Antinuclear antibodies: two-step detection strategy.

In order to evaluate the performance of the chemiluminescent immunoassay (CLIA) in antinuclear antibodies (ANA) testing, using indirect fluorescent antibody (IFA) on HEp-2 cells as a standard, 209 samples were studied from September to October/2010. The tests were conducted according to the procedures recommended by the manufacturers of the reagents. The interpretation of the IFA results was done according to the Brazilian standards. The charts of patients showing different results between the two techniques were analyzed. The CLIA efficiency was 89%, with a sensitivity of 65%, and a specificity of 94.7%. Nine (4.3%) false-positive and 14 (6.7%) false-negative results were detected. Of these, 13 (93%) represented no risk for the diagnosis and therapeutic management of the patients. The CLIA methodology reduced the need for the IFA manual technique by 77% (160/209). The ANA screening test proved to be a fast and acceptable methodology in the studied population. We established the following criteria for the introduction of an automated ANA screening: (1) Positive results must be confirmed by IFA to characterize the pattern and titer of the antibody. (2) Negative results are issued with a notice to request a new test by IFA when the clinical suspicion of autoimmune disease persists.

Usefulness of anti-dsDNA antibody screening with chemiluminescence followed by confirmation by indirect immunofluorescence.

OBJECTIVE: The purpose of this study was to evaluate the performance of a chemiluminescent immunoassay (CLIA) to detect anti-dsDNA antibodies, using the indirect immunofluorescence test (IIF) on Crithidia luciliae as a reference. METHODS: The automation system demonstrated 81% efficiency, 100% sensitivity and 82% specificity according to the intrinsic validation process performed using 179 consecutive samples from 169 patients in the beginning of 2011. These patients were subsequently divided into 3 groups according to the co-reactivity of anti-dsDNA results using the 2 methods (reactive, non-reactive and discrepant results). RESULTS: Upon data analysis, 77% (129/169) of the tests were requested by rheumatologists, and 57% (97/169) of the samples were from lupus patients. Both the reactive and non-reactive results of the CLIA were well defined and standardised, and automation reduced the manual labor required by 70% in a safe and high-quality manner. Furthermore, the high prevalence of patients with lupus and nephritis among the CLIA false-positive results corroborates the hypothesis that the actual index of CLIA false positivity is lower than that initially found in this study.

Utilidade da triagem dos anticorpos anti-dsDNA por quimioluminescência, seguida de confirmação por imunofluorescência indireta / Usefulness of anti-dsDNA antibody screening with chemiluminescence followed by confirmation by indirect immunofluorescence

OBJETIVO: Avaliar o desempenho de um imunoensaio quimioluminescente (CLIA) para os anticorpos anti-dsDNA, utilizando como referência o teste de imunofluorescência indireta (IFI) sobre Crithidia luciliae. MÉTODOS: O sistema de automação foi previamente aprovado com 81% de eficiência, 100% de sensibilidade e 82% de especificidade, por processo de validação intrínseca em 179 amostras consecutivas de 169 pacientes no início de 2011. A seguir, esses pacientes foram subdivididos em três grupos de acordo com os resultados da pesquisa dos anticorpos anti-dsDNA nas duas metodologias (reagentes, não reagentes e resultados discrepantes). RESULTADOS: Na análise dos dados: 1) 77% (129/169) dos exames haviam sido solicitados por médicos reumatologistas; 2) 57% (97/169) das amostras eram de pacientes lúpicos; 3) Os resultados de CLIA, reagentes e não reagentes, estavam bem definidos e padronizados; 4) A automação reduziu em 70% as passagens pela técnica manual com segurança e qualidade; 5) A alta prevalência de pacientes lúpicos e com nefrite entre os resultados de CLIA falso-positivos corrobora a hipótese de que o índice real de falsa positividade do CLIA seja menor que o encontrado inicialmente neste estudo.

