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1.
Rev Med Chil ; 147(3): 342-355, 2019 Mar.
Artigo em Espanhol | MEDLINE | ID: mdl-31344172

RESUMO

Idiopathic inflammatory myopathies (IIM) are a heterogeneous group of acquired immune-mediated diseases, which typically involve the striated muscle with a variable involvement of the skin and other organs. Clinically, they are characterized by proximal muscle weakness, elevation of muscle enzymes, myopathic changes on electromyography and an abnormal muscle biopsy. The different IIM have been classified according to their distinctive histopathologic features in dermatomyositis (DM), polymyositis (PM), inclusion body myositis (IBM) and immune-mediated necrotizing myopathy (IMNM). Several myositis-specific antibodies are associated with the different phenotypes, as well as with different risk of neoplastic disease and systemic complications. The basis for the treatment of DM, PM, and IMNM is immunosuppression. For IBM there are only symptomatic treatments. Steroids, associated or not with other immunosuppressant drugs, are the first line of treatment. Biologic drugs will allow future individualized therapies. The 10-year survival of DM, PM and IMNM is 62 to 90%. The leading causes of death are neoplastic, lung and cardiac complications. IBM does not impair survival, although it affects the quality of life.


Assuntos
Miosite/patologia , Anticorpos , Dermatomiosite/patologia , Eletromiografia , Humanos , Imunossupressores/classificação , Imunossupressores/uso terapêutico , Músculo Esquelético/patologia , Miosite/tratamento farmacológico , Polimiosite/patologia
2.
Muscle Nerve ; 60(3): 315-327, 2019 09.
Artigo em Inglês | MEDLINE | ID: mdl-31172530

RESUMO

INTRODUCTION: The molecular mechanism of immune-mediated necrotizing myopathy (IMNM) remains unknown. Autophagy impairment, described in autoimmune diseases, is a key process in myofiber protein degradation flux and muscle integrity and has not been studied in IMNM. METHODS: Muscle biopsies from patients with IMNM (n = 40), dermatomyositis (DM; 24), polymyositis (PM; 8), polymyositis with mitochondrial pathology (4), sporadic inclusion body myositis (8), and controls (6) were compared by immunohistochemistry. RESULTS: The proportions of myofibers containing autophagy markers LC3b and p62 were higher in IMNM than in DM or PM and correlated with creatine kinase levels. In IMNM, compartmentalized LC3b puncta were located in regenerating and degenerating myofibers surrounded by major histocompatibility complex type II+ inflammatory cells. Several IMNM myofibers accumulated ubiquitin and misfolded protein. DISCUSSION: The detection of LC3b+ or p62+ myofibers could be used in differentiating IMNM from PM. The identification of autophagy-modifying molecules potentially could improve patients' outcomes. Muscle Nerve, 2019.


Assuntos
Autofagia/imunologia , Músculo Esquelético/patologia , Miosite/imunologia , Miosite/patologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Doenças Autoimunes/imunologia , Doenças Autoimunes/patologia , Biópsia , Dermatomiosite/patologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Miosite de Corpos de Inclusão/patologia , Polimiosite/imunologia , Polimiosite/patologia
3.
Intern Med ; 58(18): 2689-2693, 2019 Sep 15.
Artigo em Inglês | MEDLINE | ID: mdl-31178484

RESUMO

A 69-year-old Japanese woman presented with mild muscle weakness of the neck and symmetrical proximal parts of the upper and lower limbs. Laboratory tests, needle electromyography, and a muscle biopsy revealed inflammatory myopathy with an apparent clinical classification of polymyositis and positive findings for anti-PM/Scl-75 antibody. This antibody is rare among Japanese populations, and most Japanese patients with the antibody are not classified with the inflammatory myopathy seen in polymyositis. The muscle biopsy also showed marked necrotic and regenerative fibers. We need to collectively investigate patients with the potential to develop this disease, and to identify any unique characteristics for Asian populations, including Japanese.


