Assessment of disease activity in juvenile systemic sclerosis (jSSc) is essential for clinical care and trial readiness, yet no validated pediatric activity measures exist. Adult systemic sclerosis activity tools, including the Scleroderma Clinical Trials Consortium Activity Index, revised CRISS, and revised EUSTAR, provide conceptual frameworks but require adaptation for developmental, physiologic, and feasibility considerations for children. At the 17th Hamburg Symposium on JSSc, an international multidisciplinary panel reviewed adult indices, evaluated corresponding variables in the jSSc Inception Cohort and the NRCOS registry, and conducted a structured Delphi process to define organ-specific indicators of clinically meaningful activity in jSSc.

Background: Orbital metastases are rare in breast cancer, representing only 3-10% of ocular metastases. This report highlights a case where orbital involvement was the first indicator of systemic metastatic spread. Case Presentation: A 72-year-old woman with a history of Estrogen Receptor (ER)-positive (5%), Progesterone Receptor (PR)-negative, Human epidermal growth factor receptor-2 (HER2)-negative breast cancer (diagnosed 3 years prior) presented with right orbital pain, diplopia, and periorbital swelling. Imaging revealed multiple myositis of the extraocular muscles, compressive displacement of the optic nerve, and right periorbital edema. Bone scintigraphy identified multifocal skeletal metastases. A navigation-assisted biopsy confirmed metastatic invasive ductal carcinoma, immunohistochemically consistent with the primary tumor (ER/PR-negative, HER2-negative). A systematic analysis using next-generation sequencing indicated aberrant activation of the phosphoinositide 3 kinase (PI3K)/AKT/mammalian target of rapamycin (mTOR) signaling pathway. Chemotherapy, targeted therapy and bisphosphonate therapy were initiated, with planned radiotherapy for symptomatic progression. Conclusion: Orbital symptoms in breast cancer survivors, even subtle ones, necessitate prompt evaluation for metastatic disease. Multimodal imaging (e.g., Computed Tomography (CT)/Magnetic Resonance Imaging (MRI)) combined with image-guided biopsy is critical for diagnosis. Early detection enables multidisciplinary palliative strategies to optimize quality of life while addressing systemic dissemination.

Myositis specific antibodies are clinically useful biomarkers in inflammatory myositis. Although immunoprecipitation is regarded as the gold standard, line immune assay (LIA) is widely used in practice. However, LIA is limited by multiple MSA positivity in up to 15% of patients, which limits its applicability in diagnosis and prognostication. We sought to interrogate the subset of patients with multiple antibody positivity on LIA to determine a new positive control band index with high specificity for the diagnosis of myositis. We retrospectively reviewed all samples tested for MSA (EUROLINE DL 1530-1601-4G) between April 2023 to April 2025. We calculated the PCBI (test reading/positive control) and compared it with optimal cutoffs as per EUROLINE and how it impacted multiple antibody positivity. Diagnosis of myositis subtype was confirmed by 2 rheumatologists. We retrieved records of 235 patients, 59.6% females with a mean age of 46.1 years. The underlying diagnosis was dermatomyositis (8.5%), juvenile dermatomyositis (2.1%), anti-synthetase syndrome (7.7%), necrotizing myopathy (2.6%), malignancy associated (4, 1.7%), MSA negative IIM or polymyositis (8, 3.4%) and CTD associated myositis (12, 5.1%) and ILD for IPAF evaluation (139, 59.1%). Multiple MSA positivity was seen in 25 patients (10.6%) with EuroLine, Using the revised PCBI cutoffs, we could discriminate between 14 of these MSA's and 11 remained positive for multiple antibodies. On correspondence analysis, both Euroline cutoffs and PCBI showed association between antibody positivity and clinical diagnosis (Chisq p values < 0.001). However, using the PCBI, 73.4% of the variability in the data compared to 69.13% with Euroline cutoffs. Rather than a single uniform cut off for all patients, a phenotype specific cut off for each clinical phenotype at the time of clinical assessment might offer a higher specificity for MSA with line immune assay.

