Hipertensión arterial pulmonar en pacientes con dermatomiositis juvenil y esclerosis sistémica en un centro pediátrico de tercer nivel

INTRODUCCIÓN: La hipertensión arterial pulmonar puede estar asociada secundariamente a enfermedades del tejido conectivo. Entre estas enfermedades, predominan la esclerosis sistémica y la dermatomiositis juvenil. MATERIALES Y MÉTODOS: Se realizó un estudio retrospectivo, descriptivo y transversal. Se incluyeron todos los pacientes con diagnóstico de dermatomiositis juvenil y esclerosis sistémica que acudieron a nuestro hospital. Posteriormente se verificaron los niveles de presión arterial pulmonar mediante ecocardiografía. RESULTADOS: Se incluyeron 58 pacientes, de los cuales sólo 17 pacientes tuvieron ecocardiografía diagnóstica. Entre ellos, dos pacientes presentaron hipertensión arterial pulmonar. CONCLUSIÓN: La detección oportuna de la hipertensión arterial pulmonar en las enfermedades del tejido conectivo es esencial. Generalmente es asintomático. Es necesario adherirse al protocolo internacional que sugiere realizar ecocardiografía en todos los pacientes con dermatomiositis juvenil y esclerosis sistémica.

INTRODUCTION: Pulmonary arterial hypertension may be secondary associated with connective tissue diseases. Among these diseases, systemic sclerosis and juvenile dermatomyositis predominate. MATERIALS AND METHODS: A retrospective, descriptive and cross-sectional study was carried out. All patients with a diagnosis of juvenile dermatomyositis and systemic sclerosis who attended our hospital were included. Pulmonary arterial pressure levels were subsequently verified by echocardiography. RESULTS: 58 patients were included, of which only 17 patients had a diagnostic echocardiography. Among them, two patients presented pulmonary arterial hypertension. CONCLUSION: Timely detection of pulmonary arterial hypertension in connective tissue diseases is essential. It is generally asymptomatic. It is necessary to adhere to the international protocol that suggests performing echocardiography in all patients with juvenile dermatomyositis and systemic sclerosis.

Research progress of juvenile dermatomyositis with anti-melanoma differentiation-associated gene 5 antibody / 中华实用儿科临床杂志

Juvenile dermatomyositis (JDM) is the most common subtype of juvenile idiopathic inflammatory myopathy (JIIM), characterized by non-suppurative inflammation of skin and muscle.JDM frequently involves important organs such as lungs.JDM with anti-melanoma differentiation-associated gene (MDA) 5 antibody has unique clinical characteristics, mainly including skin mucosal ulcer, palm papule, hair loss and arthritis.Interstitial lung disease (ILD) is its most serious complication.The levels of serum ferritin, Krebs von den Lungen-6 and interleukin-18 can be used as important indicators of disease activity and prognosis.Glucocorticoids combined with immunosuppressants are the basic treatment for the disease.Immunosuppressants include calcineurin inhibitors (Cyclosporine A and Tacrolimus), Cyclophosphamide, Azathioprine, Mycophenolate Mofetil, etc.Refractory patients can also be treated with Rituximab, Janus kinase inhibitor and human immunoglobulin.Early active treatment of JDM with anti-MDA 5 antibody can alleviate the symptoms, reverse organ damage and improve the long-term prognosis.

Juvenile dermatomyositis in Thai children: Retrospective review of 30 cases from a tertiary care center

