Polymyositis And Dermatomyositis Research Articles

Serum uric acid is independently associated with hypertension in patients with polymyositis and dermatomyositis.

Serum uric acid (UA), as an antioxidant, has been associated with hypertension in the general population. Hypertension is highly prevalent in patients with polymyositis and dermatomyositis (PM/DM). Owning elevated levels of reactive oxygen species, patients with PM/DM have lower concentrations of UA in comparison with healthy people. We explored a potential association between UA levels and hypertension in PM/DM and evaluated whether this association is independent of hypertension risk factors, PM/DM characteristics and relevant drugs. A total of 472 PM/DM patients were assessed. UA and related laboratory data were measured. Demographic, hypertension-related factors, PM/DM characteristics and drug use were assessed as potential covariates. Results were analyzed using logistic models to test the independence of the association between UA and hypertension. UA levels were higher in hypertension subjects compared to non-hypertensive PM/DM patients [284.70 (239.93-357.38) vs 264.00(222.50-322.75), p=.017]. When adjusted for hypertension risk factors, PM/DM characteristics and drugs, the odds of being a hypertensive PM/DM patient per 1μmol/L UA increase were significantly increased: odds ratio=1.473 (95% confidence interval:1.063-2.042, p=.020). This cross-sectional study suggests that UA levels are independently associated with hypertension in PM/DM patients.

Pregnancy course and outcomes of patients with polymyositis and dermatomyositis (PM/DM) managed in a single center

We aimed to determine the association between disease activity during pregnancy and pregnancy outcomes of women with polymyositis and dermatomyositis (PM/DM). Patients with PM/DM who were managed from pregnancy to delivery at Kagawa University Hospital from March 2006 to May 2021 were enrolled. Clinical data were retrospectively analyzed to evaluate the association between disease activity during pregnancy and pregnancy outcomes. Eight pregnancies in 5 women with PM/DM were analyzed. The mean age at conception was 28.3 ± 3.8 years, and mean disease duration was 6.3 ± 3.2 years. Four patients required an increased glucocorticoid dosage because of worsening disease activity (sustained elevation of creatine phosphokinase [CPK] concentration). Two patients who continuously received immunosuppressive drugs from conception to delivery showed no increase in disease activity and did not need increased glucocorticoid dosages. The pregnancy outcomes were 1 spontaneous abortion and 7 live births. The mean gestation length was 35.3 ± 5.2 weeks, and mean birthweight was 2297.7 ± 1041.4 g. Five adverse pregnancy outcomes (APOs) occurred (2 preterm births and 4 low birthweights); most of these cases had sustained elevation of CPK concentration and increased glucocorticoid dosages. No APOs occurred in the 2 patients who received continuous immunosuppressive medication. Continued use of pregnancy-compatible medications and control of disease activity with lower glucocorticoid dosages in pregnancies with PM/DM may be important to achieve good pregnancy outcomes.

Rituximab as the first-line therapy in anti-synthetase syndrome-related interstitial lung disease.

Anti-synthetase syndrome (ASS) is an idiopathic inflammatory myopathy (IIM). In comparison to interstitial lung disease (ILD) in polymyositis and dermatomyositis (PM/DM), ILD in ASS is more frequent, has a more aggressive phenotype, a greater involvement of the lungs, and a more rapid onset of pulmonary symptoms. Continuous declines in predicted forced vital capacity (FVC) and dyspnea were the main features of patients who developed end-stage ILD. The severity of ASS at diagnosis dictates when and which immunosuppressant will be started. There is an experience for the usage of RTX in the first, second, and subsequent lines, as well as for reintroduction and salvage therapies. Not all ASS patients will develop severe illness and require intense immunosuppression. Some features associated with poor prognosis include older age, acute or subacute onset, lack of response to steroids, and lower baseline values for FVC and DLCO. Here we hypothesize that RTX should be the first line of treatment for high-risk ILD in ASS to preserve lung function and then maintenance therapy should be continued with the same or another drug depending on the recovery of lung function.

