Language：English   Publisher：Modern Rheumatology Case Reports  

Dermatomyositis (DM) is classically associated with distinctive skin manifestations, such as Gottron’s sign or heliotrope rash. Cases of DM without these skin manifestations – termed DM sine dermatitis (DMSD) – have recently been reported, with an increasing association with anti-nuclear matrix protein 2 (NXP-2) antibody. We report a 26-year-old Japanese man who presented with muscle weakness and myalgia but without the characteristic skin manifestations of DM. Laboratory findings showed only mildly elevated levels of myogenic enzymes, and initial screening for myositis-specific antibodies (MSAs) was negative. However, the muscle biopsy demonstrated necrotic and regenerative muscle fibres with perifascicular expression of myxovirus resistance A. The findings were consistent with DM. Further MSA testing revealed positivity for the anti-NXP-2 antibody, confirming the diagnosis of DMSD. This case presented a diagnostic challenge because of the absence of typical skin rashes and only mild elevation of myogenic enzymes. Clinicians should be aware of DMSD as a potential diagnosis, particularly in patients with muscle symptoms but lacking the characteristic DM skin manifestations. In a myositis diagnosis, proactive muscle biopsy and MSA testing, including the anti-NXP-2 antibody, are crucial for ensuring early diagnosis and treatment.

DOI： 10.1093/mrcr/rxag023

Web of Science

Scopus

PubMed

Publisher：Frontiers in Immunology  

Background: Anifrolumab (ANI), a monoclonal antibody blocking the type I interferon (IFN) receptor, is approved for systemic lupus erythematosus (SLE); yet real-world responses vary. We aimed to identify biomarkers predicting clinical response to ANI in SLE. Methods: We prospectively enrolled patients with SLE who initiated ANI and evaluated immune cell subsets and type I IFN-associated markers by flow cytometry with respect to clinical response to ANI. Clinical response at 6 months was classified into responders and non-responders based on two criteria: achievement of a British Isles Lupus Assessment Group-based Composite Lupus Assessment (BICLA) response and successful glucocorticoid tapering. Results: Of the 31 patients analyzed, 15 were responders and 16 were non-responders. Among the ten biomarkers that showed significant changes in responders, four - CD317 expression on T cells, CD317 expression on B cells, CD169 expression on monocytes, and T-peripheral-helper-cell frequency - were already higher at baseline in responders than in non-responders. Baseline CD317 expression on T cells showed the highest discriminative power in predicting 6-month response, separating responders from non-responders with an AUC of 0.89, surpassing the four-gene IFN gene signature (IFNGS) measured by quantitative PCR (qPCR) (DeLong's test, P = 0.044). Conclusions: This study demonstrates that higher baseline CD317 expression on T cells is associated with a favorable clinical response to ANI and predicts this response more accurately than the previously proposed IFNGS in patients with SLE. These findings identify CD317 as a promising and practical candidate biomarker to guide personalized treatment strategies in SLE, contingent upon further validation.

DOI： 10.3389/fimmu.2026.1756139

Scopus

Publisher：Arthritis Research and Therapy  

Background: The efficacy of rituximab (RTX) for the treatment of systemic sclerosis-associated interstitial lung disease (SSc-ILD) has not been fully established. This study compared the efficacy and safety of RTX and intravenous cyclophosphamide (CY) for SSc-ILD. Methods: This retrospective study compared the efficacy and safety of RTX (20 patients) and CY (30 patients) after adjusting for the stabilised inverse probability of treatment weighting based on propensity scores. The efficacy endpoints were the absolute changes in forced vital capacity (FVC) and serum Krebs von den Lungen-6 (KL-6) levels from baseline to 6 and 12 months after treatment. The incidence of progression based on the definition of progressive pulmonary fibrosis was also recorded. The safety endpoint was the frequency of adverse events. Results: The clinical characteristics of the two groups were well-balanced after adjusting for confounders (i.e., FVC, nintedanib use, and newly diagnosed cases). From baseline to 6 and 12 months after the start of treatment, the median FVC increased by 50 and 60 ml in the RTX group and 40 and 15 ml in the CY group, respectively, with no difference after adjustment. The changes in serum KL-6 levels and the incidences of progression were identical in both groups after adjustment. The overall adverse events were similar in both groups after adjustment. Conclusions: RTX demonstrated comparable safety and efficacy as CY in patients with SSc-ILD. Thus, RTX may be an alternative to CY for the treatment of SSc-ILD.

DOI： 10.1186/s13075-025-03654-0

Scopus

Publisher：Medicine United States  

Takayasu arteritis (TAK) is characterized by vascular injury, in which endothelial cells and immune cells including natural killer cells, have key roles. CD226 is an activating receptor expressed on natural killer cells and T cells, and the soluble form of CD226 (sCD226) is increased in diseases involving these cells. Therefore, we investigated the utility of serum sCD226 as a biomarker for TAK. Serum sCD226 levels were measured using an enzyme-linked immunosorbent assay in 34 TAK patients and 21 giant cell arteritis (GCA) patients. The associations between sCD226 levels and the angiographic classification, disease activity, and prognosis of TAK were analyzed. Serum sCD226 levels were significantly higher in TAK patients than in GCA patients. In patients with TAK, serum sCD226 levels were significantly elevated in the group of type Ⅴ compared with type Ⅰ to Ⅳ. Serum sCD226 levels were also elevated in patients with active TAK and in those with poor responses to corticosteroids. Moreover, the cumulative probability of relapse was increased in patients with high sCD226 levels. Serum sCD226 levels differentiated TAK from GCA and were associated with disease activity and relapse of TAK. Thus, serum sCD226 might be a useful biomarker for the management of TAK.

DOI： 10.1097/MD.0000000000042844

Scopus

Language：Japanese  

Language：Japanese  

Language：Japanese  

Language：Japanese  

Language：Japanese