Cardiac imaging techniques for the assessment of immune checkpoint inhibitor-induced cardiotoxicity and their potential clinical applications.

Immune checkpoint inhibitors (ICIs) have encouraged a paradigm shift in the clinical management of patients with cancer. Despite the dramatically improved tumor response and patient prognosis, ICIs have been associated with ICI-related myocarditis, which has a high fatality rate. Cardiac imaging plays a critical role in the assessment of cardiac injury. Echocardiography, cardiac magnetic resonance imaging, and targeted tracer-based cardiac molecular imaging techniques alone or in combination reflect pathophysiology and depict different aspects of lesions at different clinical stages, i.e., they have potentially complementary value. Imaging techniques for identifying ICI-induced cardiotoxicity at the early stage may reduce the incidence of adverse cardiovascular events. Particularly in planned ICI therapy among patients with cancer, improved monitoring approaches to identify patients who are at the highest risk of ICI-related myocarditis may help in refining clinical decisions, allowing treatment to be more accurately targeted toward patients who are most likely to benefit. In this study, we systematically reviewed the studies on cardiac imaging techniques for assessing ICI-induced cardiotoxicity. We elaborated about the potential applications of cardiac imaging techniques for the optimized management of patients with ICI-related myocarditis, including risk stratification, diagnosis, and prognosis.

Risk factors associated with osteoporosis and fracture in psoriatic arthritis.

BACKGROUND: Although there are few studies mentioned there may be some relationship between psoriatic arthritis (PsA) and osteoporosis, clinical data in real world still need to be clarified in China. The aim of this study was to assess the areal and volumetric bone mineral density (BMD), frequency of fracture, and risk factors in patients with PsA. METHODS: A total of one hundred PsA patients who visited Peking University First Hospital and one hundred age- and sex-matched healthy controls with DXA data were enrolled in the study. Patients with clinical fractures confirmed by X-ray during follow-up were also recorded. Clinical characteristics of the patients were recorded and compared between the abnormal BMD group and the normal BMD group, as well as between the fracture and non-fracture groups. Risk factors for fracture and low BMD were analyzed. RESULTS: Mean BMD at the total hip and femoral neck was significantly lower in PsA patients than that in healthy controls (0.809 ±â€Š0.193 vs. 0.901 ±â€Š0.152 g/cm2, P  = 0.041; 0.780 ±â€Š0.146 vs. 0.865 ±â€Š0.166 g/cm2, P  = 0.037, respectively). Moreover, lumbar spine BMD was negatively correlated with psoriasis duration, swollen joint count and DAS28-CRP (r = -0.503, -0.580, -0.438; P < 0.05). Total hip BMD and femoral neck BMD were negatively correlated with HAQ (r = -0.521, -0.335; P < 0.05). Fractures occurred in 29 patients during the follow-up period. Logistic regression analysis showed that older age (OR 1.132 [95%CI: 1.026-1.248), P < 0.05], higher HAQ score (OR 1.493, 95%CI: 1.214-1.836, P < 0.01), higher disease activity index for psoriatic arthritis (OR 1.033, 95% CI: 1.002-1.679, P < 0.05) and hip joint involvement (OR 6.401, 95% CI: 4.012-44.180, P < 0.05) were risk factors for fracture in the multivariate model. CONCLUSIONS: Increased risks of osteoporosis and fracture were found in PsA patients compared to healthy controls. Besides age, high disease activity and hip joint involvement were risk factors for decreased BMD and fracture.

Systemic lupus erythematosus: year in review 2019.

Systemic lupus erythematosus (SLE) is an autoimmune disease with extreme heterogeneity and potentially involvement of any organ or system. Numerous unanswered questions and challenges in SLE always prompt further exploration. In 2019, great progress in various aspects of SLE emerged. Both the classification criteria and management recommendation for SLE were updated. New promising medications have been widely developed and tested, although subsequent clinical studies are warranted. As an emerging number of most notable studies in SLE were published in both clinical area and basic research in 2019, we aim to summarize the highest quality data on SLE regarding novel insights of pathogenesis, updated recommendations, hot-spot issues on clinical manifestations, new understanding of disease prognosis, and most importantly, the therapeutic advances in SLE in this review.

