Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 1.524
Filtrar
1.
Brain Nerve ; 73(2): 137-146, 2021 Feb.
Artigo em Japonês | MEDLINE | ID: mdl-33561828

RESUMO

Dermatomyositis and antisynthetase syndrome are among idiopathic inflammatory myopathies (myositis) that have skin lesions. In these disorders, skin symptoms often appear as the initial manifestations, and thus, their evaluation is critical for diagnosis. Two components, "site" and "properties," are important for understanding the skin manifestations in myositis. The rash in these disorders is induced by Koebner's phenomenon, and is most frequently observed on the hands and face. Recent studies have shown that myositis-specific autoantibodies are useful tools for the diagnosis and classification of these diseases, as they are closely correlated with the clinical and histopathological phenotypes of myositis. It has recently become clear that each antibody is associated with characteristic skin manifestations, especially related to the properties of the skin lesions.


Assuntos
Dermatomiosite , Miosite , Autoanticorpos , Dermatomiosite/complicações , Dermatomiosite/diagnóstico , Humanos , Miosite/complicações , Miosite/diagnóstico , Fenótipo
2.
Brain Nerve ; 73(2): 183-187, 2021 Feb.
Artigo em Japonês | MEDLINE | ID: mdl-33561833

RESUMO

A 66-year-old woman visited our hospital complaining of shortness of breath during exertion and progressive weakness in all her limb muscles. On admission, we noted muscle weakness in her trunk and in her proximal limb muscles, although, her muscle MRI showed no remarkable findings. However, her serum CK level (2,747U/L) was above the normal range. Histopathological examination of muscle biopsy, performed from the left biceps brachii muscle, revealed immune-mediated necrotizing myopathy (IMNM). Her serum samples were negative for myositis-associated autoantibodies (MAAs), anti-SRP, and HMGCR antibodies. However, as the anti-SS-A antibody level in her serum was high (53.2U/mL), we conducted the salivary gland biopsy and the Schirmer test on her eyes. We found lymphocytes infiltration in her salivary gland tissue, and thus, she was diagnosed with primary Sjögren syndrome (SjS). We also observed necrotizing myopathy associated with the SjS. Following her treatment with oral steroids, her symptoms and CK level improved. Although, inflammatory myositis frequently occurs in association with general collagen diseases, necrotizing myopathy has rarely been observed secondary to SjS. We report here this rare case study along with the review of the relevant literature. (Received June 24, 2020; Accepted October 12, 2020; Published February 1, 2021).


Assuntos
Doenças Autoimunes , Doenças Musculares , Miosite , Síndrome de Sjogren , Idoso , Autoanticorpos , Feminino , Humanos , Doenças Musculares/etiologia , Miosite/complicações , Síndrome de Sjogren/complicações
3.
BMJ Case Rep ; 14(1)2021 Jan 07.
Artigo em Inglês | MEDLINE | ID: mdl-33414112

RESUMO

Influenza A and B commonly cause benign respiratory disease in humans, but can cause more severe illness in high-risk populations. We report an unusual case of a previously healthy adult patient who presented with myositis and severe rhabdomyolysis secondary to influenza A infection that resulted in atraumatic compartment syndrome of all four extremities, each requiring emergent fasciotomy. The patient was subsequently managed with delayed primary closure and skin grafting in the operating room. Prompt recognition of this rare complication by the team resulted in no limb amputations. On his first follow-up appointment, 1 month after discharge, he had regained full functionality in both his hands and his feet were both close to 50% of baseline and improving with physical therapy.


Assuntos
Extremidades/patologia , Influenza Humana/complicações , Miosite/complicações , Rabdomiólise/complicações , Síndromes Compartimentais/etiologia , Síndromes Compartimentais/cirurgia , Extremidades/cirurgia , Humanos , Vírus da Influenza A , Masculino , Pessoa de Meia-Idade , Miosite/virologia , Rabdomiólise/virologia , Transplante de Pele
4.
Beijing Da Xue Xue Bao Yi Xue Ban ; 52(6): 995-1000, 2020 Dec 18.
Artigo em Chinês | MEDLINE | ID: mdl-33331304

