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Objectives We aimed to investigate the value of deep learning (DL) models based on multimodal ultrasonographic (US) images to quantify RA activity. Methods Static greyscale (SGS), dynamic greyscale (DGS), static power Doppler (SPD) and dynamic power Doppler (DPD) US images were collected and evaluated by two expert radiologists according to the EULAR–OMERACT Synovitis Scoring system. Four DL models were developed based on the ResNet-type structure, evaluated on two separate test cohorts, and finally compared with the performance of 12 radiologists with different levels of experience. Results In total, 1244 images were used for the model training, and 152 and 354 for testing (cohort 1 and 2, respectively). The best-performing models for the scores of 0/1/2/3 were the DPD, SGS, DGS and SPD models, respectively (Area Under the receiver operating characteristic Curve [AUC] = 0.87/0.95/0.74/0.95; no significant differences). All the DL models provided results comparable to the experienced radiologists on a per-image basis (intraclass correlation coefficient: 0.239–0.756, P  < 0.05). The SPD model performed better than the SGS one on test cohort 1 (score of 0/2/3: AUC = 0.82/0.67/0.95 vs 0.66/0.66/0.75, respectively) and test cohort 2 (score of 0: AUC = 0.89 vs 0.81). The dynamic DL models performed better than the static ones in most of the scoring processes and were more accurate than the most of senior radiologists, especially the DPD model. Conclusion DL models based on multimodal US images allow a quantitative and objective assessment of RA activity. Dynamic DL models in particular have potential value in assisting radiologists to improve the accuracy of RA US-based grading. [ABSTRACT FROM AUTHOR]

Objectives Mortality of dermatomyositis patients positive with anti-melanoma differentiation-related gene 5 antibody (anti-MDA5-DM) is alarming, especially during the first several months. Infection is an important cause of early death. As there are no reports regarding the effect of prophylactic use of compounded sulfamethoxazole (coSMZ; each tablet contains 400 mg of sulfamethoxazole and 80 mg of trimethoprim) in anti-MDA5-DM patients, we conducted this study to evaluate the efficacy of coSMZ in reducing the incidence of Pneumocystis jirovecii pneumonia (PJP). Methods Consecutive patients with new-onset anti-MDA5-DM from June 2018 to October 2021 in our centre were retrospectively reviewed for >12 months. They were divided into two groups—coSMZ and non-coSMZ—based on the initial use of prophylactic coSMZ. Mortality and the incidence of severe infection within 12 months were compared between two groups. Results Compared with the non-coSMZ group (n  = 93), the coSMZ group (n  = 121) had lower mortality (18.8% vs 51.1%; P   <  0.001) and a lower incidence of PJP (6.8% vs 15.2%; P  = 0.040) and fatal infection (16.1% vs 3.3%; P  = 0.001) during the first 12 months from diagnosis. After adjusting for age, gender, disease duration, peripheral blood lymphocyte count, anti-MDA5 antibody titres, ground-glass opacity scores and treatments, an inverse association was revealed between the prophylactic use of coSMZ and incidence of PJP [adjusted odds ratio 0.299 (95% CI 0.102–0.878), P  = 0.028]. Conclusion Prophylactic use of coSMZ is an effective and safe way to improve the prognosis of anti-MDA5-DM patients by preventing the incidence of PJP. [ABSTRACT FROM AUTHOR]

Objectives To describe the detailed characteristics and explore the potential risk factors of relapses in patients with adult-onset Still's disease (AOSD). Methods We enrolled patients with AOSD admitted to the Department of Rheumatology and Immunology, Ruijin Hospital from August 2016 to September 2019. Kaplan–Meier curves and the log rank test were used to estimate the cumulative relapse probability and persistent remission rate before the first occurrence of relapse. The multivariate Cox proportional hazard method was utilized to identify risk factors associated with relapses of AOSD. Results A total of 122 patients with AOSD were enrolled with a median follow-up of 12.6 months. Among them, 26 (21.3%) patients had at least one relapse. The cumulative relapse rates of AOSD patients were 14.42%, 21.79%, 24.81% and 28.57% at 6, 12, 18 and 36 months, respectively. According to the multivariate analysis, intensive treatment (odds ratio: 6.848; 95% CI: 2.441, 19.211) and macrophage activation syndrome (odds ratio: 4.020, 95% CI: 1.564, 10.322) were associated with increased risk of relapse. Conclusion Our study indicated that relapses occurred in at least one-fifth of patients with AOSD, and patients with high disease severity at initial attack may have an increased risk of relapse, which needs more intensive therapy and close follow-up. [ABSTRACT FROM AUTHOR]