OBJECTIVE: The purpose of this study was to evaluate the performance of a chemiluminescent immunoassay (CLIA) to detect anti-dsDNA antibodies, using the indirect immunofluorescence test (IIF) on Crithidia luciliae as a reference. METHODS: The automation system demonstrated 81% efficiency, 100% sensitivity and 82% specificity according to the intrinsic validation process performed using 179 consecutive samples from 169 patients in the beginning of 2011. These patients were subsequently divided into 3 groups according to the co-reactivity of anti-dsDNA results using the 2 methods (reactive, non-reactive and discrepant results). RESULTS: Upon data analysis, 77% (129/169) of the tests were requested by rheumatologists, and 57% (97/169) of the samples were from lupus patients. Both the reactive and non-reactive results of the CLIA were well defined and standardised, and automation reduced the manual labor required by 70% in a safe and high-quality manner. Furthermore, the high prevalence of patients with lupus and nephritis among the CLIA false-positive results corroborates the hypothesis that the actual index of CLIA false positivity is lower than that initially found in this study.

Interpretation of the presence of IgM and IgG antibodies in a rapid test for dengue: analysis of dengue antibody prevalence in Fortaleza City in the 20th year of the epidemic / Interpretação da presença dos anticorpos IgM e IgG nos testes rápidos para dengue: análise da prevalência dos anticorpos da dengue em Fortaleza, no vigésimo ano da epidemia

INTRODUCTION: The diagnosis of dengue and the differentiation between primary and secondary infections are important for monitoring the spread of the epidemic and identifying the risk of severe forms of the disease. The detection of immunoglobulin (Ig)M and IgG antibodies is the main technique for the laboratory diagnosis of dengue. The present study assessed the application of a rapid test for dengue concerning detection of new cases, reinfection recognition, and estimation of the epidemic attack rate. METHODS: This was a retrospective, cross-sectional, descriptive study on dengue using the Fortaleza Health Municipal Department database. The results from 1,530 tested samples, from 2005-2006, were compared with data from epidemiological studies of dengue outbreaks in 1996, 2003, and 2010. RESULTS: The rapid test confirmed 52% recent infections in the tested patients with clinical suspicion of dengue: 40% detected using IgM and 12% of new cases using IgG in the non-reactive IgM results. The positive IgM plus negative IgG (IgM+ plus IgG-) results showed that 38% of those patients had a recent primary dengue infection, while the positive IgG plus either positive or negative IgM (IgG+ plus IgM+/-) results indicated that 62% had dengue for at least a second time (recent secondary infections). This proportion of reinfections permitted us to estimate the attack rate as >62% of the population sample. CONCLUSIONS: The rapid test for dengue has enhanced our ability to detect new infections and to characterize them into primary and secondary infections, permitting the estimation of the minimal attack rate for a population during an outbreak.

INTRODUÇÃO: O diagnóstico de infecções por dengue e sua diferenciação entre infecções primárias e secundárias são importantes para monitorar a disseminação de epidemias e para identificar riscos de formas graves da doença. A detecção de anticorpos IgM e IgG tem sido o principal mecanismo para o diagnóstico laboratorial. Este estudo visa avaliar a capacidade do teste rápido para dengue para: detectar casos novos da doença, diagnosticar reinfecções e estimar taxas de ataque de epidemias. MÉTODOS: Este trabalho consiste de estudo descritivo, transversal retrospectivo, sobre dengue, que utiliza o banco de dados da Secretaria Municipal de Saúde de Fortaleza. Os resultados de 1.530 amostras testadas entre 2005-2006 foram confrontados com dados de estudos epidemiológicos relativos aos surtos de dengue em 1996, 2003 e 2010. RESULTADOS: o teste foi capaz de confirmar 52% de infecções recentes entre pacientes com suspeitas clínicas de dengue: 40% das infecções foram confirmadas pela banda IgM e 12% de casos extras foram detectados pela banda IgG reagente em amostras IgM nãoreagentes. Resultados IgM reagentes e IgG não-reagentes mostraram que 38% das infecções eram primárias, enquanto resultados IgG reagentes, com ou sem IgM reagente, indicaram que 62% das infecções recentes eram reinfecções. Esta proporção de infecções secundárias permitiu estimar a taxa de ataque como maior ou igual a 62% naquela população amostral. CONCLUSÕES: O teste rápido para dengue apresentou capacidade aumentada para diagnosticar infecções recentes e de caracterizá-las entre infecções primárias e secundárias, permitindo estimar a taxa de ataque mínima para a população amostral de um surto.

Tuberculosis infection in rheumatic patients with infliximab therapy: experience with 157 patients.

