Objective: To evaluate the association of routine complete blood count (CBC)-derived inflammatory biomarkers, including neutrophil-to-lymphocyte ratio (NLR), monocyte-to-lymphocyte ratio (MLR), platelet-to-lymphocyte ratio (PLR), and systemic inflammation response index (SIRI), with disease activity and exploratory neuropsychiatric risk stratification in patients with systemic lupus erythematosus (SLE). Methods: In this multi-center retrospective study, 579 SLE patients and 282 healthy controls (HCs) were recruited from five clinical centers between 2018 and 2025. NLR, MLR, PLR, and SIRI were calculated from routine CBC parameters. Disease activity was assessed using the SLE Disease Activity Index 2000 (SLEDAI-2K), with high activity defined as SLEDAI-2K ≥ 10. The comparison between SLE patients and HCs was performed as an exploratory descriptive analysis to characterize systemic inflammatory profiles, whereas the primary analyses focused on associations with disease activity and NPSLE-related risk stratification. Results: SLE patients exhibited significantly higher levels of SIRI, NLR, PLR, and MLR compared to HCs (all p < 0.001). In this exploratory comparison, MLR showed the largest area under the curve for distinguishing SLE patients from HCs (AUC: 0.849, Cut-off: 0.263). In regression analyses, MLR, NLR, PLR, and SIRI were positively associated with SLEDAI-2K score. In multivariable linear regression analysis, MLR was associated with a higher SLEDAI-2K score (B = 4.600, 95% CI: 2.039-7.160, p < 0.001). In patients with available neuropsychiatric data, MLR, NLR, and SIRI were higher in patients with NPSLE than in those with non-NPSLE, whereas PLR showed no significant difference. SIRI showed modest exploratory discriminatory ability for NPSLE and may provide auxiliary information for NPSLE risk stratification (AUC: 0.710, p < 0.001, Cut-off: 1.438). Conclusions: Routine CBC-derived inflammatory biomarkers, particularly MLR, NLR, and SIRI, are associated with SLE disease activity and may serve as accessible, low-cost adjunctive tools for rapid clinical assessment. SIRI may provide additional auxiliary information for identifying patients at higher risk of neuropsychiatric involvement. However, these biomarkers should be interpreted as complementary screening or risk-stratification tools rather than substitutes for established disease activity indices or organ-specific evaluations. Further prospective studies are warranted to validate their clinical utility.

BackgroundRheumatoid arthritis-associated interstitial lung disease (RA-ILD) is a severe extra-articular manifestation with limited diagnostic biomarkers. While gut microbiota dysbiosis contributes to rheumatoid arthritis (RA) pathogenesis, its specific role in RA-ILD remains poorly characterized.MethodsWe performed shotgun metagenomic sequencing on fecal samples from 41 participants: 10 RA-ILD patients, 20 RA patients without ILD (RA-non-ILD), and 11 healthy controls (HCs). We assessed alpha and beta diversity, differential abundance (Wilcoxon rank-sum tests with FDR correction), Spearman correlations with clinical parameters, microbial co-occurrence networks, and random forest classification.ResultsAlpha and beta diversity did not differ significantly among groups. After FDR correction, no genus differed significantly between RA-ILD and RA-non-ILD. Exploratory analysis (uncorrected P < 0.05) revealed enrichment of Escherichia/Shigella in RA-ILD (11.72% vs. 2.66%, P = 0.003) and depletion of Roseburia (1.05% vs. 3.77%, P = 0.005) and Ruminococcus (5.98% vs. 7.85%, P = 0.032), while Faecalibacterium showed a trend toward depletion without reaching nominal significance (4.45% vs. 4.66%, P = 0.409). Correlation analysis revealed a dichotomous pattern: pro-inflammatory genera correlated positively with disease activity, while butyrate-producing genera correlated negatively. Co-occurrence network analysis showed RA patients had a more complex network than HC and RA-ILD. Random forest classification identified Bifidobacterium, unclassified_ Oscillospiraceae, and unclassified_Lachnospiraceae as top discriminators between HC and RA, and unclassified_ Bacteroidaceae, Parabacteroides, and Blautia for RA-ILD vs RA.ConclusionsRA-ILD is associated with specific gut microbial alterations—notably Escherichia/Shigella enrichment and depletion of Roseburia and Ruminococcus—despite preserved overall diversity. These changes correlate with systemic inflammation and suggest a role for the gut microbiota in RA-ILD pathogenesis via the gut-lung axis. The identified taxa warrant validation as candidate biomarkers in larger cohorts.