To characterize the clinical and serological correlates of cardiovascular magnetic resonance (CMR)-confirmed myocarditis in idiopathic inflammatory myopathies (IIM), evaluate the diagnostic performance of high-sensitivity cardiac troponin I (hs-TnI), and quantify the independent prognostic impact of myocarditis relative to interstitial lung disease (ILD). Single-center retrospective cohort study (STROBE-compliant) of 142 consecutive adults with IIM (2019-2025). Myocarditis was confirmed by CMR according to the 2018 Lake Louise Criteria, performed both at diagnosis and during follow-up (with or without immunosuppression) upon clinical suspicion and/or elevated hs-TnI. Myositis-specific antibodies (MSA), myositis-associated antibodies (MAA), anti-Ro52 and antiphospholipid antibodies (aPL, Sydney criteria: ≥40 GPL/MPL units confirmed at ≥ 12 weeks) were systematically recorded. Associations were assessed with Fisher's exact test and logistic regression; multivariable models were pre-specified as parsimonious (two predictors) given the limited event count. Overall survival was analyzed with Kaplan-Meier curves, the log-rank test and Cox proportional-hazards regression. Myocarditis was confirmed in 9/142 patients (6.3%), without significant association with IIM class (P = 0.303) or with the antisynthetase antibody subgroup (anti-Jo-1 + PL-12 + PL-7 + EJ; 5.8% vs. 6.7%, P = 1.000). In univariable analyses, myocarditis was associated with Raynaud phenomenon (OR 13.6, 95% CI 2.3-80.0), aPL positivity (OR 10.7, 95% CI 2.6-43.9), anti-Ro52 coexisting with MSA/MAA (OR 8.7, 95% CI 2.2-34.8), anti-PM/Scl (OR 7.1, 95% CI 1.6-30.3), fever (OR 7.1, 95% CI 1.6-30.3) and ILD (OR 4.7, 95% CI 1.1-20.3). hs-TnI was markedly higher in myocarditis (median 366 vs. 3.5 ng/L; P < 0.001) with an area under the receiver-operating characteristic curve (AUROC) of 0.91 (95% CI 0.83-0.98). In the event-constrained multivariable model, Raynaud phenomenon (adjusted OR 13.1, 95% CI 1.5-112.7) and aPL (adjusted OR 7.2, 95% CI 1.4-36.0) remained independently associated. All-cause mortality was higher in myocarditis (44% vs. 9%; OR 7.95, 95% CI 2.0-31.5; log-rank P = 0.030; univariable Cox HR 3.77, 95% CI 1.03-13.78; P = 0.044), and persisted after adjustment for ILD in the logistic model (adjusted OR 5.9, 95% CI 1.3-26.1; P = 0.020), whereas ILD attenuated to a non-significant trend (adjusted OR 2.9, 95% CI 0.9-9.2; P = 0.073). In this single-center retrospective cohort, IIM-associated myocarditis was consistently associated with Raynaud phenomenon, aPL positivity and anti-Ro52 coexistence, and was associated with higher mortality than ILD. These findings support hs-TnI-guided triage to CMR as part of systematic cardiac surveillance in IIM, pending multicenter confirmation.

