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1.
Rev. baiana saúde pública ; 45(3,supl.n.esp): 98-107, 28 dec. 2021.
Article in English | LILACS | ID: biblio-1352336

ABSTRACT

Cutaneous manifestations of dermatomyositis are unusual and difficult to treat. This study aimed to report a case of cutaneous manifestations of dermatomyositis treated with hyperbaric oxygen. We present a case of dermatomyositis in a 44-year-old female with pain ulcers in her left leg for 17 months, refractory to an exclusive clinical treatment, who underwent a hyperbaric oxygen therapy (HBOT) breathing O2 100%, 90 minute sessions, six days a week, at 2.4 ATA. HBOT therapy proved to be highly efficacious in wound healing in this case and HBOT should be considered as a treatment in the assistance given to such patients.


Manifestações cutâneas da dermatomiosite são raras e difíceis de ser tratadas. O objetivo deste estudo é relatar um caso de manifestações cutâneas da dermatomiosite tratadas com oxigenioterapia hiperbárica, ocorrido em uma paciente de 44 anos, sexo feminino, com úlceras dolorosas em seu membro inferior esquerdo por 17 meses. O caso foi refratário ao tratamento clínico exclusivo e a paciente submetida a tratamento de oxigenioterapia hiperbárica (HBOT), respirando oxigênio a 100%, em sessões de noventa minutos, seis dias por semana, a 2,4 ATA. O HBOT mostrou ser altamente eficaz na cicatrização da lesão neste caso e deve ser um tratamento considerado no cuidado desses pacientes.


Las manifestaciones cutáneas de la dermatomiositis son inusuales y difíciles de tratar. El objetivo de este estudio fue reportar un caso de manifestaciones cutáneas de dermatomiositis tratadas con oxigenoterapia hiperbárica. Presentamos un caso de dermatomiositis en una mujer de 44 años con úlceras dolorosas en su pierna izquierda durante 17 meses, refractaria a un tratamiento clínico exclusivo, que se sometió a oxigenoterapia hiperbárica (HBOT) respirando O2 100%, sesiones de 90 minutos, seis días a la semana, a 2,4 ATA. La terapia con HBOT demostró ser muy eficaz en la cicatrización de heridas en este caso y la HBOT debe considerarse como un tratamiento en la asistencia brindada a estos pacientes.


Subject(s)
Humans , Female , Skin Manifestations , Ulcer , Dermatomyositis , Research Report , Hyperbaric Oxygenation
2.
Rev. cuba. med ; 60(2): e1345, tab
Article in Spanish | LILACS, CUMED | ID: biblio-1280351

ABSTRACT

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.


Subject(s)
Humans , Female , Dermatomyositis/diagnosis , Myositis/epidemiology , Epidemiology, Descriptive , Cross-Sectional Studies , Observational Study
3.
Rev. colomb. gastroenterol ; 36(supl.1): 91-97, abr. 2021. graf
Article in Spanish | LILACS | ID: biblio-1251554

ABSTRACT

Resumen Los pacientes con dermatomiositis presentan un mayor riesgo de asociación con patología maligna subyacente, sin conocerse una causa precisa de esta relación. Son múltiples las posibles localizaciones anatómicas, entre las que se incluyen los ovarios, mamas, zona gástrica, zona colorrectal, sangre, pulmones y próstata. Se presenta el caso de una mujer de 58 años que durante el estudio de pérdida anormal de peso manifestó debilidad muscular grave y alteraciones dermatológicas asociadas con el hallazgo de adenocarcinoma del colon transverso.


Abstract Patients with dermatomyositis are more likely to have an underlying malignancy, although the exact cause of this association is unknown. There are multiple possible anatomical sites, including ovaries, breasts, stomach, colorectum, blood, lungs, and prostate. We present the case of a 58-year-old woman who during abnormal weight loss study showed severe muscle weakness and skin alterations, associated with finding of adenocarcinoma of the transverse colon.


Subject(s)
Humans , Female , Middle Aged , Colorectal Neoplasms , Colon, Transverse , Dermatomyositis , Skin , Weight Loss , Literature
4.
Adv Rheumatol ; 61: 12, 2021. tab
Article in English | LILACS | ID: biblio-1152748