Assuntos
Autoanticorpos/imunologia , Complexo Multienzimático de Ribonucleases do Exossomo/imunologia , Polimiosite/imunologia , Proteínas de Ligação a RNA/imunologia , Idoso , Grupo com Ancestrais do Continente Asiático , Biópsia , Eletromiografia , Feminino , Humanos , Japão , Imagem por Ressonância Magnética , Debilidade Muscular , Miosite/diagnóstico por imagem , Miosite/imunologia , Miosite/patologia , Polimiosite/diagnóstico por imagem , Polimiosite/patologia
4.
Medicine (Baltimore) ; 98(20): e15578, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-31096460

RESUMO

Myositis-specific autoantibodies, such as anti-melanoma differentiation associated gene 5 (MDA5) and anti-anti-amino acyl-tRNA synthetases (ARS) antibodies, are associated with interstitial lung diseases (ILD), which determine the prognosis of polymyositis/dermatomyositis (PM/DM) patients. However, there is a paucity of data on the clinical correlation between anti-Sjögren syndrome-related antigen A (anti-SSA)/Ro52 antibodies in PM/DM. We investigated the prevalence of myositis-specific autoantibodies including anti-SSA/Ro52 antibody and assessed the clinical significance of these antibodies in patients with PM/DM.We retrospectively reviewed demographic data and clinical outcomes in patients with PM/DM. The study population comprised 24 patients with PM and 60 patients with DM. The presence of anti-myositis-specific antibodies (MDA5, ARS, Jo-1, SSA/Ro52) was determined by immunosorbent assay (ELISA).Anti-MDA5 antibody was detected in 18 patients with DM (n = 60). Anti-ARS/anti-SSA/Ro52 antibodies were detected in 31 and 39 patients with PM/DM (n = 84). Rapidly progressive ILD patients were mainly found in the anti-MDA5 antibody-positive DM group. During the follow-up period, 9 patients died. Kaplan-Meier analysis demonstrated that survival rates seem to be lower in DM patients with anti-MDA5 antibodies compared with those without anti-MDA5 antibodies. Furthermore, dual positivity for anti-SSA/Ro52 and anti-MDA5 antibodies was significantly higher in nonsurviving DM patients compared with survivors.Although the presence of anti-ARS or anti-MDA5 antibodies is a prognostic marker in patients with PM/DM, combined presence of anti-SSA/Ro52 and anti-MDA5 antibodies represent another marker for clinical outcome in DM patients. Our results suggest that anti-SSA/Ro52 antibody positivity in DM patients with anti-MDA5 antibody reveals a subgroup of DM patients with poor prognosis.


Assuntos
Autoanticorpos/sangue , Polimiosite/imunologia , Adulto , Idoso , Anticorpos Antinucleares/sangue , Antígenos Ly/sangue , Biomarcadores , Dermatomiosite/imunologia , Feminino , Humanos , Helicase IFIH1 Induzida por Interferon/sangue , Japão , Masculino , Pessoa de Meia-Idade , Fenótipo , Polimiosite/sangue , Polimiosite/patologia , Prognóstico , Estudos Retrospectivos , Ribonucleoproteínas/sangue , Fatores Socioeconômicos , Taxa de Sobrevida , Ativador de Plasminogênio Tipo Uroquinase/sangue
5.
Clin Rheumatol ; 38(5): 1425-1431, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30645753

RESUMO

OBJECTIVES: Tumor necrosis factor-related apoptosis-inducing ligand (TRAIL) is a member of the TNF super-family, which is involved in the regulation of immune response and pathogenesis of autoimmune diseases, including polymyositis (PM) and dermatomyositis (DM). In this study, we examined the level and origin of serum-soluble TRAIL (sTRAIL) in patients with PM and DM and analyzed its association with disease activity and clinical features. METHOD: 11 PM patients, 33 DM patients, and 20 healthy controls were enrolled in this study. Clinical features were recorded when admitted, and disease activity was evaluated by myositis disease activity assessment visual analogue scale (MYOACT). TRAIL expression in muscle tissues was detected by immunohistochemistry. Serum sTRAIL levels were measured by enzyme-linked immunosorbent assay. The expression of membrane TRAIL (mTRAIL) and its receptors, including DR4 and DR5, on circulating T cells was analyzed by flow cytometry. RESULTS: TRAIL was expressed in infiltrated inflammatory cells in muscle tissues from patients. The serum sTRAIL level was markedly increased in patients and was positively correlated with the disease activity. Serum sTRAIL was decreased after therapy in patients and was specifically higher in patients with dysphagia, but lower in patients with autoantibody Jo-1 positive. The frequency of mTRAIL and its receptors on circulating T cells from patients were significantly elevated than that from healthy controls. CONCLUSIONS: The serum sTRAIL could be a biomarker for evaluating the disease activity of PM and DM, and targeting the generation of TRAIL in T cells might be a potential approach in the treatment of PM and DM.


