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.

Miositis aguda benigna epidémica por influenza B en pacientes pediátricos / Epidemic mild acute myosistis due to influenza B in pediatric patients

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)

Clinical and immunological characteristics of myositis complicated with thromboembolism / 北京大学学报(医学版)

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.

Miositis viral, reporte de un caso pediátrico / Viral myositis, a pediatric case report

La miositis viral aguda es una complicación posterior a una infección viral causada, principalmente, por virus influenza A y B. Se caracteriza por ser un proceso inflamatorio transitorio, súbito, autolimitado y benigno. Generalmente, afecta a niños preescolares y escolares, que presentan dolor bilateral y sensibilidad en los grupos musculares de los miembros inferiores sin alteración en el examen neurológico. Puede generar alteración de la marcha o de la bipedestación. Su principal complicación es la rabdomiólisis, por lo que se debe hacer un seguimiento clínico y de los valores de la creatinfosfoquinasa. Se presenta el caso de un niño en edad escolar con diagnóstico de miositis viral aguda. Debido a que es poco frecuente y su incidencia en Latinoamérica es desconocida, se considera importante el reporte del caso y la revisión del tema, ya que su curso es benigno, de fácil manejo, y su conocimiento evita estudios y hospitalizaciones innecesarias.

Acute viral myositis is a complication after a viral infection mainly caused by Influenza A and B viruses. It is characterized as a transitory, sudden, self-limiting and benign inflammatory process. It usually affects preschool and school children presenting bilateral pain and sensitivity in the muscle groups of the lower limbs without alteration in the neurological examination. It can affect the gait or standing. The main complication is rhabdomyolysis, which is why clinical followup and values of creatine phosphokinase must be done. We present the case of a school-age child diagnosed with acute viral myositis. Because it is not a frequent entity and its incidence in Latin America is unknown, we consider important to report the case and review the topic, as its clinical course is benign, easily treated, and its knowledge can avoid unnecessary studies and hospitalizations.

Dermatopolimiositis en paciente adolescente. Reporte de caso / Dermatopolymyositis in adolescent patient. Case report

1011RESUMENLa Dermatopolimiositis (DPM) pertenece a las miopatías inflamatorias idiopáticas (MII), un grupo heterogéneo de miopatías autoin-munitarias sistémicas crónicas, asociadas con una alta morbilidad y discapacidad funcional. Comprende aquellas entidades de naturaleza adquirida que cursan con debilidad muscular y presentan de forma característica un infiltrado inflamatorio y necrosis celular en el tejido muscular estria-do. Es una enfermedad rara, con una inci-dencia global de 2­10 casos por millón de habitantes/año. Presentamos el caso de adolescente masculino de 14 años con antecedente de dermatomiositis, el cual presenta debilidad muscular proximal progresiva, acompañado de mialgias inten-sas e incapacitantes, presencia de eritema en heliotropo y pápulas de Gottron. Estu-dios laboratoriales que evidenciaron anemia, alteraciones enzimáticas, reactan-tes de fase aguda alterados, estudio electromiográfico que evidenció la presen-cia de polimiositis reactiva, y biopsia de tejido muscular que reportó cambios compatibles con DPM. El diagnóstico de miopatías inflamatorias se sospecha sobre la base de un conjunto de signos y síntomas y es confirmado mediante estudios comple-mentarios, entre los que se incluyen: eleva-ción de enzimas musculares, presencia de autoanticuerpos específicos de miositis,Dermatopolymyositis in adolescent patient. Case reportDermatopolimiositis en paciente adolescente. Reporte de casoelectromiografía con patrón miopático, hallazgos específicos en la biopsia. La PDM en niños tiene un comportamiento clínico diferente al adulto por la presencia vasculi-tis, existiendo varios desordenes que pueden confundir esta entidad y retardar su diagnóstico y tratamiento, por lo tanto, es muy importante el conocimiento de esta enfermedad en la edad pediátrica y estable-cer comparaciones con lo reportado en la literatura mundial...(AU)

Anticuerpos en miopatías inflamatorias idiopáticas / Antibodies in idiopathic inflammatory myopathies

Las miopatías inflamatorias idiopáticas (MII) comprenden un grupo de enfermedades multisistémicas de baja prevalencia que afectan tanto adultos como a niños, con ma-nifestaciones clínicas variables como lo son: debilidad muscular de predominio proxi-mal, alteraciones cutáneas (pápulas de Gottron, signo del chal, ulceras cutáneas), artritis, enfermedad pulmonar intersticial difusa (EPD), calcinosis y malignidad; en-marcadas en diferentes subtipos clínicos. Se cree que la autoinmunidad tiene un pa-pel clave en la patogénesis de estas enfermedades y como tal se han identificado autoanticuerpos en más del 50% de los pacientes con MII (algunos específicos y otros relacionados a miositis), lo cual ha permito clasificar diferentes características fenotí-picas e histológicas de estas enfermedades al igual de reconocer diferentes patrones de respuesta a tratamiento y factores pronósticos.En esta revisión mencionaremos los autoanticuerpos mas conocidos en relación a las miopatías inflamatorias idiopáticas, incluida la identificación de anticuerpos asocia-dos con las miopatía necrotizantes autoinmunes (MNA), la miositis por cuerpos de inclusión (MCI) y la asociación miositis - cáncer.

The Idiopathic inflammatory myopathies (IIM) comprise a group of low prevalence multisitemic diseases that affect both adults and children, with variable clinical man-ifestations such as muscular weakness of predominantly proximal, skin alterations (Gottron papules, sign of the shawl, skin ulcers), arthritis, diffuse interstitial lung dis-ease (ILD), calcinosis and malignancy; framed in different clinical subtypes. It is be-lieved that autoimmunity plays a key role in the pathogenesis of these diseases and as such autoantibodies have been identified in more than 50% of patients with IIM (some specific and others related to myositis), which has allowed to classify different phenotypic characteristics and histological of these diseases as well as recognizing different patterns of response to treatment and prognostic factors.In this review we will mention the most known autoantibodies in relation to idio-pathic inflammatory myopathies, including the identification of antibodies associated with autoimmune necrotizing myopathies (ANM), inclusion body myositis (IBM) and the myositis - cancer association.

Syndrome in question: antisynthetase syndrome (anti-PL-7)

Abstract: Antisynthetase syndrome is a rare autoimmune disease characterized by interstitial lung disease and/or inflammatory myositis, with positive antisynthetase antibodies (anti-Jo-1, anti-PL-7, anti-PL-12, ZO, OJ, anti-KE or KS). Other symptoms described include: non-erosive arthritis, fever, Raynaud's phenomenon, and "mechanic's hands." The first therapeutic option is corticotherapy, followed by other immunosuppressants. The prognosis of the disease is quite limited when compared to other inflammatory myopathies with negative antisynthetase antibodies.

Perfil clínico radiológico de 18 pacientes con síndrome antisintetasas atendidos en Policlínico de Pulmón Reumatológico, Instituto Nacional del Tórax / Clinic and radiologic profile of 18 patients with antisynthetase syndrome, treated in a Rheumatologic Lung Disease Clinic, Instituto Nacional del Torax

Connective Tissue Diseases (CTD) can manifest as Interstitial lung disease (ILD). ILD is a common manifestation of Anti-synthetase syndrome (AS). The main pattern of ILD in AS is nonspecific interstitial pneumonia (NSIP) with or without elements of organizing pneumonia (OP). Other less common forms include usual interstitial pneumonia (UIP) and nonspecific forms. Objectives: Describe radiological and clinical profile of 18 patients with ILD due to AS, evaluated in Instituto Nacional del Torax (INT) between 2013 and 2015. Highlighting the importance of Myositis Panel in patients being evaluated for ILD. Methods: Review of clinical records and lung CT of 76 patients with ILD and suspected AS, seen at INT between august 2013 and July 2015. Results: The diagnosis of AS was made in 18 of 76 patients, with female predominance and mean age of 46.5 years . In most patients the diagnosis of AS and ILD was simultaneous. The most frequent AS antibody was Jo-1, followed by PL-12. Less than half had ANA (+). The predominant radiographic pattern was NSIP / OP. Half of the patients had myositis. Conclusions: AS should be suspected in patients under study for ILD, especially NSIP / OP, and may occur without myositis and with negative ANA. It is essential to have new immunological tests such as a Myositis Panel, which will allow us to diagnose AS with subtle clinical features and negative or inconclusive serology...

Las Enfermedades del tejido conectivo (ETC) se pueden manifestar como Enfermedad Pulmonar Difusa (EPD). El Síndrome Antisintetasas (SAS) con mucha frecuencia presenta EPD. La forma de expresión más frecuente en SAS es la neumonía intersticial no específica (NSIP) con o sin elementos de neumonía en organización (OP). Otras formas menos frecuentes son la neumonía intersticial usual (UIP) y formas inespecíficas. Objetivos: Describir el perfil clínico radiológico de 18 pacientes con EPD secundaria a SAS, atendidos en el Instituto Nacional del Tórax (INT) entre los años 2013 y 2015. Destacar la importancia del Panel de Miositis en pacientes con EPD en estudio. Métodos: Revisión de ficha clínica y TAC pulmonar de 76 pacientes con EPD y sospecha de SAS, atendidos en INT entre agosto 2013 y julio 2015. Resultados: En 18 de 76 pacientes se hizo el diagnóstico de SAS, predominio femenino, edad promedio 46,5 años. En la mayoría el diagnóstico reumatológico y pulmonar fue simultáneo, el anticuerpo antisintetasa más frecuente fue Jo-1, seguido de PL 12. Menos de la mitad tuvo ANA (+). El patrón radiológico predominante fue NSIP/OP. La mitad de los pacientes no presentaron miositis. Conclusiones: El SAS debe ser sospechado en el estudio de pacientes con EPD, especialmente NSIP/OP y se puede presentar sin miositis y ANA (-). Es indispensable contar con nuevos exámenes inmunológicos como el Panel de Miositis, que permite diagnosticar SAS con clínica sutil y serología habitual negativa o no concluyente...

Polymyalgia rheumatica following paraspinal muscle inflammation and sacroiliitis

No abstract available.

Detection of parvovirus B19 in a case of erythema infectiosum with myositis.

A well documented case of erythema infectiosum is being reported here for the first time from India which was associated with myositis that has not been reported globally. A 9-year-old child presented with moderate to high grade fever, mild anemia, and erythematous rash involving face, trunks and limbs associated with arthralgia, myalgia and myositis. Parvovirus B19 infection was confirmed by detection of IgM antibodies (inhouse ELISA) and DNA (nested PCR) in patient's serum.

Malignancies in Korean Patients with Inflammatory Myopathy

The aim of this study was to assess the prevalence and the common type of malignancies in Korean patients with polymyositis (PM) and dermatomyositis (DM) and to evaluate the differences of clinical and laboratory findings between patients with malignancy and those without malignancy. Forty-one Korean patients, who were diagnosed as PM or DM, were enrolled in this study. They fulfilled the Bohan and Peter's criteria for a definite diagnosis of PM and DM. Patients with PM were 25 and those with DM were 16. Eleven out of 41 patients (26.8%) had malignancies. The malignancy was diagnosed simultaneously or later in 81.8% of patients with inflammatory myopathy (IM). The breast cancer was the most common malignancy. In this study, forty three years old as a screening age for malignancy had 88.9% sensitivity and 50.2% specificity. The serum levels of creatine kinase (CK) were significantly lower in patients with malignancy than those without malignancy.

Piomiositis no tropical y síndrome de superposición: lupus / Sjõgren / Nontropical pyomyositis and overlap syndrome: systemic lupus erythematosus and Sjõgren syndrome

La Piomiositis (PMS) es una infección primaria del músculo estriado, con progresión insidiosa de una colección purulenta, que se presenta generalmente en áreas tropicales denominándose por esa razón PMS tropical. La incidencia de PMS se ha incrementado últimamente en zonas templadas, denominándose a esta entidad como PMS no tropical, ya que existen algunas características que la diferencian de la PMS clásica. Presentamos el caso de una mujer de 39 años de edad con Síndrome de Superposición, Lupus Erimatoso Sistémico (LES) / Síndrome de Sjõgren (SS), que presentó durante la evolución de su enfermedad una PMS no tropical como ampliación atípica.

Tetraparesia fláccida / Flaccid tetraparesis

In this paper we review a clinical case of a man of 66 years old admitted to the medicine service of the Clinical Hospital of the University of Chile, with a clinical setting of flaccid tetraparesis associated with myalgias, dysphagia, dysphonia, reddish-violaceous eruption on the upper eyelids, periungual erythema and erythema distributed over the anterior neck and chest. We take this case to review the cases of flaccid tetraparesis that can be due to systemic, neuromuscular or psychiatric diseases. The clinical approach begins with the anamnesis: drugs consumption, or systemic diseases; continues with the physical examination orientated to stablish compromise of first or second motoneuron. Finally laboratory test as muscle enzymes, endocronologic test, and others like electromyography and muscle biopsy. We also review the actual tratments and the outcome and prognosis with each of them

Pyomyositis--with special reference to the comparison between extra- and intrapelvic muscle abscess.

Fifteen patients, nine males and six females, diagnosed with pyomyositis from 1988 to 1994, and followed for an average of 69.8 months, were reviewed. Excluding two children, the average age was 56.6 years. Eleven adults (73.3%) had underlying diseases. The lesions were multiple in five patients (33.3%) and a total of twenty-four muscle abscesses, including eleven extrapelvic and thirteen intrapelvic, were identified. When comparing extra- and intrapelvic pyomyositis, intrapelvic pyomyositis presents a diagnostic challenge requiring a high index of suspicion. Distinct clinical features such as local heat and painful swelling were all identified in extrapelvic pyomyositis, but they rarely (in only two of the thirteen lesions) emerged in intrapelvic pyomyositis. The average time from presentation to diagnosis was significantly longer in intrapelvic than in extrapelvic pyomyositis (1.4 vs 9.7 days). Although aspiration showed a high diagnostic rate in extrapelvic muscle abscesses, it was difficult to perform and was occasionally misinterpreted in intrapelvic cases. Routine X-rays were not helpful in making the diagnosis. CT scan was valuable because it provided positive diagnostic findings in all twelve patients who received one. The causative organisms in our patients were Staphylococcus aureus in eight (53.3%), Escherichia coli in three (20%), and Klebsiella in three (20%). Treatments consisted of parenteral antibiotics for all patients, image-guided aspiration in four patients, and surgical drainage in eleven patients. Two intrapelvic pyomyositis patients expired due to sepsis. At the completion of the study, twelve patients were asymptomatic without sequel, and one patient had a recurrence.

Relación entre algunos agentes microbianos y ciertas dermatosis, incluidas algunas colagenopatías / The relation between some microbial agents and some dermatoses, including collagen diseases

Basándonos en una amplia revisión bibliográfica, consideramos que la mayoría, sino todas las enfermedades del tejido conectivo tienen agentes etiológicos disparadores de fenómenos inmunes. La identificación de éstos permitirá ajustar las medidas terapéuticas y profilácticas e impedir, cuando sea posible, el contagio y/o las consecuencias alejadas de estas infecciones