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1.
Dermatol Online J ; 26(2)2020 Feb 15.
Artigo em Inglês | MEDLINE | ID: mdl-32239891

RESUMO

Dermatomyositis is an auto-immune inflammatory myopathy that primarily affects the skin and muscle and can be triggered by exposure to various environmental factors. We present a patient with active syphilis infection who developed dermatomyositis and discuss the significance of anti-NXP2 autoantibody positivity.


Assuntos
Adenosina Trifosfatases/imunologia , Doenças Autoimunes/etiologia , Proteínas de Ligação a DNA/imunologia , Dermatomiosite/etiologia , Pele/patologia , Sífilis/complicações , Adulto , Autoanticorpos/sangue , Doenças Autoimunes/patologia , Dermatomiosite/imunologia , Dermatomiosite/patologia , Humanos , Masculino
2.
Medicine (Baltimore) ; 99(15): e19741, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-32282733

RESUMO

INTRODUCTION: Immune-related adverse events (ir-AEs) are increasingly becoming a concern, as immune checkpoint inhibitors (ICIs) are used more frequently. Herein, we present a case of fulminant cytokine release syndrome (CRS) complicated by dermatomyositis after the combination therapy with ICIs. PATIENT CONCERNS: A 70-year-old male developed dermatomyositis during the course of treatment with two ICIs, nivolumab and ipilimumab. He was treated by steroid pulse therapy, but the effect was limited. Afterwards, he had acute-onset high fever, hypotension, respiratory failure, impaired consciousness, renal failure, and coagulation abnormality at the same time. C reactive protein (CRP), creatinine kinase (CK), D-dimer, and ferritin levels were considerably elevated: CRP, 24 mg/dL; CK, 40,500 U/L; D-dimer, 290 µg/mL; ferritin, 329,000 ng/mL. DIAGNOSIS: CRS induced by ICI combination therapy. INTERVENTIONS: Given that high fever and elevated CRP level indicated potential sepsis, an antibiotic was used until the confirmation of negative blood cultures. All the simultaneous acute symptoms were supposed to be CRS. He was admitted to the intensive care unit (ICU), and temporary intubation and hemodialysis were needed. Immunosuppressive therapy was reinforced by mycophenolate mofetil together with steroid, and plasma exchange was performed for the elimination of abnormal proteins. OUTCOMES: The patient's clinical symptoms and laboratory parameters gradually improved and he was discharged from the ICU in a month. CONCLUSION: Fulminant CRS can be induced by ICI combination therapy. As the initial symptoms of CRS resemble sepsis, it is important to consider CRS as a differential diagnosis and to initiate immunosuppressive therapy early when needed. In steroid-resistant cases, early introduction of other immunosuppressive therapy and plasma exchange can be effective.


Assuntos
Terapia Combinada/métodos , Síndrome da Liberação de Citocina/sangue , Síndrome da Liberação de Citocina/complicações , Dermatomiosite/etiologia , Idoso , Antibióticos Antineoplásicos/administração & dosagem , Antibióticos Antineoplásicos/uso terapêutico , Antineoplásicos Imunológicos/administração & dosagem , Antineoplásicos Imunológicos/efeitos adversos , Antineoplásicos Imunológicos/uso terapêutico , Biomarcadores/sangue , Síndrome da Liberação de Citocina/tratamento farmacológico , Dermatomiosite/sangue , Dermatomiosite/patologia , Dermatomiosite/terapia , Diagnóstico Diferencial , Humanos , Fatores Imunológicos/administração & dosagem , Fatores Imunológicos/efeitos adversos , Fatores Imunológicos/uso terapêutico , Ipilimumab/administração & dosagem , Ipilimumab/efeitos adversos , Ipilimumab/uso terapêutico , Masculino , Ácido Micofenólico/administração & dosagem , Ácido Micofenólico/uso terapêutico , Nivolumabe/administração & dosagem , Nivolumabe/efeitos adversos , Nivolumabe/uso terapêutico , Troca Plasmática/métodos , Resultado do Tratamento
3.
BMJ Case Rep ; 13(1)2020 Feb 02.
Artigo em Inglês | MEDLINE | ID: mdl-32014990

RESUMO

Mesenteric panniculitis (MP), part of the spectrum of sclerosing mesenteritis, is an often asymptomatic disorder that is characterised by chronic inflammation of abdominal mesentery. We present a case of an 83-year-old woman who presented with proximal muscle weakness and erythematous, photosensitive rash of the face and upper torso and was subsequently diagnosed with dermatomyositis based on skin biopsy, electromyography and muscle biopsy. She had radiographic evidence of panniculitis on CT scan of the abdomen and pelvis for malignancy surveillance, which improved on serial CT scan 3 months after beginning treatment for her underlying dermatomyositis with prednisone and mycophenolate mofetil. Our case highlights that MP can be associated with underlying autoimmune disease. Connective tissue disease could be considered in the differential of MP when other etiologies such as surgery, trauma and malignancy are ruled out.


Assuntos
Dermatomiosite/complicações , Paniculite Peritoneal/complicações , Idoso de 80 Anos ou mais , Anti-Inflamatórios/uso terapêutico , Dermatomiosite/diagnóstico por imagem , Dermatomiosite/tratamento farmacológico , Dermatomiosite/patologia , Feminino , Humanos , Ácido Micofenólico/uso terapêutico , Paniculite Peritoneal/diagnóstico por imagem , Paniculite Peritoneal/tratamento farmacológico , Prednisona/uso terapêutico , Radiografia , Tomografia Computadorizada por Raios X , Resultado do Tratamento
4.
Skinmed ; 17(3): 211-214, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31496481

RESUMO

A 68-year-old Indian man presented with a pruritic eruption on his neck, back, elbows, knees, and the dorsum of his hands. He was initially treated for possible Lyme's disease by his primary care physician, but without improvement. Then he developed daily chills and fevers up to 101 °F, as well as shortness of breath. A chest radiograph showed patchy airspace opacities suggestive of atypical pneumonia, and the patient was treated with levofloxacin and prednisone. Although prednisone diminished the eruption, the patient continued to experience fever, malaise, and generalized weakness, at which point he was hospitalized. Blood cultures and an antinuclear antibodies (ANA) were negative and extensive lab workup was only notable for an elevated erythrocyte sedimentation rate (ESR) (63 mm/hr, Reference Range 0-22), mild transaminitis (AST 77 U/L, Reference Range 10-40), hyponatremia (131 mEq/L, Reference Range 135-145) and elevated ferritin (440, Reference Range 20-500). The patient was discharged on 20 mg of prednisone, with referral to rheumatology and dermatology for possible autoimmune diseases.


Assuntos
Autoanticorpos/sangue , Dermatomiosite/complicações , Dermatomiosite/imunologia , Helicase IFIH1 Induzida por Interferon/imunologia , Doenças Pulmonares Intersticiais/complicações , Idoso , Biópsia , Dermatomiosite/patologia , Humanos , Masculino , Pele/patologia
6.
Internist (Berl) ; 60(8): 775-782, 2019 Aug.
Artigo em Alemão | MEDLINE | ID: mdl-31273399

RESUMO

Various specific skin alterations can occur in patients with malignant diseases. If these skin diseases occur as associated symptoms of a malignant process, they are called paraneoplastic. In this overview, obligate and frequent facultative paraneoplastic skin diseases are assigned according to the triggering type of malignancy. Some of the processes predominantly show a link with malignant diseases of the digestive tract, e.g. acanthosis nigricans, florid cutaneous papillomatosis, necrolytic migratory erythema, Leser-Trélat syndrome, palmoplantar keratoderma, panniculitis and pityriasis rubra pilaris. Others are predominantly associated with a hematolymphoid malignoma, e.g. acquired ichthyosis, exfoliative erythroderma, necrobiotic xanthogranuloma, paraneoplastic pemphigus, plane xanthoma, pyoderma gangrenosum, scleromyxedema, Sweet syndrome and leukocytoclastic vasculitis. In a third group paraneoplastic skin diseases are pooled in association with other malignancies, e.g. Trousseau's syndrome, dermatomyositis, erythema gyratum repens, hypertrichosis lanuginosa acquisita and papuloerythroderma of Ofuji. In order to initiate targeted diagnostics for detection of an underlying malignant disease, it is essential that accomplished physicians recognize the skin diseases that represent obligate or potential paraneoplasms as such.


Assuntos
Acantose Nigricans/patologia , Dermatomiosite/patologia , Síndromes Paraneoplásicas/patologia , Dermatopatias/patologia , Eritema/patologia , Humanos , Ictiose/patologia , Dermatopatias/etiologia
7.
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
8.
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
9.
Eur Radiol ; 29(12): 6708-6716, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31250167

RESUMO

OBJECTIVE: To study a muscle-to-muscle standardised uptake value (SUV) ratio with FDG-PET/CT (FDG-PET) as a marker for the detection of disease activity in dermatomyositis (DM). METHODS: Patients with DM (n = 24) who met the European Neuro-Muscular Centre diagnostic criteria were retrospectively identified over a 3-year period through a national survey. Muscle biopsy was performed in all patients. Maximum SUV was measured in proximal muscles (SUVPROX) that had the highest radiotracer uptake on visual grading as well as in the musculus longissimus thoracis (SUVMLT), whereas mean SUV was measured for the liver (SUVLIV). Muscle-to-liver SUV ratios for either muscle group were compared and a SUVPROX/SUVMLT ratio was calculated. SUVPROX/SUVMLT of DM patients were compared with age- and sex-matched control subjects (n = 24) with melanoma who had received FDG-PET scans. RESULTS: DM patients presented with proximal and symmetrical muscle uptake. Differences in SUVPROX/SUVLIV and SUVMLT/SUVLIV ratios in DM subjects were significant (p < 0.001). SUVPROX/SUVMLT ratios in DM and their controls also differed significantly (p = 0.0012). The SUVPROX/SUVMLT ratio threshold between DM subjects and controls was 1.73 with a sensitivity of 50% (CI95%, 29.1 to 70.9%) and specificity at 83.3% (CI95%, 62.6 to 95.3%). When amyopathic DM patients were removed from the analysis, specificity was increased to 95% (CI95%, 75.1 to 99.9%) with a likelihood ratio of 10 and an AUC of 83.4% (CI95%, 71.4 to 95.4%). CONCLUSION: A muscle-to-muscle SUVPROX/SUVMLT ratio with a cut-off value of 1.73 in FDG-PET imaging might serve as a non-invasive marker to determine disease activity in dermatomyositis. KEY POINTS: • [18F]-FDG PET-scanner standardised uptake value (SUV) could reflect disease activity in dermatomyositis (DM). • A ratio of SUV in proximal muscles (SUVPROX) to SUV in musculus longissimus thoracis (SUVMLT) could be used to determine active DM. • Active disease is suspected for SUV PROX /SUV MLT ratios greater than 1.73.


Assuntos
Dermatomiosite/diagnóstico por imagem , Fluordesoxiglucose F18/farmacocinética , Músculo Esquelético/diagnóstico por imagem , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons/métodos , Compostos Radiofarmacêuticos/farmacocinética , Adulto , Idoso , Idoso de 80 Anos ou mais , Dermatomiosite/metabolismo , Dermatomiosite/patologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Músculo Esquelético/metabolismo , Músculo Esquelético/patologia , Reprodutibilidade dos Testes , Estudos Retrospectivos , Sensibilidade e Especificidade
10.
Pediatr Dermatol ; 36(4): e102-e103, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31134636

RESUMO

A case of a 15-year-old male patient with a 3-year history of linear, segmental amyopathic dermatomyositis with calcinosis cutis is presented. The calcinosis was recalcitrant to treatment with topical steroids and hydroxychloroquine. Topical 10% sodium thiosulfate use for 8 weeks resulted in improvement. The use of topical sodium thiosulfate for patients in whom surgical extraction is not an option is detailed.


Assuntos
Calcinose/diagnóstico , Dermatomiosite/tratamento farmacológico , Dermatomiosite/patologia , Tiossulfatos/uso terapêutico , Administração Tópica , Adolescente , Biópsia por Agulha , Calcinose/complicações , Doença Crônica , Dermatomiosite/complicações , Dermatomiosite/diagnóstico , Humanos , Imuno-Histoquímica , Dermatoses da Perna/diagnóstico , Dermatoses da Perna/etiologia , Masculino , Dermatopatias/tratamento farmacológico , Dermatopatias/patologia , Resultado do Tratamento
11.
Brain Nerve ; 71(4): 323-328, 2019 Apr.
Artigo em Japonês | MEDLINE | ID: mdl-30988214

RESUMO

When examining the skin manifestations of dermatomyositis, it is important to systematically observe the frequently affected sites. The head and the hands are particularly important. It is critical to look not just for symptoms included in the classification criteria, such as heliotrope rash and Gottron's papules/signs, but also for other rashes. Recent studies have demonstrated that specific autoantibodies are useful tools to subgroup dermatomyositis, and the skin manifestations are also closely correlated with these autoantibodies.


Assuntos
Autoanticorpos/sangue , Dermatomiosite/patologia , Dermatomiosite/classificação , Humanos
13.
Z Gastroenterol ; 57(4): 497-500, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30873576

RESUMO

Juvenile polyposis syndrome is a rare autosomal-dominant disorder characterized by multiple hamartomatous polyps in the gastrointestinal tract. It is associated with an increased risk of gastrointestinal cancer. We report the case of a 49-year-old woman presenting with proximal muscle weakness, weight loss, severe anemia, and melena. One year before, the diagnosis of a "fundic gland polyposis" was presumed after endoscopic evaluation for iron deficiency anemia had shown numerous polyps limited to the gastric mucosa. On admission, the diagnosis of dermatomyositis was made based on laboratory results with a marked elevated creatine kinase as well as the presence of characteristic clinical findings and muscle histology. Upper endoscopy revealed multiple pedunculated, edematous polyps in the stomach without apparent cancerous lesions intraluminally. Infiltration of the muscular layer was not detectable on endoscopic ultrasound. Histopathological examination of the polyps showed smooth outer surfaces and multiple dilated cystic glands, consistent with hamartomatous juvenile-type polyps. Magnetic resonance imaging revealed a peritoneal mass close to the greater curvature of the stomach, which was identified as a poorly differentiated adenocarcinoma by laparoscopic sampling. Immunohistochemical analysis of resected polyps was remarkable for a loss of SMAD4 expression, a finding that is very commonly observed in patients with gastric juvenile polyposis syndrome. Despite initial treatment response to glucocorticoids and chemotherapy, the patient died 5 months later due to progressive illness. Patients with gastric juvenile polyposis and SMAD4 mutations are at a high risk of developing gastric cancer; hence, early gastrectomy should be considered.


Assuntos
Dermatomiosite/complicações , Polipose Intestinal/congênito , Polipose Intestinal/diagnóstico por imagem , Síndromes Neoplásicas Hereditárias/diagnóstico por imagem , Pólipos/complicações , Pólipos/genética , Proteína Smad4/genética , Neoplasias Gástricas/complicações , Neoplasias Gástricas/genética , Adenocarcinoma/diagnóstico , Adenocarcinoma/genética , Pólipos Adenomatosos/diagnóstico , Pólipos Adenomatosos/genética , Adolescente , Dermatomiosite/patologia , Endoscopia do Sistema Digestório , Evolução Fatal , Feminino , Humanos , Polipose Intestinal/patologia , Imagem por Ressonância Magnética , Pessoa de Meia-Idade , Síndromes Neoplásicas Hereditárias/diagnóstico , Síndromes Neoplásicas Hereditárias/genética , Neoplasias Gástricas/patologia
14.
J Cutan Pathol ; 46(6): 401-410, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-30737826

RESUMO

BACKGROUND: Common histopathologic findings in cutaneous dermatomyositis include vacuolar interface with dyskeratosis, mucin, and perivascular inflammation. Data examining the relationships between these and other histologic abnormalities, or their dependence on biopsy site, and medications are limited. METHODS: Using 228 dermatomyositis skin biopsies and statistical analyses including Chi-squared analyses, calculations of relative risk, and adjusted generalized estimating equation regressions, we investigated relationships between 14 histopathologic findings and the impact of clinical factors on these findings. RESULTS: In biopsies taken from sites of visible rash, interface dermatitis was seen in 91%, and 95% had at least one of perivascular inflammation, mucin, or basal vacuolization. Vascular abnormalities were not closely associated with epidermal or inflammatory findings. Concomitant prednisone significantly decreased the odds of basal vacuolization (odds ratio [OR] = 0.34, 95% confidence interval [CI]: 0.12-0.98, P-value = 0.05), perivascular inflammation (OR = 0.19, 95% CI: 0.07-0.53, P-value = 0.002), and vessel damage (OR = 0.81, 95% CI: 0.68-0.96, P-value = 0.02). CONCLUSION: Vasculopathy and classic findings of interface dermatitis may be driven by unique pathways in dermatomyositis. Corticosteroid use may impact skin biopsy findings. There is a need for clinicopathologic correlation when diagnosing dermatomyositis.


Assuntos
Dermatomiosite/patologia , Pele/patologia , Adulto , Biópsia , Dermatomiosite/tratamento farmacológico , Dermatomiosite/imunologia , Feminino , Humanos , Inflamação/tratamento farmacológico , Inflamação/imunologia , Inflamação/patologia , Masculino , Pessoa de Meia-Idade , Prednisolona/administração & dosagem , Pele/imunologia
16.
J Dtsch Dermatol Ges ; 17(2): 167-181, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30762968

RESUMO

Syndromes associated with concurrent skin and joint inflammation frequently pose a therapeutic challenge for both dermatologists and rheumatologists. In part 1 of this review, we discussed psoriatic arthritis as well as the autoinflammatory disorders SAPHO syndrome, Still's disease and Behçet's disease. Part 2 will address rheumatoid arthritis, reactive arthritis, Reiter's syndrome and Lyme borreliosis. In addition, we will discuss dermatomyositis and lupus erythematosus, two common autoimmune disorders that frequently present with both cutaneous and joint involvement. For each of the aforementioned disorders, we will highlight aspects of epidemiology, pathogenesis, clinical presentation, diagnosis and treatment.


Assuntos
Entesopatia/complicações , Inflamação/etiologia , Pele/patologia , Sinovite/complicações , Artrite Reativa/epidemiologia , Artrite Reativa/patologia , Artrite Reativa/terapia , Artrite Reumatoide/complicações , Artrite Reumatoide/epidemiologia , Artrite Reumatoide/patologia , Artrite Reumatoide/terapia , Doenças Autoimunes/epidemiologia , Doenças Autoimunes/patologia , Dermatomiosite/complicações , Dermatomiosite/epidemiologia , Dermatomiosite/patologia , Dermatomiosite/terapia , Entesopatia/epidemiologia , Entesopatia/patologia , Doenças Hereditárias Autoinflamatórias/epidemiologia , Doenças Hereditárias Autoinflamatórias/patologia , Humanos , Lúpus Eritematoso Sistêmico/complicações , Lúpus Eritematoso Sistêmico/epidemiologia , Lúpus Eritematoso Sistêmico/patologia , Lúpus Eritematoso Sistêmico/terapia , Doença de Lyme/complicações , Doença de Lyme/epidemiologia , Doença de Lyme/patologia , Doença de Lyme/terapia , Prevalência , Sinovite/epidemiologia , Sinovite/patologia , Sinovite/terapia
18.
Clin Exp Rheumatol ; 37(4): 633-640, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30620292

RESUMO

OBJECTIVES: The aim of this study was to compare muscle biopsy findings, as well as clinical and analytical features, with those of magnetic resonance imaging (MRI) studies of muscle in patients with dermatomyositis. METHODS: All patients from the Longitudinal Myopathy Cohort of the Hospital Clínic de Barcelona were prospectively included in the study from 2009 to 2016. MRI images of muscle and fascial oedema were compared with muscle pathology results using both quantitative and semi-quantitative scores. RESULTS: We found a statistically significant association between the inflammatory infiltrate and both muscle (r2=0.54, p=0.001) and fascial oedema (r2=0.54, p<0.001). In addition, muscle oedema was significantly associated with punched-out vacuoles (p=0.04) and muscle enzymes in serum (r2=0.34, p=<0.01 for CK and r2=0.22, p<0.05 for aldolase). The number of treatment drugs received at the time of MRI was inversely associated with the number of muscle inflammatory cells in the biopsy and with both muscle and fascial oedema (all p<0.05). CONCLUSIONS: Key MRI findings correlate with the main features of dermatomyositis muscle biopsy results, suggesting that MRI findings could be used as a surrogate marker of disease activity.


Assuntos
Dermatomiosite , Imagem por Ressonância Magnética , Doenças Musculares , Biópsia , Dermatomiosite/diagnóstico por imagem , Dermatomiosite/patologia , Edema , Humanos , Imagem por Ressonância Magnética/métodos , Músculo Esquelético/patologia
19.
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
20.
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
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