Precordial pain, leukocytosis and bicytopenia in a teenager with systemic juvenile idiopathic arthritis under immunosuppressive therapy / Dor precordial, leucocitose e bicitopenia em adolescente com artrite idiopática juvenil sistêmica em terapia imunossupressora

ABSTRACT Objective: To highlight the importance of the new classification criteria for the macrophage activation syndrome (MAS) in systemic juvenile idiopathic arthritis in order to reduce morbidity and mortality outcome related to this disease. Case description: A 12-year-old female patient with diagnosis of systemic juvenile idiopathic arthritis under immunosuppression therapy for two years developed cough, acute precordial chest pain, tachypnea, tachycardia and hypoxemia for two days. Chest tomography showed bilateral laminar pleural effusion with bibasilar consolidation. The electrocardiogram was consistent with acute pericarditis and the echocardiogram showed no abnormalities. Laboratory exams revealed anemia, leukocytosis and increased erythrocyte sedimentation rate, as well as C-reactive protein rate and serum biomarkers indicative of myocardial injury. Systemic infection and/or active systemic juvenile idiopathic arthritis were considered. She was treated with antibiotics and glucocorticoids. However, 10 days later she developed active systemic disease (fever, evanescent rash and myopericarditis with signs of heart failure) associated with macrophage activation syndrome, according to the 2016 Classification Criteria for Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis. She was treated for five days with pulse therapy, using glucocorticoids, immunoglobulin and cyclosporine A, with improvement of all clinical signs and laboratory tests. Comments: Myopericarditis with signs of heart failure associated with MAS is a rare clinical presentation of systemic juvenile idiopathic arthritis. Macrophage activation syndrome occurs mainly during periods of active systemic juvenile idiopathic arthritis and may be triggered by infection. Knowledge about this syndrome is crucial to reduce morbidity and mortality.

RESUMO Objetivo: Destacar a importância do conhecimento sobre os novos critérios de classificação para síndrome de ativação macrofágica (SAM) na artrite idiopática juvenil sistêmica para reduzir a morbidade e mortalidade desse desfecho. Descrição do caso: Adolescente do sexo feminino de 12 anos de idade, em terapia imunossupressora por diagnóstico de artrite idiopática juvenil sistêmica há 2 anos, com quadro de tosse, dor precordial aguda, taquipneia, taquicardia e hipoxemia há 2 dias. A tomografia de tórax evidenciou efusão pleural laminar bilateral com consolidação bibasal. O eletrocardiograma foi compatível com pericardite aguda, e o ecocardiograma foi normal. Os exames laboratoriais revelaram anemia, leucocitose e aumento da velocidade de hemossedimentação, proteína C-reativa e marcadores séricos de lesão miocárdica. Infecção sistêmica e/ou doença sistêmica em atividade foram consideradas. A paciente foi tratada com antibióticos e glicocorticoide. Entretanto, dez dias depois, evoluiu com doença sistêmica em atividade (febre, exantema e miopericardite com insuficiência cardíaca) associada à SAM, de acordo com o 2016 Classification Criteria for Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis, e necessitou de cinco dias de pulsoterapia com glicocorticoide, imunoglobulina e ciclosporina A, com melhora de todos os parâmetros clínicos e laboratoriais. Comentários: A miopericardite com sinais de insuficiência cardíaca associada à SAM é uma apresentação clínica rara da artrite idiopática juvenil sistêmica, que ocorre principalmente em períodos de atividade sistêmica da doença e pode ser deflagrada por infecções. O conhecimento sobre essa síndrome é fundamental para reduzir morbidade e mortalidade desse grave desfecho.

Inmunizaciones en pacientes con artritis idiopática juvenil / Immunizations in patients with juvenile idiopathic arthritis

Los pacientes con AIJ/ARJ presentan un riesgo mayor de infecciones inmunopreveni-bles, debido a su disfunción inmune, exacerbada por la actividad de su enfermedad y la terapia inmunosupresora. Las vacunas inactivadas han demostrado un perfil de seguridad adecuado en estos pacientes, por lo que no están contraindicadas, aunque su respuesta inmune puede ser inadecuada. Las vacunas vivas atenuadas, formal-mente contraindicadas, poseen una información creciente que permite evaluar su riesgo beneficio de manera individual. Por este motivo, debemos procurar mantener el calendario de vacunas actualizado y complementado, evitando el retraso en esque-mas de vacunación y poniéndolo al día lo antes posible, con estrategias basadas en el individuo, idealmente antes de iniciar la terapia inmunosupresora o de lo contrario durante ella. Para llevar a cabo esto debemos conocer y considerar los intervalos entre las vacunas, los esquemas acelerados, la solicitud de vacunas especiales, las aprobaciones vigentes y, finalmente, sus contraindicaciones.

Patients with JIA/JRA present a higher risk of vaccine-preventable infections, due to their immune dysfunction, exacerbated by the activity of their disease and immu-nosuppressive therapy. Inactivated vaccines have shown an adequate safety profile in these patients, so they are not contraindicated, although their immune response may be impaired. Live attenuated vaccines, formally contraindicated, have a growing information that allows to evaluate their risk benefit case by case. For this reason we must try to keep the vaccination schedule updated and supplemented, avoiding the delay in vaccination schemes and updating it as soon as possible, with taylor-based strategies, ideally before starting immunosuppressive therapy or otherwise during it. To carry out this we must manage and consider the intervals between the vaccines, the accelerated schemes, the request for special vaccines, the current approvals and, finally, their contraindications.

Evolución de pacientes con artritis idiopática juvenil en terapia biológica que cursan infección por virus varicela zoster: a propósito de 4 casos / Outcomes of patients with juvenile idiopathic arthritis on biological therapy and have a varicella-zoster virus infection

En niños inmunocomprometidos, la infección por virus varicela puede producir una enfermedad grave. Existen pocos casos publicados de varicela en pacientes con artritis idiopática juvenil (AIJ) y terapia biológica. OBJETIVO: Describir la evolución de pacientes con AIJ con terapia biológica que adquirieron el virus varicela. CASOS CLÍNICOS: Se describe la historia clínica de 4 pacientes con AIJ, de entre 3 y 12 años de edad, que presentaron infección por virus varicela zoster estando con distintas terapias biológicas: 2 con anti TNF, uno con anti IL-6 y uno con bloqueador de la coestimulación del linfocito T. Dos de ellos habían recibido la vacuna contra la varicela. Todos recibieron diferentes terapias y evolucionaron sin complicaciones, no encontrando diferencias importantes en relación con el tipo de terapia biológica ni con el antecedente de haber sido vacunados. En todos los pacientes se suspendió el tratamiento biológico por al menos 2 semanas y se reinició sin reactivación de la artritis. CONCLUSIONES: En esta serie de pacientes con AIJ tratados con terapia biológica que cursaron con infección por VVZ no se observaron complicaciones graves.

Varicella virus infection may develop into severe disease in immunocompromised children. There are few studies that describe the clinical presentation of varicella infection in patients with Juvenile Idiopathic Arthritis when on biological therapy. OBJECTIVE: Describe the outcomes of patients with a diagnosis of Juvenile Idiopathic Arthritis, who acquired a varicella virus infection during treatment with biological therapy. CLINICAL CASES: A description is presented on 4 cases of Juvenile Idiopathic Arthritis in children between 3 and 12 years old, who developed a varicella-zoster infection during treatment with different biological therapies. Two patients were taking anti-TNF agents, one an Anti IL-6 agent, and one patient a T cell costimulatory blockade agent. Two of them received varicella vaccination prior to the start of biological therapy. All of them received different therapies and had favourable outcome without developing complications. No significant differences were found as regards the type of biological therapy or history of previous vaccination. Biological therapy was suspended for at least 2 weeks in all patients, and was restarted without reactivation of arthritis. CONCLUSIONS: No serious complications were observed in this patient series of children with JIA treated with biological therapy associated with VZV infection.

Association of neopterin as a marker of immune system activation and juvenile rheumatoid arthritis activity / Associação de neopterina como marcador de ativação do sistema imunológico e atividade da artrite reumatoide juvenil

OBJECTIVE: To evaluate neopterin plasma concentrations in patients with active juvenile idiopathic arthritis (JIA) and correlate them with disease activity.METHODS: Sixty patients diagnosed as active JIA, as well as another 60 apparently healthy age- and gender-matched children as controls, were recruited from the Pediatrics Allergy and Immunology Clinic, Ain Shams University. Disease activity was assessed by the Juvenile Arthritis Disease Activity Score 27 (JADAS-27). Laboratory investigations were performed for all patients, including determination of hemoglobin concentration (Hgb), erythrocyte sedimentation rate (ESR), and C-reactive protein. Serum concentrations of tumor necrosis factor-alpha (TNF-a), interleukin-6 (IL-6), monocyte chemoattractant protein-1 (MCP-1), and neopterin were measured.RESULTS: Significant differences were found between JIA patients and controls with regard to the mean levels of Hgb, ESR, TNF-a, IL-6, and MCP-1 (p < 0.05). A statistically significant higher mean level serum neopterin concentration (p < 0.05) was found in JIA patients (20.43 ± 8.73 nmol/L) than in controls (6.88 ± 2.87 nmol/L) (p < 0.05). Positive significant correlations were detected between serum neopterin and ESR, TNF-a, IL-6, MCP-1, and JADAS-27 (p < 0.05). No correlation was found between serum neopterin and CRP (p > 0.05). Multiple linear regression analysis showed that JADAS- 27 and ESR were the main variables associated with serum neopterin in JIA patients (p < 0.05).CONCLUSION: The elevation of plasma neopterin concentrations in early JIA patients may indicate stimulation of immune response. Serum neopterin can be used as a sensitive marker for assaying background inflammation and disease activity score in JIA patients.

OBJETIVO: Avaliar as concentrações plasmáticas de neopterina em pacientes com artrite idiopática juvenil (AIJ) ativa e correlacioná-las com a atividade da doença.MÉTODOS: Foram recrutados da clínica de Alergia e Imunologia Infantil da Universidade Ain Shams 60 pacientes diagnosticados com AIJ ativa, bem como 60 crianças aparentemente saudáveis com a mesma idade e o mesmo sexo no grupo de controle. A atividade da doença foi avaliada pelo Escore de Atividade da Doença da Artrite Juvenil em 27 Articulações (JADAS-27). Foram feitas investigações laboratoriais em todos os pacientes, incluindo a determinação da concentração de hemoglobinas, a taxa de sedimentação de eritrócitos e a proteína C-reativa. Foram mensuradas as concentrações séricas do fator de necrose tumoral alfa, interleucina-6 e proteína quimiotática de monócitos-1 e neopterina.RESULTADOS: Foi encontrada uma diferença significativa entre os pacientes com AIJ e os controles quanto às médias de Hb, TSE, FNT-a, IL-6 e MCP-1 (p < 0,05). Foi encontrado um nível estatística e significativamente maior de concentração média de neopterina sérica (p < 0,05) em pacientes com AIJ (valor médio de 20,43 ± 8,73 nmol/L) do que em controles (valor médio de 6,88 ± 2,87 nmol/L) (p < 0,05). Foram detectadas correlações positivas significativas entre a neopterina sérica e TSE, FNT-a, IL-6, MCP-1 e JADAS-27 (p < 0,05). Não foi encontrada correlação entre a neopterina sérica e a PCR (p > 0,05). A análise de regressão linear múltipla mostrou que o JADAS-27 e a TSE foram as principais variáveis associadas à neopterina sérica em pacientes com AIJ (p < 0,05).CONCLUSÃO: A elevação das concentrações plasmáticas de neopterina em pacientes com AIJ precoce pode indicar um estímulo de resposta imune. A neopterina sérica pode ser usada como um indicador sensível para analisar o histórico de inflamações e o escore de atividade da doença em pacientes com AIJ.

Induction of metalloproteinases expression by TLR ligands in human fibroblast like synoviocytes from juvenile idiopathic arthritis patients.

Background & objective: Juvenile idiopathic arthritis (JIA) is characterized by chronic synovitis, cartilage damage and bone erosion. Both genetic and environmental factors and microbes probably play a role in pathogenesis. Microbes are recognized by Toll like receptors (TLRs) and activate innate immune response. We studied the ability of bacterial and viral products to produce matrix metalloproteinases (MMPs) and cytokines by fibroblast like synoviocytes (FLS) from patients with JIA. Methods: FLS were cultured from synovial fluid (SF) of patients with JIA and subsequently stimulated for 48 h by different TLR ligands [peptidoglycan (PG) for TLR2, poly(I-C) for TLR3, lipopolysaccharide (LPS) for TLR4, flagellin for TLR5, imiquimod for TLR7 and CpG DNA for TLR9]. Later the production of IL6, IL8, MMP-1, MMP-3, tissue inhibitors of metalloproteinase (TIMP1) was measured in the culture supernatants by ELISA. Expression of TLR2, TLR4, TLR7 and TLR9 was studied in FLS derived from JIA patients by RT-PCR. Results: IL6, IL8, MMP3 and MMP1 production was induced on stimulation of FLS with TLR2 ligand, TLR3 ligand, TLR4 ligand, TLR5 ligand but not with TLR7 ligand and TLR9 ligand. There was no effect of these ligands on the production of TIMP thus the balance was tilted in favour of MMPs after TLR ligation. TLR2, TLR4 and low expression of TLR9 was found but, no expression of TLR7 was found in FLS from JIA patients. Interpretation & conclusion: TLR pathway stimulation by microbial products or endogenous ligands could be involved in the production of MMPs in JIA and may contribute to disease pathology. Thus it may be beneficial to inhibit TLR pathway to reduce cartilage destruction.

Anti-cyclic citrullinated peptide antibodies in juvenile idiopathic arthritis.

Objective. Prevalence and clinical significance of anti-cyclic citrullinated peptide (CCP) antibodies in Indian patients with juvenile idiopathic arthritis (JIA). Methods. Anti-CCP antibodies were determined by enzyme-linked immunosorbent assay (ELISA) in 78 patients with JIA which included all 3 major subtypes of the disease: pauciarticular, polyarticular afld systemic onset. Values above 5 relative units were taken as positive. Associations between antiCCP antibodies and clinical and laboratory and radiological parameters were determined. Results. Anti-CCP antibodies were positive in only 2 of 34 (5.9%) patients with pauciarticular JIA and 3 of 17 (17.6%) of systemic,.pnset JIA, whereas it was positive in 13 of 27 (48.1%) of polyarticular JIA patients (p < 0.001). Furthermore, it was seen that among patients with polyarticular JIA, RF-lgM positive patients had higher rate of anti-CCP antibody positivity with 7 of 8 (87.5%) patients having positive anti-CCP antibody (p<0.001). Similarly, patients with erosions (11/19; p<0.001) and deformities (5/-10; p<0.001) were found to have significant association with anti-CCP antibody positivity. Conclusion. Anti-CCP antibodies could be detected more frequently in the sera of JIA patients with severe manifestations like- erosions and deformity. It was also more significantly associated with seropositive polyarticular JIA than other types. It can be presumed from these results that anti-CCP antibodies can be used as a marker to predict severe course of JIA at the onset to guide optimal aggressive therapy.

Soluble adhesion molecules in juvenile idiopathic arthritis: relation to activity and clinical subtype

The present research aimed to evaluate serum Adhesion Molecules [AMs] in patients with Juvenile Idiopathic Arthritis [JIA] to correlate their values with disease activity in different clinical subtypes. Serum levels of some soluble AMs [E-selectin, sICAM 1 and sVCAM 1] wereassayed by ELISA in 37 patients with JIA both during activity and after remission. Other activity parameters like sedimentation rate and leukocytic counts were tested as well. Twentyhealthy children of matched age and sex were taken as control. Serum E-selectin was found significantly higher in JIA compared to control [in all subtypes across all disease stages], with significant drop after remission, yet not reached the normal values. These changes were more evident in systemic JIA compared to other subtypes. Serum ICAM 1 and VCAM 1 showed the same changes in relation to control and to the disease activity. We can conclude that systemic JIA is associated with higher levels of soluble AMs thus explaining the perpetual inflammatory process and hence the remissions and exacerbations which are usually associated with higher morbidity in systemic JIA than in the other subtypes. We recommend following JIA patients until laboratory remission [normalization of serum AMs] to correlate AMs levels to clinical course aiming to put forward a therapeutic plan

Prevalência de anticorpos contra peptídeos cíclicos citrulinados na artrite idiopática juvenil / The prevalence of anti-cyclic citrullinated peptide antibodies in juvenile idiopathic arthritis

OBJETIVOS: Avaliar a presença de anticorpos contra peptídeos cíclicos citrulinados em uma coorte de pacientes com artrite idiopática juvenil. MÉTODOS: A presença de anticorpos contra peptídeos cíclicos citrulinados foi avaliada por ensaio imunoenzimático (ELISA) no soro de pacientes com artrite idiopática juvenil com idade inferior a 18 anos, acompanhados no ambulatório de reumatologia pediátrica do Hospital de Clínicas de Porto Alegre, com tempo de diagnóstico de doença de, no mínimo, 6 meses. Também foi estudada a presença do fator reumatóide IgM e do fator antinuclear em células Hep-2 RESULTADOS: Foram analisadas amostras séricas de 45 pacientes com artrite idiopática juvenil. A presença de títulos elevados de anticorpos contra peptídeos cíclicos citrulinados foi encontrada somente no soro de uma criança (2 por cento), a qual apresentava quadro de poliartrite com fator reumatóide reagente. CONCLUSÕES: O anticorpo contra peptídeos cíclicos citrulinados pode ser detectado em crianças com artrite idiopática juvenil, mas em freqüência muito inferior aos adultos com artrite reumatóide. Torna-se importante avaliar se anticorpos contra peptídeos cíclicos citrulinados podem identificar os pacientes com artrite idiopática juvenil com potencial de evolução para artrite reumatóide do adulto.

Síndrome de activación macrofagico en pediatría: a propósito de cuatro casos / Activated macrophage syndrome in paediatrics: 4 clinical cases

Introducción: El Síndrome de Activación Macrofágico (SAM) es una entidad poco frecuente en la práctica pediátrica que se caracteriza por una excesiva activación del sistema macrofágico y por una liberación exagerada de citoquinas por parte de los linfocitos T, y que clínicamente se manifiesta como un síndrome semejante a una falla orgánica múltiple. Existe actualmente disparidad en la nomenclatura de este síndrome, y es así como a nivel de la reumatología pediátrica se mantiene el término de SAM, mientras que para los hemato-oncólogos esta enfermedad está incluida dentro de las diferentes variedades de histiocitosis. Objetivo: Actualizar el conocimiento respecto de la etiología, clínica y tratamiento del SAM, enfermedad de baja frecuencia y alta mortalidad, en relación al análisis de 4 casos clínicos. Casos clínicos: 2 pacientes eran portadores de una enfermedad reumatológica, Artritis Idiopática Juvenil, el tercer paciente presentaba elementos de Inmunodeficiencia, y el último niño de linfohistiocitosis familiar congénita. Los factores desencadenantes del SAM fueron fármacos en dos pacientes y una posible causa infecciosa en los dos restantes. Los cuatro pacientes sobrevivieron al proceso inicial después de recibir una terapia agresiva inmunosupresora con esteroides en altas dosis y ciclosporina intravenosa. Conclusión: La importancia de dar a conocer este síndrome, radica en que un diagnóstico precoz y una terapia agresiva, preferentemente con ciclosporina y pulsos de esteroides en altas dosis, puede mejorar significativamente el pronóstico de esta enfermedad.

Síndrome de ativação macrofágica associada com artrite idiopática juvenil sistêmica / Macrophage activation syndrome associated with systemic juvenile idiopathic arthritis

OBJETIVO: Descrever as características da síndrome de ativação macrofágica associada a artrite idiopática juvenil. DESCRIÇÃO DOS CASOS: Foram analisados retrospectivamente os prontuários de 462 pacientes com artrite idiopática juvenil. Destes, sete (1,5 por cento) pacientes desenvolveram síndrome de ativação macrofágica; todos tinham a forma sistêmica da doença. A mediana de idade de início da artrite idiopática juvenil foi de 3 anos e 10 meses, e a mediana do tempo de duração da artrite idiopática juvenil antes da síndrome de ativação macrofágica foi de 8 anos e 4 meses. Todos os pacientes apresentaram febre, icterícia, hepatoesplenomegalia, sangramentos, pancitopenia e elevação das enzimas hepáticas e dos tempos de coagulação e bilirrubina direta. Três casos apresentaram infecções associadas e um caso desenvolveu a síndrome de ativação macrofágica 2 semanas após a introdução de sulfasalazina. Três pacientes morreram. Proliferação macrofágica e hemofagocitose foram evidenciadas em cinco. A terapêutica da síndrome de ativação macrofágica incluiu pulsoterapia com metilprednisolona em todos, ciclosporina em três, plasmaférese em dois e gamaglobulina endovenosa em dois. COMENTARIOS: A síndrome de ativação macrofágica é uma complicação da artrite idiopática juvenil sistêmica com alta morbidade e mortalidade.

Low levels of anti-histone antibodies in north Indian children with juvenile rheumatoid arthritis.

BACKGROUND & OBJECTIVES: Early onset pauciarticular disease with uveitis is distinctly uncommon in Indian children with juvenile rheumatoid arthritis (JRA). The occurrence of anti-histone antibodies (AHA) in serum is strongly associated with presence of uveitis. There is a paucity of information from India on the levels of AHA in patients of JRA. In this study, an attempt was made to evaluate the levels of IgG and IgM antibodies to histones in children with JRA in north India. METHODS: Serum samples of 148 children with JRA (84 boys, 64 girls) were collected. Clinical details including onset, symptoms and course of the disease in each patient were recorded. Detailed eye examination including slit lamp examination was done in all patients at presentation and yearly thereafter to rule out uveitis. The presence of antihistone IgG and IgM antibodies was studied by ELISA. Antinuclear antibodies (ANA) were measured by indirect immunofluorescence using HEP-2 cells as substrate at a screening dilution of 1:40. RESULTS: Of the 148 children, 54 had pauciarticular (12 early onset and 42 late onset), 64 polyarticular and 30 systemic onset disease respectively. ANA were present in two children. AHA were raised in 15 (10%) children, of whom 10 had IgM antibodies, 3 had IgG and 2 had both isotypes. None of the children with early onset pauciarticular disease had uveitis, ANA or AHA. INTERPRETATION & CONCLUSION: The low occurrence of AHA and uveitis in our subset of patients with JRA is in contrast to that reported from Western countries. The low occurrence is unlikely due to technical reasons as the antigen that has been used consistently showed significant binding to serum from patients with systemic lupus erythematosus (SLE). This is in accordance with the rarity of early onset pauciarticular disease and chronic uveitis in these patients. More studies from other parts of the country are required to validate this observation.

Artritis reumatoídea juvenil o artritis juvenil / Juvenile rheumatoid arthritis

Se revisaron los conceptos, clínicos y diagnósticos de la artritis reumatoidea juvenil, haciendo énfasis en la clasificación, patogénesis, aspectos genéticos. Se puntualiza sobre los posibles factores inmunológicos envueltos en la etiología del quebranto y se señala la posibilidad de denominar la enfermedad como artritis juvenil (AJ) en vez de artritis reumatoidea juvenil

Factor reumatoideo en artritis reumatoidea juvenil

Se estudiaron 60 niños afectados de artritis reumatoidea juvenil con la finalidad de observar la frecuencia de positividad del factor reumatoideo en ellos. El factor reumatoideo fue determinado cuantitativamente utilizando las pruebas de Látex y de Waler Rose. Se encontró sólo un 6.3% de postividad, tanto para la prueba de Látex como la de Waler-Rose; esta prueba fue particularmente positiva en el grupo de niños adolescentes. Se concluye que en los niños, la positividad es baja debiéndose guiar de la clínica para el establecimiento del diagnóstico. Dado que ambas pruebas dieron resultados similares se aconseja preferir al Látex cuantitativo por ser esta prueba más económica y sencilla

Defect of suppressor cell induction in patients with juvenile rheumatoid arthritis.

We studied the suppressor cell activity induced by concanavalin A (Con A) in 9 patients with acute febrile juvenile rheumatoid arthritis (JRA). The suppressor activity of JRA patients was higher than that of normal controls. However, the activity was significantly reduced by treating Con A-activated cells with mitomycin C (MMC) (P less than 0.05). On the other hand, the suppressor activity of normal controls and systemic lupus erythematosus (SLE) patients was not affected by MMC treatment. Two of 5 SLE patients showed low activity even before MMC treatment. The addition of the culture supernatant of Con A-stimulated peripheral blood mononuclear cells from a normal donor restored the induction of suppressor activity of JRA which was decreased by MMC treatment. The results indicated that patients with acute febrile type of JRA had reduced MMC resistant suppressor cell activity and that this was due to a defect in the ability of the cells to produce soluble factors needed to induce MMC resistant suppressor cells.

Enfermedad de Still en el niño y en el adulto: estudio comparativo de sus características clínicas; serológicas y de histocompatibilidad / Still disease in children and adults: comparative study of its clinical, serological and histocompatibility characteristics

Diecisiete pacientes con enfermedad de Still de comienzo juvenil y 6 pacientes con comienzo en la edad adulta fueron analizados comparativamente con el objeto de determinar la influencia de la edad en la expresión clínica de esta afección. Doce de 17 pacientes juveniles y 3 de 6 adultos eran de sexo masculino. Fiebre héctica, rash cutáneo y poliartritis se observaron en todos los pacientes de ambos grupos. Leucocitosis se observó en 13 de 17 pacientes juveniles (76%) y en 5 de 6 (83%) pacientes adultos. Pericarditis, esplenomegalia, adenomegalias, anemia y anquílosis ósea se observaron con mayor frecuencia, aunque no significativamente, en el grupo de pacientes de comienzo juvenil. El antígeno HLA DR4 se encontró en 6 de 11 (54,5%) pacientes juveniles, frecuencia significativamente aumentada con respecto a la población general. En los adulto 1 de 6 tenían HLA DR4. No existió relación entre pronóstico favorable y presencia del antígeno HLA Bw35. En este trabajo no se encontraron diferencias significativas en las manifestaciones clínicas y serológicas entre pacientes juveniles y adultos con enfermedad de Still