Inflammatory Bowel Diseases Are Associated with Polymyositis and Dermatomyositis-A Retrospective Cohort Analysis.

Background and Objectives: Polymyositis and dermatomyositis (PM/DM) are classified as polygenic autoimmune diseases, whereas inflammatory bowel disease (IBD) is considered a polygenic autoinflammatory disease. In the literature, several cases exist reporting the co-occurrence of both conditions. At the molecular level, PM/DM and IBD share common genetic determinants including interferon regulatory factor and vitamin D receptor susceptibility loci. Accumulating evidence underline several indicators that confer poor prognosis in IBD, including antinuclear antibody positivity and the presence of other autoimmune diseases, therefore the aim of this study is to assess the association between these entities. Materials and Methods: This is a population-based retrospective study using data retrieved from a large electronic medical record in Israel, the Clalit health registry. The sample included PM/DM patients and age- and sex-frequency matched controls. The prevalence of IBD in PM/DM was compared between the two groups and logistic regression was applied to control for confounding variables. Predictors of IBD in patients with PM/DM were also explored. Results: Our study included 12,278 subjects with 2085 PM/DM patients and 10,193 age- and sex- frequency-matched controls. The incidence of IBD in patients with PM/DM was significantly higher even after controlling for various confounding variables (OR of 1.73, 95% CI 1.05-2.86, p-value = 0.033). Anti-nuclear antibodies (ANA) positivity was found to be an independent predictor for IBD diagnosis in patients with PM/DM (OR 3.67, 95% CI 1.01-13.36, p = 0.048). Conclusion: Our analysis reports an association between IBD and PM/DM. Such association could point towards a common pathophysiological background. Further research is needed to further describe the clinical courses and whether a unique therapeutic approach is warranted.

The SNPs of mitochondrial DNA displacement loop region and mitochondrial DNA copy number associated with risk of polymyositis and dermatomyositis

Oxidative damage-induced mitochondrial dysfunction may activate muscle catabolism and autophagy pathways to initiate muscle weakening in idiopathic inflammatory myopathies (IIMs). In this study, Single nucleotide polymorphisms (SNPs) in the mitochondrial displacement loop (D-loop) and mitochondrial DNA (mtDNA) copy number were assessed and their association with the risk of polymyositis and dermatomyositis (PM/DM) was evaluated. Excessive D-loop SNPs (8.779 ± 1.912 vs. 7.972 ± 1.903, p = 0.004) correlated positively with mtDNA copy number (0.602 ± 0.457 vs. 0.300 ± 0.118, p < 0.001). Compared with that of the controls, the mtDNA of PM/DM patients showed D-loop SNP accumulation. In addition, the distribution frequencies of 16304C (p = 0.047) and 16519C (p = 0.043) were significantly higher in the patients with PM/DM. Subsequent analysis showed that reactive oxygen species (ROS) generation was increased in PM/DM patients compared with that in the controls (18,477.756 ± 13,574.916 vs. 14,484.191 ± 5703.097, p = 0.012). Further analysis showed that the PM/DM risk-related allele 16304C was significantly associated with lower IL-4 levels (p = 0.021), while 16519C had a trend to be associated with higher IL-2 expression (p = 0.064). The allele 16519C was associated with a positive antinuclear antibody (ANA) status in PM/DM patients (p = 0.011). Our findings suggest that mitochondrial D-loop SNPs could be potential biomarkers for PM/DM risk and these SNPs associated with cytokine expression may be involved in the development of PM/DM. Further, mtDNA copy number-mediated mitochondrial dysfunction may precede the onset of PM/DM.

Arrhythmia prevalence among patients with polymyositis-dermatomyositis in the United States: An observational study.

