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Objectives: This study aimed to depict the clinical features, including myositis specific or associated antibody (MSA/MAA) profile of systemic lupus erythematosus (SLE) patients with muscle involvement in a Chinese cohort. Methods: We retrospectively studied a cohort of 1696 SLE inpatients and screened for concurrent myositis features from January 2013 to June 2021. Propensity score matching was applied to enroll controls without myositis features from our cohort. Demographic, clinical and laboratory variables were collected. MSA/MAA panels containing 16 autoantibodies (TIF1‐γ, MDA5, NXP2, Mi‐2α/β, SAE1, Jo‐1, PL‐7, PL‐12, EJ, OJ, SRP, HMGCR, cN‐1A, PM‐Scl75/100, Ku and Ro52) were tested by line‐blotting assay. Binary logistic regression and K‐means clustering were applied. Results: Forty‐one of 1696 (2.42%) SLE patients in our SLE inpatient cohort showed features of myositis. Binary logistic regression revealed that new‐onset SLE (odds ratio [OR] = 4.77, 95% CI = 1.10–20.57), interstitial lung disease (ILD) (OR = 10.07, 95% CI = 1.65–61.51), positive anti‐U1RNP antibody (OR = 4.38, 95% CI = 1.08–17.75), and Raynaud's phenomenon (OR = 7.94, 95% CI = 1.41–44.69) were associated with muscle involvement. Except for anti‐Ro52 (50%), anti‐Ku antibody (38.2%) was the next frequently detected MSA/MAA in the panel, followed by anti‐NXP2 antibody (11.8%). It was noteworthy that multiple MSA/MAAs (≥2, excluding anti‐Ro52) coexisted in 9 patients. Patients with myositis features were clustered into 2 subgroups. Cluster 1 was characterized by anti‐Ku or anti‐Ro52 with high SLE Disease Activity Index, whereas cluster 2 presented with anti‐U1RNP, Raynaud's phenomenon and pulmonary arterial hypertension resembling mixed connective tissue disease. Conclusion: In our Chinese SLE inpatient cohort, muscle involvement was infrequent. Nevertheless, distinct features in these SLE patients deserve further study. [ABSTRACT FROM AUTHOR]

Objective To develop a short-term renal outcome prediction model for acute kidney injury (AKI) in patients with LN. Methods Two lupus AKI cohorts from two independent centres during 2013–2019 were included: a derivation cohort from a rheumatology centre and a validation cohort from a nephrology centre. Clinical characteristics and renal histologic features were obtained. The outcome measurement was the recovery of kidney function within 12 months. Lasso regression was used for feature selection. Prediction models with or without pathology were built and a nomogram was plotted. Model evaluation including calibration curve and decision curve analysis was performed. Results A total of 130 patients were included in the derivation cohort and 96 patients in the validation cohort, of which 82 and 73 patients received a renal biopsy, respectively. The prognostic nomogram model without pathology included determinants of SLE duration, days from AKI onset to treatment and baseline creatinine level [C-index 0.85 (95% CI 0.78, 0.91) and 0.79 (95% CI 0.70, 0.88) for the two cohorts]. A combination of histologic tubulointerstitial (TI) fibrosis in the nomogram gave an incremental predictive performance (C-index 0.93 vs 0.85; P  = 0.039) in the derivation cohort but failed to improve the performance in the validation cohort (C-index 0.81 vs 0.79; P  = 0.78). Decision curve analysis suggested clinical benefit of the prediction models. Conclusion The predictive nomogram models might facilitate more accurate management for lupus patients with AKI. [ABSTRACT FROM AUTHOR]

Immune thrombocytopenia (ITP) is a common complication of connective tissue diseases (CTD). However, refractory and recurrent cases are frequent, who often need intensive immunotherapy. In the real world to compare the efficacy and safety of two common options, rituximab (RTX) and cyclosporine (CsA), in patients with refractory CTD-ITP, we conducted this retrospective study. Inpatients diagnosed with CTD-ITP who experienced treatment failure with initial prednisone or other immunosuppressants and who subsequently received either RTX or CsA between 2013 and 2018 were identified. All the patients were followed up for at least 6 months. Remission was defined as sustained platelet count ≥ 50 × 10^9/L, where ≥ 100 × 10^9/L was considered complete remission and 50–100 × 10^9/L was considered partial remission. Propensity score weighting analysis was performed to balance the confounders as indication. A total of 83 patients with CTD-ITP were identified, of whom 43 had systemic lupus erythematosus, 24 had undifferentiated CTD, and 16 had primary Sjogren syndrome. The RTX group (n = 53) had a much higher remission rate than the CsA group (n = 30) after 3 months and throughout the following 3 months (3 m, 86.8% vs 63.6%, p = 0.025; 6 m, 81.8% vs 53.5%, p = 0.011). Binary logistic regression analysis confirmed that treatment with RTX predicted better outcome (OR 4.09, 1.42 ~ 11.79), while age > 50 (OR 0.31, 0.11 ~ 0.93) was a risk factor. Furthermore, we reinforced the conclusions by propensity score weighting analysis (RTX OR 4.89, 1.64 ~ 14.58; age > 50 OR 0.31, 0.12 ~ 0.83). In our real-world retrospective study, for patients with refractory CTD-ITP, RTX was superior to CsA in terms of the durable remission rate. Key Points: • Refractory cases are common in patients with immune thrombocytopenia secondary to connective tissue diseases (CTD-ITP), requiring intensive immunotherapy. • Randomized controlled trials comparing rituximab and a traditional immunosuppressive agents (IS), such as cyclosporin, are lacking in these patients. • Our real-word retrospective study indicated that rituximab was superior to cyclosporin in patients with refractory CTD-ITP. [ABSTRACT FROM AUTHOR]