ABSTRACT

Abstract Background: To assess the prevalence and clinical relevance of anti-Jo-1 autoantibodies in a representative sample of patients with definite dermatomyositis (DM). Methods: This retrospective cohort study took place from 2005 to 2020 and assessed 118 adult patients from a tertiary center who were diagnosed with definite DM. A commercial kit was used to detect anti-Jo-1 autoantibodies. Results: The presence of anti-Jo-1 autoantibodies was observed in 10 out of 118 (8.5%) patients with definite DM. The following variables were comparable between individuals with and without anti-Jo-1 autoantibodies: age at diagnosis, sex, ethnicity, disease duration, follow-up period, recurrence rate, complete clinical response, death rate, and cancer incidence. There was no difference in clinical features between groups, except for an increased prevalence of "mechanic's hands," joint involvement, and lung disease, as well as a reduced occurrence of skin findings in patients positive for anti-Jo-1 autoantibodies. No anti-Jo-1-positive patients went into remission; they required greater use of glucocorticoids and immunosuppressive drugs. Conclusions: Anti-Jo-1 positivity was found in 8.5% of patients with definite DM. This autoantibody was associated with an antisynthetase syndrome phenotype and might predict clinical outcomes in patients with definite DM.(AU)


Subject(s)
Humans , Adult , Autoantibodies/analysis , Dermatomyositis/physiopathology , Histidine-tRNA Ligase/blood , Retrospective Studies , Cohort Studies , Muscular Diseases/physiopathology
5.
Article in English | WPRIM | ID: wpr-922392

ABSTRACT

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.


Subject(s)
Autoantibodies , Dermatomyositis , Humans , Lung Diseases, Interstitial/etiology , Myositis , Prognosis
6.
Article in English | WPRIM | ID: wpr-880629

ABSTRACT

Dermatomyositis (DM) is a kind of idiopathic inflammatory myopathy characterized by chronic proximal skeletal muscle weakness and unique skin lesions. However, DM with exfoliation of esophageal mucosa is rare. A 36-year-old male patient complained of muscular soreness of extremities, dysphagia, and pharyngalgia was diagnosed with DM with exfoliation of esophageal mucosa. After treatment with glucocorticoid, immunosuppressant, acupuncture, and endoscopic submucosal dissection (ESD), the above symptoms were disappeared. During the 3-year follow-up period, the results of routine physical examination, laboratory examination, gastroscopy, and imaging examination were normal. High-dose of corticosteroid is needed in the initial treatment, but it must be reduced regularly to avoid adverse reactions. Acupuncture and ESD are also effective as adjuvant therapy.


Subject(s)
Adult , Dermatomyositis/complications , Endoscopic Mucosal Resection , Esophageal Mucosa , Esophageal Neoplasms , Gastroscopy , Humans , Male , Treatment Outcome
7.
Article in Chinese | WPRIM | ID: wpr-880063

ABSTRACT

OBJECTIVE@#To present one patient initially diagnosed with dermatomyositis(DM) who was eventually revealed to be diffuse large B-cell lymphoma(DLBCL) complicated with hemophagocytic syndrome(HPS), and to improve the understanding of the disease.@*METHODS@#The clinical characteristics, diagnostic approach, treatment of the patient were retrospectively analyzed, and some related literatures were reviewed.@*RESULTS@#A 52-year-old female patient suffered from muscle weakness, elevated serum creatine kinase activity, electromyography changes and characteristic skin rashes and diagnosed as DM. The patient was treated with glucocorticoid therapy and the muscle strength, skin rashes, and creatine kinas index turns into remission. Subsequently, subcutaneous nodules appeared during treatment, and the patient was confirmed as DLBCL based on pathological biopsy; And the patient was considered HPS because of presenting with repeated fever, splenomegaly, cytopenias, hypofibrinogenemia, hypertriglyceridemia, hyperferritinemia, high levels of sCD25, low NK-cell activity and hemophagocytosis in bone marrow. But the patient refused chemotherapy, and only treated with "DXM+VP-16" to control hemophagocytic syndrome, and unfortunately died due to the disease progression.@*CONCLUSION@#Cutaneous involvement in diffuse large B-cell lymphoma and hemophagocytic syndrome patients with first presentation of dermatomyositis is relatively rare. Malignacy screening should be performed as soon as possible after newly diagnosed DM, so that the patient can get early diagnosis and effective treatment to improve survival rate.


Subject(s)
Dermatomyositis/complications , Etoposide , Female , Humans , Lymphohistiocytosis, Hemophagocytic/diagnosis , Lymphoma, Large B-Cell, Diffuse/complications , Middle Aged , Retrospective Studies
8.
Rev. Méd. Paraná ; 79(1): 97-101, 2021.
Article in Portuguese | LILACS | ID: biblio-1282503

