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To define the prevalence and clinical phenotype of anti-cortactin autoantibodies in adult and juvenile myositis.In this longitudinal cohort study, anti-cortactin autoantibody titers were assessed by enzyme-linked immunosorbent assay in 670 adult myositis patients and 343 juvenile myositis patients as well as in 202 adult healthy controls and 90 juvenile healthy controls. The prevalence of anti-cortactin autoantibodies was compared among groups. Clinical features of patients with and those without anti-cortactin autoantibodies were also compared.Anti-cortactin autoantibodies were more common in adult dermatomyositis (DM) patients (15%; P = 0.005), particularly those with coexisting anti-Mi-2 autoantibodies (24%; P = 0.03) or anti-NXP-2 autoantibodies (23%; P = 0.04). In adult myositis, anti-cortactin was associated with DM skin involvement (62% of patients with anti-cortactin versus 38% of patients without anti-cortactin; P = 0.03), dysphagia (36% versus 17%; P = 0.02) and coexisting anti-Ro 52 autoantibodies (47% versus 26%; P = 0.001) or anti-NT5c1a autoantibodies (59% versus 33%; P = 0.001). Moreover, the titers of anti-cortactin antibodies were higher in patients with interstitial lung disease (0.15 versus 0.12 arbitrary units; P = 0.03). The prevalence of anti-cortactin autoantibodies was not different in juvenile myositis patients (2%) or in any juvenile myositis subgroup compared to juvenile healthy controls (4%). Nonetheless, juvenile myositis patients with these autoantibodies had a higher prevalence of "mechanic's hands" (25% versus 7%; P = 0.03), a higher number of hospitalizations (2.9 versus 1.3; P = 0.04), and lower peak creatine kinase values (368 versus 818 IU/liter; P = 0.02) than those without anti-cortactin.The prevalence of anti-cortactin autoantibodies is increased in adult DM patients with coexisting anti-Mi-2 or anti-NXP-2 autoantibodies. In adults, anti-cortactin autoantibodies are associated with dysphagia and interstitial lung disease.

BACKGROUND The evidence for positive effects of exercise is insufficient in dermatomyositis. This article aims to outline the effects of physical exercise with regard to structure (Disease activity, inflammation, metabolic milieu and muscle structure) as well as clinical outcome in terms of impairment, activity limitation, participation restriction according to the ICF; and QOL in an adult patient with dermatomyositis. CASE DESCRIPTION A 47-years old female diagnosed with definite DM since last 10 months was referred by a Rheumatologist for physical therapy intervention. INTERVENTION 12 weeks (Three days a week) of Physiotherapy (Moderate intensity, individualized, closely monitored) along with a structured home exercise program (Low intensity, 5 days - 6 days a week). OUTCOME MEASURES Assessment of muscular pain, muscle strength, endurance, balance, functional evaluation, and QOL along with serological investigations. RESULTS Effectiveness Patient continued to show meaningful and clinically significant improvement on standardized outcome measures: 1. A major clinical response (total improvement score of 77) on IMAC S core set measures. 2. Improvement across all domains of ICF viz. muscle strength; endurance; movement coordination; balance; aerobic endurance; confidence and performance in activities of daily living; participation in social activities. 3. Probable disease-modifying effect: Progressively tapering dose of prescribed corticosteroids medication and improvement in disease-specific biochemical markers. 4. Patient reported measures: Reduction in muscular pain, shortness of breath & fatigue; and improvement in Healthrelated QOL. SAFETY (As evident clinically and from the investigations): 1. No adverse reaction to exercises or physical activity. 2. Remained in remission - stable disease activity. CONCLUSION Moderate intensity of physiotherapy intervention along with home exercise program is effective and safe in improving functional outcomes for dermatomyositis in a stable disease activity state. This case report enhances our understanding of the effects of physical exercise and may improve treatment and outcome in patients with dermatomyositis.

The aim was to study and compare the clinical manifestations, auxiliary examinations, and therapeutic responses in patients with different myositis-specific antibody (MSA) types. We retrospectively investigated the medical records of 143 hospitalized dermatomyositis patients, all of whom were tested for MSAs, and performed follow-up. Patients were divided into groups with and without anti-nuclear matrix protein 2 (NXP2) antibodies (17 vs 126 patients). Demographic, clinical manifestation (occurring at any time during the disease course), imaging, laboratory, treatment response, and survival data were collected for statistical analyses. Adult dermatomyositis patients with anti-NXP2 antibodies were more prone to dysphagia (P 1000 IU/L, P

Background: Nailfold capillaroscopy (NFC) has been described to be a valuable instrument used in diagnosis of systemic sclerosis (SSc) and juvenile dermatomyositis (JDM) patients. Though, NFC has been infrequently assessed in adult dermatomyositis (DM) patients. Objectives: Our aim was to visualize nailfold capillary changes in dermatomyositis (DM) and polymyositis (PM) Egyptian patients and to identify a specific diagnostic capillary pattern and to assess its relevance to disease activity, clinical and laboratory findings. Patients and Methods: This study included 20 patients (12 DM, 5 PM, and 3 overlap syndrome). Routine laboratory assessment was done, disease and skin activity scores were evaluated. Assessment of the nailfold capillary circulation using the videocapillaroscope was done and capillary density score was assessed. Results: The mean age was 39±12 years and their mean disease duration was 24±19 months. The mean muscle disease activity score was 3.8±3.3, and global disease activity score (DAS) 3.92±3.09. The mean skin activity score was 2±3. The global DAS was significantly higher in patients with branched capillaries (p=0.041). Skin activity score was significantly higher in patients with capillary hemorrhage (p=0.024). More severe capillaroscopic findings were prominent in DM patients rather than PM or overlap patients. Conclusion: Capillary branching is more common in patients with higher global DAS and capillary hemorrhage is more frequent in patients with higher skin activity score. Capillaroscopic changes are evident in DM patients rather than PM or overlap patients.