It is recommended to evaluate the presence of latent tuberculosis infection (LTBI) prior to the use of antitumor necrosis factor α. The aim of this study is to assess the presence of LTBI in patients with rheumatic diseases undergoing treatment with infliximab in an endemic area for tuberculosis (TB). LTBI was searched through the contact history, chest X-ray and tuberculin skin test with purified protein derivative (PPD) ≥5 mm. We studied 157 patients in the period from May 2005 to October 2008, 99 (63.1%) were women with average age of 49 years and 58 (36.9%) were men with average age of 41 years. The group comprising 90 patients (57.3%) with rheumatoid arthritis (RA), 54 (34.4%) with ankylosing spondylitis (AS) and 13 (8.3%) with psoriatic arthritis (PsA) had PPD reactor 13.4% (21/157), being prevented by isoniazid (INH) in these patients. There are dissimilar responsiveness to the PPD between the three pathologies, and the reactivity was lower in RA (RA × AS: χ(2) = 12; P = 0.0004; and RA × PsA: χ(2) with Yates' correction = 3.6; P = 0.05). No significant difference between the reactivity of the PPD and the use of immunosuppressive drugs (P = 0.81) is observed. The immunoprophylaxis with INH showed an efficacy of 95% (20/21); three (1.9%) patients developed active TB (spondylodiscitis, meningitis and lymphadenopathy) after the use of infliximab, reaffirming extrapulmonary involvement. These results suggest that PPD has a low sensitivity for detection of LTBI in RA and that the previous use of immunosuppressive drugs does not affect the response to PPD.

Systemic Lupus Erythematosus with muscle weakness due to Myasthenia Gravis.

Systemic lupus erythematosus (SLE) and myasthenia gravis (MG) are autoimmune diseases, whose association in the same patient is rarely reported. Both pathologies share the following characteristics: affect mainly young women; alternate exacerbation and remission periods; and have positive antinuclear antibody (ANA) test. This case report assesses possible diagnostic hypotheses for the clinical findings of eyelid ptosis and proximal muscle weakness in a female patient recently diagnosed with SLE, who evolved with associated MG.

Lúpus Eritematoso Sistêmico com fraqueza muscular por Miastenia Gravis / Systemic Lupus Erythematosus with muscle weakness due to Myasthenia Gravis

O Lúpus Eritematoso Sistêmico (LES) e a Miastenia Gravis (MG) são doenças autoimunes cuja associação em um mesmo paciente é raramente descrita. Essas patologias compartilham algumas características como acometimento de mulheres jovens, positividade para anticorpos antinucleares, evolução em períodos de exacerbações e remissões. O presente relato de caso analisa as possíveis hipóteses diagnósticas para um quadro clínico de ptose palpebral e fraqueza muscular proximal em uma paciente portadora de lúpus recente que evoluiu com MG associada.

Systemic lupus erythematosus (SLE) and myasthenia gravis (MG) are autoimmune diseases, whose association in the same patient is rarely reported. Both pathologies share the following characteristics: affect mainly young women; alternate exacerbation and remission periods; and have positive antinuclear antibody (ANA) test. This case report assesses possible diagnostic hypotheses for the clinical findings of eyelid ptosis and proximal muscle weakness in a female patient recently diagnosed with SLE, who evolved with associated MG.

Low prevalence of reactive PPD prior to infliximab use: comparative study on a population sample of Hospital Geral de Fortaleza.

OBJECTIVE: To identify tuberculosis infection in rheumatic patients on infliximab by use of PPD testing prior to immunobiologic therapy. METHODS: This study comprised 157 patients undergoing infliximab treatment and 734 other patients undergoing laboratory screening for tuberculosis infection originating from several services. The Mantoux technique was used for PPD testing, and an induration of at least 5 mm was considered reactive status. RESULTS: In the infliximab group, 13% of the patients reacted to PPD, while, in the other group, 27% of the patients reacted to PPD (χ² = 13; P = 0.0003). These patients were divided into categories: adults with chronic diseases, PPD reactivity of 22%; and other controls, PPD reactivity of 31%. This shows the heterogeneous response of that population (χ² = 7; P < 0.009). In the infliximab group, subdivided according to pathologies [rheumatoid arthritis (RA), ankylosing spondylitis (AS), and psoriatic arthritis (PA)], different reactivity rates were observed, the lowest value occurring among RA patients: (RA x AS: OR = 0.13; CI: 0.03-0.47; χ ² = 12; P = 0.0004) and (RA x PA: OR = 0.16; CI: 0.02-1.04; χ² Yates corrected = 3.6; P = 0.05). The PPD reactivity in the RA subgroup (4%) was also lower as compared with that of the chronic patients group (22%) (OR = 0.16; CI: 0.05-0.49; χ² = 14; P = 0.0002), even when reclassified into four subgroups: rheumatology (OR = 0.19; CI: 0.04-0.72), kidney transplantation (OR = 0.16; CI: 0.05-0.51), infectology (OR = 0.21; CI: 0.05-0.75), and other conditions (OR = 0.13; CI: 0.04-0.44). CONCLUSION: The low prevalence of PPD reaction in this Brazilian population, mainly in chronic patients, with the worst performance among RA patients, showed that the test has limited value for diagnosis of tuberculosis infection in candidates to infliximab therapy.