Objective:To delineate the clinical characteristics, identify risk factors (including the exploratory role of anti-Ro-52 antibody), and assess the prognostic implications of malignancy in patients with anti-synthetase syndrome (ASyS). Methods:In this retrospective multicenter study, patients with idiopathic inflammatory myopathies (IIM) were analyzed, comprising 103 ASyS and 261 non-ASyS patients [including dermatomyositis (n = 195), immune-mediated necrotizing myopathy (n = 9), overlap myositis (n = 12), and other IIM subtypes (n = 45)]. Participants were stratified into four groups based on ASyS and malignancy status: ASyS with malignancy (ASyS-MAL), ASyS without malignancy, non-ASyS with malignancy (non-ASyS-MAL), and non-ASyS without malignancy. Data on demographics, clinical features, serology, and survival were collected. Multivariate logistic regression identified malignancy-associated factors, and Kaplan-Meier analysis compared survival. Results:Malignancy prevalence was 15.5% (16/103) in ASyS patients vs. 9.2% (24/261) in non-ASyS patients (P = 0.074). Multivariate analysis identified ASyS as an independent risk factor for malignancy (adjusted OR 2.65, 95% CI 1.15-6.11, P = 0.022), along with advancing age (adjusted OR 1.04 per year, 95% CI 1.01-1.07, P = 0.005). Comparative analysis showed ASyS-MAL patients had significantly higher creatine kinase (CK) levels (median 978 vs. 336 U/L, P = 0.018) and a 100% prevalence of myositis-specific antibodies (MSAs), while non-ASyS-MAL patients had a higher prevalence of heliotrope rash (75.0% vs. 37.5%, P = 0.019). The presence of malignancy was associated with worse overall survival (P < 0.001). ASyS-MAL patients had a median survival of 36.8 months, compared to 46.2 months in non-ASyS-MAL patients (log-rank P = 0.138). Conclusion:Anti-synthetase syndrome is an independent risk factor for malignancy in patients with myositis, challenging the traditional view of ASyS as a low-risk subtype. Systematic malignancy screening is warranted for all ASyS patients, particularly those over 60 years of age. The role of anti-Ro-52 as an independent predictor was not confirmed and requires further study.

Background:Lupus mesenteric vasculitis (LMV) is a rare but life-threatening gastrointestinal complication of systemic lupus erythematosus (SLE). Refractory cases pose significant therapeutic challenges due to limited treatment options and cumulative toxicity from long-term immunosuppressant therapy. Case presentation:A 46-year-old woman with SLE presented with recurrent abdominal pain, diarrhea, and malar rash over a 1-year period. Despite receiving standard immunosuppressive therapies-including glucocorticoids (GCs), cyclophosphamide, mycophenolate mofetil, and tacrolimus-she experienced multiple relapses of LMV between 2015 and 2020, confirmed by abdominal computed tomography showing bowel wall thickening and the characteristic "target sign." In September 2020, belimumab (600 mg intravenous every 4 weeks) was initiated alongside a reduced-dose GC regimen. Over a 49-month treatment period (34 doses), the patient achieved sustained remission, with complete resolution of abdominal symptoms, normalization of computed tomography findings, stable Systemic Lupus Erythematosus Disease Activity Index scores (0-4), decreasing anti-double-stranded DNA titers (<20 IU/mL), and rising serum complement C3 levels (>0.6 g/L). GC dosage was successfully tapered to 2.5 mg/day without disease relapse. Conclusions:This case demonstrates belimumab's efficacy in achieving the longest documented remission (49 months) in refractory LMV, highlighting its potential as a first-line biologic for steroid-dependent gastrointestinal vasculitis.