Background: Juvenile dermatomyositis is a rare condition, but it is the most common idiopathic inflammatory myopathy in pediatric patients. Aim: To study the clinical manifestations, investigations, treatment, clinical course, and outcomes of juvenile dermatomyositis in Thai children. Method: This retrospective study included juvenile dermatomyositis patients treated at Siriraj Hospital, a 2,300-bed national tertiary referral center in Bangkok, Thailand, from 1994 to 2019. Results: Thirty patients (22 females and 8 males) were included with a female to male ratio of 2.7:1. Median age at diagnosis was 5.1 years (range, 2.6-14.8 years). Median duration of illness before diagnosis was 6.5 months (range, 0.3-84.0 months). Acute and subacute onset occurred in the majority of patients. Presenting symptoms included muscle weakness in 27/30 (90%), skin rash in 26/30 (86.7%), muscle pain in 17/26 (65.4%), and arthralgia in 4/18 (22.2%) of patients. Dermatologic examination revealed Gottron’s rash, heliotrope rash, and periungual telangiectasia in 25/30 (83.3%), 21/30 (70.0%), and 15/24 (62.5%) of patients, respectively. Interestingly, scalp dermatitis was found in 8/21 (38.1%) of patients. The most commonly used treatment regimen in this series was a combination of prednisolone and methotrexate. During the median follow-up of 3.1 years (range, 0.0-18.5 years), only one-third of patients were seen to have monocyclic disease. Extraskeletal osteosarcoma at a previous lesion of calcinosis cutis was observed in one patient at 12 years after juvenile dermatomyositis onset. Limitations: This was a retrospective single-center study, and our results may not be generalizable to other healthcare settings. Prospective multicenter studies are needed to confirm the findings of this study. Conclusion: juvenile dermatomyositis usually poses a diagnostic and therapeutic challenge, which can be compounded by the ethnic variations in the clinical presentation, as observed in this study. Asian patients tend to present with acute or subacute onset of disease, and arthralgia and/or arthritis are less common than in Caucasian patients. Scalp dermatitis is not uncommon in pediatric juvenile dermatomyositis patients. An association between juvenile dermatomyositis and malignancy, though rare, can occur

Clinical characteristics of 11 cases of anti-melanoma differentiation associated gene 5 autoantibody-positive juvenile dermatomyositis / 中华实用儿科临床杂志

Objective:To clarity the clinical features of juvenile dermatomyositis (JDM) with positive anti-melanoma differentiation associated gene 5 (MDA5) antibody.Methods:Retrospective study.Clinical data of 11 anti-MDA5 autoantibody-positive JDM patients in the Department of Rheumatology and Immunology, Children′s Hospital Affiliated to Capital Institute of Pediatrics from January 2016 to January 2019 were retrospectively recruited for analyzing their clinical characteristics, pulmonary imaging and pulmonary function, thus summarizing treatment experiences.Results:A total of 11 children with anti-MDA5 autoantibody-positive JDM were recruited, involving 2 males and 9 females, with the average onset age of 1-13 (5.8±4.2) years.Clinical manifestations included rash in 11 cases (100.0%), arthritis in 5 cases (45.5%), and myasthenia in 4 cases (36.4%). Muscle enzyme elevated in 10 cases (90.9%) and serum ferritin (SF) elevated in 9 patients (81.8%). Ten cases (90.9%) showed interstitial lung disease (ILD), manifesting as ground glass opacity at subpleural area on CT scans, restrictive ventilation and decreased diffusion function on lung function test, while respiratory symptoms were absent.All patients were treated with glucocorticoid combined with immunosuppressor.Case 2 developed into rapid progressive pulmonary interstitial disease (RPILD), and died of respiratory failure 2 months later.The remaining was followed up for 1-2 years, and the ILD was relieved.Conclusions:All recruited children with anti-MDA5 autoantibody-positive JDM presented typical rash, and mild muscle weakness with a greater tendency to arthritis.Chinese pediatric patients are prone to complicate with ILD with no respiratory symptoms, but ground glass opacity at subpleural area on CT, and restrictive ventilation and decreased diffusion function on lung function test can be detected.Elevated SF is associated with the development of ILD.Glucocorticoid combined with immunosuppressive therapy is effective to JDM with ILD, but ineffective for RPILD.The mortality of anti-MDA5 autoantibody-positive JDM is high without an effective treatment.

Mycoplasma pneumoniae - The Culprit for Juvenile Dermatomyositis in A 9-Year-Old Girl: A Case Report

@#Mycoplasma pneumoniae is frequently known as an “atypical bacterium” that can cause wide-ranging extrapulmonary manifestations. Here, we outline a case of a child, aged 9-year-old, who presented with profound proximal muscle weaknesses and a vague rash, associated with markedly elevated serum creatinine kinase (CK). Muscle biopsy suggested Juvenile Dermatomyositis (JDM) following an upper respiratory illness of M. pneumoniae origin. The child responded exceptionally well to a combined therapy of immunoglobulin, intravenous glucocorticoid and methotrexate.