Assuntos
Dermatomiosite/sangue , Polimiosite/sangue , Linfócitos T/metabolismo , Ligante Indutor de Apoptose Relacionado a TNF/sangue , Adolescente , Adulto , Biomarcadores/sangue , Estudos de Casos e Controles , China , Dermatomiosite/patologia , Progressão da Doença , Ensaio de Imunoadsorção Enzimática , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Polimiosite/patologia , Adulto Jovem
6.
Neurol Neuroimmunol Neuroinflamm ; 6(2): e535, 2019 03.
Artigo em Inglês | MEDLINE | ID: mdl-30697585

RESUMO

Objective: To provide evidence that idiopathic inflammatory myopathy (IM) with myasthenia gravis (MG) frequently shows thymoma association and polymyositis (PM) pathology and shares clinicopathologic characteristics with IM induced by immune checkpoint inhibitors (ICIs). Methods: We analyzed the clinicopathologic features of 10 patients with idiopathic IM and MG identified in 970 consecutive patients with biopsy-proven IM. Results: Seven patients (70%) had thymoma. IM and MG were diagnosed with more than 5-year time difference in 6 thymomatous patients and within 1 year in 1 thymomatous and 3 nonthymomatous patients. Seven thymomatous patients showed rhabdomyolysis-like features with respiratory failure (4/7), dropped head (3/7), cardiac involvement (2/7), and positive anti-acetylcholine receptor (anti-AChR) and anti-titin antibodies (7/7 and 4/6, respectively) but rarely showed ocular symptoms (2/7) or decremental repetitive nerve stimulation (RNS) responses (1/7) at IM diagnosis. Three nonthymomatous patients showed acute cardiorespiratory failure with rhabdomyolysis-like features (1/3), positive anti-AChR and anti-titin antibodies (3/2 and 2/2, respectively), and fluctuating weakness of the skeletal muscle without ocular symptoms (3/3). Muscle pathology showed a PM pathology with infiltration of CD8-positive CD45RA-negative T-lymphocytes (9/9), scattered endomysial programmed cell death 1 (PD-1)-positive cells (9/9), and overexpression of programmed cell death ligand 1 (PD-L1) on the sarcolemma of muscle fibers around the infiltrating PD-1-positive cells (7/9). Conclusion: Rhabdomyolysis-like features, positive anti-AChR antibody without decremental RNS responses, and PD-L1 overexpression are possible characteristics shared by ICI-induced IM. Frequent thymoma association in patients with idiopathic IM and MG may suggest thymoma-related immunopathogenic mechanisms, including dysregulation of the immune checkpoint pathway.


Assuntos
Miastenia Gravis/complicações , Miosite/complicações , Polimiosite/complicações , Timoma/complicações , Neoplasias do Timo/complicações , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Miastenia Gravis/diagnóstico , Miastenia Gravis/patologia , Miosite/diagnóstico , Miosite/patologia , Polimiosite/diagnóstico , Polimiosite/patologia , Timoma/diagnóstico , Timoma/patologia , Neoplasias do Timo/diagnóstico , Neoplasias do Timo/patologia
7.
Muscle Nerve ; 59(5): 555-560, 2019 05.
Artigo em Inglês | MEDLINE | ID: mdl-30697788

RESUMO

INTRODUCTION: Short tau inversion recovery (STIR) sequences in whole-body MRI are usually used for detecting muscle edema (ME) in inflammatory myopathies. We evaluated b-value 800 diffusion-weighted imaging (b800 DWI). METHODS: Two radiologists independently and a consensus reader retrospectively reexamined 60 patients with inflammatory myopathies and 15 controls. For each participant, 78 muscles were analyzed with 3 sets of imaging acquisitions: T1-weighted (T1) turbo spin echo and STIR; T1 and DWI; and T1, STIR and DWI. Mean edema per patient was compared between sequences. Agreement was evaluated. RESULTS: Diffusion-weighted imaging detected more ME compared with STIR (P < 0.001). Agreement between readers was better with both sequences (k = 0.94) than with b800 DWI (k = 0.89) or STIR (k = 0.84) alone. DISCUSSION: Diffusion-weighted imaging is a valuable add-on for the study of inflammatory myopathies. Muscle Nerve 59:555-555, 2019.