Systemic inflammation has been associated with atherosclerotic cardiovascular diseases (ASCVD) and arrhythmia occurrence in rheumatologic conditions such as rheumatoid arthritis. Polymyositis and dermatomyositis (PD) are rare rheumatologic conditions characterized by symmetrical proximal muscle weakness and, in the case of dermatomyositis, cutaneous eruption. Although there is literature associating PD with ASCVD, no population-level studies have analyzed arrhythmia risk in PD. The purpose of this study was to assess the prevalence of arrhythmia and its subtypes by age and sex in patients with PD and to determine associations between arrhythmia and PD. This retrospective cohort study included adults for whom hospitalizations had been recorded in the National Inpatient Sample database in the United States between 2016 and 2018. Patients with PD were matched (1:10) by age to patients without PD. Prevalence of arrhythmia was calculated in the 2 groups and compared by sex and age groups. Associations between PD and arrhythmia were determined after adjustment for common arrhythmia risk factors. From 107,001,355 hospitalizations, 32,085 adults with PD were matched to 320,850 controls. Patients with PD aged <70 years had a higher prevalence of arrhythmia and higher adjusted odds of arrhythmia compared with controls. This increased risk was only seen for supraventricular arrhythmias. Adults with PD had increased odds of in-hospital mortality if they had an arrhythmia diagnosis (odds ratio 3.3; 95% confidence interval 2.5-4.5; P <.001). We found a higher prevalence and odds of arrhythmias, particularly supraventricular arrhythmias, in young and middle-aged patients with PD compared with matched controls. Arrhythmias were associated with significant mortality among patients with PD.

Pneumocystis jirovecii pneumonia in autoimmune rheumatic diseases: a nationwide population-based study.

ObjectiveTo compare Pneumocystis jirovecii pneumonia (PJP) risk between patients with autoimmune rheumatic diseases (ARD) and the general populationMethodsWe identified patients with ARD recorded in the National Health Insurance Research Database of Taiwan from 2002 to 2015 and randomly selected a comparison cohort from the general population matched for age and sex. We analyzed PJP risk stratified by sex, age, comorbidities, and medications using Cox proportional hazard model.ResultsWe enrolled 103,117 patients with ARD. PJP risk significantly increased in patients with any ARD and with each individual ARD like rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), Sjogren’s syndrome (SjS), polymyositis and dermatomyositis (PM/DM), systemic sclerosis (SSc), and systemic vasculitis. Patients with PM/DM showed prominent risk with incidence rate of 12.47/100,000 patient year (95% confidence interval (CI), 32.16–86.70). In a time-dependent Cox proportional hazard model with comorbidities and medications as covariates, PM/DM, SSc, SLE, and SjS significantly increased adjusted hazard ratios (aHR) of 5.40, 5.12, 4.09, and 3.64, respectively (95% CI of 2.82–10.35, 2.16–12.13, 2.41–6.95, and 2.06–6.42, respectively). AHR after adjusting for male sex, cancer, human immunodeficiency virus infection (HIV), and interstitial lung disease also significantly increased. Use of daily oral steroid dose of >10 mg conferred the highest risk followed by mycophenolate. Use of injected steroids, cyclophosphamide, biological agents, methotrexate, and cyclosporine conferred a significantly higher risk.ConclusionUnderlying ARD significantly predisposes patients to PJP, with PM/DM posing the highest threat. In addition to underlying disease, comorbidities and concomitant immunosuppressants are major risks. The strongest risk is recent daily steroid dose of >10 mg. Mycophenolate seems to be a more prominent risk factor than cyclophosphamide.Key Points• Autoimmune rheumatic diseases (ARD) significantly increased the overall risk of PJP, and so did each individual ARD.• Use of steroids, mycophenolate, cyclophosphamide, biological agents, methotrexate, and cyclosporine all significantly increased risk of PJP.• Male, elderly, malignancy, HIV, and interstitial lung disease are also related to increased risk of PJP.• Underlying ARD, comorbidities, and use of immunosuppressant should all be considered in determining the overall risk of PJP.

