Search

The Systemic Lupus Activity Questionnaire (SLAQ) is a patient-reported outcome for systemic lupus erythematosus (SLE). We aimed to translate it into Japanese and further investigate its validity and reliability. The English version of the SLAQ was translated into Japanese and administered to Japanese SLE patients at our university clinic. Physicians assessed disease activity using the SLE Disease Activity Index 2000 (SLEDAI-2K). The patients were prospectively followed for repeat assessment a year later. Ultimately, 255 patients participated. The patients’ 10-point ratings of disease activity and SLAQ scores were significantly correlated (Spearman’s ρ = 0.53). The SLAQ score was weakly correlated with the SLE Disease Activity Index 2000 (SLEDAI-2K)-nolab (omitting laboratory items; ρ = 0.18) but not with the SLEDAI-2K (ρ = 0.02). These results suggested its convergent and discriminant validity. The SLAQ demonstrated acceptable internal consistency (Cronbach’s α = 0.80), and good test–retest reliability (intraclass correlation coefficient = 0.85). The effect sizes and the standardized response means of the SLAQ were as follows: clinical worsening, 0.26 and 0.31, and improvement, −0.39 and −0.41, respectively, which indicated a small but significant responsiveness. The Japanese version of the SLAQ demonstrated acceptable reliability and validity; its performance was comparable to that of the original version. [ABSTRACT FROM AUTHOR]

Objectives: Anti-MDA5 autoantibodies are strongly associated with interstitial lung disease (ILD) and rapidly progressive ILD (RP-ILD) in Asian patients with dermatomyositis (DM) or amyopathic DM (ADM). However, this association has not yet been established in Brazilian patients with anti-MDA5(+) DM/ADM. This study aimed to investigate the phenotypic differences between Brazilian and Japanese patients with anti-MDA5(+) DM/ADM, with a particular focus on ILD., Methods: This was an international, tricentric, retrospective cohort study conducted in one Brazilian and two Japanese tertiary centres. Patients diagnosed with anti-MDA5(+) DM/ADM at the three centres were enrolled. Clinical characteristics and outcomes were collected using a pre-standardised protocol and compared between Brazilian and Japanese patients., Results: Thirty-four Brazilian and 65 Japanese patients were analysed. Brazilian patients were younger at the time of diagnosis than Japanese patients. The prevalence of muscle weakness, myalgia, dysphagia, heliotrope rash, V-sign, calcinosis, Raynaud's phenomenon, and digital ulcers was higher in Brazilian patients, whereas mechanic's hands were more prevalent in Japanese patients. The prevalence of ILD was significantly lower in Brazilian patients than in Japanese patients (50.0% vs. 98.5%, P<0.001). RP-ILD was observed in 34 (52.3%) Japanese patients and in only one (3.3%) Brazilian patient (P<0.001). Outcomes including overall survival and the frequency of relapses and complications, such as severe infection and malignancy, were comparable between the two populations., Conclusions: Brazilian patients with anti-MDA5(+) DM/ADM had a higher prevalence of skin and muscle involvement, whereas the prevalence of ILD and RP-ILD was significantly lower than in Japanese patients.

Background: Cytomegalovirus (CMV) can cause life-threatening diseases in immunosuppressed patients. Some of the patients with connective tissue disease develop CMV infection, and approximately half of this group has been reported to have received pulsed-methylprednisolone (p-MPSL) therapy. This study aimed to identify predictors of the onset of CMV infection in patients receiving p-MPSL therapy for connective tissue disease., Methods: This was a retrospective, multicenter cohort study. We included patients who received p-MPSL therapy for connective tissue disease and had CMV antigenemia measured between April 2011 and December 2020. Peripheral blood cell data before the start of p-MPSL therapy and at the start of steroid tapering were collected in addition to baseline characteristics, including age, sex, and body mass index. CMV infection was defined as detection of one or more CMV antigen-positive cells (CMV-positive). The study examined and compared the CMV-positive group with the CMV-negative group. Logistic regression analysis was used to explore the factors associated with CMV antigen positivity. Receiver operating characteristic curve analysis was used to identify the cut-off values for the factors associated with CMV antigen sensitivity., Results: Of the 200 patients included, 87 had antigen positivity. Logistic regression analysis showed that age ≥ 65 years [adjusted odds ratio (aOR): 2.75, 95% confidence interval (CI): 1.54-4.92] and platelet count less than 30.20 × 10 4 /µL [aOR: 4.38, 95%CI: 2.21-8.68] at baseline were significantly associated with CMV antigen positivity. Lymphocyte count < 1440 /µL [aOR: 5.36, 95% CI: 1.96-14.65], neutrophil-to-lymphocyte ratio (NLR) ≥ 3.42 [aOR: 7.31, 95% CI: 2.52-21.22], and platelet-to-lymphocyte ratio (PLR) ≥ 145.28 [aOR:6.10, 95% CI: 2.24-16.64] at the start of steroid tapering also increased the OR for CMV infection. The areas under the receiver operating characteristic curves for lymphocytes, NLR, and PLR were 0.742, 0.693, and 0.673 respectively., Conclusion: Platelet count, lymphocyte count, NLR, and PLR may be crucial predictors of the onset of CMV infection in patients with connective tissue disease. These easily obtainable factors may be clinically useful as predictors of CMV infection. A potential research area would be to validate the parameters in a prospective patient population., Competing Interests: Declarations. Ethics approval and consent to participate: The study was approved by the Ethics Committee of Showa University (approval number: 2600). Given the retrospective nature of the study, written informed consent from the patients was waived. In case of refusal, we posted an opt-out opportunity. Consent for publication: Not applicable. Competing interests: The authors declare no competing interests., (© 2025. The Author(s).)