Macrophage activation syndrome (MAS) is a severe and life-threatening syndrome associated with autoimmune diseases, characterized by fever, hepatosplenomegaly, and pancytopenia. Dermatomyositis (DM) is one of the causes of MAS; however, its clinical characteristics in DM patients remain unclear. This study aimed to present a case of anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive DM complicated by MAS in a 29-year-old woman and to review the literatures including similar cases. Even though symptoms and cytopenia of our patient were refractory to combination therapy, including glucocorticoids, immunosuppressants, and plasma exchange, the administration of rituximab (RTX) resulted in rapid clinical improvement and glucocorticoid reduction. The literature review revealed 18 adult patients with DM associated MAS. Most patients developed MAS within 3 months from DM onset. A monotherapy of glucocorticoid was insufficient to control the disease, and the mortality of MAS in DM was higher than that of MAS in other rheumatic diseases, despite being treated by various means. RTX may be an effective treatment for patients with DM complicated by MAS who are refractory to conventional therapy. Anti-MDA5 antibody could influence the development of MAS; however, further investigations are needed to elucidate the association between myositis-specific antibody and MAS.

Introduction: Current literature has minimal solid data to guide therapeutic intervention in adult dermatomyositis and studies that include systemic inflammatory mechanism suppression, musculoskele...

Les dermatomyosites (DM) sont des maladies auto-immunes rares du groupe des myopathies inflammatoires idiopathiques, définies par une atteinte cutanée caractéristique. Elles peuvent survenir dans l’enfance, ou chez l’adulte. Il existe des variations phénotypiques entre les DM concernant la présentation cutanéomusculaire (ex: amyopathique) mais aussi la présentation extra-cutanéomusculaire (ex: atteinte pulmonaire ou articulaire associée). Le caractère auto-immun de ces pathologies est souligné dans 60 % des cas par la présence d’anticorps spécifique de myosite. Ces derniers sont associés à la présence de caractéristiques cliniques, histologiques, mais aussi pronostiques. Ils sont au nombre de cinq, les anti-Mi2, anti-Tif1-γ, anti-NXP2, anti-MDA5 et anti-SAE. Les anti-Mi2 sont associées à une forme clinique cutanée classique, une atteinte musculaire souvent sévère au diagnostic et une bonne évolution sous traitement. Les deux suivants, fréquents chez l’enfant et l’adulte, sont associés à des formes récidivantes cutanées et sont fortement associés aux cancers chez l’adulte. Les anti-MDA5 sont les anticorps associés aux formes les plus systémiques avec une atteinte pulmonaire interstitielle rapidement progressive pouvant être très grave. Enfin, les anti-SAE n’ont été décrits que chez l’adulte, avec une atteinte classique.

ObjectiveTo evaluate SIGLEC1 expression on monocytes by flow cytometry as a type I interferon biomarker in idiopathic inflammatory myopathies (IIM).MethodsWe performed a retrospective analysis of adult and pediatric patients with the diagnosis of IIM. SIGLEC1 expression was assessed by flow cytometry and was compared with Physician Global Assessment (PGA) or Childhood Myositis Assessment Scale (CMAS) disease activity scores. Mann Whitney-U test and receiver operating characteristic (ROC) curves were used for cross-sectional data analysis (n=96), two-level mixed-effects linear regression model for longitudinal analyses (n=26, 110 visits). Response to treatment was analyzed in 14 patients within 12 months, using Wilcoxon test. SIGLEC1 was compared to ISG15/MxA status by immunohistochemical staining of muscle biopsies (n=17).Results96 patients with adult (a) and juvenile (j) dermatomyositis (DM, n=38), antisynthetase syndrome (AS, n=19), immune-mediated necrotizing myopathy (IMNM, n=8), inclusion body myositis (IBM, n=9), and overlap myositis (n=22) were included. SIGLEC1 distinguished significantly between active and inactive disease with an area under the curve (AUC) of 0.92 (95% CI: 0.83–1) in DM and correlated with disease activity longitudinally (aDM: standardized beta=0.54, pConclusionSIGLEC1 is a candidate biomarker to assess type I interferon activity in IIM and proved useful for monitoring disease activity and response to treatment in juvenile and adult DM.Key messagesWhat is already known about this subject?There is an unmet need for routine clinical biomarkers to assess type I interferon activity in rheumatic musculoskeletal diseasesSIGLEC1 is part of the type I interferon signature and transcripts were found to be upregulated in various autoimmune diseases such as systemic lupus erythematosus (SLE), Sjoegren syndrome and dermatomyositis.SIGLEC1 is expressed on the surface of monocytes and thus is easily assessable by flow cytometry, which enables straightforward monitoring of type I interferon activityWhat does this study add?An activation of the type I interferon system in IIM can be identified by flow cytometry analysing SIGLEC1 expression. SIGLEC1 correlated to disease activity and improvement under therapy in juvenile and adult dermatomyositis.How might this impact on clinical practice or future developments?SIGLEC1 expression is a suitable biomarker for monitoring type I interferon activation in rheumatic musculoskeletal diseases, which has clinical implications for patient stratification and treatment decision making especially in the context of interferon inhibitory therapies.

Objectives:The aim of this study was to determine the relationship between disease activity in adult patients with dermatomyositis (DM) and other biomarkers of disease activity such as C-reactive protein creatinine kinase and nailfold video capillaroscopy (NVC).Methods:We performed a prospective single center study of 15 adult patients with DM. Study participants underwent two assessments at least 9 months apart including clinical, laboratory and NVC evaluations. Patients received immunosuppressive medications for their dermatomyositis, and ongoing disease activity was measured by the Myositis Intention to Treat Index (MITAX). NVC evaluation included assessment of capillary density, capillary apical diameter (mm), and the number of microhemorrhages per digit.Results:Microvascular abnormalities were present in most DM patients. Of these, capillary density (4.71 vs. 6.84,p= 0.006) and mean apical diameter (56.09 vs. 27.79 μm,p= 0.003) significantly improved over the study period in concordance with improving disease control (MITAX 8.53 vs. 2.64,p= 0.002). Longitudinal analysis demonstrated that capillary density was independently associated with MITAX (β = −1.49 [CI −2.49, −0.33],p= 0.013), but not other parameters such as C-reactive protein and creatinine kinase.Conclusions:Nailfold capillary density is a dynamic marker of global disease activity in adult DM. NVC may be utilized as a non-invasive point-of-care tool to monitor disease activity and inform treatment decisions in patients with DM.