Reduced miR-146a Promotes REG3A Expression and Macrophage Migration in Polymyositis and Dermatomyositis.

Background: Growing evidence from studies elsewhere have illustrated that microRNAs (miRNAs) play important roles in polymyositis and dermatomyositis (PM/DM). However, little has been reported on their relationship with regenerating islet-derived protein 3-alpha (REG3A) as well as their associative roles in macrophage migration. Therefore, this study sought to establish the association between miR-146a and REG3A as well as investigate their functional roles in macrophage migration and PM/DM pathogenesis.Methods: Peripheral blood mononuclear cells (PBMCs) were isolated from PM/DM patients and healthy controls through density centrifugation. Macrophages were obtained from monocytes purified from PBMCs via differentiation before their transfection with miRNA or plasmids to investigate cell migration with transwell assay. An experimental autoimmune myositis murine model was used to investigate PM/DM. Real-time PCR and Western blot analysis were conducted to determine the expression levels of miR-146a, interferon gamma (IFN-γ), interleukin (IL)-17A, and REG3A.Results: The messenger RNA (mRNA) expression level of miR-146a markedly decreased, while the mRNA level of REG3A, IFN-γ, and IL-17A expression increased substantially in PBMCs from PM/DM patients compared with the healthy controls. The levels of IFN-γ and IL-17A in serum from PM/DM patients was much higher than the healthy controls. Immunohistochemistry analysis showed that REG3A expression increased in muscle tissues from patients. Consistent with clinical data, the mRNA expression level of miR-146a also decreased, whereas the mRNA and protein level of REG3A, IFN-γ, and IL-17A significantly increased in the muscle tissues of experimental autoimmune myositis mice. Moreover, miR-146a inhibited monocyte-derived macrophage migration, and REG3A promoted macrophage migration. In addition, IL-17A induced REG3A expression, while miR146a inhibited expression of REG3A in monocyte-derived macrophages from the PBMCs of the healthy donors. Notably, inhibition of macrophage migration by miR-146a was via the reduction in REG3A expression.Conclusions: Reduced miR-146a expression in PM/DM leads to increased REG3A expression that increases inflammatory macrophage migration, which may be a possible underlying mechanism of DM/PM pathogenesis.

Interleukin-23 as a therapeutic target for inflammatory myopathy

Current treatments of polymyositis and dermatomyositis (PM/DM) depend on non-specific immunosuppressants. This study was performed to elucidate the role of interleukin (IL)-23, as their possible therapeutic target. As was reported earlier in PM/DM patients, serum IL-23 levels were elevated in mice with C protein induced-myositis (CIM), a murine model of PM. IL-23 was expressed by macrophages in the PM/DM and CIM muscles and by dendritic cells and macrophages in the lymph nodes from the CIM mice. It was also expressed by macrophages in the chemically injured muscles, but not those recruited into the muscles by footpad injection of Freund’s complete adjuvant, demonstrating that IL-23 production should be associated with muscle damage. Genetic deletion of IL-23 as well as preventive and therapeutic administration of blocking antibodies against IL-23p19 subunit suppressed CIM. When lymph node cells from the CIM mice were transferred adoptively into naive wild type or IL-23p19 deficient recipient mice, both recipients developed myositis equally. Thus, elevated IL-23 should promote dendritic cells and macrophages to activate the autoaggressive T cells. Our findings suggest that IL-23 should mediate positive feedback loop from the muscle damage to the T cell activation and be a promising therapeutic target for autoimmune myositis.