Objective. To assess the diagnostic value of salivary gland ultrasonography (SGUS) for primary SS (pSS) and to compare the usefulness of two existing SGUS scoring systems. Methods. Ultrasonography examination of major salivary glands was conducted for 105 pSS patients and 41 disease control subjects without SS and 16 healthy control subjects. The imaging features were graded using two different scoring systems (0-16 and 0-48, respectively) obtained from the grades of bilateral parotid and submandibular glands. Receiver operating characteristic curves were used to describe and compare the diagnostic accuracy of the two ultrasonography echostructure scoring systems for pSS. The agreement of diagnosis for pSS between the two scoring systems was determined by /c-statistics. Results. SGUS scores for the pSS group were significantly higher than those for the non-pSS group (P< 0.001). The best score cut-off was 7 in the 0-16 system (80% sensitivity and 93% specificity, respectively), and it was 15 in the 0-48 system (88.6% sensitivity and 84.2% specificity, respectively). Compared with the 0-16 system, combined evaluation of all four glands when using the 0-48 system improved the diagnostic accuracy. Association analysis of both scoring systems showed a positive correlation of SGUS scores with RF and γ-globulin% (P<0.05, overall). Conclusion. SGUS is a feasible method for pSS diagnosis with higher sensitivity using the 0-48 system and better specificity using the 0-16 system. SGUS scores are related to RF and g-globulin%. [ABSTRACT FROM AUTHOR]

Objective. The podocyte lesion in LN is still an intriguing controversy. We assess the associations between podocyte lesions and clinico-pathological features in a large cohort of LN patients.Methods. The clinico-pathological data of 202 patients with renal biopsy-proven LN were retrospectively studied. The degree of podocyte lesions was assessed morphologically and its correlations with clinico-pathological parameters were further analysed.Results. The podocyte foot processes of most LN patients significantly effaced, reflected by the median foot process width (FPW) of 1397.39 nm, and 13 patients met the histological criteria of lupus podocytopathy. The FPW was correlated with proteinuria (r = 0.509, P < 0.001) and the cut-off value of FPW, >1240 nm, could differentiate nephrotic proteinuria from non-nephrotic proteinuria with sensitivity 81.5% and specificity 62.7%. The FPW varied significantly with different types of LN, and the patients with combined LN presented with the most severe lesions. The complete remission rate was significantly higher and the long-term renal outcome was better in the group with calcineurin inhibitors than that with other regimens in patients with FPW >1240 nm.Conclusion. Podocyte damage was common in LN. Pure lupus podocytopathy might act as an extreme form of lupus podocyte lesion, and more patients might present with severe podocyte effacement concealed in different types of LN, which needs further investigation. [ABSTRACT FROM PUBLISHER]

Intervertebral disc degeneration usually starts from the inner nucleus pulposus (NP). The majority of previous NP-related studies assessed the outcome by the expression of chondrogenic markers since NP cells are chondrocyte like. However, NP cells are unique from chondrocytes and such assessments may be inappropriate. Very recently, several investigators published their findings about the transcriptional differences between NP cells and other related cell types on a genomic scale. In this review we discuss these recent findings and summarize the molecules that may be utilized as NP-specific markers to distinguish normal NP cells from several cell types and as markers that indicate its degeneration. We will revisit markers that distinguish NP cells from the outer surrounding annulus fibrosus (AF) cells and articular chondrocytes so as to facilitate authentic NP cell engineering from stem cells. Our review indicated that N-cadherin and keratin 19 have the potential to serve as common NP markers, as they distinguish healthy NP cells from AF cells, articular cartilage cells and degenerated NP cells. [ABSTRACT FROM PUBLISHER]