Introduction Antinuclear antibodies (ANA) are frequently positive in patients with anti-melanoma differentiation-associated gene 5-positive dermatomyositis (anti-MDA5+ DM). This study aimed to investigate the association between ANA and clinical characteristics as well as prognosis in a cohort of patients with anti-MDA5+ DM. Material and methods We conducted a systematic retrospective study of medical records from a Nanjing Medical University cohort of patients with myositis-associated interstitial lung disease (ILD). Various parameters were compared and analyzed between the ANA-positive group and the ANA-negative group. Results A total of 246 patients with anti-MDA5+ DM were enrolled in this study, with 28.5% males and 71.5% females. The median age was 53.0 years, the median disease duration was 2 months, and the median follow-up period was 12.0 months. The ANA positivity rate at baseline was 52.4% in anti-MDA5+ DM patients. The ANA-positive group showed significantly higher positivity rates of anti-Ro52 antibodies (72.9% vs. 54.7%, p = 0.003) and anti-aminoacyl-tRNA synthetase (anti-ARS) antibodies (9.3% vs. 2.6%, p = 0.033) compared to the ANA-negative group, but lower ALT levels: 39.0 (21.5, 79.3) vs. 51.3 (36.5, 95.8), p = 0.006. No significant differences were observed between the two groups in terms of overall survival, rapidly progressive interstitial lung disease (RPILD) incidence, age, disease duration, and clinical characteristics. In a subgroup analysis of the ANA-positive group, MDA5+++ patients had a higher incidence of RPILD compared to the MDA5+ group ( p = 0.028). In the ANA-negative subgroup analysis, MDA5+++ patients had a higher mortality rate and worse prognosis compared to the MDA5+ group ( p = 0.026). Multivariate Cox regression analysis showed that elevated lactate dehydrogenase (LDH) levels and the presence of rapidly progressive interstitial lung disease (RPILD) were associated with poor prognosis in ANA-negative anti-MDA5+ DM patients, with hazard ratios of 1.002 (95% CI: 1.001, 1.003, p = 0.020) and 13.694 (95% CI: 15.032, 37.267, p < 0.001), respectively. Conclusions ANA is frequently found in patients with anti-MDA5+ DM. High titers of anti-MDA5 antibodies are associated with mortality and RPILD.

INTRODUCTION:Omega-3 possesses anti-inflammatory and lipid metabolism modifying effects in rheumatoid arthritis (RA), but inconsistency exists among previous studies. This meta-analysis intended to explore the effects of omega-3 supplementation on fatty acid distribution, blood lipid profiles, inflammation, and disease activity in RA patients. METHODS:This meta-analysis followed the Preferred Reporting Item for Systematic Reviews and Meta-Analyses (PRISMA) protocol. PubMed, Web of Science, and Embase databases were searched until August 31, 2023. RESULTS:Eighteen randomized controlled trials with 1018 RA patients were included. Regarding fatty acid distribution, omega-3 supplementation increased eicosapentaenoic acid (EPA) [standardized mean difference (SMD): 0.74; 95% confidence interval (CI): 0.46, 1.01; P < 0.001] and docosahexanoic acid (DHA) (SMD: 0.62; 95% CI: 0.35, 0.89; P < 0.001), but reduced omega-6:omega-3 ratio (SMD: -1.06; 95% CI: -1.39, -0.73; P < 0.001) in RA patients. Regarding blood lipid, omega-3 supplementation decreased triglyceride (TG) in RA patients (SMD: -0.47; 95% CI: -0.78, -0.16; P = 0.003). Regarding clinical symptoms, omega-3 supplementation reduced tender joint count (TJC) in RA patients (SMD: -0.59; 95% CI: -0.79, -0.39; P < 0.001). Notably, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and disease activity score on 28 joints (DAS28) score were slightly decreased by omega-3 supplementation but without statistical significance (all P > 0.05). Publication bias was low, and stability assessed by sensitivity analysis was good. CONCLUSION:Omega-3 supplementation increases EPA and DHA, but reduces the omega-6:omega-3 ratio, TG, and TJC in RA patients.