RESUMO

OBJECTIVE: To investigate and analyse the clinical and immunological features of patients with myositis complicated with thromboembolism. METHODS: We identified a cohort of 390 myositis patients diagnosed with myositis admitted to People's Hospital of Peking University from 2003 to 2019. The patients were retrospectively enrolled in this investigation. According to the outcome of the color Doppler ultrasound, CT pulmonary angiography, pulmonary ventilation and perfusion scan patients were divided into myositis with and without thromboembolism group. Demographic, clinical (heliotrope rash, Gottron's sign/papules, periungual erythema, skin ulceration, subcutaneous calcinosis, Mechanic's hands, myalgia, interstitial lung disease, pulmonary arterial hypertension), laboratory, immunological [anti-autoantibodies including melanoma differentiation associated gene 5 (anti-MDA5), anti-Mi-2, anti-transcription intermediary factor-1γ (anti-TIF-1γ, anti-nuclear matrix protein 2 (anti-NXP2), anti-small ubiquitin-like modifier activating enzyme (anti-SAE), anti-synthetase], imaging and therapeutic status data of the patients at the diagnosis of myositis with and without thromboembolism were collected and the differences in these data were analyzed. Logistic regressive analysis was used to identify the risk factors of thromboembolism. RESULTS: In the retrospective study, 390 myositis patients were investigated. The mean age of onset was (49.6±13.4) years, male to female ratio was 0.31 :1. Thromboembolism was identified in 4.62% (18/390) of the myositis patients, which was lower than the published reports. Out of 18 patients with thromboembolism, 55.6% (10/18) of them were deep venous thrombosis, followed by cerebral infarction (22.2%, 4/18), pulmonary embolism (11.1%, 2/18), renal artery embolism (5.6%, 1/18) and embolism of upper extremity (5.6%, 1/18). Fifty percent of thromboembolism events occurred 6 months after the diagnosis of myositis, 38.9% of thromboembolism events occurred 6 months within the diagnosis of myositis, 11.1% of thromboembolism events occurred 6 months before the diagnosis of myositis. As compared with the myositis patients without thromboembolism, the myositis patients complicated with thromboembolism were older [(58.3±11.7) years vs. (49.3±13.4) years, P=0.006]. C-reaction protein (CRP) (12.2 mg/L vs. 4.1 mg/L, P < 0.001), ferritin (20 085.5 µg/L vs. 216.6 µg/L, P < 0.001) and D-dimer (529.0 µg/L vs. 268.0 µg/L, P=0.002) were significantly higher in thromboembolism group. Diabetes (44.4% vs. 16.4%, P=0.006), coronary heart disease (22.2% vs. 3.0%, P=0.003) and surgery (16.7% vs. 3.5%, P=0.032) were observed more common in thromboembolism group than those without thromboembolism. Activated partial thromboplastin time (APTT) (26.9 s vs. 28.7 s, P=0.049) and albumin (32.4 g/L vs. 36.5 g/L, P=0.002) was lower in thromboembolism group. The risk factors of thromboembolism in the myositis patients were low level of albumin (OR=0.831, 95%CI: 0.736-0.939, P=0.003), diabetes (OR=4.468, 95%CI: 1.382-14.448, P=0.012), and coronary heart disease (OR=22.079, 95%CI: 3.589-135.837, P=0.001) were independent significant risk factors for thromboembolism in the patients with myositis. There was no significant difference in clinical manifestations, myositis-specific antibodies or myositis-associated antibodies between the two groups. CONCLUSION: Thromboembolism is a complication of myositis. Lower levels of albumin, diabetes, and coronary heart disease might be risk factors of thromboembolism in myositis patients.


Assuntos
Dermatomiosite , Doenças Pulmonares Intersticiais , Miosite , Tromboembolia , Adulto , Autoanticorpos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Miosite/complicações , Estudos Retrospectivos
5.
Int Heart J ; 61(5): 1005-1013, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32999188