Juvenile dermatomyositis (JDM) is a rare systemic autoimmune vasculopathy characterised by weakness in proximal muscles and pathognomonic skin rashes.1 Clinically, some patients are refractory to any available treatments or became steroids dependent.2 The adverse reactions of long-term use of steroids are severe; therefore, more effective and safer medications are urgently needed. JAK inhibitors (JAKi) can reduce interferon (IFN)-induced STAT1 phosphorylation and block the JAK-STAT pathway, demonstrating a therapeutic potential of inflammation control in JDM.3 The successful uses of JAKi were reported in adult dermatomyositis (DM) and two patients with JDM.3–5 Here, we want to share the JAKi using experiences of 25 refractory JDM cases who were diagnosed and classified according to Bohan and Peter’s criteria and treated between November 2017 and May 2019. Written informed consents were obtained from the guardians of all patients before starting the treatment. Among 25 cases, 44% (11/25) patients were female, the mean age of onset was 4.6±3.3 years and the mean age to start add-on JAKi treatment was 7.2±4.0 years. The mean disease course of the 25 JDM patients before JAKi treatment is 21.0 months (range: 14.0–36.5). All cases are refractory JDMs, including 32% (8/25) ineffective patients and 68% (17/25) glucocorticoid-dependent cases. After routine treatment fails, they received …

Anti-transcriptional intermediary factor-1γ (TIF-1γ) autoantibody may be associated with cancer in adult patients with dermatomyositis. The aim of this study was to evaluate the risk of cancer in the presence of anti-TIF-1γ autoantibody in adult dermatomyositis. A comprehensive database search of EMBASE, MEDLINE and the Cochrane Library up to May 2018 was performed using the main key words “dermatomyositis”, “”myositis”, “inflammatory myopathies” and “anti-TIF-1”. Eighteen studies, with a total of 1,962 dermatomyositis, were included in the meta-analysis. The pooled prevalence of cancer-associated dermatomyositis in patients with anti-TIF-1γ autoantibody was 0.41 (95% confidence interval (CI) 0.36–0.45). In the presence of anti-TIF-1γ autoantibody, the overall diagnostic odds ratio of cancer was 9.37 (95% CI 5.37–16.34) with low heterogeneity (Cochran’s Q: 14.88 (df = 17, p = 0.604); I2 = 0%). The results of this systematic review confirm that detection of anti-TIF-1γ autoantibody is a valuable tool to identify a subset of adult dermatomyositis patients with higher risk of cancer.

Idiopathic inflammatory myopathy encompasses a group of acquired, heterogeneous, systemic diseases of the skeletal muscle, including adult polymyositis, adult dermatomyositis, juvenile dermatomyositis, juvenile polymyositis, inclusion body myositis, and necrotizing myopathy, all resulting in muscle weakness. Granulomatous myositis (GM) is a rare myopathy disorder histologically characterized by the development of endomyseal and/or perimyseal granulomas in striated muscle. GM is often associated with sarcoidosis. GM has also been associated with myasthenia gravis, inflammatory bowel disease, thymoma, and malignancy. We are reporting a rare case of a 13-year-old girl with GM without associated disease that was refractory to multiple medications, and responded well to rituximab.

BACKGROUND In recent years, many novel myositis-specific autoantibodies (MSAs) have been identified. However, their links with the pathogenesis and clinical manifestations of inflammatory myopathies remain uncertain. OBJECTIVES To characterize the population of adult dermatomyositis (DM) and polymyositis (PM) patients treated at our center for autoimmune diseases using clinical and laboratory measures. MATERIAL AND METHODS According to the Bohan and Peter criteria, we retrospectively analyzed patients who fulfilled diagnostic criteria for DM or PM. Myositis-specific autoantibodies and myositis-associated autoantibodies (MAAs) were identified using immunoblot assays. RESULTS Fifty-one PM (71% women) and 36 DM (67% women) Caucasian patients with a median age of 58 (range: 21-88) years who met the definite or probable diagnostic criteria for myositis were included in the study. Myositis-specific autoantibodies were identified in 63 (72%) patients, whereas MAAs were observed in 43 (49%) of them. Interstitial lung disease (ILD) was characteristic of PM patients (67%, χ2 with Yates's correction (χc2) = 13.8078, df = 1, p = 0.0002), being associated with anti-Jo-1 or anti-PL-12 antibodies (fraction comparison test (FCT) 6.4878, p < 0.0001, 6.8354, p = 0.0003, respectively). Interestingly, among patients with anti-MDA5 antibodies (n = 8, 9.2%), all but one had an amyopathic form, with more frequent ILD, skin changes and arthralgias than observed in other patients (FCT 4.7029, p = 0.0228 and p = 7.7986, p = 0.0357, p = 4.7029 and p = 0.0228, respectively). Anti-signal recognition particle (SRP) was strongly associated with the Raynaud's phenomenon (FCT 4.1144, p = 0.0289) and the highest muscle injury markers (Mann-Whitney U test, z = 2.5293, p = 0.0114). Malignancy was recorded in 14 (16%) patients and was equally common in those with PM and DM. The anti-TIF-1γ was the most frequently related to cancer χ2 = 14.7691, df = 1, p < 0.0001). The anti-Mi-2α, similarly prevalent in DM and PM, was typically accompanied by skin changes (FCT 7.7986, p = 0.0357) but not ILD (FCT 8.7339, p = 0.0026). CONCLUSIONS Identification of MSAs might help to predict the clinical course of the autoimmune myopathy and malignancy risk. However, these antibodies were absent in about 30% of patients with typical PM or DM manifestations, which encourages further research in this area.

Objective: To evaluate the efficacy of tocilizumab (TCZ) in adult patients with refractory immune-mediated necrotizing myopathies (IMNMs) and investigate possible predictive biomarkers of the response to treatment with TCZ.Methods: Patients with refractory IMNM were enrolled in this open-label pilot observational study and received intravenous TCZ treatment. The clinical response was assessed after 6 months of TCZ treatment according to the 2016 American College of Rheumatology–European League Against Rheumatism (ACR–EULAR) response criteria for adult dermatomyositis and polymyositis. Muscle biopsies were performed to investigate muscle fiber regeneration by immunohistochemical staining of CD56. Serum levels of interleukin (IL)-6 were measured using a multiplex bead-based flow fluorescent immunoassay. The levels of muscle IL-6 mRNA were detected by real-time polymerase chain reaction.Results: A total of 11 patients with refractory IMNM were enrolled in the study, including 3 anti-3-hydroxy-3-methylglutaryl-CoA reductase- and 8 anti-signal recognition particle-positive patients. Seven (63.6%) of these patients achieved clinically significant responses according to the 2016 ACR–EULAR myositis response criteria. Responders had higher baseline serum IL-6 and muscle IL-6 mRNA levels and percentage of CD56-positive muscle fibers than non-responders. Baseline serum IL-6 levels and the percentage of CD56-positive muscle fibers were positively correlated with total improvement score after 6 months of TCZ treatment. Furthermore, muscle fiber necrosis and muscle fiber size variation decreased in repeated muscle biopsies in five responders.Conclusion: Patients with refractory IMNM may respond to TCZ. Baseline serum IL-6 and muscle IL-6 mRNA levels and the percentage of CD56-positive muscle fibers may predict the response to TCZ treatment in these patients.