Clinical Heterogeneity of Interstitial Lung Disease in Polymyositis and Dermatomyositis Patients With or Without Specific Autoantibodies

BackgroundThe aim of this study was to compare the heterogeneity of interstitial lung disease (ILD) in patients with polymyositis and dermatomyositis (PM/DM) according to serological type. MethodsA total of 182 patients with PM/DM-ILD were observed retrospectively. Antiaminoacyl-tRNA synthetase (ARS) and antimelanoma differentiation-associated gene5 (MDA5) antibodies were screened using immunoblotting approach. The patients with ILD were divided into 3 groups: MDA5 (with anti-MDA5 antibody), ARS (with anti-ARS antibody) and MSN (without anti-MDA5 or anti-ARS antibody) group. Pulmonary features, treatment responses and prognoses were compared among the groups. ResultsA higher percentage of rapidly progressive ILD (RP-ILD) occurrences (55.8% versus 25% versus 16.9%, P < 0.001) was observed in the MDA5 group compared to ARS and MSN groups. The MSN group experienced lower dyspnea (48.2% versus 79% versus 71.4%, P = 0.001) and fever (18.1% versus 39.5% versus 37.5%, P = 0.01) frequencies compared to MDA5 and ARS groups. Response to 6-month treatment among 95 patients showed highest deterioration ratio (70%, P = 0.001) of ILD in the MDA5 group. Additionally, the highest frequency of ILD improvement (60%, P = 0.04) was observed in the ARS group. During the observation period, 24 patients died of respiratory failure. The 5-year survival rates were significantly lower in MDA5 group (50.2%) compared to ARS group (97.7%) or the MSN group (91.4%) (P < 0.001). ConclusionsMDA5-ILD was associated with severe pulmonary manifestations, poor response to treatment and aggravated prognosis. The ARS-ILD group had favorable treatment response and prognosis. MSN-ILD patients had relatively worse treatment response and prognosis compared to the ARS group, even though they expressed milder pulmonary manifestation.

Anti-SS-A/Ro antibody positivity as a risk factor for relapse in patients with polymyositis/dermatomyositis

Objective: The objective of this study is to elucidate predictors of relapse in patients with polymyositis and dermatomyositis (PM/DM).Methods: Fifty PM/DM patients who achieved disease stabilization at Okayama University Hospital in 2004–2014 were enrolled retrospectively. Candidate predictors such as demographic factors, clinical symptoms, laboratory data, and treatment status were compared.Results: The mean age of enrolled patients was 58 years; 34 were female. The patient groupings were as follows: 21 with PM, 27 with DM, and two with clinically amyopathic DM. During a mean observation period of 685 d, 5 patients (10%) died and 20 (40%) relapsed. The relapsed patients displayed baseline muscle weakness less frequently (85% versus 100%, p = .03) and anti-SS-A/Ro antibody more frequently (65% versus 27%, p = .007). Anti-SS-A/Ro-positive patients exhibited a higher relapse rate than anti-SS-A/Ro-negative patients (log-rank test, p = .03). Anti-SS-A/Ro-positive patients also exhibited higher anti-Jo-1 antibody positivity and lower levels of serum complement. After adjusting anti-Jo-1 antibody positivity, age, sex, CK <500 IU/L, and lung involvement, anti-SS-A/Ro positivity was still an independent risk factor for higher relapse-rate (odds ratio, 5.5; 95% confidence interval, 1.4–25.1).Conclusions: Anti-SS-A/Ro antibody positivity may be a useful biomarker for prediction of relapse.

The long-term outcome of interstitial lung disease with anti-aminoacyl-tRNA synthetase antibodies