RESUMO

This study sought to evaluate clinical features, treatment patterns, and outcomes of patients with idiopathic inflammatory myopathy (IIM) complicated by heart failure (HF). Thirty-two patients with IIM-HF admitted to the Peking Union Medical College Hospital between January 1999 and January 2018 were retrospectively reviewed, including 14 patients with polymyositis, 11 with dermatomyositis, and 7 with overlap syndrome. Survivors and no-survivors were compared on clinical characteristics and treatment. Although systemic symptoms were variable, all patients presented with elevated troponin I. Rapid atrial arrhythmia was the most frequent arrhythmia. Systolic dysfunction and restrictive diastolic dysfunction were typical presentations in echocardiography. Twenty-nine patients were followed up for a median of 2.8 years (0.1 month to 11 years). We recorded 13 deaths of cardiogenic cause, 1 of serious IIM, and 3 of infective complications. The median survival time from diagnosis of IIM-HF to all-cause mortality was 8.4 months (range from 1 month to 5 years). Both all-cause deaths and cardiogenic deaths were more reported in the methotrexate-alone group than in the combination therapy group (6/7 versus 3/10, P = 0.050; 5/6 versus 2/9, P = 0.041). Combination therapy including methotrexate (HR = 0.188, 95%CI 0.040-0.871, P = 0.033) and taking ß-receptor blockers (HR = 0.249, 95%CI 0.086-0.719, P = 0.010) was associated with reduced risk of all-cause deaths. In conclusion, elevated troponin I, atrial arrhythmia, and systolic and restrictive diastolic dysfunction are typical characteristics of IIM-HF. Combined immunosuppression that includes methotrexate and ß-receptor blockers seems to be important to improve survival.


Assuntos
Imunossupressores/uso terapêutico , Metotrexato/uso terapêutico , Miosite/tratamento farmacológico , Miosite/mortalidade , Adolescente , Antagonistas Adrenérgicos beta/uso terapêutico , Adulto , Idoso , China/epidemiologia , Quimioterapia Combinada , Feminino , Glucocorticoides/uso terapêutico , Insuficiência Cardíaca/etiologia , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Masculino , Metilprednisolona/uso terapêutico , Pessoa de Meia-Idade , Miosite/complicações , Estudos Retrospectivos , Adulto Jovem
6.
BMC Infect Dis ; 20(1): 768, 2020 Oct 17.
Artigo em Inglês | MEDLINE | ID: mdl-33069216

RESUMO

BACKGROUND: Cytomegalovirus (CMV) is a double stranded DNA virus and ubiquitous in nature. Association of Guillain-Barre syndrome (GBS) and CMV is well known but CMV acute myositis is a rare condition. Restriction of movements of limbs due to severe pain in myositis may obscure the diagnosis of GBS and this may easily miss. CASE PRESENTATION: Here we describe a 29-year-old male presenting with pain and swelling of bilateral lower limbs which progressed rapidly with increasing serum creatine kinase levels with positive IgM CMV antibodies. In view of no improvement in clinical condition, patient was further evaluated and found to have concurrent GBS. He was treated with plasmapheresis and improved. CONCLUSION: Cytomegalovirus infection presenting as acute myositis is a uncommon and further association with GBS is a rare occurrence.


Assuntos
Infecções por Citomegalovirus/complicações , Infecções por Citomegalovirus/diagnóstico , Citomegalovirus/imunologia , Síndrome de Guillain-Barré/complicações , Síndrome de Guillain-Barré/diagnóstico , Miosite/complicações , Miosite/diagnóstico , Doença Aguda , Adulto , Anticorpos Antivirais/sangue , Infecções por Citomegalovirus/terapia , Erros de Diagnóstico , Síndrome de Guillain-Barré/terapia , Humanos , Imunoglobulina M/sangue , Masculino , Miosite/terapia , Miosite/virologia , Dor , Plasmaferese , Resultado do Tratamento
7.
Rev. cuba. pediatr ; 92(3): e1061, jul.-set. 2020. tab
Artigo em Espanhol | LILACS, CUMED | ID: biblio-1126766

RESUMO

Introducción: La miositis aguda benigna infantil es una complicación transitoria de la gripe causada por los virus influenza A o B, observada en pacientes escolares. Objetivos: Contribuir a divulgar las características de la miositis aguda benigna infantil en el personal médico. Métodos: Investigación descriptiva transversal en 18 pacientes con edad inferior a 19 años atendidos con el diagnóstico de esta afección, en el Hospital Pediátrico Docente Cerro desde el primero de octubre hasta 31 de diciembre del 2019. Se tomó muestra de sangre para estudios hematológicos y enzimáticos, determinación de anticuerpos antidengue y exudado nasofaríngeo en busca de virus respiratorios. Se empleó el programa SSPS versión 19. Los resultados se expresaron en valores absolutos, porcentajes, y los del laboratorio clínico, en promedios y desviación estándar. Resultados: Predominó la edad de 5-14 años (88 por ciento), sexo masculino (72,2 por ciento) y residencia en los municipios Cerro y 10 de 0ctubre (55,5 y 22,2 por ciento), respectivamente. El tipo de marcha anormal más frecuente fue la Frankenstein, con un conteo leucocitario promedio de 7,01 × 109 ± 2,16; linfocitosis 53,3 por ciento y promedio de creatininfosfoquinasa 591 µl × L ± 435,74. Todos los pacientes se recuperaron. El virus influenza B se identificó en 77,7 por ciento de las muestras. Conclusiones: La miositis constituyó una complicación por los virus de la gripe en mayor proporción el tipo B, en pacientes varones escolares, que mostraron trastornos en la marcha, examen neurológico normal y creatininfosfoquinasa elevada. Todos los enfermos se recuperaron en los primeros tres días de iniciada la afección(AU)


