Miositis aguda asociada a infección por virus influenza. Serie de casos en un hospital pediátrico / Acute myositis associated with influenza infection. Case series in a pediatric hospital

La miositis aguda benigna asociada a influenza es una complicación esporádica. En Argentina, en el año 2022, hubo un aumento temprano de la circulación de influenza y del número total de las notificaciones, con la aparición de miositis secundarias. Serie clínica retrospectiva de nueve pacientes pediátricos que consultaron por dolor e impotencia funcional de extremidades inferiores, y enzimas musculares elevadas, en el hospital Pedro de Elizalde de la Ciudad Autónoma de Buenos Aires, entre agosto y octubre del 2022. En todos se detectó infección por virus influenza y se recuperaron sin secuelas. La miositis aguda benigna es una entidad infrecuente en la infancia, cuyo diagnóstico es predominantemente clínico y de recuperación ad integrum. Debe ser sospechada en pacientes con clínica compatible en contexto de alta circulación viral. La vigilancia epidemiológica aporta herramientas para identificar los virus circulantes y sus posibles complicaciones.

Benign acute myositis associated with influenza is a sporadic complication. In Argentina, in 2022, there was an early increase in influenza circulation and the total number of notifications, with the appearance of secondary myositis. Retrospective clinical series of nine pediatric patients who consulted for pain and functional impotence of the lower extremities, and elevated muscle enzymes, at the Pedro de Elizalde hospital in the Autonomous City of Buenos Aires, between August and October 2022. In all of them, infection by influenza virus and recovered without sequelae. Benign acute myositis is a rare entity in childhood, whose diagnosis is predominantly clinical and recovery ad integrum. It should be suspected in patients with compatible symptoms in a context of high viral circulation. Epidemiological surveillance provides tools to identify circulating viruses and their possible complications.

Caracterización clínico-radiológica de pacientes con enfermedad pulmonar intersticial y panel de miositis positivo en hospital Santiago Oriente / Clinical-radiological characterization of patients with interstitial lung disease and positive myositis panel at hospital Santiago Oriente

Introducción: Las Enfermedades Pulmonares Intersticiales (EPI) afectan principalmente al intersticio pulmonar, con importante morbimortalidad asociada. Tienen un espectro de posibles etiologías que es cada vez más amplio. Hay una importante causalidad a partir de Enfermedades del Tejido Conectivo (ETC), describiéndose cada vez más casos asociados a Síndrome Antisintetasa, y con diversos patrones radiológicos según serología obtenida, agrupada en "Panel de Miositis" (PaM). El presente estudio de cohorte retrospectiva reúne PaMs realizados en el Hospital Santiago Oriente, correlacionando resultados con manifestaciones clínicas e imagenológicas. Material y Métodos: Se recuperaron 33 PaMs realizados entre 2017 y 2022, y a través de revisión de fichas de los pacientes de quienes provenían las PaMs se consignaron las principales manifestaciones clínicas, imagenológicas y de la serología reumatológica complementaria, estableciendo correlaciones entre múltiples variables. Resultados: Hubo 15 pacientes PaM positivos (45,4%), 8 de ellos (53%) ya contaban con alguna miopatía inflamatoria diagnosticada. Los principales hallazgos clínicos consignados fueron pápulas de Gottron, artritis, eritema heliotropo, Fenómeno de Raynaud y fiebre. El anticuerpo positivo más frecuente fue Ro-52. Se pudo objetivar ANA positivo en 10 casos (66,7%). Se identificó EPI en 66,7% de aquellos con PaM positivo, siendo la Neumonía Intersticial no específica fibrótica con Neumonía en Organización la manifestación más frecuente. No hubo asociación significativa entre manifestaciones imagenológicas y anticuerpos específicos. Se encontró ANA 1/80 en 66,7% de los casos, lo cual no se asoció a mayor riesgo de EPI. Conclusiones: Existe asociación entre varias ETC y las EPI. Destaca la importancia de los hallazgos clínicos para establecer un adecuado índice de sospecha, para dirigir oportunamente el estudio complementario (ej: PaM), y la eventual terapia específica.

Introduction: Interstitial Lung Diseases (ILD) mainly affect the pulmonary interstitium, with significant associated morbidity and mortality. They have a spectrum of possible etiologies that is increasingly broad. There is an important causality from Connective Tissue Diseases (CTD), describing more and more cases associated with Antisynthetase Syndrome, and with different radiological patterns according to the serology obtained, enclosed into "Panel of Myositis" (PaM). This retrospective cohort study gathers PaMs performed at Hospital Santiago Oriente, PaM results are correlated with clinical and imaging manifestations. Material and Methods: 33 PaMs performed between 2017 and 2022 were saved up and by reviewing the clinical records of the patients from whom the PaMs came, their clinical and radiological manifestations and the results of their complementary rheumatological serology were recorded to establish correlations between multiple variables. Results: There were 15 positive PaMs (45.4%), 8 (53%) of them already had some diagnosed inflammatory myopathy. The main clinical findings reported were Gottron's papules, arthritis, heliotrope erythema, Raynaud's phenomenon, and fever. The most frequent positive antibody detected was Ro-52. Positive ANA could be found in 10 cases (66.7%). PID was identified in 66.7% of those with a positive PaM, being non-specific fibrotic Interstitial Pneumonia with Organizing Pneumonia being the most frequent manifestation. There was no significant association between imaging manifestations and specific antibodies. ANA 1/80 was found in 6.7% of the cases, which was not associated with an increased risk of PID. Conclusions: There is association between several CTEs and EPIs. It is necessary to highlight the importance of the clinical findings to establish an adequate index of suspicion, in order to timely direct the complementary study (eg: PaM), and the eventual specific therapy.