ABSTRACT

INTRODUÇÃO: A calcinose é uma complicação frequente da esclerose sistêmica e da dermatomiosite, resultando em dor local, contraturas, ulcerações, infecções secundárias e limitação funcional. Não há tratamento eficaz para calcinose mas o tiossulfato de sódio tem se mostrado promissor. OBJETIVOS: Analisar a resposta terapêutica ao tiossulfato de sódio 10% intralesional nas calcinoses de pacientes com dermatomiosite e esclerose sistêmica. MÉTODOS: Estudo prospectivo, não controlado, incluindo 7 pacientes, totalizando 10 calcinoses, sendo um paciente com dermatomiosite e 6 com esclerose sistêmica. Desfechos primários: melhora da dor pela escala visual analógica e diminuição do maior e menor diâmetros das calcinoses ao raio-x. Desfechos secundários: melhora da qualidade de vida pelo SF12 (Medical Outcomes Study Short Form 12) e da função pelo HAQ (Health Assesment Questionnaire). Resultados foram expressos em médias e as variáveis contínuas comparadas pelo teste t-Student ou pelo teste de Wilcoxon. RESULTADOS: Foram realizadas 2 a 8 aplicações intralesionais (média de 3,7), com média de 8,9mg de tiossulfato por injeção a intervalos de 13 a 56 dias, com intervalo médio de 19 dias. Todos os pacientes relataram melhora da dor, porém esse resultado não foi significativamente estatístico. Não houve redução do diâmetro das calcinoses, nem melhora da qualidade de vida ou funcional. CONCLUSÃO: Baixas doses de tiossulfato de sódio administradas por injeções intralesionais, em intervalos médios de 19 dias, em número limitado de aplicações, não foram efetivas para o tratamento da calcinose


BACKGROUND: Calcinosis is a frequent complication of systemic sclerosis and dermatomyositis, resulting in local pain, contractures, ulcerations, secondary infections and functional limitations. There is no effective treatment for calcinosis, but sodium thiosulphate has shown to be promising. OBJECTIVE: To analyze the therapeutic response to 10% intralesional sodium thiosulphate on calcinosis of patients with dermatomyositis and systemic sclerosis. METHODS: Non-controlled prospective study, with 7 patients and 10 calcinoses, of which one had dermatomyositis and six had scleroderma. Primary outcomes: improvement in the pain by the visual analogue scale, and a decrease in both diameters (biggest and smallest) of calcinoses on X-ray. Secondary outcomes: improvement in quality of life by SF12 (Medical Outcomes Study Short Form 12), and of function by HAQ (Health Assessment Questionnaire). Results were expressed as means; continuous variables were compared by the t-Sudent test or by the Wilcoxon test. RESULTS: Two to 8 intralesional applications were performed (mean of 3.7), with mean of 8.9mg of thiosulphate per injection, at 13-56 days intervals (mean of 19 days). All patients reported improvement in pain, albeit such result was not statistically significant. There was no reduction in the diameters of calcinoses, nor there were quality of life or functional improvements. CONCLUSION: Low doses of sodium thiosulphate, administered through intralesional injections, in mean intervals of 19 days, in a limited number of applications, were not effective for calcinosis treatment


Subject(s)
Humans , Scleroderma, Systemic , Therapeutics , Calcinosis , Dermatomyositis , Thiosulfates
9.
Rev. cuba. reumatol ; 22(3): e749,
Article in Spanish | LILACS, CUMED | ID: biblio-1144534

ABSTRACT

La dermatomiositis es una enfermedad que afecta con mayor frecuencia a pacientes mayores de 60 años y preferiblemente del sexo femenino. Sin embargo, en algunas ocasiones aparece en la edad infantil. Se exponen los elementos medulares que permiten el diagnóstico de esta enfermedad. Se presenta el caso de una escolar de 7 años de edad que acude al servicio de emergencia con manifestaciones clínicas sugerentes de una dermatomiositis juvenil. Las manifestaciones clínicas y los resultados de los exámenes complementarios permitieron llegar al diagnóstico definitivo de la enfermedad. Fue necesario hacer el diagnóstico diferencial con otras afecciones que cursan con síntomas similares. La paciente comenzó a mostrar una evolución favorable, lo que motivó el alta hospitalaria con tratamiento para el hogar y seguimiento por consulta externa con la especialidad de Reumatología. Conclusiones: A pesar de presentar un patrón epidemiológico predominante en adultos mayores, la dermatomiositis puede aparecer en edades más tempranas. Aunque las manifestaciones clínicas orientan hacia su diagnóstico, este se confirma con la ayuda de determinados exámenes complementarios(AU)


Dermatomyositis is a disease that occurs more frequently in patients older than 60 years and preferably female; however, sometimes it occurs in children. To publicize the core elements that allow the diagnosis of this disease. The case of a 7-year-old schoolgirl who comes to the emergency service with clinical manifestations suggesting a juvenile dermatomyositis is presented. The clinical manifestations and the results of the complementary examinations allowed to reach the definitive diagnosis of the disease, it was necessary to make the differential diagnosis with other conditions that present with similar symptomatology. Conclusions: despite presenting a predominant epidemiological pattern in older adults, dermatomyositis may occur at earlier ages; although the clinical manifestations are oriented towards its diagnosis, it is confirmed with the help of certain complementary tests(AU)