Introduction: Children mild acute myositis is a transitory complication of the flu caused by the influenza A or B viruses which presents in school patients. Objectives: To contribute to spread the characteristics of children mild acute miositis in the medical staff. Methods: Descriptive cross-sectional research in 18 patients with ages under the 19 years that were attented with a diagnosis of the disease in "Cerro" Pediatric Teaching Hospital from October 1st to December 31st, 2019. There were taken blood samples to do enzymatic and hematologic studies, antidengue antibodies determination and nasopharinx exudates looking for respiratory viruses. SSPS version 19 program was used. The results were expressed in absolute values, percentages, averages and standard deviation. Results: There was predomiance of the ages from 5 to 14 years (88 percent), male sex (72.2 percent) and living in Cerro and 10 de octubre municipalities (55,5 percent and 22,2 percent, respectively). The most frquent type of abnormal walk was the Frankenstein with an average leukocytes count of 7.01 × 109 ± 2.16; lymphocytea of 53.3 percent; and an average of creatinine fosfoquinase 591 µl × L ± 435.74. All the patients recovered. The influenza B virus was identified in 77.7 percent of the samples. Conclusions: Myositis represented a complication by influenza viruses, mainly the type B, in school male patients who showed disorders in the walk, normal neurologic examination and high creatinine phosphokinase. All the patients recovered in the first 3 days after the onset of the disease(AU)


Assuntos
Humanos , Masculino , Feminino , Criança , Adolescente , Vírus da Influenza B/crescimento & desenvolvimento , Marcha/fisiologia , Miosite/complicações , Epidemiologia Descritiva , Estudos Transversais
8.
Clin Nucl Med ; 45(11): e495-e497, 2020 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-32701802

RESUMO

Graft-versus-host disease (GVHD) is a major complication of bone marrow transplantation. Polymyositis with myonecrosis is a rare manifestation of GVHD. Here, we report the case of a 32-year-old man with acute myeloid leukemia who developed GVHD after transplant. He subsequently developed polymyositis, which was diagnosed on PET/CT and confirmed on pathology. Treatment with corticosteroids resulted in the resolution of the symptoms. Abnormal muscular FDG uptake resolved on the follow-up PET/CT.


Assuntos
Doença Enxerto-Hospedeiro/complicações , Miosite/complicações , Adulto , Transplante de Medula Óssea/efeitos adversos , Doença Crônica , Gangrena/complicações , Doença Enxerto-Hospedeiro/diagnóstico por imagem , Humanos , Masculino , Tomografia Computadorizada com Tomografia por Emissão de Pósitrons , Transplante Homólogo/efeitos adversos
9.
J Neurovirol ; 26(2): 143-148, 2020 04.
Artigo em Inglês | MEDLINE | ID: mdl-32447630

RESUMO

A pandemic due to novel coronavirus arose in mid-December 2019 in Wuhan, China, and in 3 months' time swept the world. The disease has been referred to as COVID-19, and the causative agent has been labelled SARS-CoV-2 due to its genetic similarities to the virus (SARS-CoV-1) responsible for the severe acute respiratory syndrome (SARS) epidemic nearly 20 years earlier. The spike proteins of both viruses dictate tissue tropism using the angiotensin-converting enzyme type 2 (ACE-2) receptor to bind to cells. The ACE-2 receptor can be found in nervous system tissue and endothelial cells among the tissues of many other organs.Neurological complications have been observed with COVID-19. Myalgia and headache are relatively common, but serious neurological disease appears to be rare. No part of the neuraxis is spared. The neurological disorders occurring with COVID-19 may have many pathophysiological underpinnings. Some appear to be the consequence of direct viral invasion of the nervous system tissue, others arise as a postviral autoimmune process, and still others are the result of metabolic and systemic complications due to the associated critical illness. This review addresses the preliminary observations regarding the neurological disorders reported with COVID-19 to date and describes some of the disorders that are anticipated from prior experience with similar coronaviruses.