Anti-HMGCR immune-mediated necrotizing myopathy: A case report / 北京大学学报(医学版)

The patient was a 55-year-old man who was admitted to hospital with "progressive myalgia and weakness for 4 months, and exacerbated for 1 month". Four months ago, he presented with persistent shoulder girdle myalgia and elevated creatine kinase (CK) at routine physical examination, which fluctuated from 1 271 to 2 963 U/L after discontinuation of statin treatment. Progressive myalgia and weakness worsened seriously to breath-holding and profuse sweating 1 month ago. The patient was post-operative for renal cancer, had previous diabetes mellitus and coronary artery disease medical history, had a stent implanted by percutaneous coronary intervention and was on long-term medication with aspirin, atorvastatin and metoprolol. Neurological examination showed pressure pain in the scapularis and pelvic girdle muscles, and V- grade muscle strength in the proximal extremities. Strongly positive of anti-HMGCR antibody was detected. Muscle magnetic resonance imaging (MRI) T2-weighted image and short time inversion recovery sequences (STIR) showed high signals in the right vastus lateralis and semimembranosus muscles. There was a small amount of myofibrillar degeneration and necrosis, CD4 positive inflammatory cells around the vessels and among myofibrils, MHC-Ⅰ infiltration, and multifocal lamellar deposition of C5b9 in non-necrotic myofibrils of the right quadriceps muscle pathological manifestation. According to the clinical manifestation, imageological change, increased CK, blood specific anti-HMGCR antibody and biopsy pathological immune-mediated evidence, the diagnosis of anti-HMGCR immune-mediated necrotizing myopathy was unequivocal. Methylprednisolone was administrated as 48 mg daily orally, and was reduced to medication discontinuation gradually. The patient's complaint of myalgia and breathlessness completely disappeared after 2 weeks, the weakness relief with no residual clinical symptoms 2 months later. Follow-up to date, there was no myalgia or weakness with slightly increasing CK rechecked. The case was a classical anti-HMGCR-IMNM without swallowing difficulties, joint symptoms, rash, lung symptoms, gastrointestinal symptoms, heart failure and Raynaud's phenomenon. The other clinical characters of the disease included CK as mean levels >10 times of upper limit of normal, active myogenic damage in electromyography, predominant edema and steatosis of gluteus and external rotator groups in T2WI and/or STIR at advanced disease phase except axial muscles. The symptoms may occasionally improve with discontinuation of statins, but glucocorticoids are usually required, and other treatments include a variety of immunosuppressive therapies such as methotrexate, rituximab and intravenous gammaglobulin.

Significance of anti-Jo-1 antibody's clinical stratification in idiopathic inflammatory myopathy and disease spectrum / 北京大学学报(医学版)

OBJECTIVE@#To investigate the significance of anti-histidyl tRNA synthetase (Jo-1) antibody in idiopathic inflammatory myopathies (IIM) and its diseases spectrum.@*METHODS@#We enrolled all the patients who were tested positive for anti-Jo-1 antibody by immunoblotting in Peking University People's Hospital between 2016 and 2022. And the patients diagnosed with anti-synthetase antibody syndrome (ASS) with negative serum anti-Jo-1 antibody were enrolled as controls. We analyzed the basic information, clinical characteristics, and various inflammatory and immunological indicators of the patients at the onset of illness.@*RESULTS@#A total of 165 patients with positive anti-Jo-1 antibody were enrolled in this study. Among them, 80.5% were diagnosed with connective tissue disease. And 57.6% (95/165) were diagnosed with IIM, including ASS (84/165, 50.9%), immune-mediated necrotizing myopathy (7/165, 4.2%) and dermatomyositis (4/165, 2.4%). There were 23.0% (38/165) diagnosed with other connective tissue disease, mainly including rheumatoid arthritis (11/165, 6.7%), undifferentiated connective tissue disease (5/165, 3.0%), interstitial pneumonia with autoimmune features (5/165, 3.0%), undifferentiated arthritis (4/165, 2.4%), Sjögren's syndrome (3/165, 1.8%), systemic lupus erythematosus (3/165, 1.8%), systemic vasculitis (3/165, 1.8%), and so on. Other cases included 3 (1.8%) malignant tumor patients, 4 (2.4%) infectious cases and so on. The diagnoses were not clear in 9.1% (15 /165) of the cohort. In the analysis of ASS subgroups, the group with positive serum anti-Jo-1 antibody had a younger age of onset than those with negative serum anti-Jo-1 antibody (49.9 years vs. 55.0 years, P=0.026). Clinical manifestations of arthritis (60.7% vs. 33.3%, P=0.002) and myalgia (47.1% vs. 22.2%, P=0.004) were more common in the ASS patients with positive anti-Jo-1 antibody. With the increase of anti-Jo-1 antibody titer, the incidence of the manifestations of arthritis, mechanic hands, Gottron sign and Raynaud phenomenon increased, and the proportion of abnormal creatine kinase and α-hydroxybutyric dehydrogenase index increased in the ASS patients. The incidence of myalgia and myasthenia were significantly more common in this cohort when anti-Jo-1 antibody-positive ASS patients were positive for one and more myositis specific antibodies/myositis associated autoantibodies (P < 0.05).@*CONCLUSION@#The disease spectrum in patients with positive serum anti-Jo-1 antibody includes a variety of diseases, mainly ASS. And anti-Jo-1 antibody can also be found in many connective tissue diseases, malignant tumor, infection and so on.

Miositis viral aguda en un paciente pediátrico con Covid-19: a propósito de un caso / Acute viral myositis in a pediatric patient with Covid-19: about a case

Se presenta el reporte de un caso de miositis aguda en un niño de 4 años de edad con COVID-19. El paciente manifestó fiebre y dolor en ambas pantorrillas. Con sospecha de miositis se realizó análisis de CPK, encontrando valores de 4460 UI/L. Asimismo se realizó hisopado nasofaríngeo para SARS-CoV 2, confirmando la infección. El paciente recibió hiperhidratación, presentando resolución de su cuadro clínico en menos de 5 días

This case report describes a 4 year old child with COVID-19. He presented with fever and pain in both calves. Under the suspicion of myositis a CPK analysis was performed, which showed CPK: 4460 UI/L. Nasopharyngeal RT - PCR was also performed, which was positive. As a treatment, the patient received hyperhydration, achieving full recovery after five days

Miositis por Bartonella henselae: caso clínico en una adolescente / Bartonella henselae myositis: case report in an adolescent

La miositis de origen vírico o bacteriano es frecuente en la edad pediátrica. Causa dolor muscular y debilidad, con fiebre y malestar general. Una causa es la infección por Bartonella henselae, bacteria implicada en la enfermedad por arañazo de gato que, a veces, causa afectación multisistémica. Se presenta el caso de una adolescente que acudió al servicio de urgencias por mialgia intensa, malestar, adelgazamiento y esplenomegalia. En el labortorio se observaron parámetros inflamatorios elevados. Refería contacto con un gato. Entre los estudios realizados, la resonancia magnética (RM) de miembros inferiores mostró una imagen compatible con miositis inflamatoria bilateral. En la RM abdominal, se observaron tres lesiones esplénicas no detectadas previamente y el fondo de ojo mostraba una lesión compatible con oclusión arterial retiniana o vasculitis. Se indicó tratamiento antibiótico por vía intravenosa durante 21 días con cefotaxima y cloxacilina, tras los cuales desaparecieron los signos y síntomas, aunque los reactantes inflamatorios persistieron elevados. Con base en el cuadro clínico (miositis + coriorretinitis + absceso esplénico) se pensó en una posible infección por B. henselae y se inició tratamiento oral con azitromicina y rifampicina durante 14 días. Luego del tratamiento, los valores de laboratorio fueron normales, así como la RM de control, y se constató una IgG positiva para la bacteria

Infectious myositis, whether viral or bacterial, is frequent in pediatric age. It causes muscle pain and weakness, associated with fever and general malaise. One cause is Bartonella henselae, responsible for cat scratch disease, which sometimes causes systemic symptoms. We report the case of an adolescent who came to the emergency room with intense myalgia, malaise, weight loss and splenomegaly. Blood tests showed high inflammatory markers. She had been in touch with a cat. Studies were carried out including: lower limbs MRI suggestive of bilateral inflammatory myositis, abdominal MRI with three previously undetected splenic lesions and dilated fundus examination that showed possible retinal arterial occlusion or vasculitis. After 21 days of intravenous antibiotic therapy (cefotaxime + cloxaciline), she became asymptomatic, but inflammatory markers remained high. Suspecting Bartonella henselaeinfection (myositis + chorioretinitis + splenic abscess), oral azithromycin and rifampicin were prescribed for 14 days. Blood tests and control MRI became normal, and IgG was positive.

Síndrome antisintetasa: reporte de un caso / Antisynthetase syndrome: a case report

El síndrome antisintetasa es una miopatía inflamatoria idiopática (MII) de origen autoinmune, poco frecuente, que se caracteriza por la presencia de autoanticuerpos antisintetasa ARNt (generalmente anti-Jo1), asociado frecuentemente a miositis, enfermedad pulmonar intersticial, poliartritis, manos de mecánico y fenómeno de Raynaud. Se reporta el caso de una mujer de 45 años de edad que presenta este síndrome con características fenotípicas de dermatomiositis y responde de forma favorable luego de la administración del tratamiento con glucocorticoides asociado a metotrexato.

Anti-synthetase syndrome is a rare autoimmune inflammatory myopathy characterized by autoantibodies against tRNA synthetases (most commonly anti-Jo1) with clinical features that include myositis, interstitial lung disease, polyarthritis, mechanic's hands and Raynaud's phenomenon. We report a 45-year-old woman who presented with dermatomyositis phenotypical features and a significant improvement with corticosteroids and metotrexate treatment.

Síndrome antisintetasa: reporte de un caso / Antisynthetase syndrome: a case repor

El síndrome antisintetasa es una miopatía inflamatoria idiopática (MII) de origen autoinmune, poco frecuente, que se caracteriza por la presencia de autoanticuerpos antisintetasa ARNt (generalmente anti-Jo1), asociado frecuentemente a miositis, enfermedad pulmonar intersticial, poliartritis, manos de mecánico y fenómeno de Raynaud. Se reporta el caso de una mujer de 45 años de edad que presenta este síndrome con características fenotípicas de dermatomiositis y responde de forma favorable luego de la administración del tratamiento con glucocorticoides asociado a metotrexato.

Anti-synthetase syndrome is a rare autoimmune inflammatory myopathy characterized by autoantibodies against tRNA synthetases (most commonly anti-Jo1) with clinical features that include myositis, interstitial lung disease, polyarthritis, mechanic's hands and Raynaud's phenomenon. We report a 45-year-old woman who presented with dermatomyositis phenotypical features and a significant improvement with corticosteroids and metotrexate treatment.

Características clínicas, inmunológicas y daño de órganos en pacientes con miopatías inflamatorias idiopáticas / Organ damage clinical and immunological characteristics in patients with idiopathic inflammatory myopathies

