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1.
Muscle Nerve ; 60(5): 586-590, 2019 11.
Artículo en Inglés | MEDLINE | ID: mdl-31443116

RESUMEN

BACKGROUND: Several viruses have been described as causes of acquired inflammatory myopathies; however, the mechanisms by which they cause muscle disease are still unclear. The aim of this study was to describe the laboratory features of benign acute myositis in a small case series. METHODS: A detailed pathological and serological analysis was performed in five African migrants who developed an acute viral myositis complicated by rhabdomyolysis. RESULTS: Muscle biopsies clearly documented an inflammatory myopathy with histological features similar to polymyositis including CD8+ T cells surrounding and invading nonnecrotic muscle fibers, CD68+ macrophages and major histocompatibility complex class I antigen upregulation. In addition, positivity for myositis-specific antibodies (MSA), in particular anti-aminoacyl tRNA synthetases, was found in the serum of two patients. CONCLUSIONS: Our study demonstrated that T-cell mediated injury occurs in muscle of patients with acute viral myositis, and that MSA may be present in the serum of these patients.


Asunto(s)
Autoanticuerpos/inmunología , Linfocitos T CD8-positivos/inmunología , Antígenos de Histocompatibilidad Clase I/inmunología , Macrófagos/inmunología , Miositis/inmunología , Virosis/inmunología , Adolescente , Aminoacil-ARNt Sintetasas/inmunología , Anticuerpos Antivirales/inmunología , Camerún/etnología , Costa de Marfil/etnología , Creatina Quinasa/sangre , Emigrantes e Inmigrantes , Ghana/etnología , Humanos , Inmunoglobulina G/inmunología , Inmunoglobulina M/inmunología , Italia , Masculino , Miositis/complicaciones , Miositis/patología , Miositis/fisiopatología , Nigeria/etnología , Rabdomiólisis/sangre , Rabdomiólisis/etiología , Partícula de Reconocimiento de Señal/inmunología , Virosis/complicaciones , Virosis/patología
2.
Nutrients ; 11(7)2019 06 28.
Artículo en Inglés | MEDLINE | ID: mdl-31261645

RESUMEN

In recent years, the consumption of chocolate and, in particular, dark chocolate has been "rehabilitated" due to its high content of cocoa antioxidant polyphenols. Although it is recognized that regular exercise improves energy metabolism and muscle performance, excessive or unaccustomed exercise may induce cell damage and impair muscle function by triggering oxidative stress and tissue inflammation. The aim of this review was to revise the available data from literature on the effects of cocoa polyphenols on exercise-associated tissue damage and impairment of exercise performance. To this aim, PubMed and Web of Science databases were searched with the following keywords: "intervention studies", "cocoa polyphenols", "exercise training", "inflammation", "oxidative stress", and "exercise performance". We selected thirteen randomized clinical trials on cocoa ingestion that involved a total of 200 well-trained athletes. The retrieved data indicate that acute, sub-chronic, and chronic cocoa polyphenol intake may reduce exercise-induced oxidative stress but not inflammation, while mixed results are observed in terms of exercise performance and recovery. The interpretation of available results on the anti-oxidative and anti-inflammatory activities of cocoa polyphenols remains questionable, likely due to the variety of physiological networks involved. Further experimental studies are mandatory to clarify the role of cocoa polyphenol supplementation in exercise-mediated inflammation.


Asunto(s)
Antiinflamatorios/uso terapéutico , Antioxidantes/uso terapéutico , Chocolate/análisis , Metabolismo Energético/efectos de los fármacos , Ejercicio , Fuerza Muscular/efectos de los fármacos , Músculo Esquelético/efectos de los fármacos , Miositis/prevención & control , Estrés Oxidativo/efectos de los fármacos , Polifenoles/uso terapéutico , Adolescente , Adulto , Antiinflamatorios/efectos adversos , Antiinflamatorios/análisis , Antioxidantes/efectos adversos , Antioxidantes/análisis , Chocolate/efectos adversos , Humanos , Masculino , Músculo Esquelético/metabolismo , Músculo Esquelético/patología , Músculo Esquelético/fisiopatología , Miositis/etiología , Miositis/metabolismo , Miositis/fisiopatología , Polifenoles/efectos adversos , Polifenoles/análisis , Ensayos Clínicos Controlados Aleatorios como Asunto , Recuperación de la Función , Factores de Riesgo , Resultado del Tratamiento , Adulto Joven
3.
BMJ Case Rep ; 12(4)2019 Apr 16.
Artículo en Inglés | MEDLINE | ID: mdl-30996070

RESUMEN

A 61-year-old Hispanic man presented to a county hospital for subacute progressive weakness, heliotrope rash and dysphagia. There was initial suspicion for dermatomyositis (DM) given the history; however, the physical exam was not consistent. An MRI followed by a muscle biopsy revealed necrotising autoimmune myositis and anti-3-hydroxy-3-methylglutary-coenzyme A-reductase antibody titers returned positive; the patient was diagnosed with necrotising autoimmune myositis. He was treated with corticosteroids and intravenous immunoglobulin, which resulted in improvement in his weakness and functional status. This case represents a unique instance in which a cardinal feature of DM, the heliotrope rash, prompted an erroneous initial diagnosis. It highlights the necessity of developing abroad differential diagnosis and subsequent thorough investigation into patients presenting with suspected idiopathic immune-mediated myopathies.


