Publications by authors named "Mengtao Li"

162 Publications

Clinical features of central nervous system involvement in patients with eosinophilic granulomatosis with polyangiitis: a retrospective cohort study in China.

Orphanet J Rare Dis 2021 Mar 31;16(1):152. Epub 2021 Mar 31.

Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, the Ministry of Education Key Laboratory, National Clinical Research Center for Dermatologic and Immunologic Diseases, Beijing, 100730, China.

Background: Central nervous system (CNS) involvement is extremely rare in eosinophilic granulomatosis with polyangiitis (EGPA), but is associated with a poor prognosis in the five-factor score. This study aims to elucidate the clinical features and independently associated factors of EGPA with CNS involvement.

Results: CNS involvement was observed in 17.3% (19/110) of EGPA patients from Peking Union Medical College Hospital between 2007 and 2019. We retrospectively reviewed their clinical data and analyzed the independently associated factors. Their mean age was 51.7 ± 11.56 years with no male/female predominance. Ischemic lesions were the most common manifestations, accounting for 63.2% of the 19 cases, followed by posterior reversible encephalopathy syndrome (36.8%), spinal cord involvement (15.8%), medulla oblongata involvement (15.8%), and intracranial hemorrhages (15.8%). Compared to the control group, patients with CNS involvement were of older age (51.7 ± 11.56 vs. 43.7 ± 13.78 years, p = 0.019) and had a higher ratio in the digestive tract involvement (52.6% vs. 28.6%, p = 0.042). Further multivariate analysis revealed that age, disease duration, and fever were the potential independent risk factors for CNS involvement of EGPA. Glucocorticoids combined with cyclophosphamide were the strategic therapy (94.7%). Intrathecal injections of dexamethasone and methotrexate were administered to 21.1% of the patients. Although seven patients relapsed during glucocorticoid reduction, seventeen patients finally achieved clinical remission. One patient died of acute intracerebral hemorrhage within one month, and another died of gastrointestinal perforation. Outcomes and cumulative survival show no significant differences between the two groups.

Conclusions: CNS involvement is uncommon in EGPA with various manifestations, and ischemic lesions are the most frequent. Age, disease duration, and fever are independent factors associated with CNS involvement in EGPA. The therapy of glucocorticoids combined with cyclophosphamide and intrathecal injections yields favorable responses. Acute intracranial hemorrhage and gastrointestinal perforation may be the principal causes of death.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1186/s13023-021-01780-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8010942PMC
March 2021

Activation of Toll-Like Receptor 7 Signaling Pathway in Primary Sjögren's Syndrome-Associated Thrombocytopenia.

Front Immunol 2021 9;12:637659. Epub 2021 Mar 9.

Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China.

To identify the importance of the Toll-like receptor (TLR) pathway using B cell high-throughput sequencing and to explore the participation of the TLR7 signaling pathway in primary Sjogren's syndrome (pSS)-associated thrombocytopenia in patient and mouse models. High-throughput gene sequencing and bioinformatic analyses were performed for 9 patients: 3 patients with pSS and normal platelet counts, 3 patients with pSS-associated thrombocytopenia, and 3 healthy controls. Twenty-four patients with pSS were recruited for validation. Twenty-four non-obese diabetic (NOD) mice were divided into the TLR7 pathway inhibition (CA-4948), activation (Resiquimod), and control groups. Serum, peripheral blood, bone marrow, and submandibular glands were collected for thrombocytopenia and TLR7 pathway analysis. Seven hub genes enriched in the TLR pathway were identified. Compared to that in control patients, the expression of interleukin (IL)-8 and TLR7 pathway molecules in B-cells was higher in patients with pSS-associated thrombocytopenia. Platelet counts exhibited a negative correlation with serum IL-1β and IL-8 levels. In NOD mice, CA-4948/Resiquimod treatment induced the downregulation/upregulation of the TLR7 pathway, leading to consistent elevation/reduction of platelet counts. Megakaryocyte counts in the bone marrow showed an increasing trend in the Resiquimod group, with more naked nuclei. The levels of IL-1β and IL-8 in the serum and submandibular gland tissue increased in the Resiquimod group compared with that in CA-4948 and control groups. pSS-associated thrombocytopenia may be a subset of the systemic inflammatory state as the TLR7 signaling pathway was upregulated in B cells of patients with pSS-associated thrombocytopenia, and activation of the TLR7 pathway led to a thrombocytopenia phenotype in NOD mice.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.3389/fimmu.2021.637659DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7986855PMC
March 2021

Validation of the REVEAL Prognostic Models in Systemic Lupus Erythematosus-Associated Pulmonary Arterial Hypertension.

Front Med (Lausanne) 2021 4;8:618486. Epub 2021 Mar 4.

Department of Epidemiology and Bio-Statistics, Institute of Basic Medical Sciences, China Academy of Medical Sciences and Peking Union Medical College, Beijing, China.

No previous studies have investigated the predictive performance of the Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management (REVEAL) prognostic equation and simplified risk score calculator in patients with systemic lupus erythematosus-associated pulmonary arterial hypertension (SLE-PAH). We aimed to validate these prediction tools in an external cohort of patients with SLE-PAH. In this study, the validation cohort consisted of patients with SLE-PAH registered in a prospective, multicenter, nationwide database between November 2006 and May2016. The follow-up of patients was censored at 1 year. Discrimination, calibration, model fit, and risk stratification of the REVEAL prognostic equation and simplified risk score calculator were validated. As a result, a total of 306 patients with SLE-PAH were included. The 1-year overall survival rate was 91.5%. The C-index of the prognostic equation was 0.736, demonstrating reasonably good discrimination, and it was greater than that for the simplified risk score calculator (0.710). The overall calibration slope was 0.83, and the Brier score was 0.079. The risk of renal insufficiency and World Health Organization Functional Class III (WHO FC III) were underestimated, and the risk assigned to a heart rate >92 bpm in the REVEAL prognostic models was not observed in our validation cohort. Both model discrimination and calibration were poor in the very high-risk group. In conclusion, the REVEAL models exhibit good discriminatory ability when predicting 1-year overall survival in patients with SLE-PAH. Findings from both models should be interpreted with caution in cases of renal insufficiency, WHO FC III, and heart rate >92 bpm.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.3389/fmed.2021.618486DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7969505PMC
March 2021

Clinical features and long-term outcomes of interstitial lung disease with anti-neutrophil cytoplasmic antibody.

BMC Pulm Med 2021 Mar 16;21(1):88. Epub 2021 Mar 16.

Department of Respiratory and Critical Care Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, No. 1 Shuai Fu Yuan, Dongcheng District, Beijing, 100730, China.