A Rare Variant (rs933717) at FBXO31-MAP1LC3B in Chinese Is Associated With Systemic Lupus Erythematosus.

OBJECTIVE: Recent evidence from genetic, cell biology, and animal model studies has suggested a pivotal role of autophagy in mediating systemic lupus erythematosus (SLE). However, the genetic basis has not yet been thoroughly examined. Therefore, the aim of the present study was to identify additional susceptibility variants in autophagy-related genes along with their functional significance. METHODS: First, we performed a gene family-based genetic association analysis in SLE patients with the use of ImmunoChip arrays, and then we selected the most strongly associated polymorphisms for replication in additional cohorts. To identify regulatory clues, we analyzed publicly available blood expression quantitative trait locus data and Encyclopedia of DNA Elements data on transcription factor binding sites and cell type-specific differential expression. Functional effects were tested by luciferase reporter assays, electrophoretic mobility shift assays, and differential gene expression assays. RESULTS: In 14,474 samples, we observed that the rare Chinese variant rs933717T was associated with susceptibility to SLE (0.11% in cases versus 0.87% in controls; P = 2.36 × 10-10 , odds ratio 0.13). The rs933717 risk allele C correlated with increased MAP1LC3B expression; increased MAP1LC3B messenger RNA was observed in SLE patients and in lupus-prone mice. In reporter gene constructs, the risk allele increased luciferase activity up to 2.7-3.8-fold in both HEK 293T and Jurkat cell lines, and the binding of HEK 293T and Jurkat cell nuclear extracts to the risk allele was also increased. CONCLUSION: We observed a likely genetic association between light chain 3B, a widely used marker for autophagy, and susceptibility to SLE.

Image-guided stem cells with functionalized self-assembling peptide nanofibers for treatment of acute myocardial infarction in a mouse model.

AIM: To investigate the survival of bone marrow mesenchymal stem cells (BMSCs) and the therapeutic effect for acute myocardial infarction (AMI) after co-transplantation with the functionalized self-assembling peptide nanofiber RAD/PRG or RAD/KLT. METHODS: AMI of balb/c mice was induced. Mice were randomly divided into four groups, and received treatment of phosphate buffered saline (PBS) (Group A), GFP/Fluc-BMSCs (Group B), GFP/Fluc-BMSCs + RAD/PRG (Group C), and GFP/Fluc-BMSCs + RAD/KLT (Group D), respectively. Bioluminescence imaging (BLI) was performed on day 1 (d-1), d-4, d-7, d-10, and d-13 after transplantation. Magnetic resonance imaging (MRI) was performed at baseline (d-4 before transplantation) and d-29 after treatment. Mice were euthanized on d-29 following treatment. Paraffin sections were obtained from the top, mid and bottom part of the infarcted region along the long-axis of the heart. Hematoxylin and eosin (HE) staining and immunohistochemical staining were performed. The infarct ratio micro-vascular density (MVD) was quantified. RESULTS: There was a significant higher of BLI signal intensity of BMSCs in Group C than that in Group B (d-4, 9713±320 vs. 8164±378, P=0.0008; d-7, 6489±241 vs. 5417±361, P=0.0026; d-10, 3768±255 vs. 0, P < 0.0001). The left ventricular ejection fraction (LVEF) via MRI examination was significantly improved in both Group C and Group D. Infarct ratio and MVD were significantly improved in both Group C and Group D. CONCLUSION: Our data highlights BMSCs combining functionalized self-assembling peptide nanofibers RAD/PRG or RAD/KLT as promising therapy for AMI.

High remission and low relapse with prolonged intensive DMARD therapy in rheumatoid arthritis (PRINT): A multicenter randomized clinical trial.