Asunto(s)
Corticoesteroides/uso terapéutico , Hispanoamericanos , Inmunoglobulinas Intravenosas/uso terapéutico , Factores Inmunológicos/uso terapéutico , Músculo Esquelético/patología , Miositis/diagnóstico , Necrosis/patología , Autoanticuerpos/sangre , Humanos , Masculino , Persona de Mediana Edad , Miositis/tratamiento farmacológico , Miositis/fisiopatología , Necrosis/tratamiento farmacológico , Resultado del Tratamiento
4.
Neurology ; 92(13): e1416-e1426, 2019 03 26.
Artículo en Inglés | MEDLINE | ID: mdl-30824556

RESUMEN

OBJECTIVE: To define the clinical phenotype of patients with myositis with anti-U1-ribonucleoprotein (RNP) autoantibodies. METHODS: In this longitudinal cohort study, the prevalence and severity of clinical features at disease onset and during follow-up in patients with anti-U1-RNP-positive myositis were compared to those with dermatomyositis (DM), immune-mediated necrotizing myopathy (IMNM), and the antisynthetase syndrome (AS). RESULTS: Twenty anti-U1-RNP-positive patients, 178 patients with DM, 135 patients with IMNM, and 132 patients with AS were included. Anti-U1-RNP-positive patients were younger (∼37 years) and more likely to be black (60%) than patients with AS, DM, or IMNM. Muscle weakness was a presenting feature in 15% of anti-U1-RNP-positive patients; 80% eventually developed weakness. Four of 7 anti-U1-RNP-positive patients had necrotizing muscle biopsies. Arthritis occurred in 60% of anti-U1-RNP-positive patients; this was increased compared to DM (18%) or IMNM (6%) (all p < 0.01). DM-specific skin features developed in 60% of anti-U1-RNP-positive patients. Interstitial lung disease (ILD) occurred in 45% of anti-U1-RNP-positive patients; fewer patients with DM (13%) and IMNM (6%) and more patients with AS (80%) developed ILD (all p < 0.01). Glomerulonephritis and pericarditis occurred in 25% and 40% of anti-U1-RNP-positive patients, respectively, but rarely in the other groups; these features occurred only in those with coexisting anti-Ro52 autoantibodies. No anti-U1-RNP patient had cancer-associated myositis or died during the study period. CONCLUSIONS: Patients with anti-U1-RNP myositis typically present with proximal weakness and necrotizing muscle biopsies. Arthritis, dermatitis, and ILD are the most common extramuscular clinical features. Pericarditis and glomerulonephritis are uniquely found in patients with anti-U1-RNP-positive myositis.


Asunto(s)
Artritis/fisiopatología , Enfermedades Autoinmunes/fisiopatología , Glomerulonefritis/fisiopatología , Debilidad Muscular/fisiopatología , Miositis/fisiopatología , Pericarditis/fisiopatología , Adulto , Afroamericanos , Edad de Inicio , Anciano , Artritis/etiología , Autoanticuerpos/inmunología , Enfermedades Autoinmunes/complicaciones , Enfermedades Autoinmunes/etnología , Estudios de Casos y Controles , Estudios de Cohortes , Dermatomiositis/etnología , Dermatomiositis/fisiopatología , Grupo de Ascendencia Continental Europea , Femenino , Glomerulonefritis/etiología , Humanos , Estudios Longitudinales , Masculino , Persona de Mediana Edad , Debilidad Muscular/etiología , Músculo Esquelético/patología , Miositis/complicaciones , Miositis/etnología , Miositis/inmunología , Necrosis , Pericarditis/etiología , Ribonucleoproteína Nuclear Pequeña U1/inmunología , Adulto Joven
5.
Arthritis Rheumatol ; 71(8): 1371-1376, 2019 08.
Artículo en Inglés | MEDLINE | ID: mdl-30861336