We report a case involving a 73-year-old Saudi man diagnosed with dermatomyositis who subsequently developed Kaposi's sarcoma one month later. He had difficulty in rising from a chair and increased leg weakness while climbing stairs or walking. He was unable to comb his hair and had greater dysphagia with liquids than with solid foods. Laboratory tests showed elevated liver enzyme and creatine kinase levels. Right quadriceps muscle biopsy indicated inflammatory myopathy, which was consistent with adult dermatomyositis. We administered 1-g/day methylprednisolone intravenously for 3 days, followed by 60-mg oral prednisolone daily, which led to significant improvements in his muscle strength and dysphagia symptoms. The creatine phosphokinase level normalized. Computed tomography and endoscopic examinations did not reveal any evidence of an underlying malignancy. The patient was discharged and underwent close monitoring at a rheumatology clinic. We subsequently added 50-mg oral azathioprine daily to the treatment regimen. At one month after emergency room presentation, multiple new dusky violaceous macules and plaques appeared on his chest, face, upper back, neck, and upper limbs. A biopsy of one of these lesions showed findings consistent withKaposi's sarcoma. He was referred to an oncology center for chemotherapy, following which his skin lesions significantly regressed. Dermatomyositis may be considered a paraneoplastic syndrome of Kaposi's sarcoma.

Dermatomyositis is a clinically heterogenous inflammatory myopathy with unique cutaneous features. Myositis-specific antibodies can aid in diagnosis and anticipation of patient prognosis. Herein, we report a 22-year-old man who presented with multifocal erythematous plaques with violaceous papules on his bilateral elbows, neck, and face. He was diagnosed with biopsy-proven dermatomyositis and determined to be seropositive for nuclear matrix protein 2 antibody (NXP-2). He was treated with systemic corticosteroids, then intravenous methylprednisolone and azathioprine, and ultimately achieved greatest treatment response with intravenous immune globulin therapy.

Purpose of review Myositis, or idiopathic inflammatory myopathy, is an overarching concept that includes dermatomyositis, polymyositis, immune-mediated necrotizing myopathy and the antisynthetase syndrome. Glucocorticoids are still considered the mainstay of treatment of myositis but some patients require add-on immunosuppressive therapy because of insufficient response to glucocorticoids, relapses when glucocorticoids are tapered, or because they incur glucocorticoid-related side effects. Recent findings The goal of this article was to review (PubMed search from January 2019 through June 2020) the efficacy and safety of standard and novel agents used in adult dermatomyositis, polymyositis, immune-mediated necrotizing myopathy and the antisynthetase syndrome. Summary Established therapies beyond glucocorticoids continue to have a major role in managing patients with myositis. In addition, novel agents are being tried for refractory manifestations of myositis.

Background Different inflammatory cells (i.e., CD4, CD8, CD20 and CD68) are involved in pathogenesis of DM muscle. In this context, the aim of this study was to assess and compare these inflammatory cell phenotyping in muscle samples of treatment naive juvenile and adult patients with dermatomyositis. Methods This is a cross-sectional study, in which 28 untreated juvenile and 28 adult untreated dermatomyositis patients were included. Immunohistochemical analysis was performed on serial frozen muscle sections. Inflammatory cell phenotyping was analyzed quantitatively in endomysium, perimysium, and perivascular (endomysium and perimysium) area. Results Mean age at disease onset was 7.3 and 42.0 years in juvenile and adult dermatomyositis, respectively. Both groups had comparable time duration from symptom’s onset to biopsy performance. CD4 and CD8 positive cells distributions were similar in both groups in all analyzed area, except for more predominance of CD4 in perimysium at juvenile muscle biopsies. The CD20 and CD68 positive cells were predominantly observed in adult muscle biopsy sections, when compared to juvenile samples, except for similar distribution of CD20 in perivascular endomysium, and CD68 in perimysium. Conclusions These data show that the differences between juvenile and adult dermatomyositis may be restricted not only to patients’ age, but also to different inflammatory cell distribution, particularly, in new-onset disease. Further studies are necessary to confirm the present study data and to analyze meaning of the different inflammatory cell phenotyping distribution finding in these both diseases.

Presence of the characteristic heliotrope rash on eyelids, violaceus rash in sun exposed areas and the Gottron papules on knuckles, is necessary for the diagnosis of juvenile dermatomyositis Symptomatic lung diseases and association with underlying malignancy are more common in adult dermatomyositis but not juvenile dermatomyositis Calcinosis cutis and cutaneous ulcerations are seen up to 30% cases of juvenile dermatomyositis Intravenous immunoglobulin, cyclophosphamide, and mycophenolate mofetil are first-line immunosuppressive therapy in juvenile dermatomyositis Intensive physiotherapy and strict photoprotection are essential ellements in treatment of dermatomyositis

ObjectivesTo study the effects of abatacept on disease activity and on muscle biopsy features of adult patients with dermatomyositis (DM) or polymyositis (PM).MethodsTwenty patients with DM (n=9) or PM (n=11) with refractory disease were enrolled in a randomised treatment delayed-start trial to receive either immediate active treatment with intravenous abatacept or a 3 month delayed-start. The primary endpoint was number of responders, defined by the International Myositis Assessment and Clinical Studies Group definition of improvement (DOI), after 6 months of treatment. Secondary endpoints included number of responders in the early treatment arm compared with the delayed treatment arm at 3 months. Repeated muscle biopsies were investigated for cellular markers and cytokines.Results8/19 patients included in the analyses achieved the DOI at 6 months. At 3 months of study, five (50%) patients were responders after active treatment but only one (11%) patient in the delayed treatment arm. Eight adverse events (AEs) were regarded as related to the drug, four mild and four moderate, and three serious AEs, none related to the drug. There was a significant increase in regulatory T cells (Tregs), whereas other markers were unchanged in repeated muscle biopsies.ConclusionsIn this pilot study, treatment of patients with DM and PM with abatacept resulted in lower disease activity in nearly half of the patients. In patients with repeat muscle biopsies, an increased frequency of Foxp3+ Tregs suggests a positive effect of treatment in muscle tissue.