Assuntos
Edema/diagnóstico por imagem , Músculo Esquelético/diagnóstico por imagem , Miosite/diagnóstico por imagem , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Estudos de Casos e Controles , Creatina Quinase/sangue , Dermatomiosite/sangue , Dermatomiosite/diagnóstico por imagem , Dermatomiosite/patologia , Imagem de Difusão por Ressonância Magnética , Feminino , Frutose-Bifosfato Aldolase/sangue , Humanos , Masculino , Pessoa de Meia-Idade , Músculo Esquelético/patologia , Miosite/sangue , Miosite/patologia , Miosite de Corpos de Inclusão/sangue , Miosite de Corpos de Inclusão/diagnóstico por imagem , Miosite de Corpos de Inclusão/patologia , Polimiosite/sangue , Polimiosite/diagnóstico por imagem , Polimiosite/patologia , Estudos Retrospectivos , Imagem Corporal Total , Adulto Jovem
8.
Epigenomics ; 11(1): 23-33, 2019 01.
Artigo em Inglês | MEDLINE | ID: mdl-30523707

RESUMO

AIM: Dermatomyositis (DM) and polymyositis (PM) are refractory systemic autoimmune diseases with unknown pathogenesis. miRNAs is an important epigenetic mechanism to regulate gene expression. METHODS: We performed whole miRNAs analysis, transcription analysis and the association between miRNAome and mRNAome. RESULTS: For transcription and miRNAs analysis, there were common and specific mRNAs and miRNAs in the muscles of DM and PM. Among them, the expression levels of miR-196a-5p and CPM were negatively correlated in PM, miR-193b-3p and NECAP2 were negatively correlated in DM and PM. Protein carboxypeptidase M (CPM) plays roles in the degradation of extracellular proteins and in the migration and invasion of cancer cells, and protein NECAP2 plays roles in adaptor protein AP-1-mediated fast recycling from early endosomes. The functions of them in the pathogenesis of DM/PM need further studies. CONCLUSION: Our study identified and confirmed differentially miRNAs and mRNAs in DM and PM. Our observations have laid the groundwork for further diagnostic and mechanistic studies of DM and PM.


Assuntos
Dermatomiosite/genética , Perfilação da Expressão Gênica , MicroRNAs/genética , Polimiosite/genética , RNA Mensageiro/genética , Transcriptoma , Biomarcadores , Biópsia , Estudos de Casos e Controles , Biologia Computacional/métodos , Dermatomiosite/metabolismo , Dermatomiosite/patologia , Regulação da Expressão Gênica , Redes Reguladoras de Genes , Estudo de Associação Genômica Ampla , Humanos , Mioblastos/metabolismo , Polimiosite/metabolismo , Polimiosite/patologia , Interferência de RNA , Reprodutibilidade dos Testes
9.
Med Sci (Paris) ; 34 Hors série n°2: 35-38, 2018 Nov.
Artigo em Francês | MEDLINE | ID: mdl-30418144