RESUMEN

OBJECTIVE: Although more than a dozen myositis-specific autoantibodies (MSAs) have been identified, most patients with myositis are positive for a single MSA. The specific overexpression of a given myositis autoantigen in myositis muscle has been proposed as initiating and/or propagating autoimmunity against that particular autoantigen. The present study was undertaken to test this hypothesis. METHODS: In order to quantify autoantigen RNA expression, RNA sequencing was performed on muscle biopsy samples from control subjects, MSA-positive patients with myositis, regenerating mouse muscles, and cultured human muscle cells. RESULTS: Muscle biopsy samples were available from 20 control subjects and 106 patients with autoantibodies recognizing hydroxymethylglutaryl-coenzyme A reductase (n = 40), signal recognition particles (n = 9), Jo-1 (n = 18), nuclear matrix protein 2 (n = 12), Mi-2 (n = 11), transcription intermediary factor 1γ (n = 11), or melanoma differentiation-associated protein 5 (n = 5). The increased expression of a given autoantigen in myositis muscle was not associated with autoantibodies recognizing that autoantigen (all q > 0.05). In biopsy specimens from both myositis muscle and regenerating mouse muscles, autoantigen expression correlated directly with the expression of muscle regeneration markers and correlated inversely with the expression of genes encoding mature muscle proteins. Myositis autoantigens were also expressed at high levels in cultured human muscle cells. CONCLUSION: Most myositis autoantigens are highly expressed during muscle regeneration, which may relate to the propagation of autoimmunity. However, factors other than overexpression of specific autoantigens are likely to govern the development of unique autoantibodies in individual patients with myositis.


Asunto(s)
Autoanticuerpos/inmunología , Autoantígenos/metabolismo , Músculo Esquelético/inmunología , Miositis/inmunología , Regeneración/inmunología , Animales , Autoantígenos/inmunología , Biopsia , Células Cultivadas , Humanos , Ratones , Mioblastos/inmunología , Mioblastos/metabolismo , Miositis/fisiopatología , ARN/inmunología , ARN/metabolismo
6.
Indian J Pathol Microbiol ; 62(1): 61-66, 2019.
Artículo en Inglés | MEDLINE | ID: mdl-30706861

RESUMEN

Background: Juvenile idiopathic inflammatory myopathies (JIIM) are rare and heterogeneous. Subtype identification is important for treatment. Materials and Methods: Patients below 18 years diagnosed as idiopathic inflammatory myopathy (IIM) according to the Bohan and Peter criteria between January 2010 and May 2015 were evaluated with muscle biopsy in the four domains: muscle fiber, inflammation, connective tissue, and vascular, with basic panel of histochemical stains as per recommendations of the European Neuromuscular center (ENMC) workshop 2015. Immunohistochemistry with CD 31 was done to assess capillary density. Results: JIIM constituted 15.25% of IIM with juvenile dermatomyositis (JDM) being the most common subgroup (24/27) followed by juvenile overlap myositis (JOM) (3/27) in association with systemic lupus erythematosus (2) and systemic sclerosis (1). Muscle biopsy in JDM was characterized by perifascicular atrophy, necrosis, degeneration, and regeneration in all and the other features included perivascular inflammation (21), lymphoid aggregates (2), mitochondrial abnormalities (9), sarcoplasmic vacuoles (6), capillary dropout (5), capillary dilatation (6), and perimysial fibrosis (14). JOM was characterized by auto-antibodies and perivascular inflammation. Conclusion: JIIMs were rare and JDM was the most common subtype. Muscle biopsy evaluation as per ENMC criteria characterized the subgroups.


Asunto(s)
Músculos/patología , Miositis/fisiopatología , Adolescente , Corticoesteroides/uso terapéutico , Enfermedades Autoinmunes/fisiopatología , Biopsia , Niño , Preescolar , Dermatomiositis/fisiopatología , Femenino , Humanos , Inmunohistoquímica , Masculino , Metotrexato/uso terapéutico , Miositis/clasificación , Miositis/diagnóstico , Molécula-1 de Adhesión Celular Endotelial de Plaqueta/metabolismo , Estudios Retrospectivos , Esclerodermia Sistémica/fisiopatología , Vasculitis/fisiopatología
7.
Clin Ter ; 170(1): e55-e60, 2019.
Artículo en Inglés | MEDLINE | ID: mdl-30789198

RESUMEN

The chemokine monokine induced by interferon (IFN)-γ (MIG) is expressed in idiopathic inflammatory myopathies muscle. Abundant expression of MIG was observed on macrophages and T cells surrounding and invading non-necrotic muscle fibers in polymyositis and in inclusion-body myositis and in T cells in perimysial infiltrates of dermatomyositis. MIG is also localized to blood vessel endothelial cells in all inflammatory and normal muscle tissues and it exerts its biological effects mainly via binding to the chemokine (C-X-C motif) receptor (CXCR)3. Serum MIG is high in patients with inflammatory myopathies. Human skeletal muscle cells might actively self-promote muscular inflammation by eliciting MIG secretion, under the influence of cytokines (IFN-γ, tumor necrosis factor-α), which can amplify Th1 cell tissue infiltration in vivo. It has been shown that drugs able to block the MIG/CXCR3 axis can suppress inflammation in muscle.