Assuntos
Betacoronavirus/patogenicidade , Infecções por Coronavirus/epidemiologia , Encefalite Viral/epidemiologia , Meningite/epidemiologia , Pandemias , Pneumonia Viral/epidemiologia , Acidente Vascular Cerebral/epidemiologia , Betacoronavirus/genética , Betacoronavirus/metabolismo , Infecções por Coronavirus/complicações , Infecções por Coronavirus/diagnóstico , Infecções por Coronavirus/virologia , Encefalite Viral/complicações , Encefalite Viral/diagnóstico , Encefalite Viral/virologia , Cefaleia/complicações , Cefaleia/diagnóstico , Cefaleia/epidemiologia , Cefaleia/virologia , Interações Hospedeiro-Patógeno/genética , Humanos , Meningite/complicações , Meningite/diagnóstico , Meningite/virologia , Mialgia/complicações , Mialgia/diagnóstico , Mialgia/epidemiologia , Mialgia/virologia , Miosite/complicações , Miosite/diagnóstico , Miosite/epidemiologia , Miosite/virologia , Sistema Nervoso/patologia , Sistema Nervoso/virologia , Peptidil Dipeptidase A/genética , Peptidil Dipeptidase A/metabolismo , Pneumonia Viral/complicações , Pneumonia Viral/diagnóstico , Pneumonia Viral/virologia , Ligação Proteica , Receptores Virais/genética , Receptores Virais/metabolismo , Glicoproteína da Espícula de Coronavírus/genética , Glicoproteína da Espícula de Coronavírus/metabolismo , Acidente Vascular Cerebral/complicações , Acidente Vascular Cerebral/diagnóstico , Acidente Vascular Cerebral/virologia , Internalização do Vírus
10.
Scand J Rheumatol ; 49(4): 323-331, 2020 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-32286141

RESUMO

OBJECTIVE: Epidemiological studies on inflammatory myopathies (IMs) show widely variable results, and studies on Asians are lacking. Despite emerging interest in the cardiovascular disease (CVD) risk associated with IMs, the prevalence of CVD in IM patients and its impact on mortality remain unclear. We conducted a nationwide, population-based study on the incidence, mortality, and associated major CVD events of IMs in the Republic of Korea over 11 years. METHOD: Using the nationwide, population-based National Health Insurance claims database and the Rare Intractable Disease registration programme, we estimated incidence, mortality, and CVD occurrence. Survival was examined using the Kaplan-Meier method. Mortality rate in IMs with CVD was analysed by Cox proportional hazards regression. RESULTS: There were 3014 incident cases, 640 of whom died during the study period. The mean annual incidence was 7.16/106. Dermatomyositis (DM) and polymyositis (PM) had 5 year survival rates of 76.8% and 79.3%, respectively. Cardiovascular events occurred in 155 patients and 40.6% of IM patients with CVD died. Acute myocardial infarction in men had the highest risk of any CVD event in both DM [standardized incidence ratio (SIR) 4.2, 95% confidence interval (95% CI) 2.4-7.2] and PM (SIR 3.5, 95% CI 1.8-7.0). Haemorrhagic stroke had the highest hazard ratio (HR) in both DM (HR 2.31, 95% CI 1.13-4.70) and PM patients (HR 2.10, 95% CI 1.03-4.27) compared with the general population with CVD. CONCLUSION: We found persistently low incidence, poor survival, and high major CVD incidence in IMs, and increased mortality in IMs with CVD.


Assuntos
Doenças Cardiovasculares/mortalidade , Miosite/complicações , Sistema de Registros , Adulto , Idoso , Idoso de 80 Anos ou mais , Doenças Cardiovasculares/etiologia , Feminino , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , República da Coreia/epidemiologia , Adulto Jovem
11.
Artigo em Inglês | MEDLINE | ID: mdl-32268560