Introducción: Las miopatías inflamatorias idiopáticas constituyen un grupo de enfermedades musculares caracterizadas por debilidad muscular crónica e inflamación muscular de etiología desconocida. Objetivo: Identificar las características clínicas e inmunológicas y su relación con el daño de órganos en los pacientes con miopatías inflamatorias idiopáticas. Métodos: Se realizó estudio observacional, descriptivo, transversal, en 52 pacientes con diagnóstico de miopatía inflamatoria idiopática, seguidos en la consulta protocolizada de Reumatología del Hospital Clínico Quirúrgico Hermanos Ameijeiras entre enero 2016 y enero 2017. Para las variables cualitativas se calcularon los porcentajes de cada grupo. Se utilizó Chi-cuadrado de Pearson (estadístico exacto de Fisher). Nivel de significación del 95 por ciento (α = 0,05) para relacionar la presencia de anticuerpos y el tipo de miopatía así como la presencia de manifestaciones clínicas de MII. Resultados: El 80,8 por ciento fueron mujeres y 86,5 por ciento de procedencia urbana. La edad media al comienzo fue 42,8 ± 13,2 años, tiempo de demora al diagnóstico de 8,8 ± 7,0 meses, tiempo medio de evolución de la enfermedad de 7,5 ± 7,1 años. El 80,8 por ciento estaba en remisión, 50 por ciento tenía anticuerpos específicos. La hipertensión arterial se encontró en 28,8 por ciento de los pacientes y 23,1 por ciento presentó neumonía intersticial. La artritis estuvo presente en 96,2 por ciento. El 26,9 por ciento presentaron anticuerpos específicos Jo-1 y 21,2 por ciento Ro 52. Conclusiones: Predominaron los pacientes del sexo femenino en la cuarta década de la vida de procedencia urbana, los anticuerpos específicos encontrados más frecuentes fue el anti Jo-1, asociado a la presencia de neumopatía intersticial(AU)

Introduction: Idiopathic inflammatory myopathies constitute a group of muscle diseases characterized by chronic muscle weakness and muscle inflammation of unknown etiology. Objective: To identify the clinical and immunological characteristics and their relationship with organ damage in patients with idiopathic inflammatory myopathies. Methods: An observational, descriptive, cross-sectional study was carried out in 52 patients with diagnosis of idiopathic inflammatory myopathy, followed in the protocolized consultation of Rheumatology at Hermanos Ameijeiras Clinical and Surgical Hospital from January 2016 to January 2017. For the qualitative variables, the percentages of each group were calculated. Pearson's Chi-square (Fisher's exact statistic) was used. 95percent significance level (α = 0.05) was used to relate the presence of antibodies and the type of myopathy as well as the presence of clinical manifestations of MII. Results: 80.8percent were women and 86.5percent of urban origin. The mean age at the beginning was 42.8 ± 13.2 years, time delay to diagnosis was 8.8 ± 7.0 months, mean time of evolution of the disease of 7.5 ± 7.1 years. 80.8percent were in remission, 50percent had specific antibodies. Hypertension was found in 28.8percent of the patients and 23.1percent had interstitial pneumonia. Arthritis was present in 96.2percent. 26.9percent had specific Jo1 antibodies and 21.2percent had Ro 52. Conclusions: Urban female patients in the fourth decade of life predominated, the most frequent specific antibodies found was anti-Jo-1, associated with the presence of interstitial lung disease(AU)

Inclusión body myositis: A differential diagnosis to consider in patients with myopathy refractory to immunosuppressants. Two case reports / Miopatía por cuerpos de inclusión: un diagnóstico diferencial que considerar en pacientes con miopatía refractaria a inmunosupresores. Reporte de 2 casos

ABSTRACT Inclusion body myositis is part of the group of inflammatory myopathies, representing 30% of this group of diseases, and is considered an orphan disease because its estimated prevalence is less than 5 per 10,000 inhabitants. It produces weakness and atrophy of the proximal and distal muscles. The pathophysiological mechanisms are mainly autoimmune, inflammatory, and degenerative. The cases are presented of two female patients who came to : the emergency department due to progressive loss of upper and lower limb strength, and progressive asymmetric muscle weakness.

RESUMEN La miositis por cuerpos de inclusión forma parte del grupo de las miopatías inflamatorias, de las que representa el 30%; es considerada una enfermedad huérfana, ya que se estima que su prevalencia es menor a 5 por cada 10.000 habitantes. Produce debilidad y atrofia de los músculos proximales y distales. Los mecanismos fisiopatológicos son principalmente autoinmunes, inflamatorios y degenerativos. Se presentan 2 casos de mujeres, quienes acudieron a urgencias por pérdida progresiva de la fuerza en miembros superiores e inferiores, con debilidad muscular asimétrica de curso progresivo.

Cáncer de mama triple negativo asociado a dermatomiositis paraneoplásica: Reporte de caso y revisión de la literatura / Triple-negative breast cancer associated with paraneoplastic dermatomyositis: Case report and literature review

Resumen La dermatomiositis (DM) es un tipo de miopatía inflamatoria bien definida, inmunomediada, con afectación específica del músculo esquelético y con compromiso variable de piel y otros órganos. Se caracteriza por debilidad muscular proximal, lesiones cutáneas patognomónicas de dermatomiositis como el signo de Gottron, eritema violáceo o heliotropo, y evidencia de inflamación muscular por enzimas elevadas, cambios miopáticos en electromiografía y biopsia muscular anormal. Tiene una asociación bien establecida con diferentes tipos de cáncer pero es rara su asociación con cáncer de mama. Cuando se presentan de manera concomitante, su diagnóstico requiere un estudio multidisciplinario para orientar el origen paraneoplásico frente a una etiología propiamente autoinmune que requiera terapia inmunosupresora dirigida. Describimos el caso de una paciente con diagnóstico simultáneo de carcinoma infiltrante de mama triple negativo y criterios de dermatomiositis como manifestación paraneoplásica.

Abstract Dermatomyositis (DM) is a well-defined immune-mediated inflammatory myopathy, with specific involvement of skeletal muscle and variable involvement of skin and other organs. It is characterized by proximal muscle weakness, pathognomonic skin lesions of dermatomyositis such as Gottron's sign, violaceous or heliotrope rash, and evidence of muscle inflammation due to elevated enzymes, myopathic changes on electromyography, and abnormal muscle biopsy. It has a well-established association with different types of cancer, but its association with breast cancer is rare. When they occur concomitantly, their diagnosis requires a multidisciplinary study to confirm the paraneoplastic origin versus a primarily autoimmune etiology that may require targeted immunosuppressive therapy. We describe the case of a patient with a simultaneous diagnosis of triple-negative infiltrating breast carcinoma and criteria for dermatomyositis as a paraneoplastic manifestation.