Asunto(s)
Quimiocina CXCL9/metabolismo , Miositis/fisiopatología , Quimiocina CXCL9/sangre , Humanos , Células Musculares/metabolismo , Miositis/sangre , Receptores CXCR3/metabolismo , Factor de Necrosis Tumoral alfa/metabolismo
8.
BMJ Case Rep ; 12(2)2019 Feb 21.
Artículo en Inglés | MEDLINE | ID: mdl-30796068

RESUMEN

Although cocaine induced myopathy and myotoxicity are described in the literature, we report a rare case of cocaine induced paraspinal myositis presenting with acute sciatic symptoms. A 35-year-old man presented with acute left-sided sciatica and was discharged from the emergency department (ED). He subsequently attended ED the following day in severe pain and bilateral sciatic symptoms, but denied symptoms of neurogenic bowel/bladder disturbance. Clinical examination was limited by severe pain: focal midline lumbar tenderness was elicited on palpation, per rectal and limb examinations were within normal limits with no significant neurological deficit. He was admitted for observation and pain management. His blood tests revealed a leucocyte count of 21.5×109/L, C reactive protein of 89 mg/L and deranged renal function with creatinine of 293 µmol/L. An urgent lumbar spine MRI was arranged to rule out a discitis or epidural abscess. Lumbar MRI did not demonstrate any features of discitis but non-specific appearances of paraspinal inflammation raised the suspicion of a paraspinal myositis. Creatinekinase (CK) was found to be 66329 IU/L and a detailed history revealed he was a cocaine user. Paraspinal muscle biopsy confirmed histological features compatible with myositis. Other serological tests were negative, including anti-GBM, ANCA, ANA, Rheumatoid factor, Hep B, Hep C, myositis specific ENA, Treponema pallidum, Borrelia burgdorferi, Rickettsia, Leptospira, EBV and CMV. There was good clinical response to treatment with prednisolone 20 mg OD with an improvement in renal function, CK levels and CRP. He had resumed normal activities and return to work at 6-week follow-up. A detailed social history including substance misuse is important in patients presenting to the ED-especially in cases of severe musculoskeletal pain with no obvious localising features. Drug induced myotoxicity, although rare, can result in symptomatic patients with severe renal failure.


Asunto(s)
Fumar Cocaína/efectos adversos , Trastornos Relacionados con Cocaína/diagnóstico , Región Lumbosacra/patología , Miositis/diagnóstico , Dolor Intratable/etiología , Prednisolona/uso terapéutico , Adulto , Fumar Cocaína/fisiopatología , Trastornos Relacionados con Cocaína/complicaciones , Trastornos Relacionados con Cocaína/tratamiento farmacológico , Trastornos Relacionados con Cocaína/fisiopatología , Diagnóstico Diferencial , Humanos , Región Lumbosacra/diagnóstico por imagen , Masculino , Miositis/inducido químicamente , Miositis/complicaciones , Miositis/fisiopatología , Dolor Intratable/diagnóstico por imagen , Dolor Intratable/fisiopatología , Ciática , Tomografía Computarizada por Rayos X , Resultado del Tratamiento
9.
J Neuromuscul Dis ; 6(1): 99-107, 2019.
Artículo en Inglés | MEDLINE | ID: mdl-30714969

RESUMEN

BACKGROUND: Manual muscle testing has been widely used for the evaluation of muscle strength in myositis, yet less attention has been devoted to the evaluation of muscle function and endurance. OBJECTIVE: Our objective was therefore to compare the responsiveness to change of muscle strength, endurance and functional testing following induction therapy for severe myositis flare (requiring high-dose corticosteroids and combined immunotherapy) in patients with a diagnosis of dermatomyositis, immune-mediated necrotizing myopathy, or overlap myositis. METHODS: Muscle status was assessed at baseline and after mean 6.4±1.9 months, using the MRC-5 scale, along with endurance (Barre and Mingazzini maneuvers) and functional evaluation (e.g. chair rise) and responsiveness to change was evaluated using the Standardized Response Mean (SRM) and Standardized Mean Difference. RESULTS: Among the 49 patients included, the strongest responsiveness to change was observed in the muscle testing of the psoas and deltoids (SRM: 1.23 and 1.16, respectively). Noticeably, endurance testing also demonstrated strong responsiveness (SRM: 1.05 and 0.96, respectively), compensating for the poor discriminatory ability of muscle testing and permitting to overcome its ceiling effect. CONCLUSION: Functional and endurance testing provide simple and reliable measures complementing the testing of select proximal muscle groups to evaluate responsiveness to intervention in myositis patients in daily clinical practice. Interest of functional and endurance testing should be evaluated prospectively as outcome measures in myositis clinical trials.


Asunto(s)
Fuerza Muscular , Miositis/diagnóstico , Resistencia Física , Corticoesteroides/uso terapéutico , Adulto , Estudios de Cohortes , Prueba de Esfuerzo , Femenino , Humanos , Inmunoterapia , Masculino , Persona de Mediana Edad , Músculo Esquelético/fisiopatología , Miositis/fisiopatología , Miositis/terapia , Reproducibilidad de los Resultados
10.
Skeletal Radiol ; 48(8): 1209-1219, 2019 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-30810778