Objectives: The incidence of cytomegalovirus (CMV) infection or disease in patients with rheumatic musculoskeletal diseases is reported to be 2%. Over half received pulsed methylprednisolone, and some experienced a fatal outcome. In this study, we aimed to explore predictive and diagnostic biomarkers for CMV infection or disease in such patients and compare them with biomarkers reported for immune reconstitution inflammatory syndrome (IRIS) in people with HIV., Methods: In this multicentre prospective cohort study, we collected blood and saliva samples from 38 patients with rheumatic musculoskeletal disease before initiating high-dose glucocorticoid therapy, at the start of glucocorticoid tapering, at the onset of CMV infection, and 4 weeks later. Peripheral blood cell counts, flow cytometry for CD4, CD8, and Tregs, ELISA for cytokine/chemokine panels, and measurements of herpesvirus-derived DNA in saliva were performed., Results: Lower white blood cells, CD4+ cells, IL-6, and interferon-γ levels and higher interferon-inducible protein (IP)-10 and granulysin levels at baseline could be predictive biomarkers for CMV infection. Furthermore, lower platelet counts and higher IL-10, IP-10, granulysin, TNF-a, IL-1ra, and IL-15 levels at the onset of CMV infection were found as diagnostic biomarkers for CMV infection. EBV, human herpes virus (HHV)-6, and HHV-7 DNA levels in the saliva were significantly increased after high-dose glucocorticoids, regardless of CMV infection., Conclusions: We identified predictive and diagnostic biomarkers for CMV infection after high-dose glucocorticoid therapy for rheumatic musculoskeletal diseases. While similarities with IRIS biomarkers in patients living with HIV were observed, complete agreement could not be confirmed.

Objective: To investigate clinical relevance of performing lung ultrasound (LUS) in patients with connective tissue disease (CTD)-associated interstitial lung disease (ILD) in comparison with high-resolution computed tomography (HRCT)., Methods: This single-centre study enrolled eligible patients with CTD-ILD from the prospective LUS registry. Total B-lines were detected by assessment at 14 sites via LUS. Forced vital capacity, diffusing lung capacity for carbon monoxide (DLCO), DLCO/alveolar volume, 6-minute walking distance, and the ILD-GAP index were used as ILD prognostic parameters. Correlations were examined using single and multiple regression analyses., Results: Sixty-seven patients were enrolled, including 29 with idiopathic inflammatory myopathy or anti-synthetase syndrome, 25 with systemic sclerosis (SSc), 10 with rheumatoid arthritis, and 3 with mixed CTD. The total number of B-lines correlated with ILD extent on HRCT in patients with CTD-ILD (r = 0.66; P < .001), particularly in patients with SSc-ILD (r = 0.78; P < .001). Total B-lines and ILD extent on HRCT showed comparable correlations with prognostic parameters, while multiple regression analysis revealed the limited benefit of performing LUS in addition to HRCT in predicting correlations with prognostic factors., Conclusions: LUS serves as an alternative tool for assessing the severity and prognosis of patients with CTD-ILD., (© Japan College of Rheumatology 2024. Published by Oxford University Press.)

Purpose of Review: In patients with myositis, interstitial lung disease (ILD) is one of the major causes of morbidity and mortality. Given the limited evidence, there is an urgent need to refine the treatment for myositis-ILD. This review aims to highlight recent updates on the management of myositis-associated ILD, focusing on screening, risk stratification, and treatment., Recent Findings: Asian race and/or residence, dermatomyositis, mechanic's hand, antisynthetase antibodies, and antimelanoma differentiation-associated gene 5 antibodies are risk factors for ILD development. Patients with such risk factors should be screened for ILD using high-resolution computed tomography. Various prediction models for mortality or rapidly progressive ILD (RP-ILD) in patients with myositis-ILD have been proposed, but validation of these models in multiple independent studies is required. Academic societies in Japan, the United Kingdom, and the United States have proposed tentative treatment algorithms for myositis-ILD on the basis of the presence or absence of RP-ILD., Summary: Knowledge on myositis-ILD risk stratification, potentially useful for personalized management approaches in clinical practice, is accumulating. However, further global joint efforts are necessary to build a strong evidence base for consensus algorithms for myositis-ILD., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)