OBJECTIVES: To determine whether prolonged intensive disease-modifying antirheumatic drug (DMARD) treatment (PRINT) leads to high remission and low relapse rates in patients with severe rheumatoid arthritis (RA). METHODS: In this multicenter, randomized and parallel treatment trial, 346 patients with active RA (disease activity score (28 joints) [DAS28] (erythrocyte sedimentation rate [ESR]) > 5.1) were enrolled from 9 centers. In phase 1, patients received intensive treatment with methotrexate, leflunomide, and hydroxychloroquine, up to 36 weeks, until remission (DAS28 ≤ 2.6) or a low disease activity (2.6 < DAS28 ≤ 3.2) was achieved. In phase 2, patients achieving remission or low disease activity were followed up with randomization to 1 of 2 step-down protocols: leflunomide plus hydroxychloroquine combination or leflunomide monotherapy. The primary endpoints were good European League Against Rheumatism (EULAR) response (DAS28 (ESR) < 3.2 and a decrease of DAS28 by at least 1.2) during the intensive treatment and the disease state retention rate during step-down maintenance treatment. Predictors of a good EULAR response in the intensive treatment period and disease flare in the maintenance period were sought. RESULTS: A good EULAR response was achieved in 18.7%, 36.9%, and 54.1% of patients at 12, 24, and 36 weeks, respectively. By 36 weeks, 75.4% of patients achieved good and moderate EULAR responses. Compared with those achieving low disease activity and a high health assessment questionnaire (HAQ > 0.5), patients achieving remission (DAS28 ≤ 2.6) and low HAQ (≤ 0.5) had a significantly higher retention rate when tapering the DMARDs treatment (P = 0.046 and P = 0.01, respectively). There was no advantage on tapering to combination rather than monotherapy. CONCLUSIONS: Remission was achieved in a proportion of patients with RA receiving prolonged intensive DMARD therapy. Low disease activity at the start of disease taper leads to less subsequent flares. Leflunomide is a good maintenance treatment as single treatment.

Magnetic resonance imaging in early rheumatoid arthritis: a multicenter, prospective study.

To identify the magnetic resonance imaging (MRI) features of hands and wrists in early rheumatoid arthritis (RA). A total of 129 early arthritis patients (≤1 year) were enrolled in the study. At presentation, MRI of the hands was performed, with clinical and laboratory analyses. After a 1-year follow-up, clinical diagnosis of early RA or non-RA was confirmed by two rheumatologists. The characteristics of MRI variables at baseline in RA patients not fulfilling ACR 1987 criteria [RA-87(-)] were compared with those fulfilling ACR1987 criteria [RA-87(+)] and non-RA. In the 129 early arthritis patients, 90 were diagnosed with RA in a 1-year follow-up. There were 47.8 % (43/90) of the RA patients not fulfilling ACR 1987 criteria [RA-87(-)]. The scores of synovitis in RA-87(-) patients were similar with those in RA-87(+) [Synovitis score, 14.0 (IQR, 4.0-25.0) vs. 14.0 (IQR, 10.0-25.0), p > 0.05]. Compared with those in non-RA, RA-87(-) patients had higher synovitis scores and occurrence of synovitis in proximal interphalangeal (PIP) joints [synovitis score, 14.0 (IQR, 4.0-25.0) vs. 6.0 (IQR, 2.0-14.5), p = 0.046; occurrence of PIP synovitis: 53.5 vs. 27.3 %, p = 0.02]. There was no significant difference of bone marrow edema, bone erosion, and tenosynovitis between RA-87(-) and non-RA. Synovitis in PIP joints was independent predictor for RA-87(-) [OR, 3.1 (95 %CI 1.2-8.1)]. High synovitis scores and synovitis in PIP joints on MRI were important in early RA, especially those not fulfilling ACR 1987 criteria.

Efficacy and safety of weekly leflunomide for the treatment of early rheumatoid arthritis: a randomized, multi-center study.

AIM: The aim of this study was to determine the efficacy and safety of a weekly dose of leflunomide (50 mg/week) in early rheumatoid arthritis patients with mild or moderate disease activity. METHODS: The patients of early rheumatoid arthritis (ERA) with mild or moderate disease activity were randomly selected for inclusion in this study and were assigned to either the treatment group (leflunomide 50 mg/week, LEF50) or the control group (leflunomide 10 mg/day, LEF10). All patients were treated for 24 weeks. Clinical efficacy was assessed using the disease activity score in 28 joints (DAS28) - erythrocyte sedimentation rate (ESR) and European League Against Rheumatism (EULAR) response. A Chi-squared test, Fisher's exact-test and paired t-tests were used to analyze the data. RESULTS: A total of 244 patients who met the inclusion criteria and received at least one medicine dose were analyzed. At the baseline, the DAS28 (ESR) of the ERA patients were 4.41 ± 0.69 in LEF 50 group and 4.52 ± 0.64 in LEF 10 group, respectively. At week 24, the DAS28 (ESR) in two groups ( 2.94 ± 1.10 and 3.02 ± 1.14 ) were significant decreased compare with the baseline, respectively (P<0.01). There was no significant difference in DAS28 (ESR) between the LEF50 and LEF10 groups at week 24. (P > 0.05). At weeks 8, 12 and 24, the EULAR response (good responses + moderate responses) were 47.6%, 58.7% and 59.5%, in the LEF50 group and 43.2%, 49.1% and 53.4% in the LEF10 group, respectively. There was no significant different of EULAR response rates in the two groups at week 8, 12, and 24, respectively (P>0.05). There was no serious adverse events during the study. CONCLUSION: A weekly dose of 50 mg leflunomide showed similar benefits to a daily dose of 10 mg leflunomide for the treatment of mild-to-moderate early rheumatoid arthritis.

[Clinical analysis of 31 ankylosing spondylitis patients with malignancies].

OBJECTIVE: To investigate the clinical features of patients with ankylosing spondylitis (AS) and malignancies. METHODS: In the study, 31 AS patients with malignancies in Peking University First Hospital from January 2006 to July 2014 were enrolled, and their clinical features were compared with AS patients without malignancies at the same time in the hospital. Then their general characteristics, clinical and imaging findings of AS, characteristics of malignancies and prognosis were retrospectively analyzed. RESULTS: The 31 cases of AS with malignacies accounted for 11.8% of all AS patients admitted to the hospital in the same period. They comprised of 27 males (87%) and 4 females (13%). Their mean ages at diagnosis of AS were 43±17 years (range: 16-76 years), and their mean ages at diagnosis of malignancy were 60±12 years (range: 31-87 years) respectively. Malignancy was diagnosed after the establishment of AS in 27 of our patients. Furthermore, imaging changes typical of AS were found in all the 31 patients at the diagnosis of AS, but the majority of them had never received any proper treatment for AS. The malignancies of 31 AS patients included bladder cancer (7 cases), hematological malignancy (6 cases), lung cancer (5 cases), renal cancer (5 cases), prostate cancer (2 cases), carcinoma of renal pelvis (2 cases), breast cancer (2 cases), gastric carcinoma (2 cases), rectal cancer (2 cases), neuroendocrine carcinoma (2 cases), colon carcinoma (1 case), and esophagus cancer (1 case). CONCLUSION: There is risk of malignancy in AS. Malignancy is not rare in patients with AS. Bladder cancer is the most common one followed by hematological malignancy. In consequence, close attention should be paied to the conditon of AS with malignancy in clinical practice.

[Risk factors for bone mineral density changes in patients with rheumatoid arthritis and fracture risk assessment].

OBJECTIVE: To verify the fracture risk assessment tool (FRAX) to estimate the probability of osteoporotic fracture in patients with rheumatoid arthritis (RA) with or without bone mineral density (BMD), and identify associated risk factors of osteoporosis. METHODS: In the study, 200 patients with rheumatoid arthritis aged more than 40 years in Peking University First Hospital from Dec. 2009 to Dec. 2012 were recruited. Clinical information was obtained from a questionnaire of their case history and medical records. FRAX tool was administered. Their lumber spine and left femoral BMD were determined by dual energy X ray absorptiometry. The gender, age, disease duration, menopause status, body mass index (BMI) and accumulative dose of glucocorticoid were obtained in retrospect. Correlation analysis was conducted between the BMD and clinical information. RESULTS: The study population (female, 77.5%) had a mean age of 59.4 years, in which 10 (13%) patients showed a normal BMD, 67 (87%) were osteopenia or osteoporosis, while 32 patients (16%) had fragile fracture. Compared with the patients with normal BMD, the subjects with low BMD had significantly older age, longer period for corticoids usage, higher day dose and accumulated dose of corticoids.The 10-year fracture risk of sustaining major osteoporotic fractures and hip fracture was higher. No significant difference was observed between the 10-year fracture risks calculated with BMD and without BMD. The values of the different area under the receiver operating characteristic (ROC) curve (AUC) for major and hip fractures calculated in three ways: without BMD, with the femoral neck BMD, and with T-score. The best result was for FRAX tool for hip fracture with the T-score (AUC 0.899). A stepwise multivariate linear regression model was constructed to explore the relationship between the different clinical factors studied and a low BMD. Three statistically significant variables for lumber BMD were pain on visual assessment scale (VAS) (P=0.02), fracture history (P=0.003) and a higher steroid accumulated dose (P=0.008). Three statistically significant variables for left hip BMD were age (P<0.001), fracture history (P=0.05) and lower BMI (P=0.03). CONCLUSION: Low BMD is a common complication in RA patients. Risk factors for major fracture and hip fracture are increased. There is a positive correlation between FRAX calculated with and without BMD or T score. FRAX with the femoral neck T score or BMD presents a discriminatory capacity better than FRAX without BMD, according to the AUC ROC.

[Relationship between wrist bone mineral density and synovitis, erosion by ultrasonography in female rheumatoid arthritis patients].

OBJECTIVE: To find the correlation of wrist bone mineral density (BMD) to wrist synovitis and erosion, by comparing wrist BMD and ultrasonography. METHODS: A number of 80 female RA patients were examined by BMD measurement of the femoral neck, spine and non-dominant wrist using dual-energy X-ray absorptiometry (DXA). Synovitis of the wrist was examined by ultrasonography. The wrist joint (radiocarpal joint, dorsal midline, and carpoulnar joint) was assessed in the same side of DXA, with transverse and longitudinal scans for USGS synovial hypertrophy and proliferation, tenosynovitis,tendinitis and bone erosion. Colour and power doppler ultrasonography (PDUS) were used to sum the synovitis score. RESULTS: We found: (1) In the study, 80 female RA patients were enrolled, the mean age was 54.6±13.3 (27.0-80.0) years, the disease duration was 48 (12-116) months, and the body Mass Index was 23.0±4.0 (14.8-31.2) kg/m2. The Wrist BMD (g/cm2) in RA significantly reduced, compared with normal controls(0.297±0.121 vs. 0.420±0.180,P<0.01). (2) The Wrist BMD (g/cm2) exceeded in early RA compared with the established RA (0.326±0.103 vs. 0.285±0.132,P<0.01); the positive rate of severe osteoporosis in wrist was lower in early RA compared with the established RA (47.8% vs. 64.9%, P<0.05); the positive rate of bone erosion in wrist by ultrasound was lower in early RA compared with the established RA (39.1% vs. 56.1%, P<0.01). (3) The wrist BMD (g/cm2) in RA with high disease activity reduced compared with moderate and low disease activity (0.267±0.140 vs. 0.280±0.126) and (0.267±0.140 vs. 0.320±0.103) respectively, P<0.05). The percentages of positive ACPA in the high and moderate disease activity groups were significantly higher than those in the remission group (85% vs. 81.8% and 92.6% vs. 81.8%, respectively). DAS28ESR was correlated with wrist BMD (r=-0.288, P<0.01). (4) A significant positive correlation was found between wrist and spine/femur BMD (r=0.634, P<0.01, r=0.795, P<0.01), and a negative correlation between wrist and disease duration and DAS28ESR (r=-0.286, r=-0.301, P<0.01). There was a highly significant positive correlation between wrist BMD and femur BMD (r=0.95, P<0.05). (5) RA patients in wrist osteoporosis group had higher RF positive rate and ACPA rate than wrist osteopenia group (75.5% vs. 55.6%, P<0.05,100% vs. 83.3%, P<0.05). The patients of BMD osteoporosis group had higher DAS28ESR compared with osteopenia group (5.3±1.8 vs. 3.7±1.5, P<0.01). The percentages of synovitis (61.5% vs. 51.7%, P<0.05), tendenitis (14.3% vs. 10.0%, P<0.05) and bone erosion (54.2% vs. 46.2%, P<0.05) in wrist by ultrasonography in osteoporosis group were higher than those of osteopenia group. (6) The wrist BMD in negative bone erosion group by ultrasonography was lower than that in positive bone erosion group [(0.333±0.107) g/cm2 vs. (0.264±0.125) g/cm2, P<0.01], also the PDUS score was higher than positive bone erosion group (4.53±1.40 vs. 2.55±2.66, P<0.01). Compared with negative bone erosion group, the patients in positive bone erosion group had longer disease duration (96.0±104.7) months vs. (66.2±78.0) months, P<0.05), higher percentage of RF (81.0% vs. 53.8%, P<0.01), ACPA (92.7% vs. 79.5%, P<0.05). and higher DAS28ESR (5.4±1.8 vs. 4.2±2.0, P<0.05). The percentage of wrist synovitis in positive bone erosion group was higher (75.6% vs. 30.8%, P<0.01) than that of negative bone erosion group, and moreover, the percentage of severe osteoporosis in the wrist was significantly higher (75.0% vs. 46.4%, P<0.01). (7) A stepwise multivariate linear regression model was constructed to explore the relationship between the different clinical factors studied and a low wrist BMD. Statistically significant variables were age (P=0.001), disease duration (P=0.017), DAS28ESR (P=0.021), and ACPA (P=0.05). CONCLUSION: This study shows a highly significant correlation between hand BMD with disease duration and disease activity, and female RA patients with high titer of ACPA have lower wrist BMD.

Primary Sjögren syndrome in Han Chinese: clinical and immunological characteristics of 483 patients.

The epidemiological characteristics of Sjögren syndrome (SS) are significantly varied in different countries. We conducted the present study to survey the epidemiological characteristics of primary SS in China. We recruited 483 primary SS patients from 16 Chinese medical centers nationwide from January 2009 to November 2011 and assessed salivary and lacrimal gland dysfunction, organ involvement, and autoimmunity in these patients. The cohort included 456 women and 27 men (ratio, 17:1; mean age at onset, 42 ±â€Š11 years; median age at diagnosis, 49 years; range, 41-56 years). Male patients showed a lower frequency of xerophthalmia (37.0% vs 60.7%) and a higher frequency of arthritis (40.7% vs 16.4%). Young-onset patients showed a higher frequency of low C3 levels (57.7% vs 36.3%) and pancytopenia (22.2% vs 8.8%). Patients with systemic involvement had a higher frequency of immunoglobulin A (IgA) (39.4% vs 22.5%) and immunoglobulin M (IgM) (12.4% vs 37.9%). Patients with pulmonary involvement had a higher parotid enlargement (21.4% vs 10.2%), purpura (12.1% vs 5.7%) and higher anti-La/SS-B (61.7% vs 41.8%), immunoglobulin G (IgG) (80.7% vs 64.6%) and IgA (48.9% vs 30.6%) levels. Patients with anti-Ro/SSA antibodies had more frequent exocrine gland symptoms and some extraglandular symptoms and immunological alterations. Compared with previous studies performed in other countries, SS patients in China showed particular clinical manifestation, systemic involvement, and immunological alterations.

Remission of rheumatoid arthritis and potential determinants: a national multi-center cross-sectional survey.

The aim of this study is to investigate the remission rate of rheumatoid arthritis (RA) in China and identify its potential determinants. A multi-center cross-sectional study was conducted from July 2009 to January 2012. Data were collected by face-to-face interviews of the rheumatology outpatients in 28 tertiary hospitals in China. The remission rates were calculated in 486 RA patients according to different definitions of remission: the Disease Activity Score in 28 joints (DAS28), the Simplified Disease Activity Index (SDAI), the Clinical Disease Activity Index (CDAI), and the American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) Boolean definition. Potential determinants of RA remission were assessed by univariate and multivariate analyses. The remission rates of RA from this multi-center cohort were 8.6% (DAS28), 8.4% (SDAI), 8.2% (CDAI), and 6.8% (Boolean), respectively. Favorable factors associated with remission were: low Health Assessment Questionnaire (HAQ) score, absence of rheumatoid factor (RF) and anti-cyclic citrullinated peptide (anti-CCP), and treatment of methotrexate (MTX) and hydroxychloroquine (HCQ). Younger age was also predictive for the DAS28 and the Boolean remission. Multivariate analyses revealed a low HAQ score, the absence of anti-CCP, and the treatment with HCQ as independent determinants of remission. The clinical remission rate of RA patients was low in China. A low HAQ score, the absence of anti-CCP, and HCQ were significant independent determinants for RA remission.

Connective tissue disease-associated pulmonary arterial hypertension in Chinese patients.

We sought to investigate the characteristics, survival and risk factors for mortality in Chinese patients with connective tissue disease (CTD)-associated pulmonary arterial hypertension (APAH) in modern therapy era. 129 consecutive adult patients who visited one of three referral centres in China with a diagnosis of CTD-APAH confirmed by right heart catheterisation during the previous 5 years were enrolled. The end-point was all-cause death or data censoring. Systemic lupus erythematosus was the most common underlying CTD (49%) and systemic sclerosis just accounted for 6% in this cohort. The overall survival at 1 and 3 years was 92% and 80%, respectively. Pericardial effusion, a shorter 6-min walk distance, lower mixed venous oxygen saturation, higher pulmonary vascular resistance (PVR) and alkaline phosphatase (ALP), and lower total cholesterol levels were all associated with a higher risk of death among the study population. Higher PVR and ALP were independent predictors of mortality. In conclusion, unlike in western patients, systemic lupus erythematosus is the most common underlying disease in Chinese patients with CTD-APAH. The survival of Chinese patients with CTD-APAH in the modern treatment era is similar to that in western countries. Elevated PVR and ALP are independent risk factors for poor outcomes.

Shunting branch of portal vein and stent position predict survival after transjugular intrahepatic portosystemic shunt.

AIM: To evaluate the effect of the shunting branch of the portal vein (PV) (left or right) and the initial stent position (optimal or suboptimal) of a transjugular intrahepatic portosystemic shunt (TIPS). METHODS: We retrospectively reviewed 307 consecutive cirrhotic patients who underwent TIPS placement for variceal bleeding from March 2001 to July 2010 at our center. The left PV was used in 221 patients and the right PV in the remaining 86 patients. And, 224 and 83 patients have optimal stent position and sub-optimal stent positions, respectively. The patients were followed until October 2011 or their death. Hepatic encephalopathy, shunt dysfunction, and survival were evaluated as outcomes. The difference between the groups was compared by Kaplan-Meier analysis. A Cox regression model was employed to evaluate the predictors. RESULTS: Among the patients who underwent TIPS to the left PV, the risk of hepatic encephalopathy (P = 0.002) and mortality were lower (P < 0.001) compared to those to the right PV. Patients who underwent TIPS with optimal initial stent position had a higher primary patency (P < 0.001) and better survival (P = 0.006) than those with suboptimal initial stent position. The shunting branch of the portal vein and the initial stent position were independent predictors of hepatic encephalopathy and shunt dysfunction after TIPS, respectively. And, both were independent predictors of survival. CONCLUSION: TIPS placed to the left portal vein with optimal stent position may reduce the risk of hepatic encephalopathy and improve the primary patency rates, thereby prolonging survival.