RESUMEN

OBJECTIVE: To investigate muscle stiffness in patients with idiopathic inflammatory myopathies (IIM) using shear wave elastography (SWE) and to correlate the results with muscle strength and MRI features of myositis. MATERIALS AND METHODS: Muscle shear wave velocity (SWV) was measured in 23 active IIM patients (13 females, mean age 50.4 ± 16.1 years) and 23 matched healthy controls (13 females, mean age 50.7 ± 16.2 years). The investigated muscles included the vastus lateralis (VL), rectus femoris (RF), vastus medialis (VM) vastus intermedius (VI), biceps femoris (BF), semitendinosus (ST), semimembranosus (SM) and the biceps brachii (BB) scanned during relaxed resting and passive stretching positions. Participants performed multiple tests to evaluate their muscle strength. IIM patients had a thigh MRI to assess degrees of oedema, fatty infiltration and atrophy. RESULTS: In the resting position, IIM patients had a 12.9-22.2% significantly lower SWV (p < 0.05) for the quadriceps and hamstrings, but not BB. There was no difference during passive stretching. The SWV for VL, VI and BF showed moderate correlations with the muscle strength tests ranging from r = 0.47 to r = 0.70 (all p < 0.05). Lower SWV was associated with greater MRI scores of oedema (p = 0.001) and atrophy (p = 0.006). However, SWV did not correlate with fatty infiltration (r < 0.3; p = 0.28), creatine kinase (r = 0.28; p = 0.19) or disease duration (r = 0.26; p = 0.24). CONCLUSION: Shear wave elastography may detect abnormal reduced thigh stiffness in IIM patients. SWE measurements were significantly associated with muscle weakness and MRI signs of oedema and atrophy. Future research should investigate this new technology for monitoring disease activity.


Asunto(s)
Miositis/diagnóstico por imagen , Miositis/fisiopatología , Adulto , Anciano , Estudios de Casos y Controles , Diagnóstico por Imagen de Elasticidad , Femenino , Humanos , Imagen por Resonancia Magnética , Masculino , Persona de Mediana Edad , Fuerza Muscular , Músculo Esquelético/diagnóstico por imagen , Músculo Esquelético/fisiopatología , Resistencia al Corte
11.
J Electromyogr Kinesiol ; 45: 41-45, 2019 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-30798164

RESUMEN

Motor unit recruitment is abnormal in myopathies. We have addressed this subject by recording motor unit potentials (MUPs) using a standard concentric needle electrode in tibialis anterior muscles of clinically normal strength in a group of patients with myopathy (15 with myositis and 4 with facioscapulohumeral muscular dystrophy Type 1). In each recording site, a minimal voluntary contraction was sought in order to activate only 2 MUPs. At least 5 pairs of MUPs were recorded in each muscle. We analysed the recruitment rate of the first activated MUP and the mean consecutive difference (MCD) of firing frequency between the individual MUPs of each recruited pair. Results were compared with 30 healthy control subjects. In myopathy the first recorded MUs fired at similar rates to controls (8.2 vs 8.0 Hz, respectively), but the MCD of the firing rate difference between the first two recruited MUPs was less than in controls (median difference 1.78 Hz vs median difference 2.47 Hz, p = 0.02). This change suggests increased lower motor neuron excitability as a functional adaptation, since muscle strength was normal in the studied muscles. These findings are consistent with spinal cord adaptation to the functional changes associated with myopathic muscle disease, although a primary muscle fibre feedback sensing mechanism could also be involved.


Asunto(s)
Electromiografía/normas , Contracción Muscular , Distrofia Muscular Facioescapulohumeral/fisiopatología , Miositis/fisiopatología , Reclutamiento Neurofisiológico , Adulto , Estudios de Casos y Controles , Electromiografía/métodos , Femenino , Humanos , Masculino , Persona de Mediana Edad , Neuronas Motoras/fisiología , Fibras Musculares Esqueléticas/fisiología , Fuerza Muscular
12.
Neurosci Lett ; 694: 208-214, 2019 02 16.
Artículo en Inglés | MEDLINE | ID: mdl-30503926

RESUMEN

Orofacial muscle pain is a significant clinical problem because it affects eating, speaking, and other orofacial functions in patients. However, mechanisms underlying orofacial muscle pain are not fully understood. In the present study we induced orofacial muscle pain by injecting Complete Freund's Adjuvant (CFA) into masseter muscle of rats and assessed pain by the orofacial operant test. In comparison with the control group, CFA-injected animals (CFA group) showed decreases in operant behaviors, suggesting the presence of orofacial pain. Trigeminal ganglion (TG) neurons innervating masseter muscles were retrograde-labeled with DiI and their electrophysiological properties studied using patch-clamp recordings. About 20% of DiI-labeled TG neurons showed spontaneous action potentials (APs) in the CFA group but none in the control group. AP rheobase levels were significantly lower in DiI-labeled TG neurons of the CFA group than in the control group. Membrane input resistance of DiI-labeled TG neurons was significantly higher in the CFA group than in the control group. Several other membrane parameters were also different between DiI-labeled TG neurons of the CFA and control groups. Voltage-dependent currents were examined and the most significant changes following CFA were background K+ currents, which showed significantly smaller in DiI-labeled TG neurons of CFA group compared to the control group. Collectively, orofacial muscle pain in CFA model is accompanied with changes of electrophysiological properties and background K+ currents in TG neurons that innervate masseter muscles.