Objectives. Elevated expression of Siglec-1 on circulating monocytes has been reported in some inflammatory and autoimmune diseases, but its expression and role in RA has not been elucidated. The aims of this study were to determine the expression of Siglec-1 in peripheral blood and to explore its role in mononuclear cell reactivity to autoantigen in RA.Methods. Siglec-1 protein and mRNA levels in 42 RA patients, 39 OA patients, 28 SLE patients and 42 normal controls were determined by flow cytometry and quantitative RT-PCR, respectively. In addition, 10 patients with active RA received DMARDs for 12 weeks and the frequencies of Siglec-1-positive cells and the 28-joint DAS (DAS28) were assessed before and after therapy. Furthermore, TNF-α, IFN-γ and type II collagen were used to up-regulate Siglec-1. Peripheral blood mononuclear cells (PBMCs) from different groups were stimulated with mitogens or antigens and cell proliferation and cytokine production were determined.Results. The protein and mRNA levels of Siglec-1 on PBMCs and monocytes in RA patients were significantly higher than those in OA patients and healthy controls. Moreover, the expression of Siglec-1 protein on PBMCs was positively correlated with DAS28, ESR, high-sensitivity CRP and IgM-RF, but not with anti-CCP antibody. Interestingly, Siglec-1 expression was decreased in parallel with the decrease in the DAS28 after 12 weeks of anti-rheumatic treatment. Furthermore, TNF-α, IFN-γ and type II collagen can up-regulate Siglec-1 in PBMCs. Elevated PBMC proliferation and proinflammatory cytokine production to collagen stimulation in RA patients decreased when Siglec-1 was inhibited by anti-Siglec-1 antibodies.Conclusion. Elevated Siglec-1 expression in PBMCs and monocytes can potentially serve as a biomarker for monitoring disease activity in RA. Siglec-1 may also play a proinflammatory role in stimulating lymphocyte proliferation and activation in RA. [ABSTRACT FROM PUBLISHER]

Objective. Previous studies have shown that in patients with ANCA-associated vasculitis (AAV), anti-plasminogen antibodies were associated with reduced renal function and the presence of fibrinoid necrosis and cellular crescents in renal histology. The purpose of the current study was to investigate whether anti-plasminogen antibodies are associated with the systemic disease activity of AAV.Methods. One hundred and four Chinese patients with AAV were recruited. Anti-plasminogen antibodies were detected in sequential serum samples at initial onset and remission of the disease. Associations of anti-plasminogen antibodies with clinicopathological parameters were analysed.Results. The prevalence of anti-plasminogen antibodies was significantly higher in AAV patients than in healthy controls (19/104 vs 0/50, χ2 = 8.8, P = 0.003). The prevalence of anti-plasminogen antibodies was significantly higher in the active stage of AAV than in remission (19/104 vs 1/48, χ2 = 7.5, P = 0.013). The level of anti-plasminogen antibodies (expressed as a percentage of the positive controls) correlated with the ESR (r = 0.207, P = 0.042), serum creatinine (r = 0.302, P = 0.002), d-dimer (r = 0.273, P = 0.009) and the percentage of glomeruli with crescents in renal specimens (r = 0.393, P = 0.004). The level of Birmingham vasculitis activity scores and the prevalence of arthralgia and gastrointestinal involvement in patients with anti-plasminogen antibodies were significantly higher than in patients without anti-plasminogen antibodies [22.5 (s.d. 5.63) vs. 19.4 (s.d. 4.66), P = 0.015; 63.2% vs. 25.8%, P = 0.002; 57.9% vs. 21.1%, P = 0.001, respectively].Conclusion. Circulating anti-plasminogen antibodies were associated with systemic disease activity and renal disease activity of AAV. [ABSTRACT FROM PUBLISHER]