Baixa prevalência de PPD reativo prévia ao uso de infliximabe: estudo comparativo em população amostral do Hospital Geral de Fortaleza / Low prevalence of reactive PPD prior to infliximab use: comparative study on a population sample of Hospital Geral de Fortaleza

OBJETIVO: Identificar infecção tuberculosa em pacientes reumatológicos em uso de infliximabe, através do PPD realizado como pré-requisito ao início da terapia. MÉTODOS: Foram estudados 157 pacientes em uso de infliximabe e 734 outros pacientes sob triagem de infecção tuberculosa, procedentes de diversas clínicas. O PPD foi realizado pela técnica de Mantoux e considerado reator a partir de 5 mm. RESULTADOS: No grupo infliximabe, o PPD foi reator em 13 por cento e, entre os outros pacientes, a reatividade foi de 27 por cento (χ2 = 13; P = 0,0003). Estes foram subdivididos em adultos portadores de doenças crônicas, com 22 por cento de reatividade ao PPD e demais controles com 31 por cento de positividade, demonstrando heterogeneidade de resposta nessa população (χ2 = 7; P < 0,009). No grupo infliximabe, subdividido por patologias, Artrite Reumatoide (AR), EspondiliteAnquilosante (EA) eArtrite Psoriásica (AP), observaram-se reatividades dissemelhantes, sendo a reatividade na AR sempre menor (AR x EA: OR = 0,13; IC: 0,03-0,47; χ2 = 12; P = 0,0004) e (AR x AP: OR = 0,16; IC: 0,02-1,04; χ2corrigido de Yates = 3,6; P = 0,05). O PPD reator em AR (4 por cento) também foi menor quando comparado com o grupo dos usuários crônicos (22 por cento) (OR = 0,16; IC: 0,05-0,49; χ2 = 14; P = 0,0002), mesmo quando reclassificados em quatro subgrupos: reumatologia (OR = 0,19; IC: 0,04-0,72), transplante renal (OR = 0,16; IC: 0,05-0,51), infectologia (OR = 0,21; IC: 0,05-0,75) e outras clínicas (OR = 0,13; IC: 0,04-0,44). CONCLUSÃO: A baixa prevalência do PPD reativo nessa população brasileira, principalmente em pacientes crônicos, com pior desempenho em AR, mostrou que o teste tem valor limitado para o diagnóstico de infecção tuberculosa em candidatos à terapia com infliximabe.

OBJECTIVE: To identify tuberculosis infection in rheumatic patients on infliximab by use of PPD testing prior to immunobiologic therapy. METHODS: This study comprised 157 patients undergoing infliximab treatment and 734 other patients undergoing laboratory screening for tuberculosis infection originating from several services. The Mantoux technique was used for PPD testing, and an induration of at least 5 mm was considered reactive status. RESULTS: In the infliximab group, 13 percent of the patients reacted to PPD, while, in the other group, 27 percent of the patients reacted to PPD (χ2 = 13; P = 0.0003). These patients were divided into categories: adults with chronic diseases, PPD reactivity of 22 percent; and other controls, PPD reactivity of 31 percent. This shows the heterogeneous response of that population (χ2 = 7; P < 0.009). In the infliximab group, subdivided according to pathologies [rheumatoid arthritis (RA), ankylosing spondylitis (AS), and psoriatic arthritis (PA)], different reactivity rates were observed, the lowest value occurring among RA patients: (RA x AS: OR = 0.13; CI: 0.03-0.47; χ2 = 12; P = 0.0004) and (RA x PA: OR = 0.16; CI: 0.02-1.04; χ2Yates corrected = 3.6; P = 0.05). The PPD reactivity in the RA subgroup (4 percent) was also lower as compared with that of the chronic patients group (22 percent) (OR = 0.16; CI: 0.05-0.49; χ2 = 14; P = 0.0002), even when reclassified into four subgroups: rheumatology (OR = 0.19; CI: 0.04-0.72), kidney transplantation (OR = 0.16; CI: 0.05-0.51), infectology (OR = 0.21; CI: 0.05-0.75), and other conditions (OR = 0.13; CI: 0.04-0.44). CONCLUSION: The low prevalence of PPD reaction in this Brazilian population, mainly in chronic patients, with the worst performance among RA patients, shwoed that the test has limited value for diagnosis of tuberculosis infection in candidates to infliximab therapy.