Background: Patients with interstitial lung disease (ILD) are occasionally positive for anti-neutrophil cytoplasmic antibodies (ANCAs). Differences between ILDs secondary to microscopic polyangiitis (MPA) and isolated ANCA-positive idiopathic interstitial pneumonia (IIP) remain unclear. The aim of this study was to explore the differences in clinical features and outcomes between MPA-associated ILDs and isolated ANCA-positive IIPs.

Methods: We reviewed 1338 ILDs patients with available ANCA results and retrospectively analysed 80 patients who were ANCA-positive. MPA-associated ILDs (MPA-ILDs group) and isolated ANCA-positive IIPs (ANCA-IIPs group) were compared.

Results: Among 80 patients with ANCA-positive ILDs, 31 (38.75%) had MPA-ILDs, and 49 (61.25%) had isolated ANCA-positive IIPs. Compared with ANCA-IIPs group, patients in MPA-ILDs group had a higher proportion of fever (p = 0.006) and higher neutrophil count (p = 0.011), erythrocyte sedimentation rate (ESR) (p < 0.001) and C-reactive protein (CRP) (p = 0.005). Multivariable analysis showed that ESR level was an independent risk factor for mortality in all 80 ANCA-positive ILDs patients (HR 1.028, p = 0.001). Survival in MPA-ILDs group was lower than that in ANCA-IIPs group, and further stratified analysis revealed that ANCA-IIPs patients with elevated ESR or CRP had a worse prognosis than those with normal inflammation markers, with 5-year cumulative survival rates of 60.00%, 86.90% and 100.00% in MPA-ILDs and ANCA-IIPs with and without elevated inflammation markers, respectively.

Conclusions: Among patients with ANCA-positive ILDs, the prognoses of ANCA-IIPs with normal inflammation markers, ANCA-IIPs with elevated inflammation markers and MPA-ILDs were sequentially poorer. Therefore, stratified treatment should be considered in the management of ILDs patients positive for ANCAs.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1186/s12890-021-01451-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7968287PMC
March 2021

Renal Involvement and HBV Infection Are Common in Chinese Patients With Cryoglobulinemia.

Front Immunol 2021 25;12:580271. Epub 2021 Feb 25.

Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Science, Beijing, China.

This study aimed to describe the main characteristics of Chinese patients with cryoglobulinemia, especially the characteristics of patients with different causes of cryoglobulinemia. Eighty inpatients diagnosed with cryoglobulinemia from different wards in Peking Union Medical College Hospital were included in this study. Demographic, clinical, biological, and renal pathological data were collected. We analyzed the characteristics of 61 patients with different causes of cryoglobulinemia. Most patients (36/80, 45%) were diagnosed between 40 and 60 years of age. The male: female ratio was 1:1.5. Mixed (II + III) cryoglobulinemia accounted for the majority (43.8%) of cases. Renal involvement (87.5%), cutaneous involvement (57.5%), and fever (27.5%) were the most common clinical manifestations, while other manifestations included serositis and pulmonary and gastrointestinal involvement. The most common renal histopathological pattern was membranoproliferative glomerulonephritis (25/42, 59.5%). The secondary causes of cryoglobulinemia included infectious diseases (26/61, 32.5%), such as hepatitis B virus (HBV) and hepatitis C virus (HCV) infections, and connective tissue diseases (22/61, 27.5%), such as lupus and hematologic tumors (13/61, 16.3%). Patients with hematologic tumors were diagnosed at an older age ( = 0.044) and mostly had type I cryoglobulinemia ( < 0.001). No significant difference in clinical or biological manifestations was found among patients with different causes of cryoglobulinemia. This is the largest cohort of Chinese patients with cryoglobulinemia. We found that renal involvement and HBV infection might be more common in Chinese patients with cryoglobulinemia.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.3389/fimmu.2021.580271DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7947000PMC
February 2021

Rheumatic immune-related adverse events associated with immune checkpoint inhibitors compared with placebo in oncologic patients: a systemic review and meta-analysis.

Ther Adv Chronic Dis 2021 12;12:2040622320976996. Epub 2021 Feb 12.

Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing, China.

Objective: We aim to characterize the incidence and relative risk of rheumatic and systemic immune-related adverse effects (irAEs) among immune checkpoint inhibitor (ICI) therapy compared with those after placebo treatment.

Methods: Randomized clinical trial studies with placebo control with the following keywords were searched from Embase, PubMed, Cochrane databases: immune checkpoint inhibitors, neoplasms, randomized controlled trials, and adverse effects.

Results: Among the 5444 published and 316 registration records, nine placebo-controlled randomized clinical trials met our selection criteria, and included data from 5560 patients. Compared with placebo use, using ICIs increases the risk of overall-rheumatic irAEs. The incidence and relative risk of all-grade rheumatic irAEs were 18.40% [95% confidence interval (CI) 12.16-25.59%,  < 0.01] and 2.30 (95% CI 1.32-4.02), respectively, while musculoskeletal irAEs were 11.30% (95% CI 9.76-12.85%) and 1.01 (95% CI 0.84-1.22). The incidence and relative risk of severe rheumatic irAEs were 5.72% (95% CI 3.92-7.82%), and 8.29 (95% CI 3.75-18.35), respectively. Arthralgia was the most common rheumatic irAE (incidence 11.00%, 95% CI 9.55-12.64%; relative risk 0.99, 95% CI 0.82-1.19), although usually not severe. Colitis (incidence 3.23%, 95% CI 1.27-7.98%; relative risk 6.53, 95% CI 2.66-16.04) and pneumonitis (incidence 3.11%, 95% CI 1.56-6.21; relative risk 4.04, 95% CI 1.65-9.89) were commonly observed and tended to be severe. Hepatitis, hypophysitis, thyroiditis, and myositis were rare and less recorded, yet can be severe and life threatening. Other extremely rare severe rheumatic irAEs included sarcoidosis ( = 11), autoimmune arthritis ( = 8), autoimmune uveitis ( = 3), autoimmune pericarditis, bursitis, osteochondrosis, psoriasis, polymyalgia rheumatica, systemic inflammatory response syndrome, and Sjögren syndrome ( = 1, each).

Conclusion: ICI therapy increased the incidence and relative risk of all-grade and severe rheumatic irAEs. Arthralgia was the most commonly observed non-severe irAE, while colitis and pneumonitis were commonly observed severe irAEs. Rare rheumatic irAEs like hepatitis, hypophysitis, thyroiditis, and myositis warrant special attention.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1177/2040622320976996DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7887685PMC
February 2021

Plasticity of Treg and imbalance of Treg/Th17 cells in patients with systemic sclerosis modified by FK506.

Int J Immunopathol Pharmacol 2021 Jan-Dec;35:2058738421998086

Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China.