Objective. This study aims to identify the existence of, and relationship between autoantibody clusters and clinical subsets in Chinese SLE patients.Methods. Data from 1928 SLE patients from Hong Kong were analysed. Using cluster analysis, patients were grouped by autoantibodies into clusters. The frequencies of various clinical manifestations were then compared between each cluster. Separate association analyses between individual autoantibodies and clinical manifestations as well as between clinical manifestations were also performed without any prior clustering.Results. Three separate autoantibody clusters were identified, each with significantly different clinical manifestations. Cluster 1 was characterized by anti-dsDNA and the greatest prevalence of renal disorder but the lowest frequencies of other clinical manifestations. Cluster 2 was represented by the predominance of anti-Smith, anti-RNP and aPL, with greater prevalence of malar rash, oral ulcers, arthritis and serositis. Cluster 3 was characterized by anti-Ro and anti-La with greater prevalence of discoid rash, photosensitivity and haematological involvement. Individual association analysis also revealed similar findings. Patients of clusters 2 and 3 were more closely related, while cluster 1 was more distinct, associated with renal disorder only and negatively associated or not associated with other manifestations.Conclusion. We conclude that autoantibody clustering and clinical subsets exist in SLE patients of our locality. These clusters may be viewed as a bipolar spectrum of related autoantibody and clinical manifestations. At one end are patients with over-representation of anti-dsDNA and renal disorder, while at the other end are two distinct autoantibody clusters (anti-Sm/anti-RNP/aPL and anti-Ro/anti-La) with overlapping of other clinical manifestations. [ABSTRACT FROM AUTHOR]

Objective. To study the prevalence and risk factors of low BMD in patients with SSc.Methods. Consecutive patients with SSc and an equal number of age- and gender-matched healthy subjects were screened for BMD, fat and lean mass by DXA scan. BMD, body composition and osteoporosis risk factors were compared between patients and controls. Associated factors for low BMD in SSc patients were studied by linear regression.Results. A total of 84 patients with SSc were studied [89% women; age 49.4 (11.3) years; 21% diffuse subtype; disease duration 7.8 (6.4) years]. Except for significantly lower BMI (P = 0.001), fat mass (P = 0.02) and lean body mass (P = 0.006) observed in SSc patients, the prevalence of other osteoporosis risk factors was similar to controls. Fourteen (17%) and five (6%) SSc patients had low BMD expected for age (z-score <−2.0) at the lumbar spine and hip, respectively. BMD of the lumbar spine, hip, femoral neck and whole body was significantly lower in SSc patients than controls, adjusted for age, sex, menopause and BMI (P < 0.05 in all; effect size 0.44–0.54). Linear regression revealed increasing age, menopause and low BMI were independently associated with low BMD at the spine or hip in SSc patients. However, BMD did not correlate with the severity of involvement of the skin and other systems.Conclusion. BMD of the spine and hip is significantly lower in patients with SSc than in healthy subjects, which is independent of age, sex, menopause, low BMI and altered body composition. [ABSTRACT FROM PUBLISHER]

Objective. The aim of this study was to investigate serum complement factor H (CFH) and its associations with clinical and pathological features in patients with LN.Methods. Serum CFH was detected in 241 LN patients, 38 active and 11 inactive patients with SLE without clinical evidence of renal involvement and 51 normal controls. Serum CFH autoantibodies and CFH Tyr402His were screened in the 241 LN patients. CFH deposition in kidneys was detected in some patients.Results. Serum CFH levels in patients with LN at active phase were significantly lower than in 38 SLE patients or in normal controls. No serum anti-CFH autoantibodies were detected in patients with LN, and there was no significant difference in CFH Tyr402His distribution between patients with LN and normal controls. Glomerular expression of CFH was stronger than in normal controls. Serum CFH levels were mildly negatively associated with SLEDAI scores (r = −0.204, P = 0.001) and positively associated with serum C3 (r = 0.367, P < 0.001) and haemoglobulin levels (r = 0.193, P = 0.003). Patients with LN class III, subclass IV-S and those with thrombotic microangiopathy had the lowest serum CFH.Conclusion. Serum CFH levels were associated with disease activity of LN. [ABSTRACT FROM PUBLISHER]

