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.

Increased serum sFas, sTRAIL, and reduced sFasL in juvenile-onset systemic lupus erythematosus.

The aims of this study were to assess serum Fas, FasL, TRAIL, and Bcl-2 levels in patients with juvenile-onset systemic lupus erythematosus (JSLE) and to evaluate their relations with disease activity parameters and nephritis. Forty-eight JSLE patients, 33 juvenile idiopathic arthritis (JIA, inflammatory controls) patients and 40 healthy controls were enrolled. sFas, sFasL, sTRAIL, and sBcl-2 serum levels were measured by ELISA. Disease activity parameters included SLEDAI score, ESR, anti-dsDNA antibodies, C3, and C4 levels. Thirty-five JSLE patients had nephritis and 32 patients were classified as having active disease (SLEDAI ≥4). Statistical analysis methods included Mann-Whitney test and Spearman's rank test. JSLE patients had significantly increased sFas serum levels compared with healthy controls (median 177.6 vs. 117.5 pg/mL; p = 0.0001), higher sTRAIL (median 484.6 vs 270.8 pg/mL; p = 0.02), and reduced sFasL (median 0.05 vs 0.3 ng/mL; p = 0.0002). The same results were observed for JSLE patients with active disease and for patients with nephritis. Additionally, sFas levels in JSLE patients directly correlated with SLEDAI score (r = 0.40; p = 0.009), and sTRAIL levels were increased in JSLE patients with neuropsychiatric disease compared with those without this involvement (median 667.9 vs. 216.2 pg/mL; p = 0.03). Otherwise, sBcl-2 levels of JSLE patients were similar to healthy controls. JIA patients had sFas, sFasL, sTRAIL, and sBcl-2 serum levels similar to JSLE patients and to healthy controls. In summary, this study characterized in JSLE a distinct profile from adult SLE that comprises increased sFas, sTRAIL, and reduced sFasL, notably in patients with active disease and with nephritis.

Autoinflammatory diseases: mimics of autoimmunity or part of its spectrum? Case presentation.

INTRODUCTION: Autoinflammatory diseases are very rare diseases presenting within a wide clinical spectrum. Recognition of the main clinical features are challenging due to overlapping or mimicking with autoimmune diseases. DISCUSSION: A case series is reviewed to illustrate typical and atypical features and the difficulties of these diagnoses in the low prevalence areas--a typical unrecognized case of familial Mediterranean fever (FMF) in a youngster, an atypical adult case with overlapping of FMF with Behçet disease, and an early presentation of FMF in infant presenting with inflammatory colitis, as well as the overlapping features within the cryopirin diseases spectrum in an 8-year-old boy who presented with systemic onset arthritis. CONCLUSION: These cases may represent examples of a very puzzling relationship among disorders of innate and adaptive immune systems and inflammation.

Tratamento clínico da febre reumática / Clinical treatment of rheumatic fever

O tratamento de pacientes com febre reumática compreende três fases: a profilaxia primária ou erradicação dos estreptococos da orofaringe, o tratamento sintomático das manifestações clínicas, e a profilaxia secundária ou prevenção de novos surtos. Para a profilaxia primária, a droga de escolha é a penicilina; em pacientes alérgicos à penicilina, a primeira opção é a eritromicina. As vantagens da penicilina benzatina são enfatizadas e a utilização de outros antibióticos como as cefalosporinas e azitromicina deve ser evitada, pelo risco de desenvolvimento de resistência bacteriana. A artrite da febre reumática deve ser tratada com antiinflamatórios não-hormonais, como o ácido acetilsalicílico e o naproxeno, durante quatro a seis semanas. A cardite deve ser tratada com prednisona na dose inicial de 2 mg/kg/dia, com reduções progressivas, dependentes da evolução, até completar 12 semanas. O uso de corticosteróides por via oral ou parenteral e de gamaglobulina não interfere no prognóstico da cardite. Para o tratamento da coréia utilizam-se o haloperidol ou os valproatos. Os barbitúricos, a prednisona em altas doses e a carbamazepina apresentam eficácias comparáveis. A profilaxia secundária deve ser realizada com a penicilina benzatina e, nos casos de alergia à penicilina, com a sulfadiazina ou a eritromicina. Doses de 1.200.000 U devem ser recomendadas e administradas a cada três semanas. A profilaxia secundária deve se estender até os 18 anos ou, no mínimo, durante cinco anos em pacientes sem cardite. A presença de cardite indica a profilaxia durante a vida inteira ou pelo menos até os 25 anos e no mínimo durante dez anos.