RESUMO

BACKGROUND: In patients with neuromuscular disorder, only little data of myalgia frequency and characterization exists. To date, only a weak correlation between pain intensity and pressure pain threshold has been found, and it remains enigmatic whether high pain intensity levels are equivalent to high pain sensitivity levels in neuromuscular disorders. METHODS: 30 sequential patients with suspected neuromuscular disorder and myalgia were analyzed with regard to myalgia characteristics and clinical findings, including symptoms of depression and anxiety and pain- threshold. RESULTS: A neuromuscular disorder was diagnosed in 14/30 patients. Muscular pain fasciculation syndrome (MPFS) without evidence for myopathy or myositis was diagnosed in 10/30 patients and 6/30 patients were diagnosed with pure myalgia without evidence for a neuromuscular disorder (e.g., myopathy, myositis, MPFS, polymyalgia rheumatica). Highest median pain scores were found in patients with pure myalgia and polymyalgia rheumatica. Pressure pain threshold measurement showed a significant difference between patients and controls in the biceps brachii muscle. CONCLUSION: Only a weak correlation between pain intensity and pressure pain threshold has been suggested, which is concordant with our results. The hypothesis that high pain intensity levels are equivalent to high pain sensitivity levels was not demonstrated.


Assuntos
Doenças Musculares , Mialgia , Miosite , Adulto , Anoctaminas , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Músculo Esquelético , Doenças Musculares/complicações , Mialgia/diagnóstico , Mialgia/etiologia , Miosite/complicações , Limiar da Dor , Adulto Jovem
12.
Rev Med Suisse ; 16(685): 504-507, 2020 Mar 11.
Artigo em Francês | MEDLINE | ID: mdl-32167253

RESUMO

Cancer management has been revolutionised by immune checkpoint inhibitors. Their use and indications increase in parallel with the recognition of their various side effects. Arthritis, myositis, and vasculitis are among the most common rheumatologic immune-related adverse events (irAE) of immunotherapy. Rheumatological irAEs can be of late onset, occur even after the cessation of the culprit drug and persist into time. In this article we discuss the principles of cancer immunotherapy, clinical manifestations and management of the most common rheumatologic irAEs, and aspects of immune checkpoint inhibitors therapy in patients with pre-existing autoimmune disease.


Assuntos
Imunoterapia/efeitos adversos , Neoplasias/tratamento farmacológico , Neoplasias/imunologia , Doenças Reumáticas/induzido quimicamente , Artrite/induzido quimicamente , Artrite/complicações , Artrite/terapia , Humanos , Miosite/induzido quimicamente , Miosite/complicações , Miosite/terapia , Neoplasias/complicações , Doenças Reumáticas/complicações , Doenças Reumáticas/terapia
13.
J Neuropathol Exp Neurol ; 79(4): 448-457, 2020 04 01.
Artigo em Inglês | MEDLINE | ID: mdl-32100014

RESUMO

Anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) and antisignal recognition particle (SRP) antibodies are frequently associated with immune-mediated necrotizing myopathy (IMNM). However, the difference in clinical manifestations between anti-HMGCR and anti-SRP antibodies is unclear. HMGCR is an essential enzyme for cholesterol biosynthesis and is inhibited by statins that regulate apoptosis of Bcl-2-positive and beta chemokine receptor 4 (CCR4)-positive lymphoma cells. In this study, we aimed to clarify Bcl-2 and CCR4 expressions of lymphocytes in anti-HMGCR antibody-positive IMNM and explore the difference between anti-HMGCR antibody-positive myopathy and other inflammatory myopathies. We retrospectively examined Bcl-2- and CCR4-positive lymphocyte infiltrations in muscle and skin biopsy specimens from 19 anti-HMGCR antibody-positive patients and 75 other idiopathic inflammatory myopathies (IIMs) patients. A higher incidence of Bcl-2- and CCR4-positive lymphocytes was detected in the muscle and skin of anti-HMGCR antibody-positive IMNM patients (p < 0.001). In 5 patients with anti-HMGCR antibodies, Bcl-2-positive lymphocytes formed lymphocytic accumulations, which were not observed in other IIMs. Low-density lipoprotein cholesterol levels were not increased except for patients with Bcl-2-positive lymphocytic accumulations (p = 0.010). Bcl-2 and CCR4 lymphocyte infiltrations could be a pathological characteristic of anti-HMGCR antibody-positive IMNM.