Miopatía necrotizante autoinmune: Presentación de un caso clínico / Autoimmune necrotizing myositis: Presentation of a clinical case

Las miopatías inflamatorias (MI) son un grupo heterogéneo de enfermedades musculares de rara ocurrencia, caracterizadas por inflamación de los distintos componentes del tejido muscular, ya sea de forma aislada o, más comúnmente, en el contexto de una afección sistémica. Las miopatías necrotizantes inmunomediadas (MNIM) constituyen un subtipo de miopatía inflamatoria caracterizada por debilidad muscular proximal, necrosis de miofibrillas con mínimo infiltrado celular inflamatorio en la biopsia muscular e infrecuente compromiso extramuscular asociado1. Si bien existen similitudes clínicas e histopatológicas, el espectro de las miopatías inflamatorias es considerablemente variable. Por este motivo, es fundamental realizar estudios complementarios para la identificación correcta del subtipo de MI a fin de determinar su pronóstico e implementar un adecuado tratamiento. Se presenta el caso de una paciente de 29 años, sin antecedentes personales y heredofamiliares de enfermedad autoinmune ni antecedentes patológicos relevantes, que consulta a la Guardia Médica de nuestra Institución por un cuadro de dolor e impotencia funcional en los cuatro miembros, con debilidad muscular a predominio de cintura escapular y en menor medida pelviana, acompañado de astenia, tendencia al sueño e hiporreactividad.

Inflammatory myopathies (IM) or myositis are a heterogeneous group of muscle diseases of rare occurrence. Such diseases are characterized by inflammation of the different components of muscle tissue, which can occur either in isolation or, more commonly, as part of a systemic disorder. Immune-mediated necrotizing myopathies (IMNM) are a type of autoimmune myopathy characterized by proximal muscle weakness, myofiber necrosis with minimal inflammatory cell infiltrate on muscle biopsy and infrequent extramuscular involvement1. Even though there are clinical and histopathological similarities. The spectrum of inflammatory myopathies is considerably variable. Therefore, the performance of complementary studies is essential for the proper identification of the IM subtype to contribute accurately on treatment so determine the better prognosis. The present article shows the case of a young 29 years old, with no personal and family history background of autoimmune disease and no relevant pathological background. The patient consulted the medical ward of the Institution with pain, functional impairment of upper and lower extremities, muscle weakness mainly located in the pectoral girdle area and, although to a lesser degree, in the pelvic girdle area. It was also associated with asthenia, tendency to drowsiness and hyporeactivity.

Sarcoid-like granulomatous myositis-associated hypercalcemia. An infrequent case to consider / Hipercalcemia asociada a miositis granulomatosa sarcoidea simil- sarcoidea. Un caso infrecuente a considerar

Abstract PTH-independent hypercalcemia due to granulomatous disease is well-documented and sarcoidosis is the most characteristic disease, although there are others. We describe a case of sarcoid-like granulomatous myositis. An 87-year-old man was referred with tetraparesis and hypercalcemia (albumin-corrected calcium of 13.4 mg/dl) following a trip to the Caribbean. The evaluation showed a suppressed PTH, 25-hydroxy vitamin D of 7.5 ng/ml, 18F-FDG PET/CT showed markedly increased uptake in intercostal, back, shoulder, but tock and thigh muscles and a deltoid biopsy confirmed extensive granulomatous myositis. He was prescribed glucocorticoids which resulted in normalized plasma calcium levels and complete recovery from tetraparesis. Sarcoid-like granulomatous myositis should be incorporated into the differential diagnosis of PTH-independent hypercalcemia, especially in the absence of clinical features of sarcoidosis and with special emphasis on the use of 18F-FDG PET/CT to ensure a correct approach.

Resumen La hipercalcemia PTH-independiente asociada a enfermedades granulomatosas está bien documentada y la sarcoidosis es la enfermedad más característica, a pesar de que existen otras. Des cribimos un caso de miositis granulomatosa simil-sarcoidea. Un hombre de 87 años consultó por tetraparesia e hipercalcemia (calcio corregido por albúmina 13.4 mg/dl) luego de un viaje al Caribe. La evaluación mostró una PTH suprimida, 25-hidroxivitamina D 7.5 ng/ml, 18F-FDG PET/CT mostró marcado aumento de captación a nivel de musculatura intercostal, dorsal, deltoidea, glúteos y muslos. Una biopsia deltoidea confirmó una miositis granulomatosa extensa. Se prescribieron glucocorticoides, resultando en normalización del calcio plasmático y completa recuperación de la tetraparesia. La miositis granulomatosa simil-sarcoidea debe ser incorporada den tro del diagnóstico diferencial de la hipercalcemia PTH-independiente, especialmente en ausencia de hallazgos clínicos de sarcoidosis y con especial énfasis en el uso de 18F-FDG PET/CT para su correcta aproximación.

Características clínicas, inmunológicas y daño de órganos en pacientes con miopatías inflamatorias idiopáticas / Clinical and immunological characteristics and organ damage in patients with idiopathic inflammatory myopathies

