Hospitalization mortality and associated risk factors in patients with polymyositis and dermatomyositis: A retrospective case-control study

<div><p>Background</p><p>Polymyositis and dermatomyositis (PM/DM) are systemic autoimmune diseases with multiple organ involvements that manifest as muscular and cutaneous disorders, interstitial lung disease (ILD) and malignancies. However, information concerning the outcomes and associated factors for PM/DM patients who are hospitalized is limited.</p><p>Methods</p><p>We retrospectively reviewed the medical charts of PM/DM patients admitted to a Chinese tertiary referral hospital (Peking Union Medical College Hospital, PUMCH) from 2008 to 2014. The deceased group included 63 patients who had “deceased discharge” status or were confirmed to have died within two weeks of hospital discharge. The demographic data, clinical manifestations, and direct causes of death were analyzed retrospectively. Medical records for 126 age- and sex-matched PM/DM patients were selected as controls from 982 inpatients successively admitted to the same center during the same period. In addition to the comparison of clinical manifestations between the two groups, binary logistic regression was conducted to explore the risk factors related to PM/DM mortality.</p><p>Results</p><p>Over the past 6 years at PUMCH, the in-hospital mortality rate of PM/DM patients was 4.58%. The male gender and the elder patients had a high risk of death (P = 0.031 and P = 0.001 respectively). The three most frequent causes of death for PM/DM patients were pulmonary infection (35%), ILD exacerbation (21%) or both conditions (25%). Pulmonary infection (P<0.001, OR = 5.63, 95% CI, 2.37–13.36), pneumomediastinum (P = 0.041, OR = 11.02, 95%CI, 1.10–110.54), Gottron’s papules (P = 0.010, OR = 3.24, 95%CI, 1.32–7.97), and elevated erythrocyte sedimentation rate (ESR) (P = 0.005, OR = 9.9, 95%CI 2.0–49.0) were independent risk factors for in-hospital mortality of PM/DM patients.</p><p>Conclusion</p><p>PM/DM patients continue to display high in-hospital mortality. Pulmonary infection is the strongest predictor of poor prognosis in PM/DM patients, followed by pneumomediastinum, Gottron’s papules, and elevated ESR.</p></div

Comparison of clinical manifestations between deceased and control PM/DM patients [x ± SD or n (%)].

<p>Comparison of clinical manifestations between deceased and control PM/DM patients [x ± SD or n (%)].</p

Multivariate logistic regression analysis of risk factors for death of PM/DM patients.

<p>Multivariate logistic regression analysis of risk factors for death of PM/DM patients.</p

Causes of death in PM/DM in-patients.

<p>Causes of death in PM/DM in-patients.</p

The 2013 American College of Rheumatology/European League Against Rheumatism Classification Criteria for Systemic Sclerosis Could Classify Systemic Sclerosis Patients at Earlier Stage: Data from a Chinese EUSTAR Center

<div><p>Objectives</p><p>To evaluate the performance of the 2013 ACR/EULAR classification criteria for systemic sclerosis (SSc) in clinical practice in a Chinese patient cohort, and to compare outcomes with the 1980 ACR criteria.</p><p>Methods</p><p>Patients clinically diagnosed with SSc between September 2013 and May 2015 were prospectively recruited from the EUSTAR database of the Peking Union Medical College Hospital. Diagnosis of SSc was based on the evaluation of three experienced rheumatologists. Patients diagnosed with other connective tissue diseases were recruited as disease controls. The 1980 ACR and 2013 ACR/EULAR criteria were applied to the cohort, and patients who fulfilled the criteria were classified as definite SSc patients. Sensitivity and specificity were analyzed for the 2013 and 1980 criteria.</p><p>Results</p><p>A total of 143 SSc patients and 87 patients with other connective diseases were recruited. 41 (28.7%) and 102 (71.3%) cases were diffuse cutaneous SSc and limited cutaneous SSc, respectively. Although the sensitivity of the 2013 criteria (94.4%) exceeded the 1980 criteria (72.7%) (P<0.001), the 1980 and 2013 criteria sets showed no significant difference in specificity (97.7% and 93.1%, respectively, P = 0.278). The sensitivity of the 2013 criteria was significantly higher than the 1980 criteria in some SSc subgroups (e.g., lcSSc, abnormal pattern of nailfold videocapillaroscopy [NVC] and presence of Raynaud’s phenomenon [RP]) compared to others.</p><p>Conclusions</p><p>Relative to the 1980 ACR criteria, in Chinese SSc patients the new 2013 ACR/EULAR criteria had similar specificity and higher sensitivity, especially for patients with mild skin thickening or prominent microvascular diseases.</p></div

Sensitivity and specificity of 1980 ACR and 2013 ACR/EULAR criteria in different countries.

<p>Sensitivity and specificity of 1980 ACR and 2013 ACR/EULAR criteria in different countries.</p

Disease spectrum of other connective tissue diseases used as controls.

<p>Disease spectrum of other connective tissue diseases used as controls.</p

The demagraphic and clinical features of SSc patients and disease controls.