BackgroundDiffuse connective tissue diseases (DCTDs) require long-term immunosuppressive treatment, increasing the risk of varicella-zoster virus (VZV) infection. This study aims to evaluate the humoral immune status against VZV in DCTD patients and explore factors that may influence their immune levels.MethodsThis is a retrospective cohort study that collected data from adult DCTD patients (≥18 years) attending our outpatient clinic. The geometric mean concentration (GMC) of VZV-specific IgG antibodies in the patients’ sera was measured using the enzyme-linked immunosorbent assay (ELISA).ResultsA total of 280 RA patients, 272 SLE + MCTD patients and 280 healthy controls were included. SLE + MCTD patients had significantly higher VZV IgG antibody levels than RA patients (p < 0.05) but showed no significant difference compared to healthy controls (p > 0.05). Notable differences were observed particularly among female patients and those aged 30–49 years, (p < 0.05). SLE + MCTD patients in an active disease state had significantly higher VZV IgG antibody titers than RA patients (p < 0.05). Additionally, patients with a history of herpes zoster, regardless of being in the SLE + MCTD, RA, or control group, exhibited higher VZV IgG titers (p < 0.05).ConclusionAlthough DCTD patients, particularly those with SLE and MCTD, exhibit higher VZV IgG antibody levels, they still face a higher risk of developing herpes zoster (HZ), which may be related to their underlying disease and immunosuppressive treatment. The presence of antibodies alone may not provide complete protection, necessitating consideration of cellular immune mechanisms. It is recommended to enhance monitoring of VZV antibody levels in high-risk patients and consider herpes zoster vaccination to reduce HZ-related complications.

BackgroundThe prognosis of anti-melanoma differentiation-associated gene 5 positive dermatomyositis (anti-MDA5+DM) is poor and heterogeneous. Rapidly progressive interstitial lung disease (RP-ILD) is these patients’ leading cause of death. We sought to develop prediction models for RP-ILD risk in anti-MDA5+DM patients.MethodsPatients with anti-MDA5+DM were enrolled in two cohorts: 170 patients from the southern region of Jiangsu province (discovery cohort) and 85 patients from the northern region of Jiangsu province (validation cohort). Cox proportional hazards models were used to identify risk factors of RP-ILD. RP-ILD risk prediction models were developed and validated by testing every independent prognostic risk factor derived from the Cox model.ResultsThere are no significant differences in baseline clinical parameters and prognosis between discovery and validation cohorts. Among all 255 anti-MDA5+DM patients, with a median follow-up of 12 months, the incidence of RP-ILD was 36.86%. Using the discovery cohort, four variables were included in the final risk prediction model for RP-ILD: C-reactive protein (CRP) levels, anti-Ro52 antibody positivity, short disease duration, and male sex. A point scoring system was used to classify anti-MDA5+DM patients into moderate, high, and very high risk of RP-ILD. After one-year follow-up, the incidence of RP-ILD in the very high risk group was 71.3% and 85.71%, significantly higher than those in the high-risk group (35.19%, 41.69%) and moderate-risk group (9.54%, 6.67%) in both cohorts.ConclusionsThe CROSS model is an easy-to-use prediction classification system for RP-ILD risk in anti-MDA5+DM patients. It has great application prospect in disease management.

AbstractIntroductionFatigue is a common symptom that negatively affects the outcomes and functions of rheumatoid arthritis (RA) patients. This study aimed to assess the fatigue by two scales and validate their consistency, also to comprehensively evaluate fatigue‐related risk factors in RA patients.MethodsIn this case–control study, the fatigue of 160 RA patients and 60 healthy controls was evaluated by the Bristol Rheumatoid Arthritis Fatigue Multi‐Dimensional Questionnaire (BRAF‐MDQ) and the Chinese version of the Brief Fatigue Inventory (BFI‐C). The 28‐joint disease activity score using erythrocyte sedimentation rate of RA patients was assessed.ResultsThe BRAF‐MDQ and BFI‐C scores were elevated in RA patients versus healthy controls (all p < .001). Interestingly, BRAF‐MDQ global fatigue score positively correlated with BFI‐C global fatigue score in both RA patients (r = .669, p < .001) and healthy controls (r = .527, p < .001); meanwhile, Kendall's tau‐b test showed a high consistency between BRAF‐MDQ and BFI‐C global fatigue scores in RA patients (W = 0.759, p < .001) and healthy controls (W = 0.933, p < .001). Notably, higher education level (В = −4.547; 95% confidence interval: −7.065, −2.029; p < .001) and swollen joint count (В = 1.965; 95% confidence interval: 1.375, 2.554; p < .001) independently related to BRAF‐MDQ global fatigue score; higher education level (В = −0.613; 95% confidence interval: −0.956, −0.269; p = .001) and clinical disease activity index (В = 0.053; 95% confidence interval: 0.005, 0.102; p = .032) independently linked with BFI‐C global fatigue score.ConclusionFatigue commonly occurs in RA patients, which independently relates to education level and disease activity. Furthermore, BRAF‐MDQ and BFI‐C scales exhibit a high consistency in assessing fatigue.