Objective: The objective was to describe the methodology used to develop new response criteria for adult DM/PM and JDM.Methods: Patient profiles from prospective natural history data and clinical trials were rated by myositis specialists to develop consensus gold-standard ratings of minimal, moderate and major improvement. Experts completed a survey regarding clinically meaningful improvement in the core set measures (CSM) and a conjoint-analysis survey (using 1000Minds software) to derive relative weights of CSM and candidate definitions. Six types of candidate definitions for response criteria were derived using survey results, logistic regression, conjoint analysis, application of conjoint-analysis weights to CSM and published definitions. Sensitivity, specificity and area under the curve were defined for candidate criteria using consensus patient profile data, and selected definitions were validated using clinical trial data.Results: Myositis specialists defined the degree of clinically meaningful improvement in CSM for minimal, moderate and major improvement. The conjoint-analysis survey established the relative weights of CSM, with muscle strength and Physician Global Activity as most important. Many candidate definitions showed excellent sensitivity, specificity and area under the curve in the consensus profiles. Trial validation showed that a number of candidate criteria differentiated between treatment groups. Top candidate criteria definitions were presented at the consensus conference.Conclusion: Consensus methodology, with definitions tested on patient profiles and validated using clinical trials, led to 18 definitions for adult PM/DM and 14 for JDM as excellent candidates for consideration in the final consensus on new response criteria for myositis.

Objective. The aim was to assess environmental factors associated with disease flare in juvenile and adult dermatomyositis (DM). Methods. An online survey of DM patients from the USA and Canada examined smoking, sun exposure, infections, medications, vaccines, stressful life events and physical activity during the 6 months before flares, or in the past 6 months in patients without flares. Differences were evaluated by χ2 and Fisher’s exact tests, and significant univariable results were examined in multivariable logistic regression. Residential locations before flare were correlated with the National Weather Service UV index. Results. Of 210 participants (164 juvenile and 46 adult DM), 134 (63.8%) experienced a disease flare within 2 years of the survey. Subjects more often reported disease flare after sun exposure [odds ratio (OR) = 2.0, P = 0.03], although use of photoprotective measures did not differ between those with and without flare. Urinary tract infections (OR = 16.4, P = 0.005) and gastroenteritis (OR = 3.2, P = 0.04) were more frequent in the preceding 6 months in those who flared. Subjects who flared recently used NSAIDS (OR = 3.0, P = 0.0003), blood pressure medicines (OR = 3.5, P = 0.049) or medication for depression or mood changes (OR = 12.9, P = 0.015). Moving to a new house (OR = 10.3, P = 0.053) was more common in those who flared. Only sun exposure (OR = 2.2) and NSAIDs (OR = 1.9) were significant factors in multivariable analysis. Conclusion. Certain classes of environmental agents that have been associated with the initiation of DM, including sun exposure and medications, may also play a role in disease flares.

Dermatomyositis (DM) is an uncommon autoimmune disease that primarily affects the skin, muscle, and/or lungs, and remains a therapeutic challenge. We discuss recent studies evaluating efficacy of conventional treatments for clinically amyopathic DM (CADM), DM-associated interstitial lung (ILD) disease, and classic DM (CDM). We highlight several emerging new therapies with a focus on clinical trials, systematic reviews, and case series in the last 5 years. Recent studies report a significant number of patients remain refractory to antimalarials and require second- and third-line agents. Effective treatment for DM-associated ILD can vary based on patient specific antibodies. CDM requires oral glucocorticoids; recent studies have evaluated the benefits of adjunctive therapies including methotrexate and calcineurin inhibitors. New therapies target cell populations or cytokines thought to drive disease pathogenesis. Dermatomyositis is an autoimmune disease that remains challenging to treat. Many patients are refractory to conventional therapies, warranting the development and evaluation of new treatments.

Objective Published predictive models of disease outcomes in idiopathic inflammatory myopathies (IIMs) are sparse and of limited accuracy due to disease heterogeneity. Computational methods may address this heterogeneity by partitioning patients based on clinical and biological phenotype. Methods To identify new patient groups, we applied similarity network fusion (SNF) to clinical and biological data from 168 patients with myositis (64 adult polymyositis [PM], 65 adult dermatomyositis [DM], and 39 juvenile DM [JDM]) in the Rituximab in Myositis trial. We generated a sparse proof-of-concept bedside classifier using multinomial regression and identified characteristics that distinguished these groups. We conducted χ2 tests to link new patient groups with the myositis subtypes. Results SNF identified five patient groups in the discovery cohort that subdivided the myositis subtypes. The sparse multinomial regressor to predict patient group assignments (areas under the receiver operating characteristic curve = [0.78, 0.97]; areas under the precision-recall curve = [0.55, 0.96]) found that autoantibody enrichment defined four of these groups: anti-Mi-2, anti-signal recognition peptide (SRP), anti-nuclear matrix protein 2 (NXP2), and anti-synthetase (Syn). Depletion of immunoglobulin M (IgM) defined the fifth group. Each group was associated with one subtype, with adult DM being associated with anti-Mi-2 and anti-Syn autoantibodies, JDM being associated with anti-NXP2 autoantibodies, and adult PM being associated with IgM depletion and anti-SRP autoantibodies. These associations enabled us to further resolve the current myositis subtypes. Conclusion Using unsupervised machine learning, we identified clinically and biologically homogeneous groups of patients with IIMs, forming the basis of an integrated disease classification based on both clinical and biological phenotype, thus validating other approaches and what has been previously described.

