding MTX use should be made individually based on shared decision-making, and careful follow-up is necessary.
OBJECTIVES:Enthesitis-related arthritis (ERA), characterized by chronic arthritis and/or enthesitis, is a relatively rare subtype of juvenile idiopathic arthritis (JIA). Achieving sustained disease control can be challenging. We aimed to characterize ERA and to evaluate the efficacy of salazosulfapyridine (SASP) treatment. METHODS:We retrospectively reviewed 23 patients with ERA treated in our institute between 2015 and 2025. Clinical characteristics, laboratory findings, and treatment courses were analyzed descriptively. Patients were categorized into three groups based on treatment escalation following NSAID therapy. RESULTS:The median age at onset was 10 years, and median disease duration before diagnosis was 11 months; 47.8% were females. Axial involvement was present in 65.2%, and psychological comorbidities in 52.2%. Nineteen patients received SASP; four who were younger at onset and had lower CRP levels at diagnosis were maintained on SASP monotherapy without treatment escalation. Active joint and enthesitis counts generally improved during follow-up, although some patients had persistent disease activity at 12 months. No severe adverse events were observed. CONCLUSIONS:Diagnostic delay and psychological comorbidities were characteristic of ERA. SASP may be beneficial in selected patients with lower inflammatory activity at diagnosis; prospective studies are needed to better define its role in ERA management.
OBJECTIVES:This study aimed to evaluate real-world effectiveness and long-term retention of baricitinib in Japanese patients with rheumatoid arthritis (RA), including those classified as difficult-to-treat (D2T). METHODS:This 24-month observational study enrolled patients initiating baricitinib, stratified by prior use of biological/targeted synthetic disease-modifying antirheumatic drugs (b/tsDMARDs) and baseline Clinical Disease Activity Index (CDAI) scores. Key endpoints included time-to-discontinuation, achievement of and time to low disease activity (LDA) or remission. Data were analysed using logistic regression models adjusted for demographic and clinical variables. RESULTS:Baricitinib demonstrated sustained effectiveness, with 58.2% of overall patients (N=353) and 42.0% of patients with D2T RA (N=88) remaining on treatment at 24 months. CDAI remission was achieved in 36.4% of overall patients and 22.9% patients with D2T RA. Discontinuation was mainly due to primary non-response (11.0%). Mean (SD) changes in patient-reported outcomes (PROs) from baseline scores were: patient global assessment of disease activity (-21.7 [28.4] mm), Health Assessment Questionnaire-Disability Index (-0.360 [0.601]), and pain visual analogue scale (-24.9 [26.8] mm). Among baseline glucocorticoid users retained on baricitinib at 24 months, 55.9% discontinued glucocorticoids. CONCLUSION:Baricitinib showed sustained effectiveness and retention, including in patients with D2T RA; early improvements in pain and fatigue were predictive of CDAI disease remission during the 24-month follow-up.
OBJECTIVES:Total elbow arthroplasty is a common treatment for elbow stiffness due to rheumatoid arthritis. Although linked prostheses are often preferred, unlinked implants may reduce prosthetic stress and loosening risk. This study evaluated the clinical outcomes of unlinked total elbow arthroplasty in patients with rheumatoid arthritis presenting with stiff elbows. METHODS:This retrospective study included 9 women with 10 stiff elbows due to rheumatoid arthritis who underwent primary total elbow arthroplasty using an unlinked implant. Stiffness was defined as a range of motion of less than 60°. Mean age was 58 years and mean follow-up was 59 months. Outcomes included range of motion, Mayo Elbow Performance Score, pain during movement assessed with a visual analogue scale, complications, and implant loosening. RESULTS:Extension improved from -59° to -38°, flexion from 101° to 123° and arc of motion from 42° to 85°. Mayo Elbow Performance Score improved from 53 to 93, and pain score from 36 to 1. Two cases of transient ulnar neuropathy resolved without treatment. No implant loosening, dislocation, or revision surgery occurred. CONCLUSIONS:Unlinked total elbow arthroplasty improved clinical outcomes without major complications and may be a viable option for stiff elbows in patients with rheumatoid arthritis.