RESUMO

Dysimmune and inflammatory myopathies (DIMs) affect around 14/100,000 people worldwide. Based on immupour nopathological criteria, DIMs are divided in four groups: (1) polymyositis (PM)/inclusion body myositis (IBM), (2) dermatomyositis (DM), (3) immune-mediated necrotizing myopathies (IMNM) and (iv) overlapping myositis including anti-synthetase syndrome (ASS). ASS and PM/IBM are characterized by the activation of inflammation with lymphocytic infiltrations. Recently, we showed that an expression of the major histocompatibility complex class 2 (MHC2) was present in myofibers from ASS and IBM muscle biopsies. Interestingly, MHC2 expression is known to be stimulated by Interferon-gamma (IFNγ) in myogenic cells. LTCD8 cells, which are well-known producers of IFNγ, are commonly found in close vicinity to MHC2 positive myofibers. This inflammatory cytokine also inhibits myogenic differentiation in vitro by CIITA-myogenin interaction. The mechanisms involved in the lymphocyte-driven muscle toxicity in DIMs are unclear. The objectives of this project are to characterize IFNγ effects on the biology of human myogenic cells by morphological, molecular and cellular approaches. Then, we aim to investigate the role of IFNγ in these myopathies and its impact during muscular regeneration. In vitro preliminary studies have been performed using human and mouse myoblasts treated or not with IFNγ. Our results should lead to a better understanding of the role of IFNγ in the pathophysiology of DIMs, and would hopefully help identify new therapeutic targets.


Assuntos
Interferon gama/farmacologia , Músculo Esquelético/efeitos dos fármacos , Músculo Esquelético/patologia , Doenças Musculares/imunologia , Miosite/patologia , Dermatomiosite/patologia , Dermatomiosite/fisiopatologia , Antígenos de Histocompatibilidade Classe II/análise , Humanos , Interferon gama/fisiologia , Fibras Musculares Esqueléticas/química , Fibras Musculares Esqueléticas/imunologia , Doenças Musculares/patologia , Doenças Musculares/fisiopatologia , Miosite/fisiopatologia , Miosite de Corpos de Inclusão/patologia , Miosite de Corpos de Inclusão/fisiopatologia , Polimiosite/patologia , Polimiosite/fisiopatologia
11.
Clin Radiol ; 73(12): 1057.e13-1057.e18, 2018 12.
Artigo em Inglês | MEDLINE | ID: mdl-30172348

RESUMO

AIM: To explore the T2-mapping signal characteristics of the thigh muscles in patients with dermatomyositis/polymyositis (DM/PM) and to investigate the correlation between thigh muscle T2 values, clinical parameters, and serum creatinine kinase (CK). MATERIALS AND METHODS: Forty-two patients with DM/PM proven by diagnostic criteria were enrolled in the study along with 13 healthy control subjects. Both T2-mapping and conventional magnetic resonance imaging (MRI) images were obtained in the thigh musculature of all subjects. The T2 values of thigh muscles were compared between the DM/PM patients and control groups. Thirty-one DM/PM patients were evaluated with manual muscle testing (MMT) and serum CK levels. A Spearman correlation coefficient model was used to correlate the mean T2 values and clinical assessments. The Kruskal-Wallis test and receiver operating characteristic (ROC) curves were also utilised. p-Values <0.05 reflected statistical significance. RESULTS: The T2 value of all oedematous muscles was greater on average than that of the unaffected muscles of the DM/PM patients (p<0.05) and the muscles of healthy volunteers (p<0.05). The T2 value of unaffected muscles in DM/PM patients was also greater than that of the normal muscles in healthy volunteers (p<0.05). The area under the curves (AUCs) for T2 relaxation time values was 0.72 with respective sensitivity and specificity of 72.6% and 65.4%. The mean T2 relaxation time of the 31 patients group and the MMTs (p<0.05) was correlated without serum CK levels (p>0.05). CONCLUSION: T2 mapping is not only quantitatively used for subclinical muscle involvement in DM/PM, but also be used to demonstrate severity of damaged muscles in DM/PM.


Assuntos
Dermatomiosite/diagnóstico por imagem , Imagem por Ressonância Magnética , Músculo Esquelético/diagnóstico por imagem , Polimiosite/diagnóstico por imagem , Coxa da Perna/diagnóstico por imagem , Adulto , Dermatomiosite/patologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Músculo Esquelético/patologia , Polimiosite/patologia , Coxa da Perna/anatomia & histologia , Coxa da Perna/patologia , Adulto Jovem
12.
J Neuromuscul Dis ; 5(2): 109-129, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29865091