RationaleAnti-aminoacyl transfer RNA synthetase antibodies (anti-ARS) are a group of myositis-specific autoantibodies that are detected in the sera of patients with polymyositis and dermatomyositis (PM/DM) and also in those of patients with idiopathic interstitial pneumonias without any connective tissue disease (CTD), including PM/DM. Although we reported the clinical characteristics of interstitial lung disease with anti-ARS antibodies (ARS-ILD) with and without PM/DM, the long-term prognosis of ARS-ILD remains undetermined. As our previous studies revealed that ARS-ILD without PM/DM was similar to CTD-associated ILD, and that ARS-ILD with PM/DM was radiologically suggestive of a nonspecific interstitial pneumonia (NSIP) pathological pattern, we hypothesized that the prognosis of ARS-ILD might be distinct from that of idiopathic pulmonary fibrosis (IPF) without anti-ARS. ObjectivesTo elucidate the long-term outcome of ARS-ILD with and without PM/DM and compare it to that of IPF. MethodsA two-center retrospective study was conducted. The study population comprised 36 patients with ARS-ILD (8 with PM, 12 with DM, and 16 without myositis throughout the course), 100 patients with IPF without anti-ARS, and 7 patients with NSIP without anti-ARS. The presence of anti-ARS was determined by RNA immunoprecipitation using the sera obtained at the time of diagnosis before specific treatment. Measurements and main resultsDuring the observational period (median 49 months; range, 1–114 months), 7 patients with ARS-ILD (19%; 3 with PM, 1 with DM, and 3 without PM/DM) and 51 patients with IPF (51%) died. Patients with ARS-ILD had better overall survival than those with IPF (log-rank test, P < 0.001) and similar survival compared to those with NSIP (log-rank test, P = 0.59). The prognosis for patients with ARS-ILD was similar between those with and without myositis (log-rank test, P = 0.91). At the median follow-up time of 76.5 months, 14 of the 36 patients with ARS-ILD had deteriorated. Both a decline in forced vital capacity or an initiation of long-term oxygen therapy during the course (odds ratio [OR], 5.34) and acute exacerbation (OR, 28.4) significantly increased the mortality risk. ConclusionsThe long-term outcome of ARS-ILD was significantly better than that of IPF regardless of the presence or absence of myositis.

Antioxidant status of serum bilirubin and uric acid in patients with polymyositis and dermatomyositis

Objective: Oxidative stress and variations in antioxidant status are implicated in the pathogenesis of inflammatory and autoimmune diseases. Polymyositis and dermatomyositis (PM/DM) are autoimmune diseases with inflammatory cells infiltrating into skeletal muscles, and the antioxidant status is still controversial. The aim of our study was to investigate the correlation between PM/DM and the antioxidant status of serum bilirubin (Tbil, Dbil and Ibil) and uric acid (UA). Materials and Methods: We measured serum concentrations of bilirubin (Tbil, Dbil and Ibil) and uric acid in 384 individuals, including 110 PM/DM patients and 274 healthy controls. Results: We found that PM/DM patients had significantly lower serum concentrations of bilirubin (Tbil and Ibil) and uric acid than healthy controls, whether male or female. Also, after separately adjusting the covariances of age and gender, Tbil, Dbil, Ibil and UA were all relevant factors for PM/DM. Moreover, there were no significant differences in serum antioxidant molecule levels between PM and DM subgroups. Conclusion: Our study demonstrated the low serum levels of bilirubin and uric acid in patients with PM/DM. This suggested low antioxidant status in PM/DM patients with excessive oxidative stress.

Long-term follow-up of 124 patients with polymyositis and dermatomyositis: Statistical analysis of prognostic factors

Objectives. The aims of this study were to clarify the long-term outcome of patients with polymyositis and dermatomyositis (PM/DM) and to elucidate prognostic factors using statistical analysis.Methods. We enrolled patients with PM/DM who visited our department between 1990 and 2014. Diagnoses of PM/DM and clinically amyopathic DM were based on the definitions of Bohan and Peter, and Sontheimer, respectively. We also obtained clinical data, such as age of onset, sex, medications, and presence of interstitial lung disease and malignancies, as well as laboratory tests, including the values of creatine kinase, KL-6, and ferritin. The follow-up was conducted until June 2014.Results. A total of 124 patients (PM: 46, DM: 78) were enrolled. The mean age of onset was 53.5 years, and females were predominant (64.5%). Overall survival rates were 93%, 86%, and 78% for 1, 5, and 10 years, respectively. The survival rates were significantly lower in patients with higher age of onset, with malignancies, and with hyperferritinemia in univariate analysis; however, multivariate analysis identified age of onset and serum ferritin as the most significant prognostic factors.Conclusions. Our study indicates that when age of onset and serum ferritin are used in combination, we can predict prognosis of patients with PM/DM.

AB0839 Evaluation of serum levels of N-terminal prob-type natriuretic peptide (NT-PROBNP) in patients with polymyositis and dermatomyositis (PM/DM): Preliminary data

Backgroundsymptomatic clinically cardiac involvement in PM/DM is rare but seems to be one of the most important cause of death1. High Nt-proBNP levels are commonly considered a marker of heart...

Pulmonary manifestations of anti-ARS antibody positive interstitial pneumonia – With or without PM/DM

Autoantibodies against aminoacyl-tRNA synthetases (ARS) have been found to be highly specific for polymyositis and dermatomyositis (PM/DM) and to correlate strongly with complicating interstitial pneumonia (IP). The aim of the present study was to compare the clinical presentations of anti-ARS antibody-positive IP patients with or without manifestations of PM/DM. We retrospectively examined 36 IP patients with anti-ARS antibodies. Sixteen patients presented with and 20 without the features of PM/DM. They were divided into PM/DM-IP and idiopathic-IP (IIP) groups. Clinical symptoms, findings on physical examination, laboratory data, pulmonary function, computed tomography (CT), and bronchoalveolar lavage fluid (BALF) cell counts were compared. Skin findings, myalgia, and elevation of serum creatinine kinase were found in the PM/DM-IP group. Features common to both groups included: volume loss in lower bilateral lobes; ground-glass opacities, reticular shadows and traction bronchiectasis on chest CT; high percentage of lymphocytes (IIP: 44.0% ± 21.0% (mean ± SD), PM/DM-IP: 50.5% ± 23.5%) and low CD4/8 ratios (IIP: 0.36 ± 0.34, PM/DM-IP: 0.44 ± 0.42) in BALF; decreased pulmonary function, including percentage of predicted vital capacity (VC) (IIP: 80.1% ± 15.4%, PM/DM-IP: 73.6% ± 16.4%), residual volume (RV) (IIP: 70.7% ± 21.7%, PM/DM-IP: 71.5% ± 17.1%), total lung capacity (TLC) (IIP: 73.4% ± 13.6%, PM/DM-IP: 71.6% ± 13.0%), and diffusing capacity DLco (IIP: 57.5% ± 26.7%, PM/DM-IP: 46.4% ± 10.3%). Both groups achieved good responses to initial corticosteroid or immunosuppressant therapy. Patients with anti-ARS antibody-positive IP have common pulmonary manifestations regardless of the presence of PM/DM.

Predictive factors and unfavorable prognostic factorial analysis of interstitial lung disease in patients with inflammatory myopathy

To explore the clinical features, predictive factors and unfavourable prognostic factors of interstitial lung disease (ILD) in the patients with polymyositis and dermatomyositis (PM/DM) so as to provide rationales for early clinical diagnosis and treatment. The medical records of 244 PM/DM patients from Department of Rheumatology & Immunology of Anhui Medical University Affiliated Provincial Hospital from August 2001 to August 2011 were reviewed to analyze the clinical features, predictive factors and unfavourable prognostic factors of ILD in PM/DM patients. The major clinical manifestations of PM/DM-ILD included dry cough, chest tightness and shortness of breath during activities and Velcro rale in lower lung. Besides anti-Jo-1 antibody, multivariate unconditional Logistic regression analysis indicated that amyopathic dermatomyositis (ADM), arthralgia/arthritis, fever and Gottron's sign were the predictive factors for the development of ILD in PM/DM patients. ADM and Hamman-Rich-like pattern ILD were the poor prognostic factors while large dose glucocorticoids plus immunosuppressant therapy in the early time was a protective factor for PM/DM-ILD patients. Some clinical features are the predictive factors for the development of ILD. ADM and Hamman-Rich-like pattern ILD are poor prognostic factors. Large dose glucocorticoids plus immunosuppressant therapy can significantly improve the prognosis of PM/DM patients.

Anti-MDA5 antibody is associated with A/SIP and decreased T cells in peripheral blood and predicts poor prognosis of ILD in Chinese patients with dermatomyositis

The aim of this study is to determine serum anti-melanoma differentiation-associated gene 5 prevalence and their clinical associations in Chinese patients with polymyositis and dermatomyositis (PM/DM). Serum anti-MDA5 antibody was detected by ELISA in 113 adult PM/DM patients and in various controls. Flow cytometry was applied to analyze the subgroups of lymphocytes in the peripheral blood of PM/DM patients. The serum anti-MDA5-positive rate in the DM patients (22.6%) was significantly higher compared with that in PM patients (0%, P < 0.005), patients with SLE (3.3%, P < 0.05), RA (3.3%, P < 0.05), pSS (0%, P < 0.05), pulmonary infection (0%, P < 0.05) and healthy controls (0%, P < 0.001). The percentage of decreased CD4(+), CD8(+) T cell counts, raised CD4(+)/CD8(+) ratio in peripheral blood and the incidence of acute/subacute interstitial pneumonia (A/SIP) were significantly higher in anti-MDA5-positive DM group than negative group (all P < 0.05). Additionally, logistic multivariate analysis showed that anti-MDA5 is an independent risk factor for death of ILD in DM (OR = 8.46, 95% CI 1.77-40.36, P = 0.007). In conclusion, in Chinese PM/DM patients, serum anti-MDA5 antibody is mainly presented in DM patients and can be a useful marker for A/SIP in patients with DM. It can predict unfavorable prognosis in DM patients with ILD. Further studies are needed to identify how the abnormal T cells in peripheral blood participated in the generation of the anti-MDA5 antibody.

SP1-23 Characteristics of systemic lupus erythematosus patients receiving financial aid for treatment in Japan

IntroductionSystemic lupus erythematosus (SLE) is one of the most frequent autoimmune diseases. As SLE is unknown cause and difficult to treat, and the patients are forced to have difficult life,...

Infectious Complications in Polymyositis and Dermatomyositis: A Series of 279 Patients

To assess the prevalence and characteristics of severe pyogenic, nonpyogenic, and opportunistic infections in polymyositis and dermatomyositis (PM/DM) patients and to evaluate the predictive values for infections on clinical presentation and biochemical findings of PM/DM to detect patients at risk for such infections. The medical records of 279 consecutive PM/DM patients in 3 medical centers were reviewed. One hundred four severe infections occurred in our patients (37.3%), ie, pyogenic (n = 71) and nonpyogenic/opportunistic infections (n = 33). Pyogenic infections were mainly due to aspiration pneumonia (n = 46) and calcinosis cutis infection. Thirty-three PM/DM patients developed nonpyogenic/opportunistic infections that were due to the following: Candida albicans, Pneumocystis jiroveci, Aspergillus fumigatus, Geotrichum capitatum, Mycobacterium (avium-intracellulare complex, xenopi, marinum, peregrinum, tuberculosis), Helicobacter heilmanii, cytomegalovirus, herpes simplex and zoster virus, hepatitis B and C, JC virus, Leishmania major, Strongyloides stercoralis. Esophageal dysfunction, ventilatory insufficiency, malignancy, and lymphopenia were significantly more frequent in the group of PM/DM patients with infections. Our study underscores the high frequency of infections in PM/DM, resulting in an increased mortality rate. Our results suggest that prophylaxis against pyogenic infections should be routinely recommended for patients with PM/DM, including regular physical examination of lungs to depict aspiration pneumonia as well as risk factors of aspiration pneumonia. Finally, because a great variety of micro-organisms may be responsible for opportunistic infections, it seems difficult to initiate primary prophylaxis in PM/DM patients exhibiting risk factors for opportunistic infections.