Objectives. To study the relationship of 25(OH)D3 level with disease activity, vascular risk factors and atherosclerosis in SLE.Methods. Consecutive patients who fulfilled four or more ACR criteria for SLE were recruited for assay of 25(OH)D3 level. Disease activity was assessed by the SLEDAI and physicians’ global assessment (PGA). Patients with vascular risk factors were screened for atherosclerosis at the coronary or carotid arteries. Correlation between 25(OH)D3 levels and SLEDAI scores was studied by linear regression. The link between vascular risk factors, atherosclerosis and vitamin D deficiency was also examined.Results. A total of 290 SLE patients were studied [94% women; mean (s.d.) age 38.9 (13.1) years; disease duration 7.7 (6.7) years; 78% patients had clinical or serological lupus activity]. Two hundred and seventy-seven (96%) patients had vitamin D insufficiency [25(OH)D3 < 30 ng/ml] and 77 (27%) patients had vitamin D deficiency (<15 ng/ml). Levels of 25(OH)D3 correlated inversely with PGA (β −0.20; P = 0.003), total SLEDAI scores (β −0.19; P = 0.003) and subscores due to active renal, musculoskeletal and haematological disease. Subjects with vitamin D deficiency had significantly higher total/high-density lipoprotein (HDL) cholesterol ratio [3.96 (2.94) vs 3.07 (0.80); P = 0.02] and prevalence of aPLs (57 vs 39%; P = 0.007). Of 132 patients, 58 (44%) with vascular risk factors screened were positive for subclinical atherosclerosis. No association could be demonstrated between 25(OH)D3 level and atherosclerosis, which was mainly associated with increasing age, menopause, obesity and hyper-triglyceridaemia.Conclusions. In this large cross-sectional study of SLE patients, 25(OH)D3 level correlates inversely with disease activity. Vitamin D deficiency is associated with dyslipidaemia. In patients with vascular risk factors, subclinical atherosclerosis is not associated with hypovitaminosis D. [ABSTRACT FROM PUBLISHER]

Objective. We sought to evaluate the use of high-resolution three-dimensional time of flight (3D TOF) magnetic resonance angiography (MRA) at 3 Tesla in the visualization of digital arteries in SSc patients.Methods. A total of 33 patients with SSc [32 females and 1 male; mean (s.d.) age 37.0 (10.2) years; median number of years since onset of RP 3.4 (2.8) years] and 7 healthy controls [6 females and 1 male; mean (s.d.) age 30.7 (3.5) years] were examined on a 3T MR system. A modified high spatial resolution (voxel size = 0.35 × 0.35 × 0.5 mm3) 3D TOF MRA (repetition time/echo time = 19/4.4 ms, flip angle = 15°, slice thickness = 1 mm) was performed during a total scan time of 8 min 22 s. The source images and maximum intensity projection reconstruction were studied; the digital arteries count and lumen area of the selective section of the vessel were measured independently by two experienced radiologists and compared with that of the control ones; and a four-level grading system was made according to the severity. Statistical analysis was performed with t-test and P < 0.05 was used as the criterion.Results. We detected the eight digital arteries in the four fingers (without the thumb) of each case and got a 47.58% presentation in general in the SSc group, and artery No. 5 had the highest presentation rate (70.97%). Statistics showed that the digital arteries No. 1 (P = 0.058) and No. 3 (P = 0.093) had no difference in the lumen area (P > 0.05). We finally got 3 cases in Grade 1, 14 cases in Grade 2, 9 cases in Grade 3 and 5 cases in Grade 4.Conclusion. MRA of the digital arteries in the described technique is a promising method for us to judge the severity of microvascular involvement in finger vessels of SSc patients. [ABSTRACT FROM PUBLISHER]