IntroductionTo identify the clinical characteristics and risk factors for cutaneous ulcers in patients with anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis (anti-MDA5+ DM) combined with rapidly progressive interstitial lung disease (RPILD).Material and methodsWe conducted a retrospective cohort study on the medical records of patients enrolled from the Nanjing Medical University Myositis-associated ILD cohort (NMMI). The clinical characteristics of patients in the ulcer-positive group were compared with those in the ulcer-negative group by chi-square or Fisher’s exact test. Univariate and multivariate logistic regression analyses were used to assess risk factors for the development of cutaneous ulcers.ResultsA total of 246 patients with anti-MDA5+ DM were retrospectively enrolled in the study, including 176 females (176/246, 71.54%) and 70 males (70/246, 28.46%), with a female-to-male ratio of 2.51 : 1. Among the 246 patients, a total of 88 cases (88/246, 35.77%) with anti-MDA5+ DM combined with RPILD were further studied, including 55 females (55/88, 62.5%) and 33 males (33/88, 37.5%), with a female-to-male ratio of 1.67 : 1. Twelve patients (12/88, 13.64%) had cutaneous ulcers. In terms of clinical characteristics, patients in the ulcer-positive group had significantly more proximal muscle involvement (83.33% vs. 38.16%, p = 0.003) and more heliotrope rash (83.33% vs. 43.42%, p = 0.010) than in the ulcer-negative group. In univariate analysis, cutaneous ulcers were associated with proximal muscle involvement (OR = 8.103; 95% CI: 1.657–39.625; p = 0.010) and heliotrope rash (OR = 6.515; 95% CI: 1.336–31.773; p = 0.020). In multivariate analysis, cutaneous ulcers were associated with proximal muscle involvement (OR = 6.436; 95% CI: 1.274–32.524; p = 0.024), and proximal muscle involvement was an independent risk factor for cutaneous ulcers.ConclusionsWe confirmed the association between cutaneous ulcers and proximal muscle involvement and heliotrope rash in patients with anti-MDA5+ DM combined with RPILD. Proximal muscle involvement is an independent risk factor for cutaneous ulcers.

Abstract Introduction Adalimumab (ADA) and etanercept (ETN) are the most commonly applied biologics for rheumatoid arthritis (RA) management in China; however, the evidence regarding their superiority is controversial. In addition, in real‐world clinical settings, many factors may affect the application of these agents, such as dosage and administration period. Therefore, the present real‐world study aimed to compare the efficacy and safety of ADA and ETN treatment in RA patients via the propensity score matching method. Methods In total, 105 RA patients receiving ADA (n = 66) or ETN (n = 39) were reviewed in this retrospective study. The propensity score matching method was used to eliminate discrepancies in baseline features. Clinical response, low disease activity (LDA), and remission were evaluated based on the DAS28. Results Before propensity score matching, compared with ETN, ADA yielded higher rates of clinical response at W24 (97.0% vs. 84.6%, p = .021), LDA at W12 (78.8% vs. 51.3%, p = .003), and remission at W24 (75.8% vs. 46.2%, p = .002). After propensity score matching, compared with ETN, ADA only achieved a higher rate of clinical response at W24 (96.3% vs. 77.8%, p = .043), whereas the rates of LDA and remission were not different between ADA and ETN treatments at any time point (all p > .05). In addition, the incidence of adverse events was not significantly different between the ADA and ETN treatments (all p > .05). Conclusion ADA shows superiority over ETN in terms of a numerically greater response rate and equivalent adverse events.

ObjectiveInterstitial lung disease (ILD) is a common extramuscular complication contributing to significant morbidity and mortality in patients with dermatomyositis (DM) who are positive for antimelanoma differentiation–associated gene 5 antibody (anti-MDA5+). We conducted this study to investigate the association of anti-Ro52 antibodies with clinical characteristics and prognosis in patients with anti-MDA5+ DM.MethodsWe assessed a cohort of 246 patients with anti-MDA5+ DM. To calculate hazard ratios and 95% CIs for rapidly progressive ILD (RP-ILD) and death while controlling for potential confounders, variables selected by univariate Cox regression analysis were included in a multivariate Cox regression model with the stepwise forward-selection method. A 2-tailed analysis withP< 0.05 was considered to be statistically significant.ResultsA total of 246 patients with anti-MDA5+ DM were enrolled; 70 patients were male, and the patient group had an average age of 53.1 (12.4) years. Anti-Ro52 was present in 64.2% (158/246) patients. Patients with anti-MDA5+ DM who were positive for anti-Ro52 had a higher rate of RP-ILD (log-rankP< 0.001) and a higher mortality rate (log-rankP= 0.01). For patients with anti-MDA5+ DM who were positive for anti-Ro52, those with a short disease course and high inflammation were at increased risk of RP-ILD and death. The appearance of active rash was an independent protective factor of death.ConclusionAnti-Ro52 antibodies were highly prevalent in patients with anti-MDA5+ DM, and their coexistence correlated with a higher rate of RP-ILD and mortality. Patients with a short disease course, with increased inflammation, and without rash were more likely to have a poor prognosis.