To determine the effects of Tacrolimus (FK506) on Treg cells and subpopulations in SSc patients and assess the ability of FK506 to modify the immune imbalance of Treg/Th17 cells. We analyzed PBMC from five SSc patients and six healthy control by flow cytometry after cultured with 0, 0.1, 1, or 10 ng/ml FK506 in vitro. The number of Treg cells decreased in SSc patients treated with FK506. The number of FrI cells were decreased in SSc following FK506 treatment. The drug did increase the frequency of FrII/Treg cells, but not FrII cells. However, FK506 significantly decreased FrIII in both SSc patients and controls. FK506 clearly decreased the numbers of Th17 cells and FoxP3IL-17 cells. The proliferation capacity of cells was also inhibited by FK506, which had a greater effect on FoxP3 cells than FoxP3 cells. FK506 did inhibit the proliferation of FrIII cells, but not FrI or FrII cells. Our study provides that FK506 reduced the number of FoxP3 CD45RA T cells (FrIII) by inhibiting its proliferation. Therefore, FK506 modifies Treg cells and the immune imbalance between Tregs and Th17 cells in SSc patients.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1177/2058738421998086DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7917869PMC
February 2021

Early Initiation of Anticoagulation Improves the Long-Term Prognosis in Patients With Antiphospholipid Syndrome Associated Portal Vein Thrombosis.

Front Med (Lausanne) 2021 4;8:630660. Epub 2021 Feb 4.

Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Science, National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science & Technology, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China.

Portal vein thrombosis (PVT) is a rare and severe clinical phenotype of antiphospholipid syndrome (APS) with a poor prognosis. Anticoagulation therapy is efficient but is associated with potentially severe bleeding episodes, especially for those patients with thrombocytopenia. We conducted this case-control study to explore the clinical features and associated factors of PVT in APS patients, the re-canalization rate of the PVT after anticoagulation and investigate the beneficial effects of early initiation of anticoagulation in patients with APS associated PVT. We enrolled patients with APS associated PVT as the case group, and age-, and entry-time-matched APS patients without PVT (1:2) as the control group. We explored the associated factors of PVT in APS patients using multivariate logistic regression analysis. The re-canalization rate of the PVT after anticoagulation was analyzed using the survival analysis. A total of 34 patients (8 males and 26 females) with APS-PVT were enrolled, with a median follow-up time of 3 years (1.5, 7 years). Multivariate logistic regression analysis showed that thrombocytopenia (OR 6.4, 95%CI 1.561-26.218, = 0.01), hypersensitive c-reactive protein >3 mg/L (OR 4.57, 95%CI 1.426-14.666, = 0.011), anti β2GPI positive (OR 5, 95%CI 1.816-13.772, = 0.002) and aPL double-positive (OR 4.08, 95%CI 1.312-12.429, = 0.013) were independent associated factors for PVT in APS. Survival analysis revealed that effective anticoagulation could increase re-canalization rate significantly (log-rank = 0.001), with better prognosis (lower mortality rate, log-rank = 0.045). PVT could be the first presentation of APS with insidious onset and atypical clinical symptoms and easily be misdiagnosed. For patients with APS, double aPLs positive, thrombocytopenia, and inflammation could be the associated factors of PVT. Early diagnosis and anticoagulation treatment can bring thrombus re-canalization thereby significantly improving the prognosis.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.3389/fmed.2021.630660DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7890015PMC
February 2021

Cardiac manifestations of eosinophilic granulomatosis with polyangiitis from a single-center cohort in China: clinical features and associated factors.

Ther Adv Chronic Dis 2021 22;12:2040622320987051. Epub 2021 Jan 22.

Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, The Ministry of Education Key Laboratory, National Clinical Research Center for Dermatologic and Immunologic Diseases, Beijing 100730, China.

Background: Cardiac manifestations are common and life-threatening in eosinophilic granulomatosis with polyangiitis (EGPA), which remains poorly studied in China. We aim to investigate its clinical features, associated factors, treatment, and outcomes.

Methods: We reviewed the clinical records of 110 EGPA patients and examined the independent factors associated with cardiac manifestations using multivariate logistic regression. Receiver operating characteristic curves determined the cut-off values, and survival was calculated Kaplan-Meier curves.

Results: Cardiac involvement was present in 36.4% (40/110) of EGPA patients, which mainly manifested as pericardial effusion (16.4%, 18/110), myocardial involvement (13.6%, 15/110), and heart failure (8.2%, 9/110). The mean age was 42.1 ± 14.23 years with no female/male predominance. Compared with the cardiac-unaffected group, the cardiac-affected group showed a lower rate of biopsy-proved vasculitis (0% 20%,  = 0.002). The eosinophil count [odds ratio (OR) = 1.142, 95% confidence interval (CI) 1.029-1.267] was independently associated with cardiac manifestations in EGPA, with a cut-off value of 3.66 × 10/L [area under the curve (AUC) = 0.692,  = 0.001]. Regarding treatment, the cardiac-affected group displayed a higher ratio of glucocorticoid pulse combined with intravenous cyclophosphamide (CYC-IV) (40% 21.4%,  = 0.037), and intravenous immunoglobulin combined with glucocorticoid plus CYC-IV (17.5% 4.3%,  = 0.035) than the control group. Outcomes ( = 0.131) and survival ( = 0.1972) were not significantly different between the groups.

Conclusion: In this single-center Chinese EGPA cohort, cardiac manifestations are observed in 36.4% of patients, which primarily presents as myocardial involvement, pericardial effusion, and heart failure, independently associated with eosinophil count. Glucocorticoid combined with cyclophosphamide is the treatment cornerstone for EGPA patients with cardiac manifestations.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1177/2040622320987051DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7841702PMC
January 2021

Clinical Features and Outcomes of Neuropsychiatric Systemic Lupus Erythematosus in China.

J Immunol Res 2021 18;2021:1349042. Epub 2021 Jan 18.

Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science & Technology, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, No. 1 Shuaifuyuan, Beijing 100730, China.

Objective: To identify the clinical characteristics, magnetic resonance imaging (MRI) results, and prognostic factors of neuropsychiatric (NP) systemic lupus erythematosus (SLE; NPSLE) in a relatively large patient series in China.

Methods: Data of patients with NPSLE at Peking Union Medical College Hospital (PUMCH) were collected retrospectively from June 2012 to June 2016. NPSLE patients were compared with 220 non-NPSLE patients. Survival rates were evaluated using the Kaplan-Meier curves, log-rank test, and Cox proportional hazards modeling. Cranial MRI results were also studied.

Results: Of the 194 included patients, sixteen subtypes of NPSLE were identified, and the most common manifestations were seizure (36.6%), acute confusional state (25.3%), and cerebral vascular disease (15.5%). Compared with the non-NPSLE group, NPSLE patients were significantly more likely to have typical lupus symptoms, higher Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) scores ( = 0.002), and positive rate of anti-ribosomal P protein antibodies ( = 0.008). Patients with seizure were more likely to have higher SLEDAI-2K scores and positive anti-2GP1 than non-NPSLE patients. Sixteen patients died during follow-up. The most common cause of death was infection (37.5%). NPSLE significantly decreased survival rates of SLE patients. Patients with elevated serum creatinine ( = 0.001), hypocomplementemia ( = 0.031), and SLEDAI - 2K scores ≥ 15 ( = 0.014) had shorter survival periods. Eighty-two patients underwent detailed cranial MRI analysis; of these, 50 (61.0%) had abnormal results. Small vessel disease was the most common abnormal finding, followed by inflammatory-like lesions and large vessel disease.

Conclusions: High disease activity and positive rate of anti-ribosomal P protein antibodies may be risk factors for NPSLE. NPSLE decreases survival rates of SLE patients. Renal insufficiency and high disease activity are predictive of poor prognoses for NPSLE patients.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1155/2021/1349042DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7834780PMC
January 2021

MicroRNA-320a: an important regulator in the fibrotic process in interstitial lung disease of systemic sclerosis.

Arthritis Res Ther 2021 01 11;23(1):21. Epub 2021 Jan 11.

Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China.

Background: Systemic sclerosis (SSc) is an acquired autoimmune disorder characterized by excessive accumulation of collagen and progressive tissue fibrosis. Although interstitial lung disease (ILD) complicates the majority of SSc patients and is the leading cause of death, its pathogenesis remains largely unclear. In the current study, we aimed to evaluate the role of microRNAs in SSc-ILD.

Methods: miRNA expression patterns were assessed by miRNA array and real-time PCR from serum and PBMCs of SSc-ILD patients and healthy controls. Bleomycin-induced SSc-ILD mouse model was used to verify the miRNA expression in the lung tissue. The function of miRNAs in pulmonary fibroblasts was assessed using miRNA inhibitors, and mimics.

Results: miR-320a was significantly downregulated in both SSc-ILD patients and mouse models. The inhibition or overexpression of miR-320a in human pulmonary fibroblasts significantly affected the protein expression of type I collagen. Luciferase reporter assay, RT-PCR, and western blot analysis identified TGFBR2 and IGF1R as direct targets of miR-320a. Upon TGF-β stimulation, the expression of miR-320a and collagen genes were significantly upregulated.

Conclusion: miR-320a, together with its target genes, TGFBR2 and IGF1R, constituted a complex regulatory network, and played an important role in the fibrotic process of SSc-ILD. Investigation of more detailed mechanisms of miR-320a-mediated regulation of collagen expression may provide new therapeutic strategies for SSc-ILD.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1186/s13075-020-02411-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7802184PMC
January 2021

Chinese SLE Treatment and Research Group Registry (CSTAR) XIII: prevalence and risk factors for chronic scarring alopecia in patients with systemic lupus erythematosus.

Arthritis Res Ther 2021 01 11;23(1):20. Epub 2021 Jan 11.

Department of Rheumatology, State Key Laboratory of Complex Severe and Rare Diseases, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Key Laboratory of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, No. 1 Shuaifuyuan, Wangfujing Ave, Beijing, 100730, China.

Background: Scarring alopecia in systemic lupus erythematosus (SLE) patients caused reduced life quality and prolonged disease course. This case-control study aims to survey the prevalence of scarring alopecia during the disease course of SLE and evaluate the risk factors for scarring alopecia in Chinese SLE patients.

Methods: SLE patients in Chinese SLE treatment and Research group (CSTAR) were recruited. Scarring alopecia was defined according to SLICC/ACR-DI which was collected during follow-up visits or via self-reported questionnaires. We collected demographic characteristics, common comorbidities, autoantibody profiles, disease activity status, major organ involvements, and treatment strategies of these patients at registry. Univariate and multivariate logistic regression analyses were used to investigate the risk factors for scarring alopecia.

Results: We recruited 4792 SLE patients, and 374 (7.80%) patients had scarring alopecia. Mucocutaneous lesions (OR 2.062, p < 0.001), high SLICC/ACR-DI (OR 1.409, p < 0.001), and positive anti-Sm (OR 1.374, p = 0.029) were risk factors for scarring alopecia, while renal (OR 0.714, p = 0.028) and cardio-respiratory involvements (OR 0.347, p = 0.044), and immunosuppressant treatment (OR 0.675, p < 0.001) were significantly negative associated with it.

Conclusions: The prevalence of scarring alopecia in SLE patients is 7.80%. Active treatment strategies should be adopted to prevent scarring alopecia occurring.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1186/s13075-020-02407-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7802218PMC
January 2021

Comparison of Clinical Features in HLA-B27 Positive and Negative Patients With Axial Spondyloarthritis: Results From a Cohort of 4,131 Patients.

Front Med (Lausanne) 2020 23;7:609562. Epub 2020 Dec 23.

Department of Rheumatology, Xinxiang Central Hospital, Xinxiang, China.

The aim of our study was to assess the influence of the HLA-B27 status on axial spondyloarthritis (axSpA) in the largest cohort in China. An observational, cross-sectional, and analytic study of axSpA patients from the China axSpA database was performed. Demographic and clinical data were compared in terms of the HLA-B27 status. Univariate and multivariate analyses were performed to identify variables related to HLA-B27 presence. We enrolled 4,131 patients in this study; of those, 36,95 (89.4%) were HLA-B27 positive. In the multivariate analysis, male gender ( < 0.001), younger age ( < 0.001), a disease duration of more than 3 years ( < 0.001), a family history of SpA ( < 0.001), uveitis ( < 0.001), ASDAS-CRP ( < 0.001), and biologic treatment ( < 0.001) were the main variables that were independently related to HLA-B27 presence, whereas a diagnosis delay time >36 months ( < 0.001) and psoriasis ( < 0.001) were independently related to HLA-B27 absence. In Chinese axial SpA patients, presence of HLA-B27 is associated with the male sex, younger age, longer disease duration, greater family aggregation, and higher frequency of uveitis; absence of HLA-B27 is associated with longer diagnosis delay time and higher frequency of psoriasis.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.3389/fmed.2020.609562DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7785867PMC
December 2020

Tocilizumab for Refractory Rapidly Progressive Interstitial Lung Disease Related to Anti-MDA 5-positive Myositis.

Rheumatology (Oxford) 2021 Jan 7. Epub 2021 Jan 7.

Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College &Chinese Academy of Medical Sciences, ; National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID); Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1093/rheumatology/keaa906DOI Listing
January 2021

Changes in Efficacy Indicators for Adalimumab Biosimilar Candidate (HS016) for the Treatment of Active Ankylosing Spondylitis at Various Time Points.

Front Pharmacol 2020 7;11:606497. Epub 2020 Dec 7.

Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, National Clinical Research Center for Immunologic Diseases, Ministry of Science and Technology, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China.

A phase III, 24-weeks Chinese clinical trial demonstrated that efficacy and safety outcomes of treatments with 40 mg/0.8 ml HS016 ( = 416) or adalimumab ( = 232) for active ankylosing spondylitis (AS) patients was comparable. In the present study, a subanalysis of the clinical trial was conducted to determine whether also individual efficacy indicators were comparable between HS016 and adalimumab. The individual efficacy indicators total and nocturnal back pain, global assessment of disease activity, swollen joint count, Maastricht AS Enthesitis Score, Bath AS Disease Activity Index, Bath AS Functional Index, Bath AS Metrology Index and chest expansion, were assessed at baseline and every 2 weeks during the treatment period. This subanalysis revealed no significant difference between the patient groups treated with HS016 or adalimumab for any individual efficacy indicator investigated at any time point (all > 0.05) beside faster total back pain score improvements in the adalimumab group on week 10, 12 and 22, which became equal at week 24. Among these indicators, chest expansion showed a significant increase at each time point compared with baseline, whereas all other efficacy indicators showed significant decreases compared with baseline at each time point (all < 0.05). All efficacy indicators had increased or decreased rapidly by week 2, and the values continued to increase/decrease up to week 12, with subsequent smaller changes thereafter up to week 24 of treatment. The response trajectory of most individual efficacy indicators was comparable between HS016 and adalimumab at each time point during the 24 weeks of the trial. http://www.chictr.org.cn/showproj.aspx?proj=37910, identifier [ChiCTR1900022520].
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.3389/fphar.2020.606497DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7750525PMC
December 2020

Factors Associated With Renal Involvement in Primary Sjögren's Syndrome: A Meta-Analysis.

Front Med (Lausanne) 2020 26;7:614482. Epub 2020 Nov 26.

Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Science, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China.

Renal impairment is a critical complication in primary Sjögren's syndrome (pSS), resulting in chronic renal disease and even death. This meta-analysis was designed to find out the relevant factors of renal involvement in pSS. PubMed, EMBASE, Cochrane Library, Scopus, and Web of Science were systemically searched until August 30, 2019. Studies were selected according to inclusion criteria, and data was extracted by two researchers independently. The Newcastle-Ottawa Scale was applied for quality assessment. Random- and fixed-effects models were used in this meta-analysis based on the result of the heterogeneity test. Meanwhile, a sensitivity analysis was conducted to investigate the cause of heterogeneity. Publication bias was shown in the funnel plot and evaluated further by Begg's and Egger's tests. Of the 9,989 articles identified, five articles enrolling 1,867 pSS patients were included in the final analysis, 533 with and 1,334 without renal involvement. There was no statistical significance in age and gender between these two groups. According to the meta-analysis, anti-SSB antibody, and arthralgia showed a significant association with renal involvement in pSS, the overall odds ratio (OR) values of which were 1.51 (95% CI, 1.16-1.95) and 0.59 (95% CI, 0.46-0.74), respectively. On the other hand, the overall OR values of anti-SSA antibody, rheumatoid factor, dry eyes, and labial salivary gland biopsy were just 0.90 (95% CI, 0.49-1.64), 1.05 (95% CI, 0.59-1.86), 0.60 (95% CI, 0.34-1.06), and 1.38 (95% CI, 0.98-1.95), respectively. The presence of anti-SSB antibody is positively associated with renal involvement in pSS, while arthralgia is inversely associated. Large-scale prospective cohort studies are needed in the future to identify further risk factors.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.3389/fmed.2020.614482DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7726424PMC
November 2020

Association between acute phase reactants, interleukin-6, tumor necrosis factor-α, and disease activity in Takayasu's arteritis patients.

Arthritis Res Ther 2020 12 10;22(1):285. Epub 2020 Dec 10.

Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, 100730, China.

Background: To investigate the association between blood biomarkers and disease activity of Takayasu's arteritis (TAK) in a follow-up cohort.

Methods: Disease activity was assessed by clinical manifestations and repeated vascular Doppler examinations. The association between erythrocyte sedimentation rate (ESR), serum levels of high-sensitive C-reactive protein (hsCRP), interleukin-6(IL-6), and tumor necrosis factor-α (TNFα) and disease activity were analyzed by logistic regression and survival analysis. Kaplan-Meier method was used to estimate the cumulative remission rate curve, log-rank tests for group comparison, and Cox regression for estimating hazard ratios of these parameters for disease activity.

Results: 428 patients were included. 188 patients were in active disease, and 240 patients were in inactive disease at baseline. Elevation of ESR, hsCRP, and IL-6 were associated with active disease at baseline and during follow-up. Cox regression and Kaplan-Meier analysis showed that lower possibility and longer time to remission were associated with elevated ESR (hazard ratio [HR] = 0.32, 80 vs 33 weeks, p < 0.001), hsCRP (HR = 0.45, 70 vs 31 weeks, p < 0.001), and IL-6 (HR = 0.54, 66 vs 34 weeks, p < 0.01) in patients with active disease at baseline, while higher risk and shorter time for relapse were associated with elevated ESR (HR = 2.1, 59 vs 111 weeks, p < 0.001), hsCRP (HR = 2.1, 79 vs 113 weeks, p < 0.001), IL-6 (HR = 2.5, 64 vs 117 weeks, p < 0.001), and TNFα (HR = 2.7, 65 vs 114 weeks, p < 0.001) in patients with inactive disease at baseline.

Conclusions: Elevated ESR, CRP, and IL-6 are associated with active disease, lower possibility, and longer time to achieve disease remission. Elevation of any among ESR, CRP, IL-6, and TNFα is associated with high risk and short time for relapse during follow-up.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1186/s13075-020-02365-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7726865PMC
December 2020

Reduction in SLEDAI is associated with improved arterial stiffness in systemic lupus erythematosus.

Medicine (Baltimore) 2020 Nov;99(47):e23184

Department of Cardiology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing.

