Abstract Objectives Comorbidities are highly prevalent in Systemic lupus Erythematosus (SLE) and are associated with increased morbidity and mortality. We investigated the association between cumulative comorbidity burden, physical function, and activities of daily living (ADLs) in SLE patients. Methods In this cross-sectional study, we assessed comorbidity burden using the Age-Adjusted Charlson Comorbidity Index (ACCI) and Rheumatic Disease Comorbidity Index (RDCI) in 240 consecutive patients with SLE. Grip strength and dexterity were assessed using the Jamar dynamometer and Purdue pegboard test, respectively. ADLs were evaluated with the Disabilities of the Arm, Shoulder, and Hand (DASH) questionnaire and the Health Assessment Questionnaire (HAQ). We also assessed domains of the Lupus Quality of Life (LupusQoL) Questionnaire related to physical function and ADLs, including “Physical Health”, “Pain”, “Planning” and “Fatigue”. Associations were examined using multivariate linear regression models adjusted for potential demographic and clinical confounders. Results Median (IQR) ACCI and RDCI scores were 2 (1-3) and 0 (0-2), respectively. In multivariate analysis, higher ACCI scores were associated with impaired dexterity (p < 0.001), reduced grip strength (p < 0.05) across all models, and higher DASH scores and poorer scores in the “Physical Health” and “Pain” domains, after adjustment for pain intensity and fibromyalgia (p < 0.05). Higher RDCI scores were associated with impaired dexterity (p < 0.01), poorer scores in the “Physical Health” domain across all models, and higher DASH scores in three models (p < 0.05). Conclusion Comorbidity burden is independently associated with poorer physical functioning and ADLs performance in people with SLE.
Abstract Objectives Large vessel vasculitis (LVV), including giant cell arteritis (GCA) and Takayasu arteritis (TAK), are rare immune-mediated disorders characterized by inflammation of large arteries. Epidemiological patterns, marked geographic gradients, age and sex differences, and temporal clustering, suggest that environmental factors contribute to disease onset in genetically susceptible individuals, yet no systematic mapping of this evidence exists. The study sought to systematically map current evidence on environmental exposures investigated in relation to LVV onset, identify methodological gaps, and characterize research priorities. Genetic susceptibility data were additionally synthesized to contextualize gene–environment interactions. Methods We conducted a scoping review following PRISMA-ScR guidelines. PubMed and Embase were searched from inception to August 2026. Studies were eligible if they assessed environmental exposures in relation to GCA or TAK onset using an epidemiological design with a comparator group, Mendelian randomization, or mechanistic approach with direct relevance to human susceptibility. Genetic susceptibility was synthesized through a complementary narrative review. Results Of 3,596 records identified, 2,137 were screened after deduplication, and 44 studies were included in the narrative synthesis, of which 34 with established temporality and epidemiological design were summarized in a structured evidence table. Air pollution, infections, antibiotic use, hormonal exposures, and cardiometabolic factors emerge as candidate environmental modulators of LVV onset, although causal links remain uncertain. Genetic susceptibility implicates antigen presentation, immune regulation, innate immune activation, and vascular remodeling pathways, which likely modulate individual responsiveness to environmental triggers. Conclusions This review identifies multiple candidate environmental exposures in LVV and highlights critical methodological gaps regarding exposure timing and genomic integration. Integrative exposomic-genomic approaches represent the critical next step toward identifying modifiable risk factors and preventive strategies.
Abstract Objectives Dystrophic calcinosis, a frequent complication of dermatomyositis (DM) and juvenile DM (JDM) is poorly responsive to treatment. We analysed the outcomes of calcinosis in patients with idiopathic inflammatory myositis (IIM) and calcinosis treated with rituximab. Methods IIM patients with calcinosis who received at least one cycle of rituximab (750 mg/m² in adults and 575 mg/m² in children on day 1 and day 14) were included. Baseline data included MMT8, patient global assessment, physician global assessment, myositis-specific and myositis-associated antibodies, duration, location and morphological pattern on plain radiographs of calcinosis. Improvement was defined as any reduction of calcinosis on plain radiographs. Results Of 224 IIM patients, 18 had calcinosis of whom 10 received rituximab (six JDM and four adult patients). Five of 10 patients were anti-NXP2, other MSAs were anti-MDA5, anti-PL7, and anti-Mi2. Seven of 10 patients had calcinosis at presentation; three developed subsequently. Predominant morphologies (radiographically) were nodular and sheet-like; two had mixed patterns. Eight of 10 patients also had active myositis (MITAX A or B) at rituximab initiation—of these, all showed either improvement or stabilization. Of the two with inactive disease treated solely for calcinosis, one stabilised; the other worsened. The one patient who worsened had diffuse distribution and mixed morphology of calcinosis. Conclusion In this small series, rituximab initiation during active myositis was associated with improvement or stabilization of calcinosis; however, given the limited sample size, this observation should be interpreted cautiously and requires prospective validation.
Abstract Objectives To identify baseline predictors and develop a clinical risk stratification model for severe infections (SIs) in patients with idiopathic inflammatory myopathy (IIM) after remission induction therapy. Methods We enrolled patients diagnosed with IIM between 1991 and 2024 from three Japanese centres participating in the multicentre Idiopathic Inflammatory Myopathy and Anti-synthetase Syndrome (MYKO) cohort. SI was defined as an infection requiring i.v. antibiotics or hospitalization. We explored the risk factors for predicting SI in patients with IIM by comparing the initial demographic, clinical, laboratory and treatments between the SI and non-SI groups. Results Among 349 patients with IIM, 14.6% of patients developed SIs after remission induction therapy over a median follow-up of 4.7 years. Patients who developed SI exhibited significantly lower baseline albumin levels, and significantly higher baseline CRP, lactate dehydrogenase and surfactant protein (SP-D) levels than those without SI. Multivariate logistic regression analysis demonstrated that elevated CRP and SP-D levels were independently associated with SI. Serum CRP levels remained significantly higher in the SI group than in the non-SI group over the 2-year period after immunosuppressive therapy, and the serum SP-D levels tended to be higher in the SI group. The 10-year SI rate was significantly different among patients with IIM stratified by the number of risk factors, defined as CRP ≥ 0.8 mg/dl and SP-D ≥ 96.2 ng/ml. Conclusions This model, which consists of CRP ≥ 0.8 mg/dl, SP-D ≥ 96.2 ng/ml, is useful for clinical risk stratification of SI after remission induction therapy in IIM.