RESUMEN Introducción: Las miopatías inflamatorias idiopáticas constituyen un grupo de enfermedades musculares caracterizadas por debilidad muscular crónica e inflamación muscular de etiología desconocida. Objetivo: Identificar las características clínicas e inmunológicas y daño de órganos en pacientes con miopatías inflamatorias idiopáticas. Método: Se realizó estudio observacional, descriptivo, transversal en 52 pacientes con diagnóstico de miopatía inflamatoria idiopática, seguidos en la consulta protocolizada de Reumatología del Hospital Clínico Quirúrgico "Hermanos Ameijeiras" entre enero 2016 y enero 2017. Para las variables cualitativas se calcularon los porcentajes de cada grupo. Se utilizó Chi-cuadrado de Pearson (Estadístico exacto de Fisher), nivel de significación del 95 % (α=0,05) para relacionar la presencia de anticuerpos y el tipo de miopatía, así como la presencia de manifestaciones clínicas de miopatías inflamatorias idiopáticas. Resultados: Del total de pacientes estudiadas, 80,8 % fueron mujeres, 61,5 % de color de piel negra, 86,5 % de procedencia urbana. La edad media al comienzo fue 42,8 ± 13,2 años, tiempo de demora al diagnóstico de 8,8 ± 7,0 meses, tiempo medio de evolución de la enfermedad de 7,5 ± 7,1 años, 80,8 % estaban en remisión, 50 % tenía anticuerpos específicos. La hipertensión arterial se encontró en 28,8 % de los pacientes y 23,1 % presentó neumonía intersticial. La artritis estuvo presente en 96,2 %, 26,9 % presentaron anticuerpos específicos Jo1 y 21,2 % Ro 52. Conclusiones: Predominaron los pacientes del sexo femenino, en la cuarta década de la vida, de procedencia urbana. Los anticuerpos específicos encontrado con más frecuencia fue el anti Jo-1, que se asoció a la presencia de neumopatía intersticial.

ABSTRACT Introduction: Idiopathic inflammatory myopathies constitute a group of muscle diseases characterized by chronic muscle weakness and muscle inflammation of unknown etiology. Objective: To identify the clinical and immunological characteristics and organ damage in patients with idiopathic inflammatory myopathies. Method: An observational, descriptive, cross-sectional study was carried out in 52 patients with diagnosis of idiopathic inflammatory myopathy, followed up in the protocolized service of Rheumatology at Hermanos Ameijeiras Clinical Surgical Hospital from January 2016 to January 2017. The qualitative variables were calculated with the percentages in each group. Pearson's Chi-square (Fisher's exact statistic) (95% significance level (α = 0.05) was used to relate the presence of antibodies and the type of myopathy as well as the presence of clinical manifestations of idiopathic inflammatory myopathies. Results: 80.8% were women of the total patients studied, 61.5% non-white skin color, 86.5% of urban origin. The mean age at the beginning was 42.8 ± 13.2 years, time delay to diagnosis was 8.8 ± 7.0 months, mean time of evolution of the disease of 7.5 ± 7.1 years. 80.8% were in remission, 50% had specific antibodies. Hypertension was found in 28.8% of the patients and 23.1% had interstitial pneumonia. Arthritis was present in 96.2%. We found 26.9% had specific Jo1 antibodies and 21.2% Ro 52. Conclusions: Urban origin female patients predominated, in their fourth decade of life, the more frequent specific antibodies found was anti Jo-1, which was associated with the presence of interstitial lung disease.

Infecciones graves de huesos, articulaciones y tejidos blandos adquiridas en la comunidad / Severe community-acquired bone, joint, and soft tissue infections

Introducción: Las infecciones de huesos, articulaciones y partes blandas, no solo han tenido una incidencia creciente en los últimos años en el Hospital Pediátrico Universitario Paquito González Cueto de Cienfuegos, sino también una evolución más grave. Objetivo: Describir aspectos clínicos y epidemiológicos de las infecciones graves de tejidos blandos, huesos y articulaciones. Métodos: Se revisó la base de datos computadorizada del Departamento de Estadística del Hospital Pediátrico Paquito González, para identificar todos los egresos de la Unidad de Cuidados Intensivos Pediátricos, con diagnóstico de celulitis o absceso de tejidos blandos, fascitis, miositis, osteomielitis, sepsis severa y shock séptico, en el periodo de enero de 2009 a diciembre de 2019. Se estudiaron las siguientes variables: localización de la infección de piel y tejidos blandos, grupo de edad, año del egreso y sitio de origen de la sepsis. Resultados: No hubo diferencias en la incidencia entre los grupos de edad. La celulitis de los miembros fue la forma clínica más frecuente. Se encontró una incidencia creciente de las infecciones de tejidos blandos. Las infecciones de huesos y articulaciones, representaron el segundo sitio de origen más frecuente de sepsis grave y el shock séptico. Conclusiones: Ha habido un aumento de las infecciones de piel y tejidos blandos que requieren ingreso en la unidad de cuidados intensivos del citado hospital en los últimos 11 años. Estas infecciones de conjunto con las infecciones de huesos y articulaciones, constituyen un problema de salud puesto que provocan sepsis grave y shock séptico con una incidencia significativa(AU)

Introduction: Infections of bones, joints and soft parts have not only had an increasing incidence in recent years at ¨Paquito González Cueto¨ University Pediatric Hospital in Cienfuegos, but also a more serious evolution. Objective: Describe clinical and epidemiological aspects of severe soft tissues, bones and joints infections. Methods: The computerized database of the Department of Statistics of ¨Paquito González¨ Pediatric Hospital was reviewed to identify all discharges from the Pediatric Intensive Care Unit with a diagnosis of cellulite or soft tissue abscess, fasciitis, myositis, osteomyelitis, severe sepsis and septic shock, in the period from January 2009 to December 2019. The following variables were studied: location of skin and soft tissue infection, age group, year of discharge, and sepsis origin location. Results: There were no differences in the incidence between age groups. Limb cellulite was the most common clinical form. An increasing incidence of soft tissue infections was found. Bone and joint infections represented the second most frequent origin location of severe sepsis and septic shock. Conclusions: There has been an increase in skin and soft tissue infections that require admission to the intensive care unit of the aforementioned hospital in the last 11 years. These infections, together with bone and joint infections, constitute a health problem since they cause severe sepsis and septic shock with a significant incidence(AU)

Recent research on myositis-specific autoantibodies in juvenile dermatomyositis / 中国当代儿科杂志

Juvenile dermatomyositis (JDM) is an autoimmune disease manifesting as proximal muscle weakness and skin rash and can involve multiple systems and visceral organs. Myositis-specific autoantibodies (MSAs) are highly associated with various complications and prognosis in JDM. Patients with anti-Mi-2 antibodies tend to have good prognosis and typical clinical symptoms. Patients with anti-MDA5 antibodies often have diffuse interstitial lung disease and skin ulcer, with mild symptoms of myositis. Patients with anti-NXP2 antibodies often have calcinosis, and such antibodies are associated with gastrointestinal bleeding and perforation. Patients with anti-TIF1-γ antibodies have diffuse and refractory skin lesions. Anti-SAE antibodies are rarely detected in children, with few reports of such cases. This article reviews the features of clinical phenotypes in JDM children with these five types of MSAs, so as to provide a basis for the clinical treatment and follow-up management of children with JDM.