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.

Lipid profile of pediatric patients with chronic rheumatic diseases - a retrospective analysis

SUMMARY AIM To describe the prevalence of dyslipidemia in children and adolescents with autoimmune rheumatic diseases (ARDs), particularly juvenile idiopathic arthritis (JIA), juvenile systemic lupus erythematosus (jSLE), and juvenile dermatomyositis (JDM). METHODS Retrospective cross-sectional study conducted in the pediatric rheumatology outpatient clinic. We evaluated 186 children and adolescents between the ages of 5 and 19 years. The medical records were reviewed for the following data: demographic and clinical features, disease activity, and lipid profile (triglycerides (TG), total cholesterol (TC), low density lipoprotein (LDL-C), high density lipoprotein (HDL-C) and very low density lipoprotein (VLDL-C)). In addition, non-HDL cholesterol was calculated as TC minus HDL-C. The cut-off points proposed by the American Academy of Pediatrics were used to classify the lipid profile. RESULTS Dyslipidemia was observed in 128 patients (68.8%), the most common being decreased HDL-C (74 patients, 39.8%). In the JIA group there was an association between the systemic subtype and altered LDL-C and NHDL-C, which demonstrated a more atherogenic profile in this subtype (p=0.027 and p=0.017, respectively). Among patients with jSLE, the cumulative corticosteroid dose was associated with an increase in LDL-C (p=0.013) and with a decrease in HDL-C (p=0.022). CONCLUSION Dyslipidemia is common in children and adolescents with ARDs, especially JIA, jSLE, and JDM, and the main alteration in the lipid profile of these patients was decreased HDL-C.

RESUMO OBJETIVO Descrever a prevalência de dislipidemias em crianças e adolescentes com doenças reumáticas autoimunes (Drai), em particular artrite idiopática juvenil (AIJ), lúpus eritematoso sistêmico juvenil (Lesj) e dermatomiosite juvenil (DMJ). MÉTODOS Estudo transversal retrospectivo realizado no ambulatório de reumatologia pediátrica. Foram avaliados 186 crianças e adolescentes com idades entre 5 e 19 anos. Foram coletados dos prontuários dados demográficos, clínicos, atividade de doença e perfil lipídico (triglicérides (TG), colesterol total (CT) e frações LDL-c (low density lipoprotein); HDL-c (high density lipoprotein) e VLDL-c (very low density lipoprotein). Foi também calculada a fração não HDL do colesterol (CT-NHDL -c). Para classificação do perfil lipídico, foram adotados os pontos de corte propostos pela American Academy of Pediatrics. RESULTADOS A dislipidemia foi observada em 128 pacientes (68,8%), sendo a mais comum a diminuição do HDL-c em 74 (39,8%). No grupo AIJ houve uma associação entre o subtipo sistêmico com alteração de LDL-c e NHDL-c, mostrando um perfil mais aterogênico neste subtipo (p=0,027 e 0,017, respectivamente). Em relação aos pacientes com Lesj, podemos observar que a dose cumulativa de CTC teve associação com o aumento do LDL-c (p=0,013) e com a diminuição do HDL-c (p=0,022). CONCLUSÃO A dislipidemia é frequente em crianças e adolescentes com Drai, em especial, AIJ, Lesj e DMJ, e a principal alteração no perfil lipídico desses pacientes foi a diminuição do HDL-c.

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.

Dermatomiositis juvenil. Sistematización de casos / Juvenile dermatomyositis. Case systematization

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)

Juvenile idiopathic inflammatory myopathies: A clinicopathological study with emphasis on muscle histology

Background: Juvenile idiopathic inflammatory myopathies (JIIM) are rare and heterogeneous. Subtype identification is important for treatment. Materials and Methods: Patients below 18 years diagnosed as idiopathic inflammatory myopathy (IIM) according to the Bohan and Peter criteria between January 2010 and May 2015 were evaluated with muscle biopsy in the four domains: muscle fiber, inflammation, connective tissue, and vascular, with basic panel of histochemical stains as per recommendations of the European Neuromuscular center (ENMC) workshop 2015. Immunohistochemistry with CD 31 was done to assess capillary density. Results: JIIM constituted 15.25% of IIM with juvenile dermatomyositis (JDM) being the most common subgroup (24/27) followed by juvenile overlap myositis (JOM) (3/27) in association with systemic lupus erythematosus (2) and systemic sclerosis (1). Muscle biopsy in JDM was characterized by perifascicular atrophy, necrosis, degeneration, and regeneration in all and the other features included perivascular inflammation (21), lymphoid aggregates (2), mitochondrial abnormalities (9), sarcoplasmic vacuoles (6), capillary dropout (5), capillary dilatation (6), and perimysial fibrosis (14). JOM was characterized by auto-antibodies and perivascular inflammation. Conclusion: JIIMs were rare and JDM was the most common subtype. Muscle biopsy evaluation as per ENMC criteria characterized the subgroups.

Rash and weakness - A case of juvenile dermatomyositis

Juvenile dermatomyositis (JDM) is a well-known but uncommon paediatric rheumatological condition. It can be missed early in the disease manifestation if not thought of in patients presenting with atypical rash and weakness. We present a case of a 5-year-old girl who developed JDM over a span of 8 weeks before a diagnosis was made. This case demonstrates the nuances of how a paediatric patient’s presentation can differ from adults’. A high index of suspicion and early appropriate referral to paediatric rheumatology are paramount for successful management and good prognosis of the condition.

Diagnostic and therapeutic advances on severe juvenile dermatomyositis / 中华实用儿科临床杂志

In addition to skin and muscle lesions,severe juvenile dermatomyositis can also cause multiple systemic and organ damage,which directly threatens the life of children.The development of imaging and specific antibody detection techniques is beneficial to the early diagnosis of severe juvenile dermatomyositis.Early diagnosis and regular combination of continuous medication can improve its prognosis.At present,the treatment of severe juvenile dermatomyositis is mainly based on large doses of glucocorticoid.Intravenous immunoglobulin is a rapid and effective treatment and can be used as a first-line drug.It can be repeated in half a year.For children with combined organ damage,selective use of cyclosporine,mycophenolate mofetil,tacrolimus and other immunosuppressive agents,even biological agents,plasma exchange,stem cell transplantation,etc.,but the efficacy needs to be further verified.

Manifestaciones mucocutáneas del paciente pediátrico con enfermedades autoinmunes del Hospital Roberto del Río / Mucocutaneous manifestations of the pediatric patient with autoimmune diseases of the Roberto del Río Hospital

Las enfermedades autoinmunes son desórdenes heterogéneos que pueden comprometer distintos órganos. Su frecuencia es baja, se estima que 3 de cada 1.000 niños cursan con alguna condición reumatológica. Las patologías reumatológicas más comunes en la edad pediátrica son la artritis idiopática juvenil (AIJ) seguida por el lupus eritematoso sistémico (LES), dermatomiositis juvenil (DMJ), vasculitis primarias y la esclerodermia. Materiales y Métodos: Se efectuó un estudio descriptivo retrospectivo de pacientes pediátricos atendidos en el servicio de inmunología del Hospital Roberto del Rio entre los años 1990 y 2011. Se pesquisaron un total de 102 pacientes, con diagnósticos de AIJ, LES y DMJ. Se diseñó una ficha de protocolo, con los datos: edad, sexo, antecedentes familiares, manifestaciones cutáneas al diagnóstico y a lo largo de la evolución. Para el análisis estadístico de variables, se utilizó el programa STATA 8.0. Resultados: El 45,45% de los pacientes con AIJ presentó lesiones cutáneas, sin embargo, sólo un 20% de ellas, relacionadas a esta enfermedad. El 91,7% de los pacientes con LES presentó manifestaciones cutáneas, siendo la vasculitis cutánea y el eritema malar, las más frecuentes. En los pacientes con DMJ, el eritema heliotropo y pápulas de Gottron fueron las manifestaciones cutáneas más comunes. Conclusión: Los hallazgos cutáneos cobran un rol muy importante en el diagnostico enfermedades autoinmunes. Estos datos demuestran la importancia de un examen dermatólogico exhaustivo para su diagnóstico precoz y evitar sus complicaciones.

Background: Autoimmune diseases are disorders that can compromise different organs. Its frequency is low, it is estimated that 3 out of every 1,000 children are affected with any rheumatologic condition. The most common rheumatic diseases in children are juvenile idiopathic arthritis (JIA) followed by systemic lupus erythematosus (SLE), juvenile dermatomyositis (DMJ), primary vasculitis and esclerodermia.2 Materials and Methods: A retrospective study was conducted in pediatric patients seen in the Department of Immunology from the Roberto del Rio Hospital between 1990 and 2011. Records of 105 patients with the diagnosis of juvenile idiopathic arthritis (JIA), systemic lupus erythematosus (SLE) and juvenile dermatomyositis (JDM) have been included. We designed a protocol file with the given data: Age, sex, family history, skin manifestations at the diagnosis and throughout the evolution. The program STATA 8.0 was used for statistical analysis of variable. Results: 45.45% of JIA patients had some type of skin lesions, however, only 20% of them related to this disease. 91.7% of SLE patients presented cutaneous manifestations, the most common being cutaneous vasculitis and malar erythema. In patients with JDM, heliotrope erythema and papules Gottron were the most common skin manifestations. Discussion: Cutaneous manifestations have a very important role in autoimmune diseases. While the diagnosis and management of these diseases require a multidisciplinary team, these data demonstrate the importance of an exhaustive physical examination for early diagnosis and thereby reduce complications. Conclusions: This study highlights the importance of the dermatologist in an early diagnosis of rheumatic diseases.

An aporia:calcinosis in juvenile dermatomyositis / 中华实用儿科临床杂志

As the hallmark sequelae of juvenile dermatomyositis(JDM),the calcinosis still bothers approxi-mately one third of the patients,but it was considered a good prognostic sign. Today,however,it is universally acknow-ledged that the calcinosis is instead deemed a marker of the special state and inappropriate treatment for JDM. The treatment of based on disease process,including the biological agents and other immunomodulator,which have been not the radical therapy. However,it is a crucial to alleviate the calcinosis that controlling the long - term chronic inflamma-tion for JDM.

Doença periodontal em doenças reumáticas pediátricas / Periodontal disease in pediatric rheumatic diseases

Gengivite e periodontite são doenças periodontais imunoinflamatórias caracterizadas por infecções localizadas crônicas geralmente associadas a uma inflamação insidiosa. Essa revisão narrativa discute doenças periodontais e mecanismos que influenciam a resposta imune e a autoimunidade na área das doenças reumáticas pediátricas (DRP), particularmente a artrite idiopática juvenil (AIJ), lúpus eritematoso sistêmico juvenil (LESJ) e dermatomiosite juvenil (DMJ). Foi notada maior frequência de gengivite nessas doenças em comparação com controles sadios, enquanto casos de periodontite foram achados raros. Em pacientes com AIJ, a gengivite e a periodontite estavam relacionadas a fatores mecânicos, artrite crônica com incapacitação funcional, desregulação da resposta imunoinflamatória, dieta e medicamentos, principalmente corticosteroides e ciclosporina. Em pacientes com LESJ, a gengivite estava associada a períodos mais longos da doença, doses elevadas de corticosteroides, hiperativação dos linfócitos B e elevação da imunoglobulina G. São escassos os dados sobre doenças periodontais na população com DMJ; nos pacientes ativos, foi observado um padrão gengival singular, caracterizado por eritema gengival, dilatação dos capilares e formação arbustiforme. Em conclusão, gengivite foi a doença periodontal mais comum em pacientes com DRP. A associação observada com a atividade da doença reforça a necessidade de futuros estudos, com o intuito de determinar se a resolução dessa complicação irá influenciar o curso ou a gravidade da doença.

Gingivitis and periodontitis are immunoinflammatory periodontal diseases characterized by chronic localized infections usually associated with insidious inflammation. This narrative review discusses periodontal diseases and mechanisms influencing the immune response and autoimmunity in pediatric rheumatic diseases (PRD), particularly juvenile idiopathic arthritis (JIA), childhood-onset systemic lupus erythematosus (C-SLE) and juvenile dermatomyositis (JDM). Gingivitis was more frequently observed in these diseases compared to health controls, whereas periodontitis was a rare finding. In JIA patients, gingivitis and periodontitis were related to mechanical factors, chronic arthritis with functional disability, dysregulation of the immunoinflammatory response, diet and drugs, mainly corticosteroids and cyclosporine. In C-SLE, gingivitis was associated with longer disease period, high doses of corticosteroids, B-cell hyperactivation and immunoglobulin G elevation. There are scarce data on periodontal diseases in JDM population, and a unique gingival pattern, characterized by gingival erythema, capillary dilation and bush-loop formation, was observed in active patients. In conclusion, gingivitis was the most common periodontal disease in PRD. The observed association with disease activity reinforces the need for future studies to determine if resolution of this complication will influence disease course or severity.

Juvenile Dermatomyositis Presenting as Deep Skin Ulcerations as an Initial Manifestation / 대한피부과학회지

Juvenile dermatomyositis is a common idiopathic inflammatory myopathy in childhood and is characterized by symmetric proximal muscle weakness and specific cutaneous manifestations. We here report on a 12-year-old boy who presented with violaceous maculopatches with multiple deep skin ulcerations on the right arm and lower back 4 months ago. One month after the initial visit, he had difficulty climbing stairs and elevating his arms above the head. Laboratory tests showed elevation of the serum levels of skeletal muscle enzymes, and electromyogram and muscle biopsy indicated myopathy. Histopathological analysis of the ulceration indicated necrotizing vasculitis and calcification. Based on these findings, his condition was diagnosed as juvenile dermatomyositis. We believe that this case is unique because of the difficulty in diagnosing the condition, as skin ulceration is a rare cutaneous manifestation of dermatomyositis.

Disease Patterns of Juvenile Dermatomyositis From Western India.

A retrospective assessment of clinical characteristics, complications/ associations, laboratory investigations, treatment modalities and outcome in an inceptional cohort of 22 (male-13) children with juvenile dermatomyositis (JDM) receiving treatment at Jaslok Hospital, Mumbai during 1997- 2012 was performed . Mean age at diagnosis was 7.52 ± 3.99 years. Typical skin rash and muscle weakness were present in all children. Common complications included cutaneous ulcers (27.27%), dysphagia (22.72%) and calcinosis (18.18%).All patients presented with at least one of the serum muscle enzymes elevated. Absence of mortality and cardio-pulmonary complications and a monocyclic course in 72.7% of our patients are at variance from Western series.

Juvenile Dermatomyositis with Calcinosis Cutis.

A 6-year-old girl presented with irregular fever for last two months and difficulty in standing from sitting position for last 1½ months. She had pathognomonic heliotrope rashes on both eyelids, Gottron’s papules in proximal interphalangeal and metacarpophalangeal joints of both hands and papules on elbow, knee and ankle joints. She had elevated serum muscle enzyme levels and electromyogram was in favor of juvenile dermatomyositis (JDM). She is now under steroid treatment and showing signs of improvement.

Juvenile dermatomyositis complicated by myelodysplastic syndrome:a case report / 临床儿科杂志

Objectives A rare case of juvenile dermatomyositis (JDM) complicated by myelodysplastic syndrome (MDS) was reported in order to improve the understanding of the disease in its clinical signs, differential diagnosis and treatment. Meth-ods The course of the disease, clinical characteristics and the process of diagnosis and treatment were analyzed. Specimens of bone marrow, lymph nodes, muscles were examined by histopathological or immunohistopathological means and lfow cytometry. Results The diagnosis of JDM complicated by MDS was made based on the clinical manifestations, related laboratory results and the accompanying changes in hematological and bone marrow examinations. Conclusions JDM complicated by MDS is very special and rarely seen in China and its pathological mechanism and strategy of diagnosis and treatment should be further explored.

Therapeutic advances on juvenile dermatomyositis / 中国小儿急救医学

The current management for juvenile dermatomyositis includes the initial use of corticosteroids followed by various conventional second-line treatments such as methotrexate and azathioprine.Intravenous immunoglobulin is a reasonable short-term treatment with proven benefit.Cyclosporine or tacrolimus have shown efficacy in juvenile dermatomyositis including those patients with interstitial lung disease,whereas mycophenolate mofetil is effective in both polymyositis and refractory dermatomyositis.The curative effect of biological agents needs to be further studied.