Lipid abnormalities are an important cause of premature atherosclerosis in patients with systemic lupus erythematosus (SLE). This longitudinal study investigates the changes in lipid profile and arterial stiffness with SLE disease activity index (SLEDAI) reduction.Fifty one female SLE patients with baseline SLEDAI ≥ 6 and SLEDAI reduction >3 at 1-year follow-up were included. Neutrophil-to-lymphocyte ratio (NLR), erythrocyte sedimentation rate (ESR), high-sensitivity C-reactive protein (hsCRP), total cholesterol (TC), low-density lipoprotein cholesterol (LDL-C), high-density lipoprotein cholesterol (HDL-C), triglyceride (TG), and mean brachial-ankle pulse wave velocity (baPWV) were measured and compared between baseline and 1-year follow-up. Correlations between inflammation biomarkers, SLEDAI, mean baPWV and lipid profile were assessed.We observed significant decreases in ESR, mean baPWV, TG and TC to HDL-C ratio compared with baseline at 1-year follow up, while HDL-C, hsCRP, and NLR were not significantly changed. Significant correlations were found between the reductions in ESR and TG, and SLEDAI and mean baPWV, with adjustment to age, disease duration, blood pressure, and medications (prednisone, immunosuppressants and ARB/ACEI).SLE patients experiencing SLEDAI reductions showed improvements in arterial stiffness. This finding may provide insight into the beneficial effects of reducing SLEDAI on atherosclerosis risk in SLE.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1097/MD.0000000000023184DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7676556PMC
November 2020

Sirolimus for patients with connective tissue disease-related refractory thrombocytopenia: a single-arm, open-label clinical trial.

Rheumatology (Oxford) 2020 Nov 17. Epub 2020 Nov 17.

Department of Rheumatology, Peking Union Medical College Hospital, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Peking Union Medical College and Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Disease (NCRC-DID), Beijing, China.

Objectives: Connective tissue disease-related thrombocytopenia (CTD-TP) is a problematic disorder in clinical practice. Because the first-line therapy of glucocorticoid and/or immunosuppressants is not effective for refractory cases, alternative treatment approaches are urgently needed. The present study investigated the efficacy and safety of sirolimus in refractory CTD-TP patients.

Methods: This single-centre, single-arm, phase II study enrolled 20 refractory CTD-TP patients between September 2017 and September 2018 (registered on ClinicalTrials.gov: NCT03688191). Oral sirolimus administration was dose-adjusted to maintain a therapeutic range of 6-15 ng/ml for 6 months. The primary endpoints were partial and complete remission rates at 6 months.

Results: Twelve (60%) patients achieved the primary end point with a 50% complete remission rate after 6 months. Among the 14 SLE patients, the overall response rate was 71.4%, with a complete remission rate of 64.3%. None of the primary Sjögren's syndrome cases responded to sirolimus. There was no significant difference in baseline clinical characteristics or lymphocyte subpopulations between responders and non-responders. No severe side effect was detected during the study.

Conclusion: Sirolimus is an effective and safe treatment option for refractory CTD-TP patients.

Trial Registration: https://clinicaltrials.gov, NCT03688191.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1093/rheumatology/keaa645DOI Listing
November 2020

Relationship between disease activity, organ damage and health-related quality of life in patients with systemic lupus erythematosus: A systemic review and meta-analysis.

Autoimmun Rev 2021 Jan 22;20(1):102691. Epub 2020 Oct 22.

Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science & Technology, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China. Electronic address:

Objectives: Systemic lupus erythematosus (SLE) is a common systemic autoimmune disease that may lead to considerable physical, psychological, and socioeconomical burden. In previous studies, inconsistent results were reported for the association of disease activity and organ damage with health-related quality of life (HRQoL). This paper aimed to explore the relationship between disease activity, organ damage, and HRQoL measured by SF-36, EQ-5D, LupusQoL, and LupusPRO and investigate whether the correlation is region-specific.

Methods: We systematically searched for studies reporting the association between SLE disease activity, organ damage, and HRQoL in MEDLINE, EMBASE, PsycINFO, World of Science, the Cochrane Library, and CINAHL from inception to December 2019. A meta-analysis and region subgroup analysis were performed with a random-effects model to estimate pooled correlation coefficients and heterogeneity.

Results: Forty articles were included representing of 6079 adult SLE patients. The meta-analysis of SF-36 and LupusPRO studies revealed mild to moderate negative correlations between disease activity and domains of these HRQoL measurements (correlation coefficient r ranging from -0.27 to -0.07). Likewise, negative correlations were found between organ damage and domains of SF-36 and LupusPRO (r ranging from -0.25 to -0.08). The pooled correlation coefficient is relatively higher in physical functioning related domains than mental health. In the region subgroup analysis, disease activity had strong negative correlations with SF-36 domains in African and European SLE patients, while organ damage had the strongest negative correlation with SF-36 domains in Asian SLE patients (p < 0.010).

Conclusion: This study provides the first comprehensive assessment of the relationship between disease activity, organ damage, and four popular HRQoL instruments, which provides useful insight into the target therapy in SLE management.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.autrev.2020.102691DOI Listing
January 2021

Tofacitinib as a possible treatment for skin thickening in diffuse cutaneous systemic sclerosis.

Rheumatology (Oxford) 2020 Nov 14. Epub 2020 Nov 14.

Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Science, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science & Technology, Beijing, China.

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.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1093/rheumatology/keaa613DOI Listing
November 2020

Multimodal photoacoustic/ultrasonic imaging system: a promising imaging method for the evaluation of disease activity in rheumatoid arthritis.

Eur Radiol 2020 Nov 12. Epub 2020 Nov 12.

Department of Ultrasound, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Shuaifuyuan No.1, Dongcheng District, Beijing, 100730, China.

Objectives: We aimed to assess the clinical value of multimodal photoacoustic/ultrasound (PA/US) articular imaging scores, a novel imaging method which can reflect the micro-vessels and oxygenation level of inflamed joints of rheumatoid arthritis (RA).

Methods: Seven small joints were examined by the PA/US imaging system. A 0-3 scoring system was used to semi-quantify the PA and power-Doppler (PD) signals, and the sums of PA and PD scores (PA-sum and PD-sum scores) of the seven joints were calculated. The relative oxygen saturation (SO) values of the inflamed joints were measured and classified into 3 PA+SO patterns. The correlations between the PA/US imaging scores and the disease activity scores were assessed.

Results: Thirty-one patients of RA and a total of 217 joints were examined using the PA/US system. The PA-sum had high positive correlations with the standard clinical scores of RA (DAS28 [ESR] ρ = 0.754, DAS28 [CRP] ρ = 0.796, SDAI ρ = 0.836, CDAI ρ = 0.837, p < 0.001), which were superior to the PD-sum (DAS28 [ESR] ρ = 0.651, DAS28 [CRP] ρ = 0.676, SDAI ρ = 0.716, CDAI ρ = 0.709, p < 0.001). For the patients with high PA-sum scores, significant differences between hypoxia and hyperoxia were identified in pain visual analog score (p = 0.020) and patient's global assessment (p = 0.026). The PA+SO patterns presented moderate and high correlation with PGA (ρ = 0.477, p = 0.0077) and VAS pain score (ρ = 0.717, p < 0.001).