Long-term rituximab treatment of refractory idiopathic inflammatory myopathy: A report of 3 cases / 北京大学学报(医学版)

Idiopathic inflammatory myopathies are a group of rare but serious diseases. The treatment of refractory idiopathic inflammatory myopathy is always challenging, especially in children. Three cases of refractory idiopathic inflammatory myopathy treated by rituximab were reported and discussed with the review of relevant literature. All were female with on-set age of 8 years and 6 months, 11 years and 7 months, 4 years and 2 months old, respectively. All had acute onset, presenting with progressive and severe muscle weakness. All lost ambulation within 1 or 2 months, with difficult swallowing and low voice. Respiratory distress occurred in case 2 after an attack of asphyxia due to an aspiration of sputum, and ventilator support was required for 1 month. Rashes were detected at the initial stage of the disease in cases 2 and 3. Patient 2 showed facial erythematous papules, spreading to her neck and hands. Patient 3 showed purplish eyelids with peri-orbital swelling, generalized edema involving all her limbs. Creatine kinase (CK) levels were markedly elevated in all the patients, ranging from 6 000 IU/L to 28 819 IU/L. Anti-SRP antibody was identified in cases 1, and anti-NXP2 antibodies were confirmed in cases 2 and 3. MRI of both thighs in all the patients showed profound muscle and fascial edema. Muscle pathology of patient 1 showed prominent fiber variation and endomysial fibrosis, with overexpression of MHC-Ⅰ. While muscle pathology in patients 2 and 3 showed scattered fiber necrosis, regeneration, endomysial edema without inflammatory cell infiltration. All the patients were diagnosed with idiopathic inflammatory myopathy and failed to the initial treatment including adequate glucocorticoids and high-dose immunoglobulin therapy. Other immunosuppressants (methotrexate, cyclophosphamide) were also tried in cases 2 and 3 with poor response. Then all the patients were treated with rituximab combined with glucocorticoids. Patient 1 regained normal strength and discontinued rituximab at the end of her last follow-up (2 years and 7 mouths). Though calcinosis developed during the follow-up period, significant improvement was noticed in cases 2 and 3 (both regained the ability to walk independently) at the end of their last follow-up after 2 years and 8 months, 3 years and 2 months respectively. Long-term rituximab therapy may improve the prognosis of refractory idiopathic inflammatory myopathy, especially with positive anti-SRP and anti-NXP2 antibodies.

Clinical and immunological characteristics of 88 cases of overlap myositis / 北京大学学报(医学版)

OBJECTIVE@#To investigate the clinical and immunological characteristics of overlap myositis (OM) patients.@*METHODS@#The data of 368 patients with idiopathic inflammatory myopathies (IIMs) admitted to Peking University People's Hospital from January 2004 to August 2020 were analyzed retrospectively, including demographic characteristics, clinical characteristics (including fever, Gottron' s sign/papules, Heliotrope rash, V-sign, Shawl sign, Mechanic' s hands, skin ulceration, periungual erythema, subcutaneous calcinosis, dysphagia, myalgia, myasthenia, arthritis, Raynaud' s phenomenon, interstitial lung disease, pulmonary hypertension and myocardial involvement), laboratory characteristics, immunological characteristics [including antinuclear antibodies, rheumatoid factors, myositis-associated autoantibodies (MAAs) and myositis-specific autoantibodies (MSAs)] and survival. The clinical and immunological characteristics and prognostic differences of OM and non-OM were compared. The Kaplan-Meier and Log Rank methods were used to analyze the survival.@*RESULTS@#A total of 368 patients were included. 23.9% (88/368) of IIMs patients were OM patients. Among the 88 OM patients, 85.2% (75/88) of them were female, and the median interval between disease onset and diagnosis was 13.5 months. The incidence of overlapped connective tissue diseases in the OM patients was dermatomyositis (DM) in 60.2%, polymyositis (PM) in 3.4%, immune-mediated necrotizing myopathy (IMNM) in 2.3% and anti-synthetase syndrome (ASS) in 34.1%. Compared with the non-OM patients, the proportion of the females in the OM patients was higher (85.2% vs. 72.1%, P=0.016), the OM patients had longer disease duration [13.5(4.5, 48.0) months vs. 4.0(2.0, 12.0) months, P < 0.001]. As for clinical characteristics, compared with the non-OM patients, the incidence of V-sign (25.0% vs. 44.6%, P=0.001) and periungual erythema (8.0% vs. 19.6%, P=0.013) were lower; the incidence of Raynaud's phenomenon (14.8% vs. 1.8%, P < 0.001), interstitial pneumonia (88.6% vs. 72.1%, P=0.001), pulmonary hypertension (22.7% vs. 7.5%, P < 0.001) and myocardial involvement (18.2% vs. 9.3%, P=0.033) were higher. As for immunological characteristics, compared with the non-OM patients, the incidence of elevated aspartate aminotransferase (AST) (31.8% vs. 45.0%, P=0.035) was lower and elevated C-reactive protein (CRP) (58.0% vs. 44.6%, P=0.037) was higher; the positive rates of antinuclear antibodies (ANA) (85.1% vs. 63.4%, P=0.001) and rheumatoid factors (RF) (40.2% vs. 17.8%, P < 0.001) and anti-Ro-52 (71.6% vs. 56.1%, P=0.038) in serum were higher. There was no significant difference in the survival between the OM patients and non-OM patients.@*CONCLUSION@#Pulmonary hypertension and myocardial involvement were frequently observed in OM.