This study aimed to identify the clinical characteristics of systemic lupus erythematosus (SLE) patients with complicated with lupus mesenteric vasculitis (LMV), and to determine the impacts of the first-visit department, the number and duration of digestive system symptoms on disease activity, and imaging features of LMV patients. We expected that our study would keep clinicians across specialties vigilant for SLE-LMV, enabling early diagnosis and prompt treatment, thereby improving prognosis and reducing mortality from this potentially fatal condition. Clinical data of 174 patients with LMV admitted to the First Affiliated Hospital of Zhengzhou University from January 2015 to December 2023 were collected and analyzed. The baseline clinical characteristics and laboratory data were analyzed and compared by the first-admission department: either the Rheumatology Department or other departments. In addition, the impact of the numbers and durations of various digestive system symptoms in these patients, as well as laboratory results, disease activity, and imaging findings, was analyzed. The independent-samples t-test and Mann-Whitney U-test were used to compare differences in continuous variables. In contrast, the Kruskal-Wallis test, chi-square test, or Fisher's exact test was used to compare differences in disease activity-related indicators, imaging features, and treatment regimens. A total of 174 patients with an initial diagnosis of LMV were included in this study. Among these patients, 87 (50.0%) had a prior SLE diagnosis, 76 (43.7%) were first diagnosed in the Rheumatology Department, and 36 (20.7%) and 31 (17.8%) were first diagnosed in the Departments of Gastroenterology and Emergency Medicine, respectively. All patients had mild to severe digestive symptoms, including abdominal pain, diarrhea, nausea, vomiting, and abdominal distension. Multiple digestive symptoms occurred in 151 (86.8%) patients. The patients were divided into two groups based on whether they were first diagnosed in the rheumatology department or other departments. The results showed that patients who were not initially diagnosed in the rheumatology department had longer hospital stays and lower disease activity. The patients were further categorized into groups based on the number and time of various digestive symptoms. Compared with those with fewer flares, the proportions of leukopenia, hypocomplementemia, and abdominal effusion were higher in those with frequent LMV recurrences. The white blood cell (WBC) and neutrophil counts in patients with abdominal pain of less than 7 days were higher than those with abdominal pain of more than 7 days. However, statistical analysis did not reveal significant differences among other inflammatory markers and the indices of disease activity. In terms of imaging features obtained by contrast-enhanced CT, a statistical difference was detected in patients with abdominal pain over 7 days compared to those with less than 7 days with respect to thickening of both small and large bowel walls. Due to atypical clinical manifestations of SLE patients with concomitant LMV, only approximately 50% patients were first diagnosed at the Rheumatology Department, with the rest being first diagnosed in other departments, which resulted in delayed diagnosis and treatments, prolonged hospital stay, and potentially, poor prognosis. Evaluation of the impacts of the number and duration of digestive symptom flares on disease activity and imaging features demonstrated that patients with multiple symptoms had more severe radiological findings. Therefore, clinicians, particularly rheumatologists, should keep vigilant when patients have multiple digestive system symptoms and frequent recurrence, as early diagnosis of LMV with prompt and aggressive treatments may improve the prognosis of these patients.

Immune checkpoint inhibitor-associated Triple M overlap syndrome (TMOS), defined by concurrent myocarditis, myositis, and myasthenia gravis, is a rare but life-threatening immune-related adverse event. We report a single-center case series of 8 consecutive patients who developed TMOS during immune checkpoint inhibitor therapy for solid malignancies between 2023 and 2025. Median age was 76 years and median time to symptom onset was 14.5 days after exposure. All patients had myositis symptoms; 5 required mechanical ventilation and 1 subsequently required tracheostomy. Cardiac involvement was characterized by troponin elevation and frequent electrical abnormalities, including complete atrioventricular block, ventricular tachyarrhythmias, and bundle branch block, despite preserved left ventricular systolic function on imaging. Corticosteroid monotherapy was insufficient in practice, and all patients required additional immunomodulatory therapy. TMOS requires early recognition, close monitoring, and rapid multidisciplinary escalation of care.

Idiopathic inflammatory myopathies represent a group of acquired and treatable myopathies characterized by distinct clinical features that should be distinguished from other neuromuscular diseases presenting with proximal muscle weakness or serum creatine kinase elevation. The approach to neuromuscular disorders requires a comprehensive evaluation of history and clinical phenotype. Depending on the presentation, diagnostic strategies vary from basic laboratory tests to complementary electrophysiological studies, muscle imaging, histopathology, and genomic tests. Since the approach to individualized treatments varies according to the accurate diagnosis, a close collaboration between pediatric neurologists and rheumatologists is crucial for the management and follow-up. This review aims to provide a structured diagnostic approach to pediatric myositis mimics, emphasizing the importance of accurate differential diagnosis and highlighting frequently overlooked conditions to improve clinical outcomes.