Objective To investigate the clinical features and survival of patients with anti-melanoma differentiation-associated gene 5 positive dermatomyositis（MDA5 + DM）. Methods The clinical data of 249 MDA5 + DM patients, obtained from the Inflammatory Myopathy and Connective Tissue Disease-Associated Interstitial Lung Disease Alliance of Nanjing Medical University between December 2014 and December 2021, were retrospectively analyzed. Kaplan-Meier method was used to calculate the cumulative survival rate, univariate log-rank method and multivariate COX model was used to analyze the factors related to the survival of patients. Results The fatality rate of MDA5 + DM patients in this series was 24.1%（60/249）. The proportion of males and average age in the death group were higher than those in the non-death group. The levels of AST, LDH, CK, CRP and SF, the positive rate of anti-Ro52 antibody and the proportion of high-titer anti-MDA5 antibody were significantly higher in the death group. The incidence of rapidly progressive interstitial lung disease（RPILD） was higher than that of non-death group, but the incidence of arthritis was lower than that of non-death group（P＜0.05）. Log-rank univariate analysis showed that anti-MAD5 antibody titer, sex, age, LDH, CK, CRP, SF, positive anti-Ro52 antibody, arthritis, rash, RPILD were significantly associated with survival of MDA5 + DM patients（P＜0.05）; age, CK, CRP and arthritis were correlated with survival of univariate patients with RPILD（P＜0.05）. COX multivariate analysis showed that increased CK, CRP and positive anti-Ro52 antibody were independent risk factors for poor prognosis of MDA5 + DM patients（P＜0.05）; the elevated CK and CRP were independent risk factors for poor prognosis of MDA5 + DM combined with RPILD（P＜0.05）, while arthritis was a factor for favorable prognosis（HR=0.427, P＜0.05）. Conclusion MDA5 + DM patients have high fatality, and patients with RPILD have shorter survival. Increased CK, CRP and positive anti-Ro52 antibody may increase the risk of poor diagnosis, while the presence of arthritis may be a factor of favorable prognosis.

OBJECTIVE:To investigate the impact of sex differences on the clinical characteristics and prognosis of patients with anti-melanoma differentiation-associated gene 5-positive dermatomyositis (MDA5+ DM).METHODS:We retrospectively analyzed a cohort of 251 patients with MDA5+ DM, including 71 in the male group and 180 in the female group. A multivariate logistic regression model was built to analyze independent risk factors for RPILD in each group. An ROC curve was drawn to evaluate the predictive value of independent risk factors. Kaplan‒Meier analysis was used to compare the cumulative survival rates, while the log-rank test was used to test for significant differences between the two groups.RESULTS:Patients in the male group had a significantly higher prevalence of heliotrope rash, V sign, severe interstitial lung disease (ILD), and rapidly progressive interstitial lung disease (RPILD) than those in the female group. Anti-Ro52 positivity, high CRP level and short disease were identified as independent risk factors for RPILD in both male and female groups by multivariate logistic regression analysis. The mortality rates of males and females were 33.8% and 22.0%, respectively, and the survival time of patients in the male group was shorter than that in the female group.CONCLUSION:Male patients with MDA5+ DM exhibit an increased risk of RPILD, elevated mortality rates and reduced overall survival time compared to their female counterparts, and anti-Ro52 positivity may be an unfavorable prognostic factor for these patients. Key Points • The prevalence of solar rash, V sign, severe interstitial lung disease (ILD) and rapidly progressive interstitial lung disease (RPILD) in anti-MDA5-positive female patients was significantly lower than that in male patients. • Positive Anti-Ro52, high CRP level, and short course of disease were independent risk factors for RPILD in both men and women. • Female patients exhibited a lower mortality rate than male patients (22.0% vs 33.8%) and demonstrated longer survival time.

目的 分析抗黑色素瘤分化相关基因 5(MDA5)抗体阳性皮肌炎患者临床特征及抗体滴度对快速进展性间质性肺病(RPILD)预后的影响.方法 回顾性分析南京医科大学炎性肌病及结缔组织病相关间质性肺病专病联盟所收集的352 例皮肌炎患者的临床资料,其中抗MDA5 抗体阳性组249 例,抗MDA5 抗体阴性组103 例,比较两组的临床特点及实验室指标.采用 Kendall's W 相关分析抗 MDA5 抗体滴度与RPILD发生率及死亡率的相关性,采用Logistic回归分析抗MDA5 抗体滴度对预后的风险变化,生存分析采用Kaplan-Meier法.结果 抗MDA5 抗体阳性组皮疹、技工手、关节炎及间质性肺病(ILD)较抗MDA5 抗体阴性组更常见,而抗MDA5 抗体阴性组肌无力更明显,差异有统计学意义(P＜0.001).与抗MDA5 抗体阴性组比较,抗MDA5 抗体阳性组谷草转氨酶(AST)、血沉(ESR)、血清铁蛋白(SF)水平及抗Ro52 抗体阳性率均较高,而乳酸脱氢酶(LDH)及肌酸激酶(CK)水平较低,两组之间差异有统计学意义(均P＜0.05).抗MDA5 抗体阳性滴度与RPILD的发生率存在相关性(r =0.180,P =0.003),Logistic回归分析显示,抗MDA5 抗体高滴度组发生RPILD的风险是抗MDA5 抗体低滴度组的 4.765 倍[OR(95%CI)=4.765(1.883,12.058),P＜0.001].抗MDA5 抗体高滴度组死亡的风险是抗 MDA5 抗体低滴度组的 3.878 倍[OR(95%CI)=3.878(1.706,8.815),P =0.001].结论 抗MDA5 抗体阳性皮肌炎患者合并RPILD的风险增高,且随着抗MDA5抗体阳性滴度升高,RPILD的发生率及死亡率增加,可能是皮肌炎的预后不良因素.

OBJECTIVE:There is substantial heterogeneity among the phenotypes of patients with anti-melanoma differentiation-associated gene 5 antibody-positive (anti-MDA5+) dermatomyositis (DM), hindering disease assessment and management. This study aimed to identify distinct phenotype groups in patients with anti-MDA5+ DM and to determine the utility of these phenotypes in predicting patient outcomes. METHODS:A total of 265 patients with anti-MDA5+ DM were retrospectively enrolled in the study. An unsupervised hierarchical cluster analysis was performed to characterize the different phenotypes. RESULTS:Patients were stratified into 3 clusters characterized by markedly different features and outcomes. Cluster 1 (n = 108 patients) was characterized by mild risk of rapidly progressive interstitial lung disease (RPILD), with the cumulative incidence of non-RPILD being 85.2%. Cluster 2 (n = 72 patients) was characterized by moderate risk of RPILD, with the cumulative incidence of non-RPILPD being 73.6%. Patients in cluster 3 (n = 85 patients), which was characterized by a high risk of RPILD and a cumulative non-RPILD incidence of 32.9%, were more likely than patients in the other 2 subgroups to have anti-Ro 52 antibodies in conjunction with high titers of anti-MDA5 antibodies. All-cause mortality rates of 60%, 9.7%, and 3.7% were determined for clusters 3, 2, and 1, respectively (P < 0.0001). Decision tree analysis led to the development of a simple algorithm for anti-MDA5+ DM patient classification that included the following 8 variables: age >50 years, disease course of <3 months, myasthenia (proximal muscle weakness), arthritis, C-reactive protein level, creatine kinase level, anti-Ro 52 antibody titer, and anti-MDA5 antibody titer. This algorithm placed patients in the appropriate cluster with 78.5% accuracy in the development cohort and 70.0% accuracy in the external validation cohort. CONCLUSION:Cluster analysis identified 3 distinct clinical patterns and outcomes in our large cohort of anti-MDA5+ DM patients. Classification of DM patients into phenotype subgroups with prognostic values may help physicians improve the efficacy of clinical decision-making.