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

OBJECTIVE: To describe the characteristics of macrophage activation syndrome associated with juvenile idiopathic arthritis. DESCRIPTION: This is a retrospective study involving 462 patients with juvenile idiopathic arthritis. Seven (1.5%) of those patients suffered from systemic onset juvenile idiopathic arthritis and developed macrophage activation syndrome. The median age of the juvenile idiopathic arthritis onset was 3 years and 10 months and the median duration of juvenile idiopathic arthritis before macrophage activation syndrome was 8 years and 4 months. All of them presented with fever, jaundice, hepatosplenomegaly, bleeding, pancytopenia, abnormal liver function tests and abnormal coagulation profile. Three cases presented associated infections and one patient developed macrophage activation syndrome two weeks after the administration of sulfasalazine. Three patients died and the macrophage hemophagocytosis was present in five. The treatment of macrophage activation syndrome included pulse therapy with methylprednisolone in all of them, cyclosporine A in three, plasma exchange in two and intravenous immunoglobulin in two. COMMENTS: Macrophage activation syndrome is a complication of the systemic onset juvenile idiopathic arthritis with a high morbidity and mortality rate.

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.

Nitric oxide-derived species in synovial fluid from patients with juvenile idiopathic arthritis.

OBJECTIVE: To evaluate superoxide anion (O2-), nitrite/nitrate (NO2-/NO3-), and nitrotyrosine (NT) production and the contribution of myeloperoxidase (MPO) to the production of NT-containing proteins in the synovial fluid (SF) of patients with juvenile idiopathic arthritis (JIA). The affected tissues in inflammatory arthritis produce large amounts of nitric oxide (NO) or peroxynitrite (ONOO-) but there are no reports of NO or ONOO- participation in JIA. We also attempted to correlate our findings with variables of disease activity and articular damage. METHODS: We analyzed 40 patients with JIA, mean age 12.7 years, mean disease duration 7.8 years. O2- production was measured by cytochrome C reduction after incubation of 106 synovial fluid (SF) cells with or without phorbol myristate acetate (PMA), formyl-methionyl-leucyl-phenylalanine (FMLP) or opsonized zymosan. SF and serum NO2-/NO3- levels were measured by Griess reaction; NT was detected by Western blot. Myeloperoxidase (MPO) activity was estimated spectrophotometrically. Clinical and laboratory variables [erythrocyte sedimentation rate, C reactive protein (CRP), and radiological score] and interleukin 6 (IL-6) levels were evaluated. RESULTS: NO2-/NO3- production was greatly enhanced in the joints of JIA patients (54.6 +/- 3.2 micro M) when compared with serum (13.9 +/- 0.6 micro M; p < 0.001). NO2-/NO3- levels in SF were positively correlated with the number of infiltrating lymphomononuclear cells. NT-modified proteins detected in the SF showed a high correlation with radiological score, disease duration, CRP, and IL-6. CONCLUSION: Our results confirm the increased oxidative stress in children with JIA, suggesting a high in situ production of NO. The positive correlation between the expression of NT-modified proteins and variables of disease activity and damage is additional evidence that nitrogen and oxygen species may be involved in the joint destruction seen in patients with JIA.

Antiphospholipid antibodies in 57 children and adolescents with systemic lupus erythematosus.

OBJECTIVE: To investigate the frequencies and behavior of antiphospholipid antibodies in 57 children and adolescents with systemic lupus erythematosus. METHODS: Anticardiolipin antibodies were investigated by ELISA and lupus anticoagulant antibodies by the international tests recommended. The antiphospholipid antibodies analyses were performed in frozen samples (mean of 5.3 samples per patient obtained during a mean follow-up period of 3 years and 7 months) and on blood samples collected between January 1997 and November 1998 (mean of 2.5 samples per patient during a 2-year follow-up period). RESULTS: The frequencies of antiphospholipid antibodies (anticardiolipin and lupus anticoagulant) were similar in the samples collected prospectively and in the frozen samples (retrospective study): 63.2% and 75.4% respectively. Positivity for these antibodies fluctuated during the follow-up period and was not associated with any clinical or laboratory parameters of lupus erythematosus, including autoantibodies and also including disease activity and/or severity scores. CONCLUSIONS: The frequencies of antiphospholipid antibodies in children and adolescents with lupus erythematosus were similar to those observed in adults. The positivity fluctuated during the follow-up and was not correlated with clinical and/or laboratory disease parameters.

Inter- and intrareader variability in the interpretation of two radiographic classification systems for juvenile rheumatoid arthritis.

OBJECTIVE: To evaluate the inter- and intrareader variability for interpretation of a modified Larsen's radiographic classification system for juvenile rheumatoid arthritis (JRA) focused on osteochondral lesions and a conventional Larsen's classification system, compared to a reference MR scoring system of corresponding images. MATERIALS AND METHODS: Seventy-five radiographs of 60 children with JRA, performed within a short interval of time from the MR examinations, were independently evaluated by three experienced radiologists, three diagnostic imaging residents and three rheumatologists, in two separate sessions, according to the two different classification methods, blinded to the corresponding MR images. RESULTS: The inter- and intrareader concordance rates between the two radiographic classification systems and the MR-related radiographs were respectively poor and poor/moderate. The interobserver range of weighted kappa values for the conventional and the modified Larsen's system respectively was 0.25-0.37 vs 0.19-0.39 for radiologists, 0.25-0.37 vs 0.18-0.30 for residents and 0.19-0.51 vs 0.17-0.29 for rheumatologists. The intrareader rate ranged from 0.17-0.55 for radiologists, 0.2-0.56 for residents, and 0.14-0.59 for rheumatologists. CONCLUSION: Although the proposal of a new radiographic classification system for JRA focused on osteochondral abnormalities sounds promising, the low inter- and intrareader concordance rates with an MR-related radiographic system makes the clinical applicability of such a radiographic system less suitable.

Fibrodisplasia ossificante progressiva (FOP) em crianças: relato de três casos / Fibrodysplasia ossificans progressive (FOP) in children: report of three cases

Fibrodisplasia ossificante progressiva (FOP) é uma doença do tecido conectivo associada com ossificação endocondral, levando à imobilização permanente. Os autores descrevem as manifestações clínicas, alterações laboratoriais, achados radiológicos e terapêuticas utilizadas em três crianças com diagnóstico de FOP. O diagnóstico de FOP foi estabelecido quando o paciente apresentava malformações congênitas (hipoplasia de háluces e polegares e hálux valgus) associadas à ossificação de músculos estriados. Dois pacientes foram submetidos a biópsia cutânea e muscular. A idade do início da doença variou de 6 meses a 11 anos (mediana = 2 anos); dois pacientes eram do sexo feminino. Edema inicial ocorreu em todos os pacientes e precedeu as assificações em todos os casos. Uma paciente evoluiu para imobilização permanente, após trauma (necessitando de cadeira de rodas). Não foram observadas alterações laboratoriais nos três pacientes. Um paciente apresentou doença pulmonar restritiva e dois casos evoluíram com litíase renal. As biópsias cutâneas e muscular realizadas em dois casos mostraram fibromatose, e cursaram com ossificação no local da incisão cirúrgica. Apesar da terapêutica instituída (colchicina, EDTA, alendronato e/ou metrotrexato), todos os pacientes evoluíram com novas ossificações. Edema pode ser uma manifestação inicial de FOP. O diagnóstico é clínico e radiológico, devendo-se evitar traumas e biópsias para prevenir novas ossificações. Nenhum medicamento foi eficaz nesses pacientes

Antiphospholipid antibodies in 57 children and adolescents with systemic lupus erythematosus

OBJECTIVE: To investigate the frequencies and behavior of antiphospholipid antibodies in 57 children and adolescents with systemic lupus erythematosus. METHODS: Anticardiolipin antibodies were investigated by ELISA and lupus anticoagulant antibodies by the international tests recommended. The antiphospholipid antibodies analyses were performed in frozen samples (mean of 5.3 samples per patient obtained during a mean follow-up period of 3 years and 7 months) and on blood samples collected between January 1997 and November 1998 (mean of 2.5 samples per patient during a 2-year follow-up period). RESULTS: The frequencies of antiphospholipid antibodies (anticardiolipin and lupus anticoagulant) were similar in the samples collected prospectively and in the frozen samples (retrospective study): 63.2 percent and 75.4 percent respectively. Positivity for these antibodies fluctuated during the follow-up period and was not associated with any clinical or laboratory parameters of lupus erythematosus, including autoantibodies and also including disease activity and/or severity scores. CONCLUSIONS: The frequencies of antiphospholipid antibodies in children and adolescents with lupus erythematosus were similar to those observed in adults. The positivity fluctuated during the follow-up and was not correlated with clinical and/or laboratory disease parameters

Analysis of human leukocyte antigens class II-DR in Brazilian children and adolescents with systemic lupus erythematosus

OBJECTIVE: To analyze the frequency of human leukocyte antigens class II-DR in children and adolescents with systemic lupus erythematosus. PATIENTS AND METHODS: Fifty-fiveBrazilian systemic lupus erythematosus children and adolescents and 308 healthy individuals were studied. Gender, race, and age of onset of systemic lupus erythematosus were recorded. The human leukocyte antigens typing of class II-DR was carried out by polymerase chain reaction amplification with sequence-specific primers (PCR-SSP). Data were analyzed statistically using the chi square test with Yates' correction, Fisher's exact test, and Bonferroni's correction. RESULTS: Human leukocyte antigen-DR 15 was the most frequently detected antigen in this group of children and adolescents, and it also occurred more frequently in the female group, in children with onset of systemic lupus erythematosus between 0 and 9 years and between 10 to 14 years, and in the Black race group, but these associations were not statistically significants. CONCLUSION: In this group of children and adolescents with a high degree of racial admixture, we could not verify a significant association between human leukocyte antigens class II-DR and systemic lupus erythematosus

Immunohistological analysis of CD59 and membrane attack complex of complement in muscle in juvenile dermatomyositis.

OBJECTIVE: To assess the presence of CD59 and the deposition of membrane attack complex (MAC) of complement system in skeletal muscle from patients with juvenile dermatomyositis (JDM), in comparison to patients with muscular dystrophies (MD) and children with normal muscle biopsies. METHODS: Muscle specimens obtained for diagnostic purposes from 10 patients with JDM, 6 with MD, and 7 children whose biopsies showed normal histology were analyzed. Immunohistological staining was performed using Mab against CD59 (YTH 53.1) and MAC (WU 7.2). RESULTS: Immunohistochemical staining for CD59 was weak and irregularly distributed on muscle fibers of all patients with JDM. Two of the 9 biopsies that allowed analysis of vessels showed negative CD59 staining in all vessels; in the remaining 7 patients, there was weak staining in a proportion of the vessels. In contrast, uniform and strong or moderate immunoreactivity was detected on the sarcolemma and in intramuscular endothelium in all normal and MD samples. Immunostaining for MAC was strong in JDM muscle vessels, and weak in normal or MD muscle. An inverse relation was found between MAC deposition and presence of CD59 in vessels in 6/9 JDM biopsies and in all normal and MD samples. CONCLUSION: Decreased CD59 expression in JDM muscle fibers and vessels may be associated with muscle lesions mediated by deposition of MAC of complement in JDM.

Angiomatose óssea difusa: relato de uma caso e revisão da literatura / Diffuse angiomatosis of bone: a case report and review of the literature

Relatamos o caso de uma menina com história de fraqueza muscular progressiva e artrite, cuja investigação demonstrou lesões líticas ósseas e diagnóstico de angiomatose óssea difusa. A angiomatose é uma malformação vascular rara, que pode acometer a pele, o tecido subcutâneo, os músculos, os ossos, entre outros. Apresenta cursos clínicos variáveis e diretamente relacionados aos órgãos e sistemas acometidos. Quando as lesões líticas ocorrem exclusivamente nos ossos, as manifestações clínicas mais freqüentes são dores e edema das áreas afetadas e fraturas patológicas. As alterações radiológicas são freqüentemente características e indicam o diagnóstico. O tratamento com radioterapia e/ou quimioterapia é pouco eficaz. A angiomatose óssea difusa tem progressão lenta, eventualmente acompanhada de esclerose dos cistos ósseos.

Analysis of human leukocyte antigens class II-DR in Brazilian children and adolescents with systemic lupus erythematosus.

OBJECTIVE: To analyze the frequency of human leukocyte antigens class II-DR in children and adolescents with systemic lupus erythematosus. PATIENTS AND METHODS: Fifty-fiveBrazilian systemic lupus erythematosus children and adolescents and 308 healthy individuals were studied. Gender, race, and age of onset of systemic lupus erythematosus were recorded. The human leukocyte antigens typing of class II-DR was carried out by polymerase chain reaction amplification with sequence-specific primers (PCR-SSP). Data were analyzed statistically using the chi square test with Yates' correction, Fisher's exact test, and Bonferroni's correction. RESULTS: Human leukocyte antigen-DR 15 was the most frequently detected antigen in this group of children and adolescents, and it also occurred more frequently in the female group, in children with onset of systemic lupus erythematosus between 0 and 9 years and between 10 to 14 years, and in the Black race group, but these associations were not statistically significants. CONCLUSION: In this group of children and adolescents with a high degree of racial admixture, we could not verify a significant association between human leukocyte antigens class II-DR and systemic lupus erythematosus.

Correlation between osteochondral changes depicted by magnetic resonance imaging and disease progression

PURPOSE: To determine the consequences of the chronic use of systemic corticosteroids in children with juvenile rheumatoid arthritis by means of evaluating osteochondral effects depicted by magnetic resonance imaging. PATIENTS AND METHODS: We reviewed clinical and magnetic resonance imaging findings in 69 children (72 knees) with juvenile rheumatoid arthritis. Two groups were studied. Group I: 34 (49.3 percent) children had previous or current use of systemic corticotherapy (22 girls; 12 boys; mean age: 11.3 years; mean disease duration: 5.9 years; mean corticotherapy duration: 2.9 years; mean cumulative dose of previous corticosteroids: 5000 mg); Group II: 35 (50.7 percent) children had no previous use of corticosteroids (27 girls; 8 boys; mean age: 11.7 years; mean disease duration: 5.3 years). The groups were compared statistically. RESULTS: In the group that had received corticotherapy (Group I), osteochondral abnormalities were significantly correlated to long-standing disease (>3.5 years; p<0.001). This correlation was not found in the group that had no previous history of corticotherapy (Group II). No correlations were established between median dose of corticosteroids and magnetic resonance imaging findings. CONCLUSION: It is important to further investigate the long-term intra-articular effects of systemic corticotherapy to ensure that the side effects of the aggressive therapy will not be more harmful for the joints than the symptoms suffered over the natural course of the disease

Síndrome da fibromialgia em crianças e adolescentes. Estudo Clínico de 34 casos / Fibromyalgia syndrome in children and adolescents. Clinical features of 34 cases

Objetivo: A síndrome da fibromialgia é uma doença crônica, não inflamatória, caracterizada por dor difusa, fadiga e presença de pontos dolorosos em localizações específicas. A frequência da síndrome da fibromialgia na infância vem aumentando à medida que esta entidade se torna mais conhecida. Métodos: Este estudo analisou, retrospectivamente, 34 prontuários de pacientes com diagnóstico de fibromialgia e descreveu seus principais sinais, sintomas e o tipo de terapêutica utilizada. Vinte e quatro pacientes eram do sexo feminino (71 por cento) e 10 do masculino (29 por cento). A idade média de início da doença foi de 10,4 anos e o tempo de seguimento variou de dois a 120 meses. Resultados: Os pontos dolorosos mais frequentes foram: borda medial da escápula (88,2 por cento), epicôndilo lateral do cotovelo (82,3 por cento) e borda medial do joelho (73,5 por cento). Todos os pacientes apresentavam sintomas associados; os mais frequentes foram cefaléia (28 por cento) e dor abdominal (47 por cento). Dezenove pacientes (56 por cento) apresentavam fatores de melhora, sendo os principais massagem (58 por cento) e uso de analgésicos (42 por cento). Vinte e dois pacientes (65 por cento) apresentavam fatores de piora, sendo os principais exercício (55 por cento) e frio (50 por cento). Trinta e um pacientes (91 por cento) foram tratados com uma ou mais modalidades terapêuticas. Vinte e sete (87 por cento) com antiinflamatórios não hormonais, 10 (32 por cento) com fisioterapia, 10 (32 por cento) com acupuntura, nove (29 por cento) com psicoterapia, sete (23 por cento) com atividade física e sete (23 por cento) com antidepressivos. Todos os pacientes tratados apresentaram melhora de sintomas. Conclusão: A síndrome da fibromialgia é pouco diagnosticada em crianças e adolescentes, especialmente pelo desconhecimento dos sintomas e sinais a ela associados. Sua identificação permite a instituição de medidas terapêuticas que melhoram de maneira significativa a qualidade de vida destes pacientes

Análise comparativa entre aquisiçäo TRI (3D) e bidimensional (2D) em ressonância clínica em joelhos em artrite reumatóide juvenil (ARJ) / Comparative analysis between two-dimensional (3D) and three-dimensional (2D) MRI acquisitions-clinical applications for the evalution of the kness in juvenille rheumatoid arthritis (JAR)

Os autores pretendem, neste estudo transversal ("cross-sectional") de pacientes co artrite reumatóide juvenil, comparar a análise bidimensional de espessura sinovial máxima obtida de cortes de sequencia axial sinovial (tridimensional) obtida de cortes da sequencia axial T1 SPIR ("spectral presaturation with inversion recovery") SE pós-gadolino. Estas duas formas de avaliaçäo imagenológica säo associadas a parâmetros clínicos, laboratoriais e á presença de realce sinovial á ressonância magnética, com objetivo de avaliaçäo de seu custo-benefício.

Estudo da uveíte na artrite reumatóide juvenil / Study of iveitis in juvenile rheumatoid arthritis

Objetivo: Estudar a incidência de uveíte em pacientes com artrite reumatóide juvenil (ARJ) do Hospital das Clínicas da Faculdade de Medicina da USP bem como a freqüência da uveíte nos diferentes subtipos de ARJ e possíveis fatores de risco para desenvolvimento da uveíte. Métodos: Foi realizado exame oftalmológico completo em 160 crianças com idade inferior a 16 anos e diagnóstico de ARJ, sendo 52 com a forma sistêmica, 35 com a forma poliarticular e 73 com a forma pauciarticular. Resultados: Na forma sistêmica observou-se fator anti-núcleo (FAN) positivo em 8,5 por cento e fator reumatóde (FR) positivo em 2,2 por cento; na forma poliarticular, FAN e FR foram positivos em 8,6 por cento; e na forma pauciarticular encontrou-se FAN positivo em 25,8 por cento e FR positivo em 0 por cento. Na forma sistêmica näo foi observado nenhum tipo de uveíte. Na forma poliarticular foi encontrada iridociclite bilateral em 5,7 por cento, sendo que um deles evoluiu com catarata e ceratopatia em faixa em ambos os olhos. Na forma pauciarticular foi observada iridociclite em 6,8 por cento, tendo um caso unilateral e quatro bilaterais, tendo um olho evoluído com catarata. Conclusöes: A incidência de uveíte foi de 4,4 por cento, acometendo principalmente os portadores da forma pauciarticular. FAN positivo e idade do início da artrite foram fatores de risco para o desenvolvimento da uveíte.