Wegener's granulomatosis: prevalence of the initial clinical manifestations--report of six cases and review of the literature.

OBJECTIVES: To describe the initial clinical manifestations of Wegener's Granulomatosis (WG) in Brazil. PATIENTS AND METHODS: Retrospective analysis of six medical records of WG patients followed-up at the Rheumatology Department of Hospital Geral of Fortaleza (HGF), as well as a bibliographic survey of cases of WG in Brazil on LILACS, SciELO, and MEDLINE databases. RESULTS: The study identified 49 patients, 15 (31%) males and 34 (69%) females. Systemic disease was observed in 35 patients (73%): 28 adults, 5 children, and 2 teenagers. Limited disease was observed in 13 adults and 1 child. The average age of onset in adults was 42.2 years (18 to 65 years). Acute clinical manifestations, with the onset of symptoms less than three months before the diagnosis, were observed in 41% (20/49) of the patients, and the insidious presentation in 59% (29/49) of the patients. The prevalence of the initial clinical manifestations in adults with systemic disease (n = 28) was 64% (18/28), upper airways, 36% (10/28), lungs, 18% (5/28), kidneys, 25% (7/28), eyes, 11% (3/28) skin, 25% (7/28), musculoskeletal, and 7% (2/28), neurological. In adults (n = 13) with limited disease, prevalent symptoms included: upper airway, 84% (10/13), eyes, 23% (3/13), and lungs, 15% (2/13). CONCLUSION: The prevalence of the initial clinical manifestations of WG in Brazil was similar to that reported in the literature. The lack of specific symptoms may delay diagnosis cases with insidious presentation of the disease and increase the morbidity and mortality in acute disease.

Anti-TNF therapy in renal amyloidosis in refractory rheumatoid arthritis: a new therapeutic perspective.

Amyloidosis is a heterogeneous group of diseases characterized by extracellular deposits of a material composed of aggregates of amyloid--a poorly coupled protein--far from the site of synthesis, causing target organ dysfunction and clinical disease. Systemic amyloidosis A (AA), secondary to infections and chronic inflammation, especially rheumatoid arthritis (RA), is the most common form of amyloid deposition. Treatment of AA consists in the control or resolution of the baseline condition. The objective of the present study was to report a case of secondary renal amyloidosis in a patient with long-term refractory RA who presented sustained clinical improvement after the use of anti-TNFα (etanercept).

Use of the abatacept in a patient with psoriatic arthritis.

Psoriatic arthritis (PA) is an inflammatory seronegative arthritis of unknown origin. Classically, PA has five clinical forms, and asymmetric oligoarthritis is the most common type. We describe the case of a patient with PA refractory to disease-modifying drugs, who developed drug-induced hepatitis after chemoprophylaxis with isoniazid, administered prior to the treatment with an anti-TNFα agent. Due to the risk of activating latent tuberculosis with the administration of anti-TNFα and hepatotoxicity onset caused by the TB treatment and based on the fact that the treatment of PA is similar to the treatment of rheumatoid arthritis, a decision was made to use the empirical treatment with abatacept. Approximately twenty days after the second infusion of the drug, the patient showed clinical improvement, resolution of the arthritis, almost complete disappearance of the skin lesions and improvement of anemia and inflammatory tests.