Subject(s)
Humans , Female , Child , Rheumatology , Dermatomyositis , Academic Performance , Diagnosis, Differential
10.
Rev. colomb. reumatol ; 27(3): 224-229, jul.-set. 2020. tab, graf
Article in Spanish | LILACS | ID: biblio-1251663

ABSTRACT

RESUMEN Los síndromes paraneoplásicos neurológicos se presentan en menos del 1% de los tumores sólidos y son infrecuentes en linfomas. Se asocian a tumores con alta actividad biológica y condicionan deterioro funcional y discapacidad. La dermatomiositis se asocia a cáncer, por tanto obliga al estudio de neoplasias ocultas; su diagnóstico como síndrome paraneoplásico se establece con criterios específicos. El pronóstico funcional depende del diagnóstico oportuno, control del cáncer y de la regulación de la respuesta inmunológica. Se presenta el caso de una mujer de 65 años con dermatomiositis en el curso de un linfoma B marginal variante convencional de primario cutáneo.


ABSTRACT Neurological paraneoplastic syndromes occur in less than 1% of solid tumours and are uncommon in lymphomas. They are related to tumours with high biological activity and cause functional impairment and disability. Dermatomyositis is associated with cancer, and requires the study of hidden neoplasms. Its diagnosis as a paraneoplastic syndrome is established with specific criteria. Functional prognosis depends on early diagnosis, cancer control, and regulation of the immune response. The case is presented of a 65 year-old woman with dermatomyositis during the course of a conventional variant of a primary cutaneous B marginal lymphoma.


Subject(s)
Humans , Female , Middle Aged , Dermatomyositis , Lymphoma , Paraneoplastic Syndromes , Diagnosis , Neoplasms
11.
Rev. méd. Chile ; 148(2): 160-167, feb. 2020. tab, graf
Article in Spanish | LILACS | ID: biblio-1115772

ABSTRACT

Background: Certain associated and specific myositis antibodies are related to certain clinical phenotypes of dermatomyositis (DM), disease severity and the presence of cancer. Aim: To describe the clinical profile of Chilean patients with DM and their associated and specific myositis antibodies. Material and Methods: Review of medical records of 15 patients with DM aged 31 to 72 years. Their clinical characteristics, laboratory tests and complementary tests were reviewed. In serum samples from each patient the presence of 16 specific antibodies was analyzed by immunoblot technique (Myositis Profile Euroline Blot test kit). Results: Fourteen (93.3%) patients had skin manifestations, five (33.3%) had pulmonary involvement, two (13.3%) had an associated cancer and nine (60%) had specific antibodies associated with myositis. Conclusions: These patients with DM had a clinical profile similar to what has been described elsewhere. The profile of myositis specific antibodies was different from reports in other populations.


Subject(s)
Humans , Male , Female , Middle Aged , Aged , Autoantibodies/blood , Dermatomyositis/diagnosis , Skin/immunology , Skin/pathology , Autoantibodies/immunology , Dermatomyositis/etiology , Dermatomyositis/blood
12.
Rev. argent. reumatolg. (En línea) ; 31(1): 12-17, 2020. ilus
Article in Spanish | LILACS, BINACIS | ID: biblio-1123749

ABSTRACT

Las Miopatías Inflamatorias Idiopáticas (MII) son un grupo heterogéneo de enfermedades que se caracterizan por debilidad muscular e inflamación subyacente en la biopsia muscular. Los principales órganos afectados son el músculo, la piel y también puede afectarse el pulmón. Se distinguen dentro de los subtipos clínicos como Polimiositis (PM), Dermatomiositis (DM), DM con la variante Dermatomiositis Clínicamente Amiopática (DMCA), el Síndrome Antisintetasa (SAS), la Miositis Necrotizante Inmunomediada, la Miositis por Cuerpos de Inclusión (MCI) y la Miositis Asociada a Neoplasia. La presencia de ciertos anticuerpos específicos y asociados predispone al desarrollo de manifestaciones clínicas, determinando el pronóstico de la enfermedad. Se presentan 4 pacientes del Registro de MII de la Sociedad Argentina de Reumatología (SAR) con estas características: un paciente con PM y anti Jo-1 positivo y tres pacientes con DM (uno con DMCA y anti- RO 52 y dos pacientes con anti-PL7 y anti-TIF1γ respectivamente).


Idiopathic Inflammatory Myopathies (MII) are a heterogeneous group of diseases characterized by muscle weakness and inflammation underlying muscle biopsy. The main organs affected are muscle, skin and the lung can also be affected. They are distinguished within clinical subtypes such as Polymyositis (PM), Dermatomyositis (DM), DM with the variant Clinically Amiopathic Dermatomyositis (DMCA), the Syndrome Antisynthetase (SAS), Immune-mediated Necrotizing Myositis, Body Myositis Inclusion (MCI) and Neoplasia-Associated Myositis. The presence of certain specific and associated antibodies predisposes to the development of clinical manifestations, determining the disease prognosis. 4 patients from the Registry of MII of the Argentine Society of Rheumatology (SAR) are presented with these characteristics: one patient with PM and anti Jo-1 positive and three patients with DM (one with DMCA and anti-RO 52 and two patients with anti-PL7 and anti-TIF1γ respectively).


Subject(s)
Humans , Myositis , Rheumatology , Dermatomyositis , Lung Diseases
14.
Article in Chinese | WPRIM | ID: wpr-828933

ABSTRACT

OBJECTIVE@#To investigate the classification of idiopathic inflammatory myopathies (IIM) based on clinical manifestations and myositis- specific antibodies using cluster analysis.@*METHODS@#We retrospectively analyzed the data of patients with IIM admitted in Nanfang Hospital in 2015-2019. The clinical data of the patients including serum creatine kinase (CK), interstitial lung disease (ILD), cancer, and myositis-specific antibodies were collected for two-step cluster analysis to identify the distinct clusters of patients, whose clinical characteristics were subsequently analysed.@*RESULTS@#A total of 71 patients with IIM were included in this study, including 30 (42.3%) with polymyositis (PM), 20 (28.2%) with classic dermatomyositis (DM), 16 (22.5%) with amyopathic dermatomyositis (CADM), and 5 (7.0%) with immune-mediated necrotizing myopathy (IMNM). Two-step cluster analysis identified 3 distinctive subgroups: Cluster 1 of 15 (51.7%) patients characterized by rash, positive anti-MDA5 antibody and hypoproteinemia ( < 0.05) with normal or slightly elevated CK level, mainly corresponding to CADM; Cluster 2 of 4 (57.1%) patients with significantly elevated CK and positive anti-SRP antibody ( < 0.001) corresponding to IMNM; and Cluster 3 of 17 (48.6%) patients consisting primarily of patients with PM, characterized by positivity for anti- aminoacyl transfer RNA synthetases antibodies (=0.022) corresponding to antisynthetase syndrome (ASS).@*CONCLUSIONS@#Patients with IIM can be divided into 3 subgroups based on their clinical and serological characteristics (especially myositis-specific antibodies), and among them ASS may represent an independent IIM subgroup with unique clinical characteristics.


Subject(s)
Antibodies , Autoantibodies , Dermatomyositis , Humans , Lung Diseases, Interstitial , Myositis , Retrospective Studies
15.
Article in Chinese | WPRIM | ID: wpr-828088

ABSTRACT

Xiaoxuming Decoction is an ancient classic herbal formula for the treatment of stroke. In ancient times, the connotation of stroke was very extensive, including facial neuritis, acute cerebral infarction, acute cerebral hemorrhage, sequelae of cerebral hemorrhage, unexplained weakness of limbs, cervical spondylosis, acute myelitis, acute radiculitis, Guillain Barre syndrome, multiple sclerosis, myasthenia gravis, motor neuron disease, dermatomyositis, hypokalemic paralysis peripheral neuritis. It has been identified that: ①Xiaoxuming Decoction is very common in the treatment of cerebrovascular diseases, such as cerebral infarction, cerebral hemorrhage and other cerebrovascular diseases, facial neuritis, acute myelitis, acute radiculitis, Guillain Barre syndrome, unexplained limb weakness, multiple sclerosis, motor neuron disease, myasthenia gravis, and rheumatic and immune system diseases, such as dermatomyositis, and can not only alleviate symptoms, but also improve prognosis and the long-term survival rate. ②Sudden limb failure, facial paralysis, and hypoalgesia without heat syndrome are the key indications of Xiaoxuming Decoction. ③This is a special prescription for the treatment of acute facial neuritis, and can cure in one week in the combination with moxibustion. ④In the treatment of facial neuritis complicated with hypertension or acute cerebrovascular disease, Xiaoxuming Decoction generally has a certain antihypertensive effect, without any hypertensive effect, which reflected its two-way regulatory effect for blood pressure. ⑤In the treatment of unknown limb weakness, acute myelitis, acute radiculitis, Guillain Barre syndrome, Xiaoxuming Decoction can rapidly alleviate the symptoms. ⑥This is the basic formula for multiple sclerosis and motor neuron disease. Long term use of Xiaoxuming Decoction can alleviate the symptom of limb weakness, reduce the occurrence of complications and delay the progress of the disease, but with a poor long-term prognosis. ⑦In the treatment of myasthenia gravis, Xiaoxuming Decoction can significantly improve muscle strength, and gradually help stop hormone reduction. After thymoma surgery, Xiaoxuming Decoction is also applicable to some patients with recurrent myasthenia gravis. ⑧Xiaoxuming Decoction also plays a role in the treatment of dermatomyositis and cervical spondylopathy. ⑨Raw ephedra is the monarch drug of Xiaoxuming Decoction, which is the key to the effect. The dosage starts with 6 g is titrated in a small dose and increases gradually. In addition, this formula is forbidden for those with red face, fast heart rate, high blood pressure, blocked stool, red tongue, yellow fur, wiry and rapid pulse or powerful pulse, and spout pulse.


Subject(s)
Dermatomyositis , Facial Nerve Diseases , Guillain-Barre Syndrome , Humans , Motor Neuron Disease , Multiple Sclerosis , Myasthenia Gravis , Myelitis , Radiculopathy , Spondylosis
16.
Rev. cuba. reumatol ; 22(supl.1): e849, graf
Article in Spanish | LILACS, CUMED | ID: biblio-1280395

ABSTRACT

La incidencia de la miopatía inflamatoria idiopática es de 4 a 15 casos por cada millón de habitantes y su prevalencia de 60 por cada millón de habitantes. La dermatomiositis idiopática es más frecuente en las mujeres, aunque su asociación a fibrosis pulmonar es muy rara y solo se reporta en un 2 por ciento de los casos. Se describe el caso de un paciente de 50 años de edad, femenina, que presentó debilidad a nivel de la cintura escapular acompañada de fatiga. Tenía lesiones de rascado en diferentes regiones del cuerpo por prurito y lesiones eritematosas en la piel en ambos muslos. Además, se quejaba de dolores articulares generalizados con impotencia funcional y mialgias generalizadas progresivas e hipotrofia muscular de varios grupos musculares. El estudio analítico reveló enzimas musculares elevadas. La biopsia de piel y músculo mostró elementos sugestivos de dermatomiositis. Con la espirometría se detectó trastornos ventilatorios restrictivos de grave intensidad. Mediante la radiografía de tórax se halló infiltrado difuso peribroncovascular asociado a un trayecto fibroso y la tomografía axial computarizada precisó el pulmón con consolidación alveolar y discreto engrosamiento pleural. La paciente fue tratada con prednisona a 1 mg/kg/día asociado con azatioprina 1,5 mg/kg/día. Este tratamiento fue muy eficaz, y se logró una notable recuperación clínica y por estudios de laboratorio. Reportamos el caso de una paciente con dermatomiositis idiopática y fibrosis pulmonar. Esta asociación constituye un hallazgo infrecuente en nuestro medio y más aun con el paciente asintomático(AU)


The incidence of Idiopathic Inflammatory Myopathy is from 4 to 15 cases per million inhabitants and its prevalence of 60 per million inhabitants. Idiopathic dermatomyositis is more frequent in women; Although its association with pulmonary fibrosis is described, it is very infrequent, it is only reported in 2 percent of cases. To describe a diagnosed case of idiopathic dermatomyositis and pulmonary fibrosis. A 50-year-old patient presented weakness at the level of the shoulder girdle accompanied by fatigue. Physical examination: Skin: scratching lesions in different regions of the body due to pruritus, erythematous lesions at the level of the skin on both thighs. Osteomyoarticular system: generalized joint pains with functional impotence and progressive generalized myalgias and muscular hypotrophy of several muscle groups. The analytical study revealed elevated muscle enzymes. The skin and muscle biopsy showed elements suggestive of dermatomyositis. Chest X-ray: diffuse peribronchovascular infiltrate associated with fibrous path. Spirometry: restrictive ventilatory disorders of severe intensity. Computed tomography of the lung with alveolar consolidation and discrete pleural thickening. We report the case of a patient with idiopathic dermatomyositis and pulmonary fibrosis. This association is an uncommon finding in our environment and even more so when the patient is asymptomatic(AU)


Subject(s)
Humans , Female , Middle Aged , Spirometry/methods , Azathioprine/therapeutic use , Prednisone/therapeutic use , Dermatomyositis/diagnosis , Myositis/epidemiology , Fatigue
17.
Rev. chil. reumatol ; 36(4): 115-119, 2020.
Article in Spanish | LILACS | ID: biblio-1282551

ABSTRACT

La Dermatomiositis Juvenil representa el 75-80% de las miopatías inflamatorias juveniles. Si bien, tiene baja incidencia y prevalencia, presenta importante morbilidad dada por sus manifestaciones cutáneas, musculares, pulmonares, gastrointestinales, cardiacas, entre otras. Corresponde a un desorden poligénico con múltiples factores gatillantes, que determina el desarrollo de una vasculopatía que lleva a atrofia muscular, inflamación y activación de vías del IFN-1. Actualmente su diagnóstico se basa en las guias EULAR/ACR (2017). En los últimos años, se han descubiertos distintos subtipos de la enfermedad, basados en el perfil de autoanticuerpos específicos de miositis, lo que ha permitido establecer pronóstico y estrategias terapéuticas personalizadas. El manejo farmacológico continúa basándose principalmente en el uso de corticoesteroides y DMARDs, así como también terapia biológica; en los últimos años, los inhibidores JAK han mostrado resultados promisorios, convirtiéndose en la más nueva alternativa terapéutica para el control de la enfermedad.


Juvenile Dermatomyositis represents 75-80% of juvenile inflammatory myopathies. Although it has a low incidence and prevalence, it presents significant morbidity due to its cutaneous, muscular, pulmonary, gastrointestinal and cardiac manifestations, among others. It corresponds to a polygenic disorder with multiple triggering factors, which determines the development of a vasculopathy that leads to muscle atrophy, inflammation and activation of IFN-1 pathways. Currently its diagnosis is based on the EULAR/ACR guidelines (2017). In recent years, different subtypes of the disease have been discovered, based on the profile of myositis-specific autoantibodies, which has made it possible to establish prognosis and personalized therapeutic strategies. Pharmacological management continues to be based mainly on the use of corticosteroids and DMARDs, as well as biological therapy; In recent years, JAK inhibitors have shown promising results, becoming the newest therapeutic alternative for disease control.


Subject(s)
Humans , Dermatomyositis/classification , Dermatomyositis/diagnosis , Dermatomyositis/therapy , Biological Therapy , Adrenal Cortex Hormones/therapeutic use , Antirheumatic Agents/therapeutic use , Dermatomyositis/epidemiology , Janus Kinase Inhibitors
18.
J. bras. nefrol ; 41(4): 580-584, Out.-Dec. 2019. graf
Article in English | LILACS | ID: biblio-1056602

ABSTRACT

Abstract In kidney biopsies reviews, scleroderma renal crisis (SRC) is characterized by vascular endothelial injuries, C4d deposits on peritubular vessels, and acute and chronic injuries coexisting on the same biopsy. The clinical signs of thrombotic microangiopathy (TMA) are described in systemic sclerosis (SSc), nevertheless, it has not been related to acute injuries described on kidney biopsies. We report a case of SRC in a patient with scleroderma-dermatomyositis overlap syndrome, which also showed clinical and histopathological data of TMA. On fundus examination, a severe acute hypertensive retinopathy was found. The kidney biopsy showed severe endothelial damage with widening of mucoid cells at the level of the intima, focal concentric proliferation on most small arterioles, and C3, C4d, and IgM deposits along the capillary walls. The genetic study of complement only showed the presence of membrane cofactor protein (MCP) risk haplotypes, without other genetic complement disorders. We understand that in a patient with TMA and SSc, the kidney damage would be fundamentally endothelial and of an acute type; moreover, we would observe clear evidence of complement activation. Once further studies correlate clinical-analytical data with anatomopathological studies, it is likely that we will be forced to redefine the SRC concept, focusing on the relationship between acute endothelial damage and complement activation.


Resumo Nas revisões de biópsias renais, a crise renal esclerodérmica (CRE) é caracterizada por lesões endoteliais vasculares, depósitos de C4d em vasos peritubulares e lesões agudas e crônicas que coexistem na mesma biópsia. Os sinais clínicos de microangiopatia trombótica (MAT) são descritos na esclerose sistêmica (ES); no entanto, não foram relacionados às lesões agudas descritas nas biópsias renais. Relatamos um caso de CRE em um paciente com síndrome de superposição de esclerodermia-dermatomiosite, que também apresentou dados clínicos e histopatológicos de MAT. No exame de fundo do olho, foi encontrada uma retinopatia hipertensiva aguda grave. A biópsia renal mostrou lesão endotelial grave com alargamento das células mucoides ao nível da íntima, proliferação concêntrica focal na maioria das pequenas arteríolas e depósitos de C3, C4d e IgM ao longo das paredes dos capilares. O estudo genético do complemento mostrou apenas a presença de haplótipos de risco da proteína cofator de membrana (PCM), sem outros distúrbios genéticos do complemento. Entendemos que em um paciente com MAT e ES, o dano renal seria fundamentalmente endotelial e do tipo agudo; além disso, observaríamos evidências claras de ativação do complemento. Uma vez que novos estudos correlacionam dados clínico-analíticos com estudos anatomopatológicos, é provável que sejamos forçados a redefinir o conceito de CRE, enfocando a relação entre dano endotelial agudo e ativação do complemento.


Subject(s)
Humans , Male , Middle Aged , Raynaud Disease/complications , Vision Disorders/etiology , Acute Kidney Injury/etiology , Kidney/blood supply , Capillaries/metabolism , Angiotensin-Converting Enzyme Inhibitors/therapeutic use , Immunohistochemistry , Papilledema/pathology , Dermatomyositis/complications , Dermatomyositis/immunology , Hypertensive Retinopathy/diagnosis , Hypertensive Retinopathy/pathology , Hypertensive Retinopathy/drug therapy , Acute Kidney Injury/diagnosis , Anemia, Hemolytic/diagnosis , Anemia, Hemolytic/etiology , Kidney/pathology , Kidney/diagnostic imaging
19.
Acta pediátr. hondu ; 10(1): 1011-1020, abr.-sept. 2019. ilus, tab
Article in Spanish | LILACS | ID: biblio-1050914

ABSTRACT

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)


Subject(s)
Humans , Male , Adolescent , Muscle Weakness , Dermatomyositis/diagnosis , Myositis/complications , Erythema
20.
Rev. cuba. reumatol ; 21(2): e60, mayo.-ago. 2019.
Article in Spanish | LILACS, CUMED | ID: biblio-1093819

ABSTRACT

Introducción: La búsqueda bibliográfica realizada estableció una problemática a atender: la escasez de estudios de caso de dermatomiositis juvenil, por lo que la presente investigación pretende arrojar luz sobre esta patología, poco reflejada en la literatura médica. Nótese, además, que la sistematización puede servir de reservorio bibliográfico para estudios de posgrados de especialistas médicos. Dermatomiositis juvenil. Sistematización de casos: sistematizar y comparar 10 casos de dermatomiositis juvenil, publicados en las principales revistas médicas en cuanto a la edad del paciente, antecedentes de salud, cuadro clínico, resultados de complementarios, diagnóstico diferencial, manejo. Dermatomiositis juvenil. Sistematización de casos: hasta un 30 por ciento de los pacientes con dermatomiositis juvenil puede presentar calcinosis, especialmente en puntos de presión como codos, rodillas, dedos y glúteos. La calcinosis puede estar presente en el momento del diagnóstico, pero corrientemente se establece luego de 1 a 3 años y puede provocar la aparición de úlceras cutáneas, mengua de los rangos articulares, dolor e inflamación local. Alrededor del 10 por ciento de los pacientes con dermatomiositis juvenil puede presentar úlceras cutáneas. El estudio de su evolución suele anunciar un curso severo de la enfermedad con debilidad constante, calcinosis extensa y mala respuesta al tratamiento. Conclusiones: resulta importante sistematizar los estudios relacionados con casos de alteraciones dermatológicas de la dermatomiositis juvenil, ya que la enfermedad constituye una manifestación notable, tanto como marcador de actividad como de su daño derivado. Así también, pueden coadyuvar a lograr una percepción estadística más clara de la tasa de morbilidad y su consecuente relación con los pronósticos(AU)


Introduction: Literature search established a problem to be addressed: the scarcity of case studies of juvenile dermatomyositis, which is why this research aims to shed light on this pathology, little reflected in the medical literature. Note also that systematization can serve as a bibliographic reservoir for postgraduate studies of medical specialists. Dermatomiositis juvenil. Sistematización de casos: to systematize and compare 10 cases of juvenile dermatomyositis, published in the main medical journals regarding patient's age, health history, clinical picture, complementary results, differential diagnosis, management. Dermatomiositis juvenil. Sistematización de casos: up to 30 percent of patients with juvenile dermatomyositis can present calcinosis, especially in pressure points such as elbows, knees, fingers and buttocks. Calcinosis may be present at the time of diagnosis but is usually established after 1 to 3 years and may cause the appearance of skin ulcers, decreased joint ranges, pain and local inflammation. About 10 percent of patients with juvenile dermatomyositis may have skin ulcers. The study of its evolution usually announces a severe course of the disease with constant weakness, extensive calcinosis and poor response to treatment. Conclusions: it is important to systematize the studies related to cases of dermatological alterations of juvenile dermatomyositis, since the disease constitutes a remarkable manifestation, both as a marker of activity and of its derived damage. Likewise, they can help to achieve a clearer statistical perception of the morbidity rate and its consequent relationship with prognosis(AU)


Subject(s)
Humans , Skin Ulcer/etiology , Calcinosis/diagnostic imaging , Dermatomyositis/diagnosis , Diagnosis, Differential , Joints/injuries , Dermatomyositis/epidemiology
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