Systemic lupus erythematosus (SLE) gastrointestinal (GI) complication is characterized by multi-segment and multi-compartment involvement. The aim of this study is to develop a computed tomography (CT) image-based system for disease evaluation. SLE patients with GI involvement from two independent cohorts were retrospectively included. Baseline abdominal CT scan with intravenous and oral contrast was obtained from each individual. A CT scoring system incorporating the extent of GI tract involvement and intestinal wall thickness, along with extra-GI compartment involvement, was developed and validated. The outcome measurement was the time to GI functional recovery, defined as the time to tolerable per os (PO) intake ≥50% of ideal calories (PO50). A total of 54 and 37 patients with SLE GI involvement were enrolled in the derivation and validation cohorts, respectively. The CT scores for SLE GI involvement were positively correlated with patients' time to PO50 (r = 0.57, p < 0.0001, derivation cohort; r = 0.42, p = 0.0093, validation cohort). Patients with a CT score ≤ 3 had a shorter time to PO50 (median time of 0 day) in pooled cohort, whereas those with a CT score > 3 incurred a significantly prolonged recovery with a median time to PO50 of 13 days (p < 0.0001). The CT-based scoring system may facilitate more accurate assessment and individualized management of SLE patients with GI involvement. [ABSTRACT FROM AUTHOR]

Thrombotic microangiopathy (TMA) includes a series of life-threatening disorders. Systemic lupus erythematosus (SLE) is one of the most common acquired causes. To identify predictors of prognosis in patients with TMA secondary to SLE, we conducted a single-center historical study. From January 2013 to June 2016, of 2182 SLE hospitalized patients in the Ren Ji Hospital, a total of 21 consecutive patients with TMA secondary to SLE were identified. The 90-day short-term mortality was 33.3%. The kidney involvement (66.7%) was associated with poor prognosis, while the administration of rituximab ( n = 13) was an independent protective factor according to logistic regression analysis. Compared to conventional treatment, i.e., plasma exchange, high-dose glucocorticoids, and intravenous immunoglobulin, the overall survival is significantly higher among patients receiving rituximab add-on (92.2 vs 33.3%, p = 0.0173); however, five out of seven patients with renal involvement in the rituximab group were eventually hemodialysis dependent. Our data indicated that add-on rituximab in the background of conventional therapy may improve the overall but not the renal survival in SLE-TMA patients. [ABSTRACT FROM AUTHOR]

To compare the performance of different commercial anti-dsDNA autoantibody assays, including multiplex-based immunoassay (Bio-Plex), Farr radioimmunoassay (Farr), ELISA, chemiluminescent immunoassay (CLIA), and Crithidia Luciliae indirect immunofluorescence test (CLIFT) in Chinese patients with systemic lupus erythematosus (SLE). SLE patients ( n = 119) as well as healthy controls ( n = 200) and disease controls ( n = 100) were recruited, and serum anti-dsDNA autoantibodies were detected by Bio-Plex, Farr, two ELISA assays (Medical & Biological Laboratories-ELISA, EUROIMMU-ELISA), CLIA, and a standard CLIFT. The correlation of anti-dsDNA autoantibody levels to SLE disease activity was calculated, and the specificity and sensitivity of these methods were measured by receiver-operator characteristic (ROC) curve analysis. In ROC curve analysis, Bio-Plex showed the largest area under the curve (AUC) over other assays. Cutoff adjustment according to ROC enhanced the performance of all quantitative assays. Overall, Bio-Plex and CLIFT have higher specificity (>90.00%). ELISA and CLIA results are correlated with disease activity, and Bio-Plex results have the strongest correlation with SLEDAI score and active renal involvement. Bio-Plex assay has better overall performance in anti-dsDNA detection over Farr, ELISA, and CLIA methods in Chinese SLE patients. [ABSTRACT FROM AUTHOR]

Background: Infection remains a major cause of morbidity and mortality in patients with systemic lupus erythematosus (SLE). This study aimed to establish a clinical prediction model for the 3-month all-cause mortality of invasive infection events in patients with SLE in the emergency department. Methods: SLE patients complicated with invasive infection admitted into the emergency department were included in this study. Patient's demographic, clinical, and laboratory characteristics on admission were retrospectively collected as baseline data and compared between the deceased and the survivors. Independent predictors were identified by multivariable logistic regression analysis. A prediction model for all-cause mortality was established and evaluated by receiver operating characteristic (ROC) curve analysis. Results: A total of 130 eligible patients were collected with a cumulative 38.5% 3-month mortality. Lymphocyte count <800/ul, urea >7.6mmol/l, maximum prednisone dose in the past ⩾60 mg/d, quick Sequential Organ Failure Assessment (q S OFA) score, and age at baseline were independent predictors for all-cause mortality (LUPHAS). In contrast, a history of hydroxychloroquine use was protective. In a combined, odds ratio-weighted LUPHAS scoring system (score 3–22), patients were categorized to three groups: low-risk (score 3–9), medium-risk (score 10–15), and high-risk (score 16–22), with mortalities of 4.9% (2/41), 45.9% (28/61), and 78.3% (18/23) respectively. ROC curve analysis indicated that a LUPHAS score could effectively predict all-cause mortality [area under the curve (AUC) = 0.86, CI 95% 0.79–0.92]. In addition, LUPHAS score performed better than the qSOFA score alone (AUC = 0.69, CI 95% 0.59–0.78), or CURB-65 score (AUC = 0.69, CI 95% 0.59–0.80) in the subgroup of lung infections (n = 108). Conclusions: Based on a large emergency cohort of lupus patients complicated with invasive infection, the LUPHAS score was established to predict the short-term all-cause mortality, which could be a promising applicable tool for risk stratification in clinical practice. [ABSTRACT FROM AUTHOR]