RESUMO

Inflammatory disorders of the skeletal muscle include polymyositis (PM), dermatomyositis (DM), (immune mediated) necrotizing myopathy (NM), overlap syndrome with myositis (overlap myositis, OM) including anti-synthetase syndrome (ASS), and inclusion body myositis (IBM). Whereas DM occurs in children and adults, all other forms of myositis mostly develop in middle aged individuals. Apart from a slowly progressive, chronic disease course in IBM, patients with myositis typically present with a subacute onset of weakness of arms and legs, often associated with pain and clearly elevated creatine kinase in the serum. PM, DM and most patients with NM and OM usually respond to immunosuppressive therapy, whereas IBM is largely refractory to treatment. The diagnosis of myositis requires careful and combinatorial assessment of (1) clinical symptoms including pattern of weakness and paraclinical tests such as MRI of the muscle and electromyography (EMG), (2) broad analysis of auto-antibodies associated with myositis, and (3) detailed histopathological work-up of a skeletal muscle biopsy. This review provides a comprehensive overview of the current classification, diagnostic pathway, treatment regimen and pathomechanistic understanding of myositis.


Assuntos
Músculo Esquelético/diagnóstico por imagem , Miosite/diagnóstico , Biópsia , Dermatomiosite/classificação , Dermatomiosite/diagnóstico , Dermatomiosite/patologia , Dermatomiosite/terapia , Gerenciamento Clínico , Eletromiografia , Humanos , Imagem por Ressonância Magnética , Músculo Esquelético/patologia , Músculo Esquelético/fisiopatologia , Miosite/classificação , Miosite/patologia , Miosite/terapia , Miosite de Corpos de Inclusão/classificação , Miosite de Corpos de Inclusão/diagnóstico , Miosite de Corpos de Inclusão/patologia , Miosite de Corpos de Inclusão/terapia , Polimiosite/classificação , Polimiosite/diagnóstico , Polimiosite/patologia , Polimiosite/terapia
13.
Discov Med ; 25(136): 75-83, 2018 02.
Artigo em Inglês | MEDLINE | ID: mdl-29579414

RESUMO

The inflammatory myopathies, which include dermatomyositis, polymyositis, and the immune-mediated necrotizing myopathies, are a heterogeneous group of autoimmune diseases that manifest with muscle, skin, or lung damage. Collectively, these autoimmune diseases result from loss of tolerance to a select group of self-antigens, although the precise mechanism through which this occurs is not known. Infection, malignancy, and certain medications including statins and the immune checkpoint inhibitors used in cancer therapy have been identified as potential immunologic triggers of the inflammatory myopathies. Some of these triggers are classically associated with specific myositis-specific autoantibodies (MSAs). The strong association between certain triggers and MSAs provides insights into how an immunologic event can lead to loss of tolerance to specific self-antigens, resulting in autoimmune disease. In this review, we discuss the proposed triggers of the inflammatory myopathies and their associations with MSAs, and provide insights into how these triggers may result in the inflammatory myopathies.


Assuntos
Antineoplásicos/efeitos adversos , Doenças Autoimunes , Dermatomiosite , Inibidores de Hidroximetilglutaril-CoA Redutases/efeitos adversos , Polimiosite , Animais , Antineoplásicos/uso terapêutico , Autoanticorpos/imunologia , Autoantígenos/imunologia , Doenças Autoimunes/induzido quimicamente , Doenças Autoimunes/imunologia , Doenças Autoimunes/patologia , Dermatomiosite/induzido quimicamente , Dermatomiosite/imunologia , Dermatomiosite/patologia , Humanos , Inibidores de Hidroximetilglutaril-CoA Redutases/uso terapêutico , Pulmão/imunologia , Pulmão/patologia , Músculos/imunologia , Músculos/patologia , Polimiosite/induzido quimicamente , Polimiosite/imunologia , Polimiosite/patologia , Pele/imunologia , Pele/patologia
14.
PLoS One ; 13(1): e0190411, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29293605

RESUMO

Recent studies have suggested that neutrophil-to-lymphocyte ratio (NLR) and C-reactive protein-to-albumin ratio (CAR) are emerging markers of disease activity and prognosis in patients with chronic inflammatory diseases, cardiovascular diseases, or malignancies. Therefore, we investigated the clinical significance and prognostic value of the NLR and CAR in adult patients with polymyositis and dermatomyositis. The medical records of 197 patients with newly diagnosed polymyositis/dermatomyositis between August 2003 and November 2016 were retrospectively reviewed. Survival and causes of death were recorded during an average 33-month observational period. Clinical and laboratory findings were compared between survivors and non-survivors. Using receiver operating characteristic curves, the NLR and CAR cut-off values for predicting survival were calculated. Univariate and multivariate analyses using Cox proportional hazard models were performed to identify factors associated with survival. Twenty-six patients (13.2%) died during the study period, and the 5-year survival-rate was estimated to be 82%. The non-survivor group exhibited older age and a higher prevalence of interstitial lung disease (ILD), acute interstitial pneumonia, and acute exacerbation of ILD compared to that in the survivor group. NLR and CAR values were significantly higher in the non-survivors and in patients with polymyositis/dermatomyositis-associated ILD, and the death rates increased across NLR and CAR quartiles. Furthermore, when stratified according to the NLR or CAR optimal cut-off values, patients with a high NLR (>4.775) or high CAR (>0.0735) had a significantly lower survival rate than patients with low NLR or CAR, respectively. In addition, old age (>50 years), the presence of acute interstitial pneumonia, hypoproteinemia (serum protein <5.5 g/dL), and high NLR (but not high CAR) were independent predictors for mortality. The results indicate that a high NLR is independently associated with worse overall survival. Thus, the baseline NLR level may be a simple, cost-effective prognostic marker in patients with polymyositis/dermatomyositis.


Assuntos
Dermatomiosite/sangue , Linfócitos/patologia , Neutrófilos/patologia , Polimiosite/sangue , Adulto , Dermatomiosite/patologia , Feminino , Humanos , Masculino , Polimiosite/patologia , Estudos Retrospectivos , Análise de Sobrevida
16.
Arthritis Res Ther ; 19(1): 272, 2017 Dec 08.
Artigo em Inglês | MEDLINE | ID: mdl-29216907

RESUMO

BACKGROUND: We previously demonstrated that fasciitis is a common lesion in dermatomyositis (DM) and that DM-associated fasciitis is detectable, as the result of the increased vascularity in the fascia, by power Doppler ultrasonography. We aimed to investigate whether angiogenesis and vascular endothelial growth factor (VEGF)-expressing cells in the fascia are histologically demonstrated during the early phase of DM, and whether inflammation is involved in angiogenesis and an increased number of VEGF-expressing cells. METHODS: We prospectively evaluated 22 patients with DM and 11 patients with polymyositis (PM). Immunohistochemical staining for CD31, VEGF, and tumor necrosis factor-α (TNF-α) were performed on paraffin-embedded sections. The total vascular inflammation score (TVIS), angiogenesis score (AS), and numbers of VEGF-expressing and TNF-α-expressing cells were analyzed in the fascia and muscle. RESULTS: Significant fasciitis was detected in most of the patients DM with or without myositis-specific/associated antibodies, while mild fasciitis was detected in four patients with PM, two of whom were positive for anti-aminoacyl-tRNA synthetase (anti-ARS) antibodies. The AS and the number of VEGF-expressing cells in the fascia of patients with DM were significantly greater than those of patients with PM; no significant difference was observed in muscle in patients with DM and PM. The number of VEGF-expressing cells in the fascia correlated with the AS of DM patients. In early-phase DM, the AS, the number of VEGF-expressing cells, and the TVIS in the fascia were significantly higher than in muscle. However, no significant differences were observed in these scores excluding the TVIS between muscle and the fascia in late-phase DM. In DM patients, the TVIS correlated with the AS in the fascia, while the number of TNF-α-expressing cells correlated with the TVIS and the number of VEGF-expressing cells in the fascia. CONCLUSION: Angiogenesis, the number of VEGF-expressing cells, and the degree of inflammation were higher in the fascia in DM than in PM, and were increased predominantly in the fascia rather than in the muscle in early-phase DM. The degree of inflammation correlated with that of angiogenesis in the fascia of DM. The fascia can therefore be a primary site of inflammation and angiogenesis in the pathogenesis of DM.


Assuntos
Dermatomiosite/patologia , Fáscia/patologia , Fasciite/patologia , Neovascularização Patológica/patologia , Neovascularização Fisiológica/fisiologia , Fator A de Crescimento do Endotélio Vascular/biossíntese , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Polimiosite/patologia
18.
Autoimmun Rev ; 16(10): 1044-1048, 2017 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-28778709

RESUMO

Idiopathic inflammatory myopathies (IIM) are a group of rare and heterogeneous autoimmune diseases, and the most common subtypes are dermatomyositis (DM) and polymyositis (PM). Despite extensive efforts, the underlying mechanism of IIM remains unclear. Recent efforts to understand the pathogenesis of IIM have included genomics, epigenetics, transcriptomics, proteomics and autoantibody studies. This review focuses on recent studies in DM/PM research based on multi-omics. This integrated analysis of multi-omics profiling will provide useful insights into DM/PM pathogenesis and recommendations for therapeutic targets and biomarkers development.


Assuntos
Dermatomiosite/terapia , Metabolômica , Polimiosite/terapia , Dermatomiosite/patologia , Humanos , Polimiosite/patologia
19.
Intern Med ; 56(16): 2155-2158, 2017 Aug 15.
Artigo em Inglês | MEDLINE | ID: mdl-28781324

RESUMO

A 58-year-old man with a recent history of generalized myalgia and muscle weakness was transferred to our hospital because of acute progressive dyspnea. The patient underwent left ventricular (LV) assist device (LVAD) implantation due to cardiogenic shock with a LV ejection fraction (LVEF) of 6%. The histological findings obtained from LV apex showed the infiltration of multinucleated giant cells and severe myocardial contusion. Combining this histological finding with our experienced neurologists comments, resulted in a final diagnosis of fulminant giant cell myocarditis associated with polymyositis. A day after LVAD implantation, the patient received corticosteroid and immunosuppressive therapy, and the LVEF recovered to 68%.


Assuntos
Células Gigantes/patologia , Coração Auxiliar , Miocardite/terapia , Polimiosite/terapia , Quimioterapia Combinada , Glucocorticoides/uso terapêutico , Humanos , Imunossupressores/uso terapêutico , Masculino , Pessoa de Meia-Idade , Miocardite/complicações , Miocardite/patologia , Miocárdio/patologia , Polimiosite/complicações , Polimiosite/patologia , Indução de Remissão , Choque Cardiogênico/etiologia , Choque Cardiogênico/terapia , Função Ventricular Esquerda/fisiologia
20.
Neuromuscul Disord ; 27(9): 804-815, 2017 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-28687435

RESUMO

Acquired myasthenia gravis (MG) is relatively uncommon in cats. In humans, MG may be associated with other immune-mediated disorders, in particular polymyositis (PM). In this study, we described in-depth electrodiagnostic findings and pathological changes in muscles of cats diagnosed with MG, and assessed the presence of concurrent PM. Six cats with confirmed acetylcholine receptor antibody seropositive MG, and two suspected cases with clinical signs and electrophysiological changes consistent with MG, were reviewed. All animals presented with severe typical signs of generalized weakness and/or fatigability, resembling late-onset MG in humans, in addition to regurgitation. Five cats presented a cranial mediastinal mass, with 3 confirmed as thymoma. Repetitive nerve stimulation revealed a decrement of the compound muscle action potential in all tested cases, starting from low frequencies of stimulation. Serum creatine kinase activity was increased in 6/8 cats. Muscle biopsies performed in 5 cats revealed varying degrees of mixed mononuclear cell infiltrates, positive for the leukocyte markers CD3/CD4/CD8 and CD11b. Further MHC-1/C5b-9 positive sarcolemmal deposits were identified in all tested cases, with or without thymoma. This study documents an association of MG and PM in cats, and provides further support for feline MG as a relevant animal model of human MG.


Assuntos
Miastenia Gravis/complicações , Miastenia Gravis/veterinária , Polimiosite/complicações , Polimiosite/veterinária , Animais , Antígenos CD/metabolismo , Gatos , Creatina Quinase/sangue , Eletrodiagnóstico , Potencial Evocado Motor , Feminino , Masculino , Camundongos , Músculo Esquelético/metabolismo , Músculo Esquelético/patologia , Miastenia Gravis/patologia , Exame Neurológico , Exame Físico , Polimiosite/patologia
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