Calcinosis in Juvenile Dermatomyositis-Epidemiology, Pathogenesis, Clinical Features, and Treatment: A Systematic Review.

PURPOSE OF REVIEW: We performed a systematic review of the literature on the epidemiology, pathogenesis, clinical and laboratory characterization, and treatment of calcinosis in patients with juvenile dermatomyositis (JDM). A qualitative systematic review was conducted from January 1975 to April 2023 according to the PRISMA protocol using three electronic databases: PubMed, Web of Science, and Scopus. Studies were analyzed based on the following eligibility criteria: at least one combination of the terms described in the search strategy appeared in the title, written in English, Portuguese, or Spanish, and addressed the epidemiology, pathogenesis, diagnosis, and treatment of calcinosis in juvenile dermatomyositis. Systematic or scoping reviews, letters, clinical images, book chapters, abstracts, inflammatory myopathy in other connective tissue diseases, idiopathic inflammatory myopathies in adults, and purely qualitative studies were excluded. RECENT FINDINGS: Seventy-five studies were included. According to the literature, calcinosis is common in women, around five years old, with three years of disease in association with osteoarticular, cutaneous, pulmonary manifestations, and fever. The pathogenesis is still unknown, but the participation of interleukin 1 and 6, tumor necrosis factor alpha, and innate immunity dysregulation seem to be involved. Common autoantibodies are anti-NXP-2, anti-MDA-5, and anti-Mi-2, and their treatment remains controversial. Prospective, randomized, controlled studies are needed to evaluate treatment protocols and map the natural history of this serious complication. Calcinosis seems to be more common in White female children with muscle weakness, fever, arthritis, severe pulmonary, and skin involvement with anti-NXP-2, anti-MDA-5, and anti-Mi-2 autoantibodies. The multitargets and aggressive treatment is recommended.

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.

Juvenile Dermatomyositis in Afro-Caribbean children: a cohort study in the French West Indies.

INTRODUCTION: The epidemiology of Juvenile Dermatomyositis (JDM) in non-Caucasian population is poorly described. We performed a study of patients followed up in the French West Indies for JDM. We aimed to describe clinical and biological specificities during childhood. METHODS: Retrospective study covering the period from Januarys 2000-2023. Listings of patients were obtained from multiple sources, namely computerized hospital archives, registry of referent pediatricians and adult specialists in internal medicine and the French National Registry for rare diseases. JDM and organ involvement were defined according to the international ILAR criteria. RESULTS: Twenty-one patients were included over a 23 year-period. Median age at onset was 8.1 years (Range: 2.5-13.9) with a median follow up of 8 years (Range: 2-19). Two-thirds (14/21) had dysphagia at onset and 33% had respiratory involvement. Thirteen had specific autoantibodies (58%), most frequently anti-Mi-2. The median number of flares during childhood was three (1-9). During childhood, 76% had calcinosis lesions. Clinical evolution seemed to be more aggressive for boys than girls (respectively 4.2 versus 2.2 flares (p = 0.04) and 50% vs 18% needing more than one background therapy, p = 0.03). CONCLUSION: This retrospective study is the largest cohort of pediatric patients of Afro-Caribbean and Black African descent treated for JDM in a high-income health system, and the first to describe the incidence and immunological profile in a population of African descent. They had higher rate of calcinosis and similar respiratory involvement. Overall outcomes during childhood were similar to North America and European countries.

Subcutaneous calcinosis: Is it different between systemic sclerosis and dermatomyositis?

Calcinosis cutis is the deposition of insoluble calcium in the skin and subcutaneous tissues. It is a manifestation of several autoimmune connective tissue diseases, most frequently with systemic sclerosis and juvenile dermatomyositis, followed by adult dermatomyositis. Autoimmune connective tissue disease-associated calcinosis is of the dystrophic subtype, which occurs at sites of damaged tissue in the setting of normal serum calcium and phosphate levels. In juvenile dermatomyositis, calcinosis is considered a marker of ongoing disease activity and possibly inadequate treatment, while in adult dermatomyositis, it is a hallmark of skin damage due to chronic rather than active disease. Calcinosis is associated with long disease duration in systemic sclerosis and dermatomyositis, anti-polymyositis/sclerosis autoantibodies in systemic sclerosis and NXP-2 and melanoma differentiation-associated gene 5 in dermatomyositis. Calcinosis in systemic sclerosis occurs most frequently in the hands, particularly the fingers, whereas in dermatomyositis, it affects mainly the trunk and extremities. The primary mineral component of calcinosis is hydroxyapatite in systemic sclerosis and carbonate apatite in dermatomyositis. Calcinosis in dermatomyositis and systemic sclerosis share some pathogenic mechanisms, but vascular hypoxia seems to play a more important role in systemic sclerosis, whereas the release of calcium from mitochondria in muscle cells damaged by myopathy may be a primary mechanism contributing to dermatomyositis-related calcinosis. Multiple treatment strategies for dermatomyositis and systemic sclerosis-related calcinosis have been used with variable results. Early aggressive treatment of underlying myositis in patients with dermatomyositis may improve long-term outcomes of calcinosis. A better understanding of the pathogenesis of calcinosis is needed to improve treatment options.

Early-onset juvenile dermatomyositis: A report of two cases and review of the literature.

Juvenile dermatomyositis (JDM) is an uncommon disease in children younger than 3 years of age. The clinical manifestations may be different than in older children, often delaying the diagnosis. We present two patients with early-onset JDM and review the literature describing the unique clinical characteristics in this age group.

Echocardiographic study of juvenile dermatomyositis patients: new insights from speckle-tracking-derived strain.

OBJECTIVES: This study aimed to investigate subclinical left ventricle (LV) systolic dysfunction in juvenile dermatomyositis (JDM) using two-dimensional speckle-tracking echocardiography (2DST). Possible associations between LV deformation impairment and disease activity/cumulative damage were also evaluated. METHODS: Thirty-five consecutive JDM patients without cardiac symptoms and 35 healthy volunteers were enrolled. Clinical data were collected from medical records, and echocardiograms were performed by a pediatric cardiologist, unaware of patients' conditions. RESULTS: Patients and controls had similar age (12.6 ± 0.7 vs.12.5 ± 0.6; p = 0.97) and gender (11F:24M vs.11F:24M; p = 1.0). Median of JDM duration was 4.6 (0.04-17.6) years, and only 6/35 (17%) had active disease (disease activity score (DAS > 3)). Conventional echocardiogram revealed preserved LV ejection fraction (EF) (≥ 55%) in all individuals. In JDM, 2DST identified reduction of LV longitudinal [-22(-17.2 to -27.9) % vs. -23(-20.8 to -27.4) %; p = 0.028)] and circumferential -23.9 ± 2.8% vs. -26.7 ± 2.9%; p = 0.0002) strain. Lower longitudinal strain was associated with DAS >3 -19.9(-17.2 to -26.5)% vs. -22.1-18.9 to -27.9)%; p = 0.046], MDI extent > 0 [-19(-17.2 to -22.5)% vs. -22.1-19.2 to -27.9)%; p = 0.0008], MDI severity > 0 [-19(-17.2 to -22.1)% vs. -22.3(-20.3 to -27.9)%; p = 0.0001] and calcinosis[-20.6(-17.2 to -23)% vs. -22.3(-20.3 to -27.9)%; p = 0.03]. Lower circumferential strain was associated with MDI extent > 0 (-22.1 ± 3.87% vs. -24.4 ± 2.3%; p = 0.039), MDI severity > 0 (-21.7 ± 3% vs. 24.7 ± 2.3%; p = 0.004) and calcinosis (-22.5 ± 3.3% vs. -24.8 ± 2.1%; p = 0.02). There was a negative correlation between longitudinal strain and cumulative dose of prednisone (r = -0.44; p = 0.009) and methotrexate (r = -0.33; p = 0.0008). CONCLUSIONS: LV 2DST detected early systolic myocardial compromise in asymptomatic pediatric JDM patients, with preserved EF. Longitudinal strain impairment was associated with disease activity and cumulative damage, whereas circumferential strain impairment was associated exclusively with cumulative damage. KEY POINTS: • Serious cardiac involvement is rare but has been associated with death in juvenile dermatomyositis. • Two-dimensional speckle tracking stands out for the identification of subclinical myocardial compromise in juvenile dermatomyositis. • Longitudinal strain impairment is associated with disease activity and cumulative damage, whereas circumferential strain impairment is associated exclusively with cumulative damage.

Safety and immunogenicity of the quadrivalent human papillomavirus vaccine in patients with juvenile dermatomyositis: a real-world multicentre study.

BACKGROUND: Concerns about the safety and efficacy of vaccines in patients with autoimmune diseases (AID) have led to contradictions and low vaccination coverage in this population, who are at a higher risk of infections, including by human papillomavirus (HPV). Although HPV vaccines have been recommended for immunocompromised patients, there is still a lack of data to support its use for AID patients, such as juvenile dermatomyositis (JDM) patients. The aim of this study was to assess the safety and immunogenicity of the quadrivalent HPV (qHPV) vaccine in a cohort of JDM patients. METHODS: JDM patients aged from 9 to 20 years and healthy controls (HC) were enrolled to receive a 3-dose schedule of qHPV vaccine from March/2014 to March/2016. Study visits were performed before the first dose, 1 month after the second and third doses, and 6 months after the third dose. Participants completed a diary of possible adverse events for 14 days following each dose of vaccination (AEFV). Disease activity and current therapy were analyzed at each visit for JDM patients. In addition, serum samples from all participants were collected to test antibody concentrations against HPV16 and 18 at each visit. Participant recruitment was conducted in ten Brazilian centres. From 47 eligible JDM patients and 41 HC, 42 and 35, respectively, completed the 3-dose schedule of the vaccine, given that five JDM patients and two HC had received doses prior to their inclusion in the study. RESULTS: The AEFVs presented by the participants were mild and in general did not differ between JDM and HC groups. No severe AEFVs were related to the vaccination. Disease activity was stable, or even improved during the follow-up. One month after the third dose of the vaccine the JDM group presented seropositivity of 100% for HPV16 and 97% for HPV18, similarly to the HC group, who presented 100% for both serotypes (p = 1.000). Six months after the third dose the seropositivity for the patient group was 94% for both HPV types. CONCLUSIONS: The HPV vaccination in this cohort of JDM patients was safe and immunogenic. Since the seropositivity against HPV16 and 18 was very high after the 3-dose schedule, this regimen should be recommended for JDM patients. TRIAL REGISTRATION: Brazilian Clinical Trials Registry, number: RBR-9ypbtf . Registered 20 March 2018 - Retrospectively registered.

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)

Increased Soluble Cytoplasmic Bcl-2 Protein Serum Levels and Expression and Decreased Fas Expression in Lymphocytes and Monocytes in Juvenile Dermatomyositis.

OBJECTIVE: To evaluate soluble Fas antigen (sFas), sFas ligand (sFasL), soluble tumor necrosis factor-related apoptosis-inducing ligand, and soluble cytoplasmic Bcl-2 protein (sBcl-2) serum levels, Fas and Bcl-2 expressions in T and B lymphocytes and monocytes and relations with erythrocyte sedimentation rate, C-reactive protein (CRP), Childhood Myositis Assessment Scale, and manual muscle testing in juvenile dermatomyositis (JDM). METHODS: Serum levels were determined by ELISA and peripheral cell expressions by flow cytometry for patients with JDM or juvenile idiopathic arthritis (JIA), and healthy controls. RESULTS: Patients with JDM had increased sBcl-2, which correlated with CRP. Expression of Bcl-2 was increased and expression of Fas was decreased in CD3+, CD4+, and CD8+ T lymphocytes compared with JIA and/or healthy controls. CONCLUSION: Patients with JDM presented a unique apoptosis-related proteins profile, which may contribute to disease development.

Comparison between treatment naive juvenile and adult dermatomyositis muscle biopsies: difference of inflammatory cells phenotyping.

BACKGROUND: Different inflammatory cells (i.e., CD4, CD8, CD20 and CD68) are involved in pathogenesis of DM muscle. In this context, the aim of this study was to assess and compare these inflammatory cell phenotyping in muscle samples of treatment naive juvenile and adult patients with dermatomyositis. METHODS: This is a cross-sectional study, in which 28 untreated juvenile and 28 adult untreated dermatomyositis patients were included. Immunohistochemical analysis was performed on serial frozen muscle sections. Inflammatory cell phenotyping was analyzed quantitatively in endomysium, perimysium, and perivascular (endomysium and perimysium) area. RESULTS: Mean age at disease onset was 7.3 and 42.0 years in juvenile and adult dermatomyositis, respectively. Both groups had comparable time duration from symptom's onset to biopsy performance. CD4 and CD8 positive cells distributions were similar in both groups in all analyzed area, except for more predominance of CD4 in perimysium at juvenile muscle biopsies. The CD20 and CD68 positive cells were predominantly observed in adult muscle biopsy sections, when compared to juvenile samples, except for similar distribution of CD20 in perivascular endomysium, and CD68 in perimysium. CONCLUSIONS: These data show that the differences between juvenile and adult dermatomyositis may be restricted not only to patients' age, but also to different inflammatory cell distribution, particularly, in new-onset disease. Further studies are necessary to confirm the present study data and to analyze meaning of the different inflammatory cell phenotyping distribution finding in these both diseases.

Juvenile dermatomyositis: is periodontal disease associated with dyslipidemia?

BACKGROUND: Association between periodontal disease and dyslipidemia was recently reported in healthy adults. However, a systematic evaluation of concomitant periodontal diseases and lipid profile was not carried out in juvenile dermatomyositis (JDM). A cross-section study was performed in 25 JDM patients and 25 healthy controls, assessing demographic data, periodontal evaluation, fasting lipoproteins and anti-lipoprotein lipase antibodies. Disease parameters, laboratorial tests and treatment were also evaluated in JDM patients. RESULTS: The mean current age was similar in patients and controls (11.5 ± 3.75 vs. 11.2 ± 2.58 years,p = 0.703). Regarding lipid profile, the median triglycerides [80(31-340) vs. 61(19-182)mg/dL,p = 0.011] and VLDL[16(6-68) vs. 13(4-36)mg/dL,p = 0.020] were significantly higher in JDM patients versus controls. Gingival vasculopathy pattern was significantly higher in the former group (60% vs. 0%,p = 0.0001), as well as the median of gingival bleeding index (GBI) [24.1(4.2-69.4) vs. 11.1(0-66.6)%,p = 0.001] and probing pocket depth (PPD) [1.7(0.6-2.4) vs.1.4(0-2.12)mm,p = 0.006]. Comparison between JDM patients with and without dyslipidemia revealed that the median of dental plaque index (PI) [100(26.7-100) vs. 59(25-100)%,p = 0.022], PPD[1.9(0.6-2.4) vs. 1.4(1.2-1.8)mm,p = 0.024] and clinical attachment level (CAL) [1.31(0.7-1.7) vs. 0.8(0.6-1.7)mm,p = 0.005] were significantly higher in patients with dyslipidemia. Further analysis between JDM patients with and without gingivitis revealed that the median of current age [12.4 (8.3-18.4) vs. 9.2 (5.5-17.5) years, p = 0.034] and disease duration [7.09 ± 3.07 vs. 3.95 ± 2.1 years, p = 0.008] were significantly higher in the former group. CONCLUSION: Our study showed that gingival inflammation seems to be related to dyslipidemia in JDM patients, suggesting underlying mechanisms for both complications.

State of the art on nailfold capillaroscopy in dermatomyositis and polymyositis.

OBJECTIVES: To provide an overview of the main nailfold capillaroscopy (NFC) changes described in dermatomyositis (DM) and polymyositis (PM) and to discuss the current evidence supporting its clinical relevance and applications in daily practice. METHODS: All relevant literature in the field of NFC and DM and PM published in the last 30 years has been systematically reviewed. A systematic research was performed in the electronic databases PubMed and EMBASE. RESULTS: A total of 540 publications were identified according to the proposed filters and 27 were included for the review. The articles have been critically analyzed with a focus on technical aspects, examined anatomical areas, main pathological capillaroscopy findings ,and the relationship between NFC alterations and critical parameters of DM and PM. CONCLUSIONS: The overview confirms that NFC is a safe and noninvasive tool able to help the clinician in the diagnosis of DM and PM and to better characterize the phase of disease activity of these patients.

Race, Income, and Disease Outcomes in Juvenile Dermatomyositis.

OBJECTIVE: To determine the relationships among race, income, and disease outcomes in children with juvenile dermatomyositis (JDM). STUDY DESIGN: Data from 438 subjects with JDM enrolled in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Legacy Registry were analyzed. Demographic data included age, sex, race, annual family income, and insurance status. Clinical outcomes included muscle strength, presence of rash, calcinosis, weakness, physical function, and quality of life measures. Disease outcomes were compared based on race and income. RESULTS: Minority subjects were significantly more likely to have low annual family income and significantly worse scores on measures of physical function, disease activity, and quality of life measures. Subjects with lower annual family income had worse scores on measures of physical function, disease activity, and quality of life scores, as well as weakness. Black subjects were more likely to have calcinosis. Despite these differences in outcome measures, there were no significant differences among the racial groups in time to diagnosis or duration of disease. Using calcinosis as a marker of disease morbidity, black race, annual family income <$50 000 per year, negative antinuclear antibody, and delay in diagnosis >12 months were associated with calcinosis. CONCLUSION: Minority race and lower family income are associated with worse morbidity and outcomes in subjects with JDM. Calcinosis was more common in black subjects. Further studies are needed to examine these associations in more detail, to support efforts to address health disparities in subjects with JDM and improve disease outcomes.

Cutaneous improvement in refractory adult and juvenile dermatomyositis after treatment with rituximab.

OBJECTIVE: The aim was to assess the efficacy of rituximab for the cutaneous manifestations of adult DM and JDM. METHODS: Patients with refractory adult DM (n = 72) and JDM (n = 48) were treated with rituximab in a randomized placebo-phase-controlled trial [either rituximab early drug (week 0/1) or rituximab late arms (week 8/9), such that all subjects received study drug]. Stable concomitant therapy was allowed. Cutaneous disease activity was assessed using the Myositis Disease Activity Assessment Tool, which grades cutaneous disease activity on a visual analog scale. A myositis damage assessment tool, termed the Myositis Damage Index, was used to assess cutaneous damage. Improvement post-rituximab was evaluated in individual rashes as well as in cutaneous disease activity and damage scores. The χ2 test, Student's paired t-test and Wilcoxon test were used for analysis. RESULTS: There were significant improvements in cutaneous disease activity from baseline to the end of the trial after rituximab administration in both adult DM and JDM subsets. The cutaneous visual analog scale activity improved in adult DM (3.22-1.72, P = 0.0002) and JDM (3.26-1.56, P <0.0001), with erythroderma, erythematous rashes without secondary changes of ulceration or necrosis, heliotrope, Gottron sign and papules improving most significantly. Adult DM subjects receiving rituximab earlier in the trial demonstrated a trend for faster cutaneous response (20% relative improvement from baseline) compared with those receiving B cell depletion later (P = 0.052). CONCLUSION: Refractory skin rashes in adult DM and JDM showed improvement after the addition of rituximab to the standard therapy in a clinical trial.

Efficacy and safety of creatine supplementation in juvenile dermatomyositis: A randomized, double-blind, placebo-controlled crossover trial.

INTRODUCTION: It has been suggested that creatine supplementation is safe and effective for treating idiopathic inflammatory myopathies, but no pediatric study has been conducted to date. The objective of this study was to examine the efficacy and safety of creatine supplementation in juvenile dermatomyositis (JDM) patients. METHODS: In this study, JDM patients received placebo or creatine supplementation (0.1 g/kg/day) in a randomized, crossover, double-blind design. Subjects were assessed at baseline and after 12 weeks. The primary outcome was muscle function. Secondary outcomes included body composition, aerobic conditioning, health-related quality of life, and muscle phosphocreatine (PCr) content. Safety was assessed by laboratory parameters and kidney function measurements. RESULTS: Creatine supplementation did not affect muscle function, intramuscular PCr content, or any other secondary outcome. Kidney function was not affected, and no side effects were reported. CONCLUSIONS: Twelve weeks of creatine supplementation in JDM patients were well-tolerated and free of adverse effects, but treatment did not affect muscle function, intramuscular PCr, or any other parameter.

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.

[Devic's disease in an adolescent girl with juvenile dermatomyositis]. / Neuromielite óptica em uma adolescente com dermatomiosite juvenil.

Devic's disease, also known as neuromyelitis optica, is an autoimmune inflammatory demyelinating disorder of the central nervous system that mainly affects the optic nerve and spinal cord. Recently, Devic's disease was demonstrated to be a channelopathy due to the presence of antibodies against the water channel aquaporin-4 in the blood-brain barrier. There have been reports of Devic's disease in infancy, but there are few reported associations of Devic's disease with other diseases. The association of Devic's disease with dermatomyositis has not yet been described in the literature. The aim of this paper is to describe the first case of Devic's disease in an adolescent with juvenile dermatomyositis.

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.