<p>The demagraphic and clinical features of SSc patients and disease controls.</p

DataSheet_1_Efficacy and safety of immunosuppressive agents for adults with lupus nephritis: a systematic review and network meta-analysis.zip

IntroductionVarious immunosuppressive regimens have been developed for the treatment of lupus nephritis (LN). This study aimed to compare the efficacy and safety of immunosuppressive regimens in adults with LN.MethodsWe systematically searched the PubMed, Embase, and Cochrane Central Register of Controlled Trials databases, including conference proceedings, trial registries, and reference lists, from inception until July 10, 2022. The effects of treatment were compared and ranked using the surface under the cumulative ranking curve (SUCRA). The primary endpoint was total remission. The secondary endpoints were complete remission, systemic lupus erythematosus disease activity index (SLEDAI), relapse, all-cause mortality, end-stage renal disease (ESRD), infection, herpes zoster, ovarian failure, myelosuppression, and cancer.ResultsSixty-two trials reported in 172 studies involving 6,936 patients were included in the network meta-analysis. The combination of tacrolimus (TAC), mycophenolate mofetil (MMF), and glucocorticoid (GC) provided the best result for the total remission rate (SUCRA, 86.63%) and SLEDAI (SUCRA, 91.00%), while the combination of voclosporin (VCS) , MMF and GC gave the best improvement in the complete remission rate (SUCRA, 90.71%). The combination of cyclophosphamide (CYC), MMF and GC was associated with the lowest risk of relapse (SUCRA, 85.57%) and cancer (SUCRA, 85.14%), while the combination of obinutuzumab (OTB), MMF and GC was associated with the lowest risk of all-cause mortality (SUCRA, 84.07%). Rituximab (RTX) plus MMF plus GC was associated with the lowest risk of ESRD (SUCRA, 83.11%), while the risk of infection was lowest in patients treated with azathioprine (AZA) plus CYC plus GC (SUCRA, 68.59%). TAC plus GC was associated with the lowest risk of herpes zoster (SUCRA, 87.67%) and ovarian failure (SUCRA, 73.60%). Cyclosporine (CsA) plus GC was associated with the lowest risk of myelosuppression (SUCRA, 79.50%), while AZA plus GC was associated with the highest risk of myelosuppression (SUCRA, 16.25%).DiscussionThis study showed that a combination of TAC, MMF and GC was the best regimen for improving the total remission rate. The optimal regimen for specific outcomes should be highlighted for high-risk patients.</p

DataSheet_1_Prevalence and diagnostic value of non-criteria antiphospholipid antibodies for antiphospholipid syndrome in Chinese patients.docx

BackgroundThe presence of antiphospholipid antibodies (aPLs) plays a pivotal role in the pathogenesis of antiphospholipid antibody syndrome (APS). This study aimed to examine the diagnostic value of a set of non−criteria aPLs and their relevance with APS-related criteria and extra-criteria manifestations.MethodsFrom a prospectively constructed database, consecutive APS patients consisting of 114 primary APS (PAPS group), 54 with APS secondary to SLE (SAPS group), 9 seronegative APS (SNAPS), as well as 209 patients with systemic lupus erythematosus (SLE) and 88 healthy controls were included in this study. Levels of criteria aPLs, baseline information, and APS-related criteria and extra-criteria features were extracted from the database. Serum levels of non-criteria aPLs including aPC IgG/IgM, aPI IgG/IgM, aPE IgG/IgM/IgA, aPG IgG/IgM/IgA, anti-phosphatidic acid (aPA) IgG/IgM, aSM IgG/IgM, and aPS/PT IgG/IgM were analyzed with AESKULISA® ELISA Test Kits.ResultsThe addition of aPC IgG/M, aPI IgG/M, aPE IgG/M/A, aSM IgG/M, and aPA IgG/M to aCL or aβ2GPI IgG/M could significantly increase diagnostic sensitivity and accuracy. A significant difference between PAPS or SAPS and HC was presented in all non-criteria aPLs except for aSM IgM and aPG IgA. Eight out of nine SNAPS patients were positive for at least 1 aPL. Pregnancy morbidity was associated with aSM IgM (r = 0.22) and aSM IgG (r = 0.15). Pre-eclampsia or premature birth was associated with aSM IgG (r = 0.16), aPI IgG (r = 0.22), aPC IgG (r = 0.16), and aPG IgG (r = 0.18). Stroke was associated with aPI IgG (r = 0.2). The clinical association was also observed in DVT with aPS/PT IgG (r = 0.17). Valve lesion was positively associated with aSM IgM (Fisher test p = 0.039), APS nephropathy was associated with aPC IgG (OR 3.797), and livedo reticularis was associated with aPE IgM (OR 15.391).ConclusionAdditional detection of non-criteria aPLs including aPC IgG/M, aPE IgG/M/A, aPI IgG/M, aSM IgG/M, and aPA IgG/M could assist in APS diagnosis. The positivity of certain aPLs was statistically associated with both criteria and extra-criteria APS clinical manifestations.</p