Clinical and immunological characteristics of patients with anti-synthetase syndrome complicated with cardiac involvement / 北京大学学报(医学版)

OBJECTIVE@#To investigate the clinical and immunological features of cardiac involvement in patients with anti-synthetase syndrome (ASS).@*METHODS@#In the study, 96 patients diagnosed with ASS hospitalized in the Department of Rheumatology and Immunology, Peking University People's Hospital from April 2003 to November 2020 were included. The patients were divided into two groups according to whether they were accompanied with cardiac involvement. Demographic features, clinical characteristics (Gottron's sign/papules, muscle damage, etc.), comorbidities, laboratory indices (creatine kinase, inflammatory indicators, immunoglobulin, complement, lymphocyte subset, autoantibodies, etc.) were collected and the differences between the two groups were analyzed statistically.@*RESULTS@#The prevalence of cardiac involvement in the patients with ASS was 25.0% (24/96). The ASS patients complicated with cardiac involvement presented with elevated cardiac troponin I (cTnI, 75.0%, 18/24), pericardial effusion (33.3%, 8/24), reduction of left ventricular function (33.3%, 8/24) and valves regurgitation (33.3%, 8/24). The age of onset of the patients with cardiac involvement was older than that of the patients without cardiac involvement [(54.58±10.58) years vs. (48.47±13.22) years, P=0.043). Arthritis was observed less frequently in the patients with cardiac involvement than those without cardiac involvement (37.5% vs. 61.1%, P=0.044). In addition, rapidly progressive interstitial lung disease (54.2% vs. 30.6%, P=0.037) was observed more frequently in the patients with cardiac involvement than those without cardiac involvement. As compared with the ASS patients without cardiac involvement, C-reactive protein (CRP) [(13.55 (8.96, 38.35) mg/L vs. 4.60 (1.37, 17.40) mg/L, P=0.001], and lactate dehydrogenase (LDH) [408.0 (255.0, 587.0) U/L vs. 259.5 (189.8, 393.8) U/L, P=0.007] were significantly higher in the patients with cardiac involvement. Anti-Ro-52 antibody was detected more commonly in the ASS patients with cardiac involvement compared with the patients without cardiac involvement (91.7% vs. 69.4%, P=0.029). No significant differences were found in the comorbidities, alanine transaminase (ALT), aspartate transaminase (AST), creatine kinase (CK), erythrocyte sedimentation rate (ESR), ferritin (Fer), immunoglobulin G (IgG), complement 3 (C3), complement 4 (C4), lymphocyte subset between the two groups.@*CONCLUSION@#Cardiac involvement is common in ASS, mainly manifested as myocardial damage. It is necessary to be aware of cardiac complications in patients with elevated CRP, elevated LDH and positive anti-Ro-52 antibody.

Punica granatum and amygdalin extracts plus cobalamin combined with albendazole reduce larval burden and myositis in experimental trichinosis / Extratos de Punica granatum e amígdalina mais cobalamina combinado com albendazol reduzem a carga larvar e a miosite na triquinose experimental

Abstract Trichinellosis is a zoonosis results from eating raw or semi-cooked meat of infected animals. Medicinal plants have been used lately as alternatives and/or combined therapies to resolve some drawbacks of the current regimens. This work analyzed the effect of albendazole monotherapy on Trichinella spiralis experimental infection (group A), in comparison to P. granatum and amygdalin extracts +cobalamin (group B), plus its combination with albendazole (group C). The study revealed that the extracts alone or combined with albendazole had an inferior effect to albendazole monotherapy regarding number of adult worms (40.83 ±3.82, 18.67 ±1.86 and 16.83 ±2.32, respectively). However, their effect was more obvious in muscle phase combined with albendazole, achieving the lower number of larvae/mL tissue homogenate (22.33 ±3.27 in comparison to 39.67 ±2.58 achieved by albendazole monotherapy). The extracts exerted a significant immunomodulatory effect by reducing the local CD4+ expression in the intestine as well as in muscle phase (1.15 ±0.25 and 3.80 ±0.65 in comparison to 4.97 ±0.37 and 12.20 ±0.87 with albendazole monotherapy, respectively). So, these extracts improved the therapeutic efficacy of albendazole, specifically in muscle phase and counteracted the inflammatory reaction caused by albendazole monotherapy, thus extensively alleviating the resulting myositis.

Resumo Trichinellosis é uma zoonose resultante da ingestão de carne crua ou semicozida de animais infectados. As plantas medicinais têm sido usadas, ultimamente, como alternativas e/ou terapias combinadas, para resolver algumas desvantagens dos regimes atuais. Este trabalho analisou o efeito da monoterapia albendazole na infecção experimental por Trichinella spiralis (grupo A), em comparação com extratos de P. granatum e amígdalina +cobalamina (grupo B), além de sua combinação com albendazol (grupo C). O estudo revelou que os extratos sozinho ou combinado com albendazol teve efeito inferior à monoterapia albendazol em relação ao número de vermes adultos (40,83 ±3,82, 18,67 ±1,86 e 16,83 ±2,32, respectivamente). No entanto, seu efeito foi mais óbvio na fase muscular combinado com o albendazol, alcançando o menor número de larvas/mL homogeneizado de tecido (22,33 ±3,27 em comparação com 39,67 ±2,58 obtidos pela monoterapia albendazol). Os extratos exerceram um efeito imunomodulatório significativo, ao reduzir a expressão local CD4+ no intestino, bem como na fase muscular (1,15 ±0,25 e 3,80 ±0,65 em comparação com 4,97 ±0,37 e 12,20 ±0,87 com monoterapia albendazol, respectivamente). Assim, esses extratos melhoraram a eficácia terapêutica do albendazol, especificamente na fase muscular e neutralizaram a reação inflamatória causada pela monoterapia albendazol, aliviando extensivamente a miosite resultante.