Conclusion: The PA scores have significant correlations with standard clinical scores for RA, and the PA+SO patterns are also related with clinical scores that reflect pain severity. PA may have clinical potential in evaluating RA.

Key Points: • Multimodal photoacoustic/ultrasound imaging is a novel method to assess micro-vessels and oxygenation of local lesions. • Significant correlations between multimodal imaging parameters and clinical scores of RA patients were verified. • The multimodal PA/US system can provide objective imaging parameters, including PA scores of micro-vessels and relative SO value, as a supplementary to disease activity evaluation.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00330-020-07353-zDOI Listing
November 2020

Guidelines for the diagnosis and treatment of osteoarthritis in China (2019 edition).

Ann Transl Med 2020 Oct;8(19):1213

Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, China.

Osteoarthritis (OA) is a degenerative disease of middle-aged and elderly people, contributed a higher burden of disease in China and the world. In 2017, under the support of the Rheumatology and Immunology Expert Committee of the Cross-Strait Medical and Health Exchange Association. The objective was to develop an evidence-based diagnosis and treatment guideline for OA in China based on emerging new evidence. The guideline was registered at International Practice Guidelines Registry Platform (IPGRP-2018CN028). The grading of recommendations assessment, development and evaluation (GRADE) approach was used to rate the quality of evidence and the strength of recommendations, and the RIGHT (Reporting Items for Practice Guidelines in Healthcare) checklist was followed to report the guideline. The guideline provides recommendations for the OA diagnosis, disease risks monitoring and evaluate, treatment purpose and physical, medical and surgical interventions. This guideline is intended to serve as a tool for Chinese clinicians for the best decisions-making on diagnosis and treatment of OA.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.21037/atm-20-4665DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7607097PMC
October 2020

Risk and prognosis factors for systemic sclerosis with lung cancer: A single-centre case-control study in China.

Int J Clin Pract 2021 Apr 20;75(4):e13819. Epub 2020 Nov 20.

Department of Rheumatology and Clinical Immunology of Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China.

Objective: To analyse clinical characteristics, risk and prognosis factors for systemic sclerosis (SSc) patients with lung cancer.

Methods: SSc patients with lung cancer admitted to Peking Union Medical College Hospital from February 1992 to December 2018 were included. Age and sex-matched controls were selected from a pool of SSc patients without lung cancer during the same period. Conditional logistic regression and Cox proportional-hazard regression were used to identify risk factors and prognosis factors. The Kaplan-Meier method was used to draw the survival curve and calculate median survival.

Results: Nineteen SSc patients with lung cancer and 76 controls were included. The mean age at lung cancer diagnosis was 54.4 ± 10.2 years. In all 19 cases the lung cancer had been diagnosed after SSc and the median interval between SSc onset and lung cancer onset was 10.5 years (range 2.0-36.2 years). Among SSc patients with lung cancer, the median follow-up time and median survival were 2.6 years and 1.4 years, respectively. In the sex and age-matched conditional logistic multivariable regression analysis, family history of malignancy (OR 4.930, 95%CI 1.926-12.619, P = .001), ILD (OR 7.701, 95%CI 1.009-58.767, P = .049) were independent risk factors for lung cancer among SSc patients, and considering sex and age of SSc onset, SSc patients with more advanced staging of lung cancer (HR 3.190, 95%CI 1.127-6.126, P = .06) had poorer prognosis.

Conclusion: Lung cancer is not uncommon in SSc patients, especially those with family histories of malignancy or ILD. Early detection of lung cancer is of vital importance for better prognosis.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/ijcp.13819DOI Listing
April 2021

Development and formulation of the classification criteria for osteoarthritis.

Ann Transl Med 2020 Sep;8(17):1068

Department of Rheumatology, Peking Union Hospital, Chinese Academy of Medical Sciences, Beijing, China.

Background: The classification criteria of osteoarthritis (OA) is lack of the support of relevant research evidence and there is no standardized protocol for detailed steps of the development or clinical verification of classification criteria has yet been established. This study aims to describe the development process of the Categorization of Osteoarthritis CHecklist (COACH), which is designed to choose the precise treatment option for patients with OA.

Methods: A multidisciplinary panel was established to gather opinions. We conducted questionnaire survey and literature review to generate and COACH Panel members were invited to review the drafted classification criteria and optimize classification criteria. The final list of items was discussed and reached the agreement by the core group of the panel.

Results: Thirty-six experts participated in COACH Panel including rheumatologist (80.6%; 29/36), orthopedist (13.9%; 5/36), methodologist (2.8%; 1/36) and rehabilitation physician (2.8%; 1/36) for classification factors generating and optimizing. The main body of the final classification criteria consists of six types of OA pathogenesis, eight types of medical findings (which can be grouped into two categories), and six types of the location. The final criteria include load-based type, structure-based type, inflammation-based type, metabolic-based type, systemic factor based type and mixed type.

Conclusions: COACH can better help clinicians quickly classify OA patients and help to choose the best treatment option from the aspects of types, findings and locations. What's more, the classification criteria are also helpful to promote the basic medical research and targeted prevention of OA.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.21037/atm-20-4673DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7575945PMC
September 2020

Immunoglobulin A Isotype of Antiphospholipid Antibodies Does Not Provide Added Value for the Diagnosis of Antiphospholipid Syndrome in a Chinese Population.

Front Immunol 2020 5;11:568503. Epub 2020 Oct 5.

Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, Key Laboratory of Rheumatology & Clinical Immunology, Ministry of Education, Beijing, China.

Objective: Antiphospholipid syndrome (APS) is characterized by the presence of anti-phospholipid (aPL) antibodies. However, the relationship between the immunoglobulin (Ig) A isotype of aPL positivity and its clinical utility in APS diagnosis is controversial. Presently, we determine the clinical utility of IgA-aPL from consecutive patients in a large cohort from the Chinese population and patients with APS whose aPL profiles were obtained.

Methods: The detection of anticardiolipin (aCL) and anti-β glycoprotein-Ⅰ (aβGPⅠ) antibodies of the IgA/IgG/IgM isotype by paramagnetic particle chemiluminescent immunoassay was carried out in sera from 7293 subjects. 153 primary APS (PAPS) patients and 59 patients with secondary APS (SAPS) were included in this study.

Results: In total, 1,082 out of 7,293 (2.55%) subjects had a positive IgA-aPL test, and the prevalence of isolated IgA-aPL was 0.29% (21/7,293) in the general population. The prevalence of IgA-aPL in the PAPS patients was 12.42% (19/153); however, only one patient (0.65%) presented with isolated IgA-aPL. Fifty (25.9%) of the SAPS had IgA-aPL, none of whom lacked IgG/IgM-aPL. The combination of the IgA isotype and the IgG/IgM isotype did not increase the diagnostic performance when compared with the IgG/IgM isotype of aCL or aβGPⅠ, respectively. IgA-aPL was not associated with clinical manifestation in patients with APS.

Conclusion: Isolated IgA-aPL is rare in the general population as well as in patients with APS. Whether in the laboratory or in clinical practice, the presence of IgA-aPL does not provide added value for the diagnosis of APS in the Chinese population.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.3389/fimmu.2020.568503DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7573363PMC
October 2020

Health Assessment Questionnaire-Disability Index (HAQ-DI) use in modelling disease progression in diffuse cutaneous systemic sclerosis: an analysis from the EUSTAR database.

Arthritis Res Ther 2020 10 28;22(1):257. Epub 2020 Oct 28.

Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland.

Background: Patients with diffuse cutaneous systemic sclerosis (dcSSc) have a poor prognosis. The importance of monitoring subjective measures of functioning and disability, such as the Health Assessment Questionnaire-Disability Index (HAQ-DI), is important as dcSSc is rated by patients as worse than diabetes or hemodialysis for quality of life impairment. This European Scleroderma Trials and Research (EUSTAR) database analysis was undertaken to examine the importance of impaired functionality in dcSSc prognosis. The primary objectives were to identify predictors of death and HAQ-DI score progression over 1 year. HAQ-DI score, major advanced organ involvement, and death rate were also used to develop a comprehensive model to predict lifetime dcSSc progression.

Methods: This was an observational, longitudinal study in patients with dcSSc registered in EUSTAR. Death and HAQ-DI scores were, respectively, analyzed by Cox regression and linear regression analyses in relation to baseline covariates. A microsimulation Markov model was developed to estimate/predict natural progression of dcSSc over a patient's lifetime.

Results: The analysis included dcSSc patients with (N = 690) and without (N = 4132) HAQ-DI score assessments from the EUSTAR database. Baseline HAQ-DI score, corticosteroid treatment, and major advanced organ involvement were predictive of death on multivariable analysis; a 1-point increase in baseline HAQ-DI score multiplied the risk of death by 2.7 (p <  0.001) and multiple advanced major organ involvement multiplied the risk of death by 2.8 (p <  0.05). Multivariable analysis showed that baseline modified Rodnan Skin Score (mRSS) and baseline HAQ-DI score were associated with HAQ-DI score progression at 1 year (p <  0.05), but there was no association between baseline organ involvement and HAQ-DI score progression at 1 year. HAQ-DI score, major advanced organ involvement, and death were successfully used to model long-term disease progression in dcSSc.

Conclusions: HAQ-DI score and major advanced organ involvement were comparable predictors of mortality risk in dcSSc. Baseline mRSS and baseline HAQ-DI score were predictive of HAQ-DI score progression at 1 year, indicating a correlation between these endpoints in monitoring disease progression. It is hoped that this EUSTAR analysis may change physician perception about the importance of the HAQ-DI score in dcSSc.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1186/s13075-020-02329-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7592571PMC
October 2020

Aberrant expression of cell-free nucleosomes in dermatomyositis/polymyositis.

Dermatol Ther 2020 11 10;33(6):e14460. Epub 2020 Nov 10.

Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, China.

Nucleosomes are typically located intracellularly, and extracellular levels of nucleosomes indicated the degree of cell death. We postulated aberrant nucleosomes expression in dermatomyositis (DM) and polymyositis (PM). To assess the aberrant expression of circulating nucleosomes in DM and polymyositis. 76 consecutive DM and PM patients from 1 September 2017 to 31 October 2018 were enrolled in this study, in addition to 20 healthy controls. The levels of circulating nucleosomes, with 16 kinds of detectable myositis-specific antibodies (MSAs) were detected in IIMs patients using enzyme-linked immunosorbent assay kit (ELISA). Receiver operating characteristic (ROC) curve analysis was performed for evaluating the ability of this candidate marker for detecting DM/PM. The results showed that the levels of circulating nucleosomes in DM/PM patients were significantly higher than that in normal individuals. Specifically, elevated levels of nucleosomes were associated with MDA5 Ab, ARS, and TIF1γAb. In addition, elevated levels of circulating nucleosomes correlated with skin eruption. This study has evidenced nucleosomes as a potential new biomarker of DM/PM. Aberrant nucleosomes expression occurs in myositis patients and the difference in nucleosomes expression between patients with and without skin eruption is statistically significant. A unique expression profile of elevated serum nucleosomes was detected in DM/PM-MDA5 Ab(+), ASS, and TIF1γAb(+).
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/dth.14460DOI Listing
November 2020

Clinical characteristics and prognosis of concomitant systemic lupus erythematosus and primary biliary cholangitis.

Clin Rheumatol 2020 Oct 16. Epub 2020 Oct 16.

Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College & Chinese Academy of Medical Sciences, National Clinical Research Center for Dermatologic and Immunologic Diseases, Ministry of Science & Technology, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, 100730, China.

Objective: This study aimed to identify the clinical characteristics of systemic lupus erythematosus-primary biliary cholangitis (SLE-PBC) patients and to compare the manifestations and prognosis with systemic lupus erythematosus (SLE) patients.

Methods: Twenty-one inpatients with concomitant SLE and primary biliary cholangitis (PBC) were identified in our hospital. Baseline clinical manifestations, laboratory results, disease activity, and organ damage, as well as changes in disease manifestations and therapies, were retrospectively analyzed. Baseline clinical characteristics, survival rate, and flare-ups were compared with 254 SLE patients also from our hospital.

Results: The prevalence of concomitant PBC in SLE inpatients was 0.27%. Over half of the patients were diagnosed with SLE and PBC simultaneously. Compared with SLE patients, SLE-PBC patients started the symptom of SLE at an older age, with a longer delay before the diagnosis of SLE (P < 0.05). Hematological and muscular involvement, pulmonary arterial hypertension, and interstitial lung disease were more common in SLE-PBC patients (P < 0.05). Kaplan-Meier estimate showed a significantly lower survival rate in SLE-PBC group, with 3-year survival rate at 88.4%.

Conclusion: Concomitant PBC might have a negative impact on the survival of SLE, with older age at SLE onset, longer delay before SLE diagnosis, and higher baseline damage. More intensive therapy and prevention of hepatic toxicity need to be considered. Key Points • Hematological and muscular involvement, PAH, and ILD were more common in SLE PBC than in SLE. • The study firstly reported the survival rate of SLE PBC patients. • More intensive therapy and prevention of hepatic toxicity are needed for SLE-PBC.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s10067-020-05457-xDOI Listing
October 2020