Asunto(s)
Condicionamiento Operante , Dolor Facial/fisiopatología , Músculo Masetero/fisiopatología , Mialgia/fisiopatología , Miositis/fisiopatología , Neuronas/fisiología , Ganglio del Trigémino/fisiopatología , Potenciales de Acción , Animales , Conducta Animal , Dolor Facial/inducido químicamente , Dolor Facial/psicología , Adyuvante de Freund/administración & dosificación , Masculino , Músculo Masetero/inervación , Mialgia/inducido químicamente , Mialgia/psicología , Miositis/complicaciones , Ratas Sprague-Dawley
14.
Clin Rheumatol ; 38(3): 803-815, 2019 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-30392161

RESUMEN

OBJECTIVE: To study prognostic factors in different types of idiopathic inflammatory myopathies (IIM) associated with interstitial lung disease (ILD). PATIENTS AND METHODS: Multicenter retrospective study of a Spanish cohort of patients diagnosed with IIM. Patients were classified into four categories: polymyositis (PM), dermatomyositis (DM), antisynthetase syndrome (ASS), and overlap myositis (OM). Sociodemographic data, clinical characteristics, antibodies, and treatments were collected. Cox regression models were calculated to identify factors associated with mortality, the necessity for long-term oxygen therapy (LTOT), and deterioration in respiratory function tests (RFT). RESULTS: The number of patients included was 478, of whom 112 (23.4%) suffered from ILD: 17% PM, 16% DM, 45% ASS, and 22% OM. Factors associated with mortality in the multivariate analysis were clinically meaningful progression of ILD after 3 months (CMP 3m) (hazard ratio (HR) 9.48, p = 0.005), severe infections (HR 6.41, p = 0.016), heliotrope erythema (HR 31.1, p = 0.002), delay in diagnosis (HR 1.29; p = 0.011), and Raynaud's phenomenon (HR 11.9, p = 0.007). However, being female (HR 0.19, p = 0.044) and positivity solely for ANAs (HR 0.08, p = 0.008) presented a protective effect. CMP 3m (HR 22.7, p = 0.027) was associated with the need for LTOT, while basal aldolase (HR 0.90; p = 0.049) had a protective effect. Likewise, joint manifestations (HR 0.04, p = 0.034) were shown to reduce risk of deterioration in RFT. CONCLUSIONS: CMP 3m, severe infections, delay in diagnosis, heliotrope erythema, and Raynaud's phenomenon were identified as factors of poor prognosis in different IIM associated with ILD.


Asunto(s)
Enfermedades Pulmonares Intersticiales/fisiopatología , Mortalidad , Miositis/fisiopatología , Terapia por Inhalación de Oxígeno/estadística & datos numéricos , Adolescente , Adulto , Anciano , Anticuerpos Antinucleares/inmunología , Diagnóstico Tardío/estadística & datos numéricos , Dermatomiositis/epidemiología , Dermatomiositis/inmunología , Dermatomiositis/fisiopatología , Progresión de la Enfermedad , Eritema/epidemiología , Femenino , Fructosa-Bifosfato Aldolasa/metabolismo , Humanos , Estimación de Kaplan-Meier , Estudios Longitudinales , Enfermedades Pulmonares Intersticiales/epidemiología , Enfermedades Pulmonares Intersticiales/terapia , Masculino , Persona de Mediana Edad , Análisis Multivariante , Miositis/epidemiología , Miositis/inmunología , Polimiositis/epidemiología , Polimiositis/inmunología , Polimiositis/fisiopatología , Pronóstico , Modelos de Riesgos Proporcionales , Factores Protectores , Capacidad de Difusión Pulmonar , Enfermedad de Raynaud/epidemiología , Pruebas de Función Respiratoria , Estudios Retrospectivos , Factores de Riesgo , Factores Sexuales , España/epidemiología , Capacidad Vital , Adulto Joven
15.
Internist (Berl) ; 60(2): 186-192, 2019 Feb.
Artículo en Alemán | MEDLINE | ID: mdl-30218200

RESUMEN

A 61-year-old woman was admitted with a suspected diagnosis of atypical pneumonia. Extended diagnostic measures revealed interstitial lung disease (ILD), polyserositis, polyarthritis and myopathy. With detection of an antibody against PL-7 (anti-threonyl-transfer-RNA synthetase) the diagnosis of anti-synthetase syndrome (ASS) was established. ASS are rare inflammatory myopathies which frequently present as multisystemic diseases with severe organ involvement. An immunosuppressive regimen with steroids and cyclophosphamide led to successful induction of disease remission. Maintenance therapy will be conducted with azathioprine. ASS should always be considered in the differential diagnosis of myopathies and ILDs.


Asunto(s)
Aminoacil-ARNt Sintetasas/inmunología , Enfermedades Pulmonares Intersticiales/diagnóstico , Pulmón/patología , Miositis/diagnóstico , Insuficiencia Respiratoria/diagnóstico , Anticuerpos Antinucleares , Autoanticuerpos/inmunología , Azatioprina/uso terapéutico , Terapia Combinada , Ciclofosfamida/uso terapéutico , Femenino , Humanos , Inmunosupresores/uso terapéutico , Enfermedades Pulmonares Intersticiales/tratamiento farmacológico , Metilprednisolona/uso terapéutico , Persona de Mediana Edad , Músculo Esquelético/fisiopatología , Miositis/fisiopatología , Insuficiencia Respiratoria/tratamiento farmacológico , Resultado del Tratamiento
16.
Mod Rheumatol ; 29(2): 351-356, 2019 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-29532710

RESUMEN

OBJECTIVES: The aim of our study is to clarify the association of myositis-specific autoantibodies (MSAs) with clinical and laboratory features in Japanese patients with juvenile idiopathic inflammatory myopathies (JIIMs). METHODS: We retrospectively analyzed the frequency of MSAs and their association with clinical or laboratory findings in 25 Japanese patients with JIIMs in Hokkaido district. RESULTS: Eighteen of the 25 patients (72%) were positive for MSAs; seven with anti-melanoma differentiation associated gene (MDA) 5 (28%), five with anti-transcriptional intermediary factor (TIF)-1γ (20%), four with anti-MJ/nuclear matrix protein (NXP)-2 (16%), two with anti-Jo-1 (8%), one with anti- HMG-CoA reductase, one with anti-signal recognition peptide (SRP) antibodies (4% each), including co-existence and transition of MSAs in one patient each. Anti-MDA5 antibodies were related to interstitial lung disease (ILD) and arthritis but not to amyopathic juvenile dermatomyositis. Drug-free remission was achieved, once ILD was overcome in this group. Anti-TIF-1γ antibodies were associated with typical rashes and mild myositis. Anti-MJ/NXP2 and anti-SRP antibodies were associated with severe muscle weakness. No patient was complicated with malignancy. CONCLUSION: Anti-MDA5 antibodies are prevalent and closely associated with ILD in our series compared with other countries. There was no apparent difference in clinical features associated with other MSAs among races.


Asunto(s)
Artritis , Autoanticuerpos , Helicasa Inducida por Interferón IFIH1/inmunología , Enfermedades Pulmonares Intersticiales , Miositis , Adolescente , Artritis/epidemiología , Artritis/etiología , Artritis/inmunología , Autoanticuerpos/sangre , Autoanticuerpos/clasificación , Niño , Preescolar , Correlación de Datos , Proteínas de Unión al ADN/inmunología , Femenino , Histidina-ARNt Ligasa/inmunología , Humanos , Hidroximetilglutaril-CoA Reductasas/inmunología , Japón/epidemiología , Enfermedades Pulmonares Intersticiales/epidemiología , Enfermedades Pulmonares Intersticiales/etiología , Enfermedades Pulmonares Intersticiales/inmunología , Masculino , Miositis/complicaciones , Miositis/inmunología , Miositis/fisiopatología , Prevalencia , Estudios Retrospectivos , Factores de Transcripción/inmunología
17.
Muscle Nerve ; 59(1): 70-75, 2019 01.
Artículo en Inglés | MEDLINE | ID: mdl-30028529

RESUMEN

INTRODUCTION: It is unclear whether quantitating muscle endurance adds nonredundant information useful for the care of patients with muscular disease. METHODS: Records were retrospectively reviewed for all Johns Hopkins Myositis Center patients with a muscle endurance assessment (n = 128, 226 patient-visits). Muscle endurance and strength were quantitated with the Myositis Functional Index-2 (FI2) and manual muscle testing (MMT), respectively. RESULTS: Composite FI2 muscle endurance scores were comparable in inclusion body myositis (n = 58), dermatomyositis (n = 31), and polymyositis (n = 39). Overall, muscle endurance correlated with and evolved similarly to strength, inversely to serum creatine kinase. However, in patients with normal or near-normal strength (mean MMT > 9.75/10), muscle endurance was typically abnormal and highly variable (mean FI2, 5.6/10; interquartile range, 3.3-7.8/10). DISCUSSION: Muscle endurance testing may identify muscle impairment inadequately described by MMT, particularly in patients with high MMT scores. Muscle Nerve 59:70-75, 2019.


Asunto(s)
Fuerza Muscular/fisiología , Miositis/fisiopatología , Resistencia Física/fisiología , Anciano , Creatina Quinasa/sangre , Dermatomiositis , Femenino , Humanos , Masculino , Persona de Mediana Edad , Miositis/sangre , Miositis por Cuerpos de Inclusión , Polimiositis , Estudios Retrospectivos , Índice de Severidad de la Enfermedad , Factores de Tiempo
18.
Med Sci (Paris) ; 34 Hors série n°2: 35-38, 2018 Nov.
Artículo en Francés | MEDLINE | ID: mdl-30418144

RESUMEN

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.