Uso do abatacepte em uma paciente com artrite psoriásica / Use of the abatacept in a patient with psoriatic arthritis

Artrite psoriásica (AP) é uma artrite inflamatória soronegativa de causa desconhecida. Classicamente, a AP apresenta cinco formas clínicas, sendo a oligoartrite assimétrica a mais comum. Descrevemos o caso de uma paciente com AP refratária às drogas modificadoras da doença, que evoluiu com hepatite medicamentosa após quimioprofilaxia com isoniazida, administrada previamente ao tratamento com anti-TNFα. Em virtude do risco de ativação de tuberculose (TB) latente pela administração de anti-TNFα, da hepatotoxicidade decorrente do tratamento da TB, e baseado no fato de o tratamento da AP se assemelhar ao da artrite reumatoide, optou-se pelo tratamento empírico com abatacepte. Aproximadamente vinte dias após a segunda dose do biológico, a paciente evoluiu com importante melhora clínica, resolução da artrite, regressão das lesões de pele e melhora da anemia e das provas de atividade inflamatória.

Psoriatic arthritis (PA) is an inflammatory seronegative arthritis of unknown origin. Classically, PA has five clinical forms, and asymmetric oligoarthritis is the most common type. We describe the case of a patient with PA refractory to disease-modifying drugs, who developed drug-induced hepatitis after chemoprophylaxis with isoniazid, administered prior to the treatment with an anti-TNFα agent. Due to the risk of activating latent tuberculosis with the administration of anti-TNFα and hepatotoxicity onset caused by the TB treatment and based on the fact that the treatment of PA is similar to the treatment of rheumatoid arthritis, a decision was made to use the empirical treatment with abatacept. Approximately twenty days after the second infusion of the drug, the patient showed clinical improvement, resolution of the arthritis, almost complete disappearance of the skin lesions and improvement of anemia and inflammatory tests.

Terapia com anti-TNF na amiloidose renal em artrite reumatoide refratária: uma nova perspectiva terapêutica / Anti-TNF therapy in renal amyloidosis in refractory rheumatoid arthritis: a new therapeutic perspective

As amiloidoses são um grupo heterogêneo de doenças caracterizadas pelo depósito extracelular de uma substância amiloide composta por agregados de proteínas mal acopladas que se depositam longe do sítio de síntese, causando disfunção do órgão-alvo e doença clínica. A forma sistêmica mais comum é a amiloidose A (AA) secundária às infecções e às inflamações crônicas, sendo a artrite reumatoide (AR) a causa mais frequente. O tratamento da amiloidose AA consiste no controle ou na resolução da doença de base. O objetivo do presente estudo é relatar um caso de amiloidose renal secundária em paciente com AR refratária de longa duração que apresentou melhora clínica sustentada após o uso de anti-TNFα (etanercepte).

Amyloidosis is a heterogeneous group of diseases characterized by extracellular deposits of a material composed of aggregates of amyloid - a poorly coupled protein - far from the site of synthesis, causing target organ dysfunction and clinical disease. Systemic amyloidosis A (AA), secondary to infections and chronic inflammation, especially rheumatoid arthritis (RA), is the most common form of amyloid deposition. Treatment of AA consists in the control or resolution of the baseline condition. The objective of the present study was to report a case of secondary renal amyloidosis in a patient with long-term refractory RA who presented sustained clinical improvement after the use of anti-TNFα (etanercept).

Prevalência das manifestações clínicas iniciais da granulomatose de Wegener no Brasil: relato de seis casos e revisão da literatura / Wegener's granulomatosis: prevalence of the initial clinical manifestations: report of six cases and review of the literature

OBJETIVOS: Descrever as manifestações clínicas iniciais da Granulomatose de Wegener (GW) diagnosticada no Brasil. PACIENTES E MÉTODOS: Análise retrospectiva de seis prontuários do Serviço de Reumatologia do Hospital Geral de Fortaleza (HGF), assim como a realização de um levantamento bibliográfico dos casos de GW descritos no Brasil obtidos dos bancos de dados LILACS, SciELO e MEDLINE. RESULTADOS: O estudo identificou 49 pacientes; 15 (31 por cento) do sexo masculino e 34 (69 por cento) do sexo feminino. A forma sistêmica ocorreu em 35 pacientes (73 por cento): 28 adultos, cinco crianças e dois adolescentes. A doença limitada ocorreu em 13 adultos e uma criança. A média da idade adulta no início da doença foi de 42,2 anos (18 a 65 anos). O quadro clínico agudo, com sintomas há menos de três meses do diagnóstico, ocorreu em 41 por cento (20/49) da casuística e a forma insidiosa, em 59 por cento (29/49) dos pacientes. A prevalência das manifestações clínicas iniciais nos adultos com doença sistêmica (n = 28) foi 64 por cento (18/28) das vias aéreas superiores (VAS), 36 por cento (10/28) pulmonares, 18 por cento (5/28) renais, 25 por cento (7/28) oculares, 11 por cento (3/28) cutâneas, 25 por cento (7/28) musculoesqueléticas e 7 por cento (2/28) neurológicas. Na forma limitada do adulto (n = 13), os sintomas prevalentes foram 84 por cento (11/13) VAS, 23 por cento (3/13) oculares e 15 por cento (2/13) pulmonares. CONCLUSÃO: No Brasil, a prevalência das manifestações clínicas iniciais da GW foi semelhante aos resultados da literatura. A falta de especificidade dos sintomas pode retardar o diagnóstico na forma insidiosa da doença e aumentar a morbimortalidade das formas agudas.

OBJECTIVES: To describe the initial clinical manifestations of Wegener's Granulomatosis (WG) in Brazil. PATIENTS AND METHODS: Retrospective analysis of six medical records of WG patients followed-up at the Rheumatology Department of Hospital Geral of Fortaleza (HGF), as well as a bibliographic survey of cases of WG in Brazil on LILACS, SciELO, and MEDLINE databases. RESULTS: The study identified 49 patients, 15 (31 percent) males and 34 (69 percent) females. Systemic disease was observed in 35 patients (73 percent): 28 adults, 5 children, and 2 teenagers. Limited disease was observed in 13 adults and 1 child. The average age of onset in adults was 42.2 years (18 to 65 years). Acute clinical manifestations, with the onset of symptoms less than three months before the diagnosis, were observed in 41 percent (20/49) of the patients, and the insidious presentation in 59 percent (29/49) of the patients. The prevalence of the initial clinical manifestations in adults with systemic disease (n = 28) was 64 percent (18/28), upper airways, 36 percent (10/28), lungs, 18 percent (5/28), kidneys, 25 percent (7/28), eyes, 11 percent (3/28) skin, 25 percent (7/28), musculoskeletal, and 7 percent (2/28), neurological. In adults (n = 13) with limited disease, prevalent symptoms included: upper airway, 84 percent (10/13), eyes, 23 percent (3/13), and lungs, 15 percent (2/13). CONCLUSION: The prevalence of the initial clinical manifestations of WG in Brazil was similar to that reported in the literature. The lack of specific symptoms may delay diagnosis cases with insidious presentation of the disease and increase the morbidity and mortality in acute disease.

Abatacept as an option therapy in difficult to treat psoriatic arthritis.

Psoriatic arthritis (PA) is an inflammatory arthritis associated with cutaneous psoriasis. Treatment consists of non-steroidal anti-inflammatory drugs, corticosteroids at low doses and disease-modifying anti-rheumatic drugs. We relate a case of PA that after the fault of the handlings convecionais to sick we opted for an empiric treatment with Abatacept, based on the current knowledge on the physiopathology of PA and its low hepatotoxicity found in an animal model.

Retinite por citomegalovirus (CMV) após terapia imunossupressora para vasculite leucocitoclástica / Cytomegalovirus (CMV) retinitis after immunossupressive therapy for leukocytoclastic vasculitis

A retinite por citomegalovírus (CMV) é uma doença rara que acomete principalmente pacientes com a síndrome da imunodeficiência adquirida (AIDS). No entanto, outros pacientes imunossuprimidos, como os transplantados, os que estão em uso de quimioterápicos, pacientes com lúpus eritematoso sistêmico (LES) ou em tratamento com drogas imunossupressoras também podem ser acometidos. O quadro clínico caracteriza-se por visão turva, diminuição da acuidade ou alterações de campo visual, geralmente unilateral, podendo ocorrer deslocamento de retina. A perda visual é progressiva, evolui em ritmo variável até a completa amaurose do olho acometido. O presente relato de caso descreve um paciente com vasculite leucocitoclástica grave submetido à terapia com corