A new proposal for phenotypic classification and outcome assessment of dermatomyositis based on clinical manifestations and serological testing.

BACKGROUND: Dermatomyositis (DM) is an infrequent disease subgroup of idiopathic inflammatory myopathies characterized by distinct skin lesions. However, high heterogeneity makes clinical diagnosis and treatment of DM very challenging. OBJECTIVES: Unsupervised classification in DM patients and analysis of key factors related to clinical outcomes. METHODS: This retrospective study was conducted between 2017 and 2022 at the Department of Rheumatology, Xiangya Hospital, Central South University. 162 DM patients were enrolled for unsupervised hierarchical cluster analysis. In addition, we divided the clinical outcomes of DM patients into four subgroups: withdrawal, stabilization, aggravation, and death, and compared the clinical profiles amongst the subgroups. RESULTS: Out of 162 DM patients, three clusters were defined. Cluster 1 (n = 40) was mainly grouped by patients with prominent muscular involvement and mild Interstitial Lung Disease (ILD). Cluster 2 (n = 72) grouped patients with skin rash, anti-Melanoma Differentiation Associated protein 5 positive (anti-MDA5+), and Rapid Progressive Interstitial Lung Disease (RP-ILD). Cluster 3 (n = 50) grouped patients with the mildest symptoms. The proportion of death increased across the three clusters (cluster 3 < cluster 1 < cluster 2). STUDY LIMITATIONS: The number of cases was limited for the subsequent construction and validation of predictive models. We did not review all skin symptoms or pathological changes in detail. CONCLUSIONS: We reclassified DM into three clusters with different risks for poor outcome based on diverse clinical profiles. Clinical serological testing and cluster analysis are necessary to help clinicians evaluate patients during follow-up and conduct phenotype-based personalized care in DM.

Clasificación y manejo actual en dermatomiositis juvenil / Classification and current management in juvenile dermatomyositis

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.

Dermatomiosite canina-símile em cão sem raça definida: relato de caso / Canine dermatomyositis-like disease in a mixed-breed dog: case report

A dermatomiosite canina é uma vasculopatia inflamatória da pele e músculos, com manifestações cutâneas envolvendo a face, orelhas, extremidades da cauda e distais por sobre proeminências ósseas. O envolvimento muscular manifesta-se sob a forma de mioatrofia cefálica, dificuldade de deglutição, redução do reflexo do vômito e marcha atípica. A dermatomiosite canina é classificada em dermatomiosite canina familiar e em forma variante ou dermatomiosite-símile. Neste relato descreve-se um caso de dermatomiosite-símile em cadela de dois anos de idade, sem raça definida, que apresentava necrose isquêmica dos pavilhões auriculares e atrofia dos músculos temporal e masseter. O exame dermo-histopatológico realizado em fragmentos de pele dos pavilhões auriculares, região cefálica e amostras de tecido muscular (temporal e masseter) confirmou a existência de dermatite perivascular e foliculite de interface pobre em células, focos de atrofia folicular e de fibras musculares e moderada fibrose dérmica. Portanto a histopatologia, associada às manifestações clínicas do animal, permitiu o estabelecimento do diagnóstico de dermatomiosite-símile, enfermidade pouco descrita na dermatologia veterinária brasileira.(AU)

Canine dermatomyositis is a skin and muscles inflammatory vasculopathy with cuta-neous manifestations involving face, ears, tail and distal ends over bony prominences. Mus-cle involvement manifests itself in the form of muscles of the head, difficulty in swallowing, reduction of reflex of vomit and atypical gait. Canine dermatomyositis is classified into variant form or dermatomyositis-like. This report describes a case of dermatomyositis-like in a two-year-old femalemixed-breed dog that presented ischemic necrosis of pinna and temporal and masseter muscle atrophy. In histopathological examinations performed with skin fragments of pinna, cephalic region and muscle samples (temporal and masseter), it was detected a perivascular dermatitis and folliculitis with poor cell interface, foci of follicular atrophy and muscle fibers with moderate dermal fibrosis confirming the diagnosis of derma-tomyositis-like, relatively little described in brazilian veterinary dermatology.(AU)

Dermatomiosite canina-símile em cão sem raça definida: relato de caso / Canine dermatomyositis-like disease in a mixed-breed dog: case report

A dermatomiosite canina é uma vasculopatia inflamatória da pele e músculos, com manifestações cutâneas envolvendo a face, orelhas, extremidades da cauda e distais por sobre proeminências ósseas. O envolvimento muscular manifesta-se sob a forma de mioatrofia cefálica, dificuldade de deglutição, redução do reflexo do vômito e marcha atípica. A dermatomiosite canina é classificada em dermatomiosite canina familiar e em forma variante ou dermatomiosite-símile. Neste relato descreve-se um caso de dermatomiosite-símile em cadela de dois anos de idade, sem raça definida, que apresentava necrose isquêmica dos pavilhões auriculares e atrofia dos músculos temporal e masseter. O exame dermo-histopatológico realizado em fragmentos de pele dos pavilhões auriculares, região cefálica e amostras de tecido muscular (temporal e masseter) confirmou a existência de dermatite perivascular e foliculite de interface pobre em células, focos de atrofia folicular e de fibras musculares e moderada fibrose dérmica. Portanto a histopatologia, associada às manifestações clínicas do animal, permitiu o estabelecimento do diagnóstico de dermatomiosite-símile, enfermidade pouco descrita na dermatologia veterinária brasileira.

Canine dermatomyositis is a skin and muscles inflammatory vasculopathy with cuta-neous manifestations involving face, ears, tail and distal ends over bony prominences. Mus-cle involvement manifests itself in the form of muscles of the head, difficulty in swallowing, reduction of reflex of vomit and atypical gait. Canine dermatomyositis is classified into variant form or dermatomyositis-like. This report describes a case of dermatomyositis-like in a two-year-old femalemixed-breed dog that presented ischemic necrosis of pinna and temporal and masseter muscle atrophy. In histopathological examinations performed with skin fragments of pinna, cephalic region and muscle samples (temporal and masseter), it was detected a perivascular dermatitis and folliculitis with poor cell interface, foci of follicular atrophy and muscle fibers with moderate dermal fibrosis confirming the diagnosis of derma-tomyositis-like, relatively little described in brazilian veterinary dermatology.

Dermatomyositis: analysis of 109 patients surveyed at the Hospital das Clínicas (HCFMUSP), São Paulo, Brazil.

BACKGROUND: Dermatomyositis affects striated muscles, skin and other organs. OBJECTIVE: To characterize the disease from January 1992 to December 2002, assessing its classification, cutaneous and systemic manifestations, and also laboratory results, therapeutic and prognostic findings compared to those in the literature. METHODS: Data were obtained from medical records of 109 patients who were classified into five groups: 23 juvenile dermatomyositis; 59 primary idiopathic dermatomyositis; 6 amyopathic dermatomyositis; 7 dermatomyositis associated with neoplasms and 14 dermatomyositis associated with other connective tissue diseases. RESULTS: Sixty patients were classified as "definite" diagnosis; 33 as "possible"; four as "probable" and 12 and as amyopathic. The average age at diagnosis was 36 years. Cutaneous manifestations occurred in all patients; the most frequent symptom was loss of proximal muscle strength; the most common pulmonary disorder was interstitial lung disease, and gastritis was the most prevalent digestive manifestation. Tumors were documented in 6.42% of cases. Lactate dehydrogenase was the muscle enzyme most frequently elevated in the majority of cases. Skin biopsies were performed in 68 patients; muscle biopsies in 53; and electroneuromyographies in 58 patients. The most commonly used treatment was corticotherapy and the mortality rate was 14.7%. CONCLUSION: in this sample, the disease appeared in younger individuals, was more frequent in women and the association with cancer was small.

Dermatomyositis: analysis of 109 patients surveyed at the Hospital das Clínicas (HCFMUSP), São Paulo, Brazil

BACKGROUND: Dermatomyositis affects striated muscles, skin and other organs. OBJECTIVE: To characterize the disease from January 1992 to December 2002, assessing its classification, cutaneous and systemic manifestations, and also laboratory results, therapeutic and prognostic findings compared to those in the literature. METHODS: Data were obtained from medical records of 109 patients who were classified into five groups: 23 juvenile dermatomyositis; 59 primary idiopathic dermatomyositis; 6 amyopathic dermatomyositis; 7 dermatomyositis associated with neoplasms and 14 dermatomyositis associated with other connective tissue diseases. RESULTS: Sixty patients were classified as "definite" diagnosis; 33 as "possible"; four as "probable" and 12 and as amyopathic. The average age at diagnosis was 36 years. Cutaneous manifestations occurred in all patients; the most frequent symptom was loss of proximal muscle strength; the most common pulmonary disorder was interstitial lung disease, and gastritis was the most prevalent digestive manifestation. Tumors were documented in 6.42% of cases. Lactate dehydrogenase was the muscle enzyme most frequently elevated in the majority of cases. Skin biopsies were performed in 68 patients; muscle biopsies in 53; and electroneuromyographies in 58 patients. The most commonly used treatment was corticotherapy and the mortality rate was 14.7%. CONCLUSION: in this sample, the disease appeared in younger individuals, was more frequent in women and the association with cancer was small. .

A Brazilian registry of juvenile dermatomyositis: onset features and classification of 189 cases.

OBJECTIVE: To describe onset features, classification and treatment of juvenile dermatomyositis (JDM) and juvenile polymyositis (JPM) from a multicentre registry. METHODS: Inclusion criteria were onset age lower than 18 years and a diagnosis of any idiopathic inflammatory myopathy (IIM) by attending physician. Bohan & Peter (1975) criteria categorisation was established by a scoring algorithm to define JDM and JPM based on clinical protocol data. RESULTS: Of the 189 cases included, 178 were classified as JDM, 9 as JPM (19.8: 1) and 2 did not fit the criteria; 6.9% had features of chronic arthritis and connective tissue disease overlap. Diagnosis classification agreement occurred in 66.1%. Median onset age was 7 years, median follow-up duration was 3.6 years. Malignancy was described in 2 (1.1%) cases. Muscle weakness occurred in 95.8%; heliotrope rash 83.5%; Gottron plaques 83.1%; 92% had at least one abnormal muscle enzyme result. Muscle biopsy performed in 74.6% was abnormal in 91.5% and electromyogram performed in 39.2% resulted abnormal in 93.2%. Logistic regression analysis was done in 66 cases with all parameters assessed and only aldolase resulted significant, as independent variable for definite JDM (OR=5.4, 95%CI 1.2-24.4, p=0.03). Regarding treatment, 97.9% received steroids; 72% had in addition at least one: methotrexate (75.7%), hydroxychloroquine (64.7%), cyclosporine A (20.6%), IV immunoglobulin (20.6%), azathioprine (10.3%) or cyclophosphamide (9.6%). In this series 24.3% developed calcinosis and mortality rate was 4.2%. CONCLUSION: Evaluation of predefined criteria set for a valid diagnosis indicated aldolase as the most important parameter associated with definite JDM category. In practice, prednisone-methotrexate combination was the most indicated treatment.

Dermatomiositis juvenil: presentación de 10 casos

Se presenta una serie de 10 pacientes con Dermatomiositis juvenil diagnosticados en el Hospital Universitario Ramón Gonzalez Valencia (HURGV) de Bucaramanga en el periódo de 13 años, estudio retrospectivo cuyo objeto es analizar las características clínicas, de laboratorio terapéutica y evolución. Los resultados mostraron predominio de mujeres (9 casos), procedentes de áreas rurales de Santander con una edad promedio de 8.8 años (rango de 6-12), con manifestaciones clínicas múltiples: lesión en piel, debilidad muscular, artralgia, fiebre, fotosensibilidad, disfagia, fenómeno de Raynaud y epistaxis. La velocidad de sedimentación globular (VSG), creatinkinasa (CK) estuvieron elevadas en todos los pacientes; la electromiografía mostró un patrón miopático. En la mayoría de pacientes hubo buenas respuestas a esteroides y evolución clínica satisfactoria. La elaboración detallada de la historia clínica con el examen físico minucioso y la elevación de enzimas musculares son de gran ayuda para el diagnóstico de esta enfermedad

Dermatomiositis y neoplasias: revisión de la literatura a proposito de un caso / Dermatomyositis and neoplasia: report of a case and review of the literature

La internación en nuestro servicio de un paciente con dermatomiositis y el hallazgo de una neoplasia concomitante, de localización poco frecuente, motivó la revisión de la literatura al respecto y la posterior comunicación de este caso

Dermatomiositis y neoplasias: revisión de la literatura a proposito de un caso / Dermatomyositis and neoplasia: report of a case and review of the literature

La internación en nuestro servicio de un paciente con dermatomiositis y el hallazgo de una neoplasia concomitante, de localización poco frecuente, motivó la revisión de la literatura al respecto y la posterior comunicación de este caso (AU)

Dermatomyositis of children and adults

Los autores refieren un caso de dermatomiositis del niño y uno del adulto, ambos de sexo femenino. Se establecen las características de la afección más notorias en estas dos etapas de la vida y se hace referencia a la clasificación de las miositis de Bohan y colaboradores

Dermatomyositis of children and adults

Los autores refieren un caso de dermatomiositis del niño y uno del adulto, ambos de sexo femenino. Se establecen las características de la afección más notorias en estas dos etapas de la vida y se hace referencia a la clasificación de las miositis de Bohan y colaboradores (AU)