Asunto(s)
Interferón gamma/farmacología , Músculo Esquelético/efectos de los fármacos , Músculo Esquelético/patología , Enfermedades Musculares/inmunología , Miositis/patología , Dermatomiositis/patología , Dermatomiositis/fisiopatología , Antígenos de Histocompatibilidad Clase II/análisis , Humanos , Interferón gamma/fisiología , Fibras Musculares Esqueléticas/química , Fibras Musculares Esqueléticas/inmunología , Enfermedades Musculares/patología , Enfermedades Musculares/fisiopatología , Miositis/fisiopatología , Miositis por Cuerpos de Inclusión/patología , Miositis por Cuerpos de Inclusión/fisiopatología , Polimiositis/patología , Polimiositis/fisiopatología
19.
Am J Physiol Renal Physiol ; 315(6): F1658-F1669, 2018 12 01.
Artículo en Inglés | MEDLINE | ID: mdl-30280599

RESUMEN

Muscle dysfunction is an important cause of morbidity among patients with chronic kidney disease (CKD). Although muscle fibrosis is present in a CKD rodent model, its existence in humans and its impact on physical function are currently unknown. We examined isometric leg extension strength and measures of skeletal muscle fibrosis and inflammation in vastus lateralis muscle from CKD patients ( n = 10) and healthy, sedentary controls ( n = 10). Histochemistry and immunohistochemistry were used to assess muscle collagen and macrophage and fibro/adipogenic progenitor (FAP) cell populations, and RT-qPCR was used to assess muscle-specific inflammatory marker expression. Muscle collagen content was significantly greater in CKD compared with control (18.8 ± 2.1 vs. 11.7 ± 0.7% collagen area, P = 0.008), as was staining for collagen I, pro-collagen I, and a novel collagen-hybridizing peptide that binds remodeling collagen. Muscle collagen was inversely associated with leg extension strength in CKD ( r = -0.74, P = 0.01). FAP abundance was increased in CKD, was highly correlated with muscle collagen ( r = 0.84, P < 0.001), and was inversely associated with TNF-α expression ( r = -0.65, P = 0.003). TNF-α, CD68, CCL2, and CCL5 mRNA were significantly lower in CKD than control, despite higher serum TNF-α and IL-6. Immunohistochemistry confirmed fewer CD68+ and CD11b+ macrophages in CKD muscle. In conclusion, skeletal muscle collagen content is increased in humans with CKD and is associated with functional parameters. Muscle fibrosis correlated with increased FAP abundance, which may be due to insufficient macrophage-mediated TNF-α secretion. These data provide a foundation for future research elucidating the mechanisms responsible for this newly identified human muscle pathology.


Asunto(s)
Contracción Isométrica , Fuerza Muscular , Debilidad Muscular/etiología , Miositis/etiología , Músculo Cuádriceps/fisiopatología , Insuficiencia Renal Crónica/complicaciones , Anciano , Estudios de Casos y Controles , Colágeno/metabolismo , Estudios Transversales , Femenino , Fibrosis , Estado de Salud , Humanos , Mediadores de Inflamación/metabolismo , Masculino , Persona de Mediana Edad , Debilidad Muscular/diagnóstico , Debilidad Muscular/metabolismo , Debilidad Muscular/fisiopatología , Miositis/diagnóstico , Miositis/metabolismo , Miositis/fisiopatología , Músculo Cuádriceps/metabolismo , Músculo Cuádriceps/patología , Insuficiencia Renal Crónica/diagnóstico , Insuficiencia Renal Crónica/fisiopatología , Índice de Severidad de la Enfermedad
20.
Clin Exp Rheumatol ; 36 Suppl 114(5): 74-81, 2018.
Artículo en Inglés | MEDLINE | ID: mdl-30296982

RESUMEN

OBJECTIVES: To describe imaging modalities for diagnosing and monitoring of patients with idiopathic inflammatory myopathies. METHODS: A detailed literature search summarising recent data documenting the contribution of different imaging techniques to current management of idiopathic inflammatory myopathies was performed. RESULTS: An overview of methods most frequently used for evaluation of inflammatory myopathies and the description of their role in the diagnostic and monitoring process is presented. CONCLUSIONS: MRI is currently the most useful method capable of demonstrating both inflammatory and post-inflammatory changes in the muscles and surrounding soft tissue. Several studies have documented potential usefulness of other imaging techniques, such as ultrasonography, positron emission tomography, scintigraphy, and dual-energy x-ray absorptiometry.


Asunto(s)
Imagen por Resonancia Magnética/métodos , Músculo Esquelético/diagnóstico por imagen , Miositis/diagnóstico por imagen , Reumatología/métodos , Absorciometría de Fotón , Humanos , Músculo Esquelético/fisiopatología , Miositis/fisiopatología , Miositis/terapia , Tomografía de Emisión de Positrones , Valor Predictivo de las Pruebas , Pronóstico , Reproducibilidad de los Resultados , Índice de Severidad de la Enfermedad , Ultrasonografía
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