Assuntos
Hidroximetilglutaril-CoA Redutases/imunologia , Linfócitos/imunologia , Doenças Musculares/imunologia , Doenças Musculares/patologia , Miosite/imunologia , Miosite/patologia , Proteínas Proto-Oncogênicas c-bcl-2/imunologia , Receptores CCR4/imunologia , Adulto , Anticorpos/imunologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Músculo Esquelético/imunologia , Músculo Esquelético/patologia , Doenças Musculares/complicações , Miosite/complicações , Dermatopatias/imunologia , Dermatopatias/patologia
14.
Autoimmun Rev ; 19(4): 102498, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-32062029

RESUMO

Myastenia-Inflammatory Myopathy (MG-IM) association has been described in less than 50 cases, as isolated reports or in few case series. In most cases, MG and IM onset occur simultaneously even if the overlapping clinical manifestations could lead to delay the diagnosis in the early stage of disease. In these cases, thymic pathology is present in more than 50% of cases. Pathological findings can be consistent of polymyositis (63%), dermatomyositis (25%) or granulomatosis (12%). Accurate clinical manifestations and severity of IM in MG, including muscle specific antibodies (MSA) and muscle MRI, have not been systematically investigated and focal or mild subclinical myositis have not been reported. We observed that focal myositis or asymptomatic CK elevation can also occur in MG. In this review we have also retrospectively re-analyzed the clinical, serological, pathological and muscle imaging data from 13 patients with MG- IM from our cohort of 441 MG patients (2,9%). Clinical onset occurred simultaneously in 10/13 patients, whereas in 2 patients the IM appeared later in MG disease course (range 10-14 years) and conversely in 1 patient MG symptoms occurred later in IM disease course (4 years). Median age at disease onset was 51 year (range 24-73 years) regardless of clinical onset (MG or IM). Median clinical follow-up was 88 months (range 31-237 months). IM was suspected by CK elevation in all patients (ranging 800-3000 UI/L at first detection) and non-fatigable muscle weakness unresponsive to acetylcholinesterase inhibitors. All the patients presented mild to moderate MG symptoms. Three main categories of muscle involvement, sometimes overlapping, were recognizable: distal, proximal and subclinical myositits, leading to three main clinical groups (A,B,C) and two overlapping subgroups (A/B and B/C). Thymus pathology was present in 10/13 patients. Anti-AChR was detected in al all patients associated with anti-Titin and -RyR1 in those patients with thymoma. No MSA, nor MAA antibodies were detected. Muscle biopsy confirmed IM in all patients. In conclusion we redefined the clinical spectrum of muscle involvement in MG-IM association, which represent a continuum among 3 main clinical groups: distal, proximal and subclinical muscle involvement. Minimal muscle involvement and focal myositis could be underestimated among myasthenic patients and early aggressive immunotherapy could be required in focal group.


Assuntos
Debilidade Muscular/complicações , Debilidade Muscular/fisiopatologia , Miastenia Gravis/complicações , Miastenia Gravis/fisiopatologia , Humanos , Miosite/complicações , Miosite/fisiopatologia , Estudos Retrospectivos , Timoma/complicações
18.
J Clin Neurosci ; 74: 32-35, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31982271

RESUMO

Interstitial lung disease (ILD) is an extramuscular manifestation associated with increased mortality in idiopathic inflammatory myopathy (IIM). To identify risk factors for ILD in patients with IIM, this study retrospectively enrolled 117 eligible patients from a university medical center. After a comprehensive chart review, 56 patients were stratified into ILD (n = 28) and non-ILD (n = 28) groups. Clinical features, laboratory data, concomitant diseases, and serology profiles were compared. Patients with ILD had high prevalence of anti-Jo1 antibodies (p = 0.002), anti-Ro52 antibodies (p < 0.001), both anti-Jo1 and anti-Ro52 antibodies (p = 0.008), anti-Jo1 or anti-Ro52 antibodies (p < 0.001), and lower initial creatine kinase (CK) levels (p = 0.006). Moreover, patients with anti-Ro52 antibodies and either anti-Ro52 or anti-Jo1 antibodies had 9.17-fold (95% confidence interval [CI]: 2.858-33.487, p < 0.001) and 13.44-fold (95% CI: 4.008-52.757, p < 0.001) increased odds of developing ILD, respectively. By contrast, patients with higher CK levels had 0.99-fold (95% CI: 0.999-0.999, p = 0.011) increased odds of developing ILD. Both anti-Ro52 and anti-Jo1 antibodies were independent serological risk factors for IIM-associated ILD. Because these serology tests are commonly available, they can be used to guide pulmonary screening for patients with IIM to increase neurologist proactivity in recognizing and treating extramuscular conditions.


Assuntos
Doenças Pulmonares Intersticiais/diagnóstico , Miosite/complicações , Adulto , Idoso , Autoanticorpos/sangue , Creatina Quinase/sangue , Feminino , Histidina-tRNA Ligase/imunologia , Humanos , Doenças Pulmonares Intersticiais/sangue , Doenças Pulmonares Intersticiais/etiologia , Masculino , Pessoa de Meia-Idade , Miosite/diagnóstico , Estudos Retrospectivos , Ribonucleoproteínas/imunologia , Fatores de Risco
19.
Sci Rep ; 10(1): 1415, 2020 Jan 29.
Artigo em Inglês | MEDLINE | ID: mdl-31996780

RESUMO

MUC5B rs35705950 (G/T) is strongly associated with idiopathic pulmonary fibrosis (IPF) and also contributes to the risk of interstitial lung disease (ILD) in rheumatoid arthritis (RA-ILD) and chronic hypersensitivity pneumonitis (CHP). Due to this, we evaluated the implication of MUC5B rs35705950 in antisynthetase syndrome (ASSD), a pathology characterised by a high ILD incidence. 160 patients with ASSD (142 with ILD associated with ASSD [ASSD-ILD+]), 232 with ILD unrelated to ASSD (comprising 161 IPF, 27 RA-ILD and 44 CHP) and 534 healthy controls were genotyped. MUC5B rs35705950 frequency did not significantly differ between ASSD-ILD+ patients and healthy controls nor when ASSD patients were stratified according to the presence/absence of anti Jo-1 antibodies or ILD. No significant differences in MUC5B rs35705950 were also observed in ASSD-ILD+ patients with a usual interstitial pneumonia (UIP) pattern when compared to those with a non-UIP pattern. However, a statistically significant decrease of MUC5B rs35705950 GT, TT and T frequencies in ASSD-ILD+ patients compared to patients with ILD unrelated to ASSD was observed. In summary, our study does not support a role of MUC5B rs35705950 in ASSD. It also indicates that there are genetic differences between ILD associated with and that unrelated to ASSD.


Assuntos
Doenças Pulmonares Intersticiais/genética , Mucina-5B/genética , Miosite/genética , Adulto , Feminino , Seguimentos , Humanos , Incidência , Doenças Pulmonares Intersticiais/epidemiologia , Masculino , Pessoa de Meia-Idade , Miosite/complicações , Polimorfismo de Nucleotídeo Único , Regiões Promotoras Genéticas/genética
20.
Clin Exp Rheumatol ; 38(1): 67-73, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-31365334

RESUMO

OBJECTIVES: To investigate anti-TIF1-γ antibodies in longitudinally followed patients with myositis and cancer. METHODS: Serum levels of anti-TIF1-γ antibodies at different time-points in relation to myositis and cancer diagnosis were analysed by ELISA in 79 patients from a Swedish cohort with polymyositis (PM) and dermatomyositis (DM) and a Spanish cohort restricted to DM patients. Anti-TIF1-γ positive and negative patients were compared with Fisher's exact test, student t-tests and Wilcoxon test. RESULTS: Thirty-six patients (17 from cohort 1 and 19 from cohort 2) with myositis and cancer were anti-TIF1-γ antibody positive; all had DM. In 88% of anti-TIF1-γ positive patients, cancer was diagnosed within 3 years from DM diagnosis compared to 63% in anti-TIF1-γ negative. Four DM patients, anti-TIF1-γ positive at cancer diagnosis had positive serum samples even antedating cancer diagnosis up to five years. In cohort 1 the median (interquartile range) antibody level was higher, 2.13 au (1.82-2.15), in the seven patients who died <1 year after cancer diagnosis, compared to the seven that died >1 year after cancer diagnosis, 1.34 au (0.92-1.59), (p=0.004). Three patients were still alive and in remission from cancer and DM 14-16 years after cancer treatment of whom two became negative for anti-TIF1-γ antibodies. In the second cohort remission of cancer coincided with remission of DM and low or negative serum levels of autoantibodies. CONCLUSIONS: Anti-TIF1-γ antibodies may be detected before clinical symptoms of cancer and may disappear after successful treatment of cancer with remission of DM supporting DM being a paramalignant phenomenon.


Assuntos
Autoanticorpos , Dermatomiosite , Miosite , Neoplasias , Proteínas Nucleares , Polimiosite , Fatores de Transcrição , Humanos , Estudos Longitudinais , Miosite/complicações , Miosite/imunologia , Miosite/terapia , Neoplasias/complicações , Neoplasias/imunologia , Neoplasias/terapia , Proteínas Nucleares/imunologia , Fatores de Transcrição/imunologia
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA