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Objectives To analyse the effectiveness of tofacitinib for the treatment of refractory skin thickening in dcSSc. Methods Data from 10 patients with dcSSc treated with tofacitinib (5 mg twice daily) were analysed. A total of 12 dcSSc patients treated with intensive conventional immunosuppressants were selected as the historical comparator group. A clinically relevant response was defined as a decrease in the modified Rodnan skin score (mRSS) of >5 points and ≥25% from baseline. Clinical indicators were compared between the two groups to evaluate the effect of tofacitinib. Results The mRSS significantly improved the first month after tofacitinib treatment, with a mean change in the mRSS of −3.7 (95% CI −5.52, −1.88; P = 0.001) and greater than the comparators at 6 months [−10.0 (95% CI −14.74, −5.26) vs −4.1 (95% CI −7.49, −0.73), P = 0.026]. Tofacitinib-treated patients had a significantly shorter response time than the comparators (P = 0.015 by log-rank test), with overall response rates of 20% (2/10) vs 0% (0/12) and 60% (6/10) vs 16.7% (2/12) at 1 and 3 months, respectively. Conclusion Our results indicate that tofacitinib may be as effective as or even better than intensive conventional immunosuppressants, with a quicker and higher response rate in refractory dcSSc patients with progressive skin thickness.

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Objective Pulmonary arterial hypertension (PAH) is a life‐threatening complication of systemic lupus erythematosus (SLE). However, there is no algorithm to identify those at high risk. This study was undertaken to develop a prediction model for PAH in patients with lupus that provides individualized risk estimates. Methods A multicenter, longitudinal cohort study was undertaken from January 2003 to January 2020. The study collected data on 3,624 consecutively evaluated patients diagnosed as having SLE. The diagnosis of PAH was confirmed by right‐sided heart catheterization. Cox proportional hazards regression and least absolute shrinkage and selection operator were used to fit the model. Model discrimination, calibration, and decision curve analysis were performed for validation. Results Ninety‐two lupus patients (2.54%) developed PAH during a median follow‐up of 4.84 years (interquartile range 2.42–8.84). The final prediction model included 5 clinical variables (acute/subacute cutaneous lupus, arthritis, renal disorder, thrombocytopenia, and interstitial lung disease) and 3 autoantibodies (anti‐RNP, anti‐Ro/SSA and anti‐La/SSB). A 10‐year PAH probability‐predictive nomogram was established. The model was internally validated by Harrell’s concordance index (0.78), the Brier score (0.03), and a satisfactory calibration curve. According to the net benefit and predicted probability thresholds, we recommend annual screening in high‐risk (>4.62%) lupus patients. Conclusion We developed a risk stratification model using routine clinical assessments. This new tool may effectively predict the future risk of PAH in patients with SLE.

Objective Interstitial lung disease (ILD) is a common extramuscular complication contributing to significant morbidity and mortality in anti-melanoma differentiation associated gene 5 positive dermatomyositis (anti-MDA5+ DM). We conducted this study to investigate the association of anti-Ro52 antibodies with clinical characteristics and prognosis in anti-MDA5+ DM patients. Methods We assessed a cohort of 246 patients with anti-MDA5+ DM. To calculate hazard ratios (HRs) and 95% confidence intervals (95% CIs) for 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 p value <0.05 was considered to indicate statistical significance. Results 246 anti-MDA5+ DM patients were enrolled, 70 cases male, with an average age of 53.10±12.35 years. Anti-Ro52 coexisted in 64.22% (158/246) patients. Anti- Ro52 autoantibodies positive anti-MDA5+ DM patients had a higher rate of RP-ILD (log-rank p<0.001) and a higher mortality rate (log-rankp=0.010). For anti-MDA5+ DM patients with positive anti-Ro52 antibodies, patients with a short disease course, and high inflammation are at increased risk of RP-ILD and death. The appearance of the active rash is an independent protective factor of death. Conclusion Anti-Ro52 antibodies are highly prevalent in anti-MDA5+ DM patients and their coexistence correlates with a higher rate of RP-ILD and mortality. Patients with a short disease course, increased inflammation and without rash are more likely to have a poor prognosis.

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.

Objectives Anti-melanoma differentiation-associated gene 5 positive (anti-MDA5+) dermatomyositis (DM) has a close relationship with rapidly progressive interstitial lung disease (RPILD) and is associated with high mortality. However, data regarding the time-dependent risk of RPILD and deaths during disease progression are limited. We conducted this study to investigate whether the risk of RPILD and death were time-dependent or not in anti-MDA5+ DM. Methods We assessed a cohort of 272 patients with anti-MDA5+ DM. The clinical characteristics of patients with anti-MDA5+ were collected, and COX regression was used to analyze independent risk factors for RPILD and death. We also described changes in risk of RPILD and death over time and their potential clinical implications. Results There were 272 anti-MDA5+ DM patients enrolled in this study. According to the multivariate cox regression analysis, short disease course, high CRP level, anti-Ro52 positive, and anti-MDA5 titer ( ++∼ +++) were independent risk factors of RPILD. High CK level, high CRP level, and RPILD were independent risk factors for death. &gt;90% RPILD and 84% mortality occurred in the first 6 months after disease onset. Notably, the first 3-months is a particularly high-risk period, with 50% RPILD and 46% death occurring. Hazards regarding RPILD and mortality diminished over time during a median follow-up of 12 months. Conclusion These results suggest significant, time-dependent changes in RPILD and mortality risk in anti-MDA5+ DM patients, providing a cutoff time window to estimate disease progression and poor prognosis.