International audience; Objective: Anti-transcription intermediary factor 1γ (anti-TIF1γ) antibodies are the main predictors of cancer in dermatomyositis (DM). Yet, a substantial proportion of anti-TIF1γ-positive DM patients do not develop cancer. This study was undertaken to identify biomarkers to better evaluate the risk of cancer and mortality in DM.Methods: This multicenter study was conducted in adult anti-TIF1γ-positive DM patients from August 2013 to August 2017. Anti-TIF1γ autoantibody levels and IgG subclasses were identified using a newly developed quantitative immunoassay. Age, sex, DM signs and activity, malignancy, and creatine kinase (CK) level were recorded. Risk factors were determined by univariate and multivariate analysis according to a Cox proportional hazards regression model.Results: Among the 51 adult patients enrolled (mean ± SD age 61 ± 17 years; ratio of men to women 0.65), 40 (78%) had cancer and 21 (41%) died, with a mean ± SD survival time of 10 ± 6 months. Detection of anti-TIF1γ IgG2 was significantly associated with mortality (P = 0.0011) and occurrence of cancer during follow-up (P < 0.0001), with a 100% positive predictive value for cancer when the mean fluorescence intensity of anti-TIF1γ IgG2 was >385. None of the patients developed cancer after 24 months of follow-up. Univariate survival analyses showed that mortality was also associated with age >60 years (P = 0.0003), active DM (P = 0.0042), cancer (P = 0.0031), male sex (P = 0.011), and CK level >1,084 units/liter (P = 0.005). Multivariate analysis revealed that age >60 years (P = 0.015) and the presence of anti-TIF1γ IgG2 (P = 0.048) were independently associated with mortality.Conclusion: Our findings indicate that anti-TIF1γ IgG2 is a potential new biomarker of cancer that should be helpful in identifying the risk of mortality in anti-TIF1γ-positive DM patients.

International audience; Dermatomyositis pathophysiology is complex. In recent years, medical research has identified molecules associated with disease activity. Besides providing insights into the driving mechanisms of dermatomyositis, these findings could provide potential biomarkers. Activity markers can be used to monitor disease activity in clinical trials and may also be useful in daily practice. This article reviews molecules that could be used as biomarkers for diagnosis and monitoring dermatomyositis disease activity.

Objective.To identify differentially expressed microRNA (miRNA) in muscle biopsies (MBx) from 15 untreated children with juvenile dermatomyositis (JDM) compared with 5 controls.Methods.Following MBx miRNA profiling, differentially expressed miRNA and their protein targets were validated by quantitative real-time PCR (qRT-PCR) and immunological assay. The association of miRNA-10a and miRNA-10b with clinical data was evaluated, including Disease Activity Score (DAS), von Willebrand factor antigen (vWF:Ag), nailfold capillary end row loops, duration of untreated disease, and tumor necrosis factor (TNF)-α-308A allele.Results.In JDM, 16/362 miRNA were significantly differentially expressed [false discovery rate (FDR) < 0.05]. Among these, miRNA-10a was the most downregulated miRNA in both FDR and ranking of fold change: miRNA-10a = −2.27-fold, miRNA-10b = −1.80-fold. Decreased miRNA-10a and miRNA-10b expressions were confirmed using qRT-PCR: −4.16 and −2.59 fold, respectively. The qRT-PCR documented that decreased miRNA-10a expression was related to increased vascular cell adhesion molecule 1 in 13 of these JDM cases (correlation −0.67, p = 0.012), unlike miRNA-10b data (not significant). Concurrent JDM plasma contained increased levels of interleukin (IL) 6 (p = 0.0363), IL-8 (p = 0.0005), TNF-α (p = 0.0011), and monocyte chemoattractant proteins 1 (p = 0.0139). Decreased miRNA-10a, but not miRNA-10b, was associated with the TNF-α-308A allele (p = 0.015). In the 15 JDM, a trend of association of miRNA-10a (but not miRNA-10b) with vWF:Ag and DAS was observed.Conclusion.MiRNA-10a downregulation is an important element in untreated JDM muscle pathophysiology. We speculate that muscle miRNA expression in adult dermatomyositis differs from muscle miRNA expression in untreated childhood JDM.

Background:Juvenile dermatomyositis (JDM) is a systemic, autoimmune inflammatory muscle disorder and vasculopathy that affects children younger than 18 years. Although the cause of JDM remains unknown it is clear that genetic and environmental influences play a role in the aetiology. New treatments are becoming available and being tested through international multicentre trials. Increasing evidence suggests a role for types I and II IFN in juvenile and adult dermatomyositis, including elevated IFN-response gene signatures in the muscle, skin and blood. It has recently been reported that patients with refractory JDM responded well to treatment with tofacitinib, a JAK inhibitor, with corresponding downregulation in selected IFN-response genes.Objectives:In this study, we evaluated our cases with resistant JDM who received tofacitinib treatment.Methods:Six patients who received tofacitinib because of severe skin involvement of JDM were included in the study. The data were obtained retrospectively from the hospital records.Results:The age ranges of the cases were between 7-17 years and the ratio of girls and boys was 1 (3/3). The age of diagnosis was between 2-13 years, and the follow-up period was between 3-9 years. Calcinosis cutis in 5 cases, decreased muscle strength in 3 cases, joint involvement in 4 cases were detected.. Systemic steroids, methotrexate, and non-steroid anti-inflammatory drugs were given in all cases before tofacitinib treatment. Pamidronate was used in 4 cases because of severe skin calcinosis, high dose intravenous immunoglobulin in 4 cases, mycophenolate mofetil in 3 cases, rituximab in 3 cases and cyclophosphamide in 1 case previously. Tofacitinib treatment (10mg/gün) was started in six cases with treatment-resistant JDM. Five cases had been treated with tofacitinib for 6-24 month intervals. The treatment was discontinued in one case because of severe allergic reaction. Variable level of improvement were detected in the skin findings of all cases during the therapy period. The treatment was interrupted for 1 month in only one case due to neutropenia.Conclusion:Tofacitinib seems to be an effective and safe treatment option in patients with JDM who are resistant to conventional treatments. More studies are needed on this subject.Disclosure of Interests:None declared

Background:Juvenile dermatomyositis (JDM) is a multisystem inflammatory disease of childhood with variable demographics, clinical features and outcomes. No studies have described the characteristics of JDM patients from Southeast Asia population.Objectives:To describe the clinical characteristics and outcomes of JDM patients in Singapore over a 20-year period.Methods:Patients diagnosed with JDM from 1999 to 2019 at KK Women’s and Children’s Hospital, Singapore, were recruited. Nonparametric descriptive statistics were used to described data. Kaplan-Meier analyses were used to estimate the probability of remission. Multivariate logistic and Cox regression analyses were used to determine predictors as appropriate. The significant level was set at < 0.05.Results:32 JDM were identified. Clinical characteristics and treatment used are shown in Table 1.All (n=32)Monophasic (n=17)Polyphasic (n=14)Male14 (43.8)7 (41.2)7 (50.0)Age at onset (yrs)*6.4 (4.5 – 9.8)5.4 (4.1 – 8.5)7.4 (5.6 – 12.3)Lag period (mo)*3.5 (1.0 – 12.5)2.0 (1.0 – 16.8)6.8 (1.2 – 15.8)Heliotrope16 (50)9 (52.9)7 (50)Gottron papule23 (71.9)12 (70.6)11 (78.6)Malar rash19 (61.3)9 (52.9)10 (71.4)Vasculitic rash19 (61.3)8 (47.1)11 (78.6)Arthritis10 (31.3)3 (17.6)7 (50)Nailfold changes28 (87.5)15 (88.2)13 (92.9)Calcinosis9 (28.1)4 (23.5)5 (35.7)Positive ANA17 (53.1)10 (58.8)7 (50)Positive Myositis antibodies4 (12.5)2 (11.8)2 (14.3)Laboratory at diagnosis, U/L* CK324 (134 – 2229)746 (139 – 2965)351 (155 – 2622) LDH650 (450 – 943)650 (398 – 1015)714 (512 – 944) ALT28 (16 – 106)35 (18 – 109)38 (15 – 104) AST50 (30 -108)50 (31 – 113)85 (36 – 215) Aldolase13.7 (8.1 – 28.0)14.2 (8.4 – 26.6)16.3 (8.6 – 38.5)CMAS score at diagnosis29 (21 – 43)28 (21 – 35)35 (11 – 43)*median (IQR), otherwise - n (%)Pulse methylprednisolone (pMP) was used in 53.1% of patients after diagnosis. Median time to inactive disease (ID) was 5.3 months (IQR 2.8 – 12.8). Male, older age and patients on pMP (p = 0.003-0.044) achieved ID sooner. Older patients also developed disease flare sooner after achieving ID (p =0.024). No clinical features nor lab investigations predicted JDM disease course. Malay patients was associated with higher risk of calcinosis (p = 0.017).Compared to adult dermatomyositis patients in Singapore1, our cohort had more cutaneous manifestations including malar rash, vasculitic rash and nailfold changes. Table 2 shows the time for each muscle enzymes to normalise.CK1.49 (0.69 – 3.53)LDH4.53 (2.35 – 25.04)ALT1.71 (0.90 – 4.13)AST0.97 (0.53 – 3.12)Aldolase3.12 (2.35 – 8.30)median in months (IQR)Conclusion:Our cohort of JDM patients had more calcinosis compared to other Asian population2. Malay population is at higher risk of this complication. It is crucial to achieve ID state in the shortest time possible to avoid significant morbidity. Our study suggests that early treatment with pMP is associated with shorter time to ID. There is no predictor identified for disease course, similar to previous studies3.References:[1]Liu, Wen Chun, et al. “An 11-year review of dermatomyositis in Asian patients.” Annals Academy of Medicine Singapore 39.11 (2010): 843.[2]Sun, Chi, et al. “Juvenile dermatomyositis: a 20-year retrospective analysis of treatment and clinical outcomes.” Pediatrics & Neonatology 56.1 (2015): 31-39.[3]Stringer, Elizabeth, Davinder Singh Grewal, and Brian M. Feldman. “Predicting the course of juvenile dermatomyositis: significance of early clinical and laboratory features.” Arthritis & Rheumatism: Official Journal of the American College of Rheumatology 58.11 (2008): 3585-3592.Disclosure of Interests:None declared

Idiopathic inflammatory myopathies (IIMs) happened to be the group of heterogeneous, systemic rheumatic diseases including adult polymyositis (PM), adult dermatomyositis (DM), myositis accompanied with another connective disease or cancer. A 52 years old male patient with known history of type 2 diabetes mellitus presented with complaints of muscle pain and swelling over left arm. These cases was successfully treated by using Corticosteroids. This cases study described the clinical presentation and features of inflammatory myositis. Although this is a rare case, its clinical features and treatment procedure helps in management of similar cases.

Objectives: Subcutaneous involvement, including calcinosis and panniculitis, is a more common complication in juvenile dermatomyositis (JDM) than in adult dermatomyositis. Magnetic resonance imagin...

Background: To analyze the frequency and clinical relevance of anti-Mi-2 autoantibody in a representative sample of patients with dermatomyositis. Methods: This longitudinal inception cohort study, from 2001 to 2017, included 87 definite adult dermatomyositis. Anti-Mi-2 analysis was performed using a commercial kit. Results: Seventeen patients (19.5%) had anti-Mi-2 and 70 (80.5%) did not have this autoantibody. The following parameters were equally distributed between the patients with versus without anti-Mi-2: mean age at the disease diagnosis onset, median follow-up time, constitutional symptoms (baseline), cutaneous cumulative lesions, dysphagia, joint and pulmonary involvement. There was also no difference between the groups in relation to follow-up time, disease relapsing, treatment, disease status, deaths and occurrence of neoplasia. In contrast, patients with anti-Mi2 antibodies had higher frequency of elevated serum levels of muscle enzymes at disease onset (median: creatine phosphokinase 6240 [3800-9148] U/L and aldolase 60.0 [35.0-138.0] U/L), lower frequency of pulmonary involvement at disease onset (5.9%), less current glucocorticoid dose (median: 0 [0-10] mg/day), and higher frequency of disease remission during follow-up (58.8%) in comparison with patients without anti-Mi-2 autoantibody (484 [115-4880] and 12.1 [6.3-70.0] U/L, 40.0%, 0 [0-10] mg/day, 27.1%, respectively). Conclusion: The anti-Mi-2 autoantibody was found in one fifth of patients with dermatomyositis. This autoantibody was associated with a lower occurrence of pulmonary involvement, a higher frequency of disease in remission, and elevated levels of muscle enzymes. There was also no correlation regarding the frequency of disease relapsing or neoplasia development.

Background: Idiopathic inflammatory myopathies (IIM) is a heterogeneous group of chronic, inflammatory diseases with varying response to treatment. To date there is no biomarker to predict such response. Autoantibodies are found in up to 80% of patients with IIM; myositis associated (MAA) and myositis specific antibodies (MSA) are associated with certain clinical features. Whether MSAs can be used as biomarkers to predict response to treatment is unknown. Objectives: To investigate predictors of response according to the New ACR/EULAR 2016 response criteria for idiopathic inflammatory myositis using a national rheumatology register. Methods: Patients with IIM who were included in the Swedish Rheumatology Quality Register (SRQ) between January 1st, 2003 and December 31st, 2015 within 12 months of diagnosis were included in this study. Response was assessed at the visit registered closest to one year after inclusion. IIM subgroup (dermatomyositis (DM), polymyositis (PM) and overlap myositis), autoantibody profile, time to diagnosis and treatment were categorized into dichotomous variables. Time to diagnosis (from firts symptoms) less than 3 months was considered as early; initial dose of glucocorticoids was considered high when >45 mg. The ACR/EULAR 2016 criteria for Clinical Response were applied to measure response, and it was categorized in minimal (20–39/100), moderate (40–59/100) and major (>60/100) response. The association between response and clinical characteristics was assessed by multinomial logistic regression. Results: 179 patients were included. Median age at inclusion was 58.9 years (Interquartile range (IQR) 46.7–68.6), 65% were female and 49% had ever smoked. Thirty-three percent had DM, 45% PM and 22% overlap myositis. Lung disease was present in 37% and cancer within ±3 years from IIM diagnosis was present in 13%. Ninety-one percent were given glucocorticoid treatment, 72% immunosuppressive drugs, 22% cyclophosphamide and 13% a biological drug. Response rates. Two thirds (62%) were responders, 23% had minimal, 20% moderate and 18% major response. Minimal response was associated with high initial glucocorticoid dose (OR 3.4, CI95% 1.4–8.1); moderate response to high initial glucocorticoid dose (4.8 CI95% 1.9–11.8) and major response to early diagnosis (OR 3.9 CI95% 1.3–11.9), and high initial glucocorticoids dose (OR 9.5 CI95% 3.0–29.8). No associations between IIM subgroup, autoantibody profile and response rates were observed. Conclusions: Early and intensive treatment with high doses of glucocorticoids was associated with high rates of clinical response. These data suggest that early intensive immunosupressive treatment is important in IIM. References 1. Betteridge Z, McHugh N. Myositis-specific autoantibodies: an important tool to support diagnosis of myositis. J Intern Med2016;280:8–23. 2. Aggarwal R, et al. 2016American College of Rheumatology/European League Against Rheumatism criteria for minimal, moderate and major clinical response in adult dermatomyositis and polymyositis. Ann Rheum Dis2017;76:792–801. Acknowledgements: Borje Dahlin Foundation. Disclosure of Interest: None declared

AIM To assess the frequency and risk factors of asymptomatic vertebral fractures in inflammatory myositis. PATIENTS AND METHODS Dorsal and lumbar spine lateral radiographs were taken for adults with inflammatory myositis and scored using Genant's semi-quantitative technique. Demographic data, weight, height, postmenopausal status, duration of corticosteroid use, drug intake, co-morbidities and past history of fractures were recorded. Bone mineral density (BMD) was assessed using dual-energy X-ray absorptiometry (DEXA). Myositis Damage Index (MDI) was also assessed. All results are expressed in median and interquartile range. RESULTS One hundred patients (82 female) with myositis of median age 35.5 (28.5-46) years and disease duration 3.0 (1.81-8.0) years were studied. Thirty-five patients had adult dermatomyositis (DM), 26 polymyositis, 31 connective tissue disease-associated myositis and eight had juvenile onset myositis. Seventeen were postmenopausal women. Forty-six patients had asymptomatic vertebral fractures and 19 had more than one fracture. Half the fractures occurred in those with disease duration of

Objective: To investigate the possible association between HLA and Dermatomyositis (DM) in the Mexican mestizo population. Methods: HLA class I (A and B) and class II (DRB1* and DQB1*) were determined in 36 Mexican mestizo patients with DM and 72 healthy controls. Results: A positive association was identified among alleles HLA A*01:01 (OR 3.5 (1.30-9.54), p

Autoimmune muscle diseases (myositis) comprise a group of complex phenotypes influenced by genetic and environmental factors. To identify genetic risk factors in patients of European ancestry, we conducted a genome-wide association study (GWAS) of the major myositis phenotypes in a total of 1710 cases, which included 705 adult dermatomyositis; 473 juvenile dermatomyositis; 532 polymyositis; and 202 adult dermatomyositis, juvenile dermatomyositis or polymyositis patients with anti-histidyl tRNA synthetase (anti-Jo-1) autoantibodies, and compared them with 4724 controls. Single-nucleotide polymorphisms showing strong associations (P < 5 × 10−8) in GWAS were identified in the major histocompatibility complex (MHC) region for all myositis phenotypes together, as well as for the four clinical and autoantibody phenotypes studied separately. Imputation and regression analyses found that alleles comprising the human leukocyte antigen (HLA) 8.1 ancestral haplotype (AH8.1) defined essentially all the genetic risk in the phenotypes studied. Although the HLA DRB1*03:01 allele showed slightly stronger associations with adult and juvenile dermatomyositis, and HLA B*08:01 with polymyositis and anti-Jo-1 autoantibody-positive myositis, multiple alleles of AH8.1 were required for the full risk effects. Our findings establish that alleles of the AH8.1haplotype comprise the primary genetic risk factors associated with the major myositis phenotypes in geographically diverse Caucasian populations.

Objective. Myositis-specific autoantibodies(MSAs) are useful tools for identifying clinical subsets of patients with idiopathic inflammatory myopathies(IIMs). There have been few reports on antibodies to some DNA mismatch repair enzymes (MMREs) inpatients with IIMs. This study was undertaken to determine the frequencies and clinical associations of antibodies to 7 types of MMREs (MLH1, MLH3, MSH2,MSH3, MSH6, PMS1, and PMS2) in patients with IIMs and other systemic autoimmune diseases.Methods. Clinical data and serum samples were collected from 239 Japanese patients with IIMs (147 with adult dermatomyositis, 13 with juvenile dermatomyositis,57 with polymyositis, and 22 with myositis overlap syndrome). One hundred patients with other diseases, including 40 with systemic lupus erythematosus(SLE), were assessed as disease controls. The presence of anti-MMRE antibodies in serum was examined by immunoprecipitation, enzyme-linked immunosorbenassay, and immunoprecipitation/Western blotting.Results. Anti-MMRE antibodies were found in 1 patients with IIMs and 3 patients with SLE. They were restricted to MLH1, PMS1, MSH2, and PMS2, with simultaneous positivity for more than one of these antibodies occurring in some patients. Nine IIM patients with anti-MMREs also had other MSAs and their associated clinical features. All patients with anti-MMREs were still living at the time of the present analysis.Conclusion. Anti-MMRE antibodies, which often co exist with other MSAs, may be serologic markers forgood prognosis in IIMs.