Objective. To validate the clinical value of the new Ankylosing Spondylitis Disease Activity Scores (ASDASs) in assessing the disease activity and efficacy of TNF-α inhibitor in AS and uSpA patients in China.Methods. Two hundred and thirty patients were included in our study. They consisted of patients with active AS (n = 87) and uSpA (n = 30) participating in a double-blind placebo-controlled randomized clinical trial of etanercept and patients with active AS (n = 58) and uSpA (n = 55) treated with infliximab. The disease activity and treatment effects were assessed by ASDAS, BASDAI, patient global and the acute inflammation score of lumbar and SI joints by MRI. Discriminatory ability of all the measures was analysed by standardized mean difference and t-score.Results. In both the AS and uSpA groups, ASDAS correlated well with patient global score (AS group: r = 0.65–0.72; uSpA group: r = 0.52–0.62), ESR (AS group: r = 0.57–0.81; uSpA group: r = 0.63–0.85) and CRP (AS group: r = 0.51–0.70; uSpA group: r = 0.61–0.76) both at baseline and in changes from baseline to 6 weeks after TNF-α inhibitor treatment. The ASDAS scores outperformed BASDAI, patient global score, ESR, CRP and the acute inflammation score by MRI in differentiating patients with different levels of disease activity and patients with different levels of change in both AS and uSpA groups. There was little difference in performance between the two versions of the ASDAS.Conclusion. The new ASDAS is a highly effective measure in assessing disease activity and a great discriminatory measurement to assess the efficacy of TNF-α inhibitor in Chinese AS patients and uSpA patients. [ABSTRACT FROM PUBLISHER]

Objective. To estimate the direct and indirect costs among a cohort of Chinese patients with RA in Hong Kong.Methods. A cost-of-illness study using cross-sectional and retrospective data was conducted on 144 patients with RA. Costs were estimated from the societal perspective. Participants completed questionnaires regarding demographics, employment status and out-of-pocket expenses. Health resources consumption was recorded by review of medical records. Functional disability was assessed using the HAQ score.Results. The cohort had a mean age of 49 years and mean disease duration of 10.8 years. Average total costs of RA were estimated at $9286 (2006 US dollars) per patient-year, >60% of which was attributable to indirect costs due to productivity losses. Patients’ out-of-pocket expenses and costs of inpatient care dominated direct costs, each representing 11% of total direct costs. Younger age, poorer physical and mental health independently predicted high direct costs. Older age, lower education level and more functional disability independently predicted high indirect costs.Conclusion. RA is associated with both high direct and indirect costs, imposing a considerable economic burden on patients and society. The hope is that effort will be gathered not only from patients and physicians, but more importantly from government, to improve the care and treatment of patients with RA and to help reduce or avoid the considerable societal costs of RA. [ABSTRACT FROM PUBLISHER]

Objective. SS is an autoimmune disease characterized by salivary and lacrimal gland dysfunction leading to dry mouth (xerostomia) and dry eyes (xerophthalmia). Anti-muscarinic acetylcholine type-3 receptor (anti-M3R) autoantibodies have been shown to be a good serum marker in primary SS (pSS). The aim of this study was to assess the clinical correlations of anti-M3R-derived peptide antibodies in patients with pSS.Methods. Sequences of the first to fourth cycle-M3R (c1M3R–c4M3R)-derived peptide was synthesized by a solid-phase technique on an Applied Biosytems Peptide Synthesizer. Synthesized cM3R peptide (cM3RP) was used as substrate in an ELISA to detect IgG anti-cM3RP antibodies in serum samples of patients and controls. The clinical and biological parameters of the diseases were also evaluated. The EULAR SS disease activity index (ESSDAI) score was used to measure disease activity in patients with primary SS.Results. (i) Anti-c2M3RP antibodies were highly prevalent in pSS patients, and the titre is much higher than anti-c1,3,4M3RP antibodies. (ii) The prevalence of anti-c2M3RP antibodies in pSS, SLE, RA and healthy controls was 62.2, 7.1, 5.3 and 1.6%, respectively. The prevalence of anti-linear-2-M3RP antibodies in pSS, SLE and RA patients and healthy controls were 56.1, 20.0, 14.7 and 9.4%. (iii) The specificity of anti-c2M3RP antibodies was 95.1%, much higher than that of linear polypeptide (84.7%) for pSS diagnosis. (iv) In pSS patients, anti-c2M3RP positivity had significantly increased frequency in patients who were RF or ANA positive, and had several haematological abnormalities, such as leucopenia, anaemia and thrombocytopenia. Furthermore, the ESSDAI score was significantly higher in anti-c2M3RP-positive pSS patients (P < 0.05).Conclusion. Anti-c2M3RP antibody was highly specific for patients with pSS. The presence of anti-c2M3RP antibody in pSS indicates that c2M3RP may act as an autoantigen that may play a role in the pathogenesis of pSS. [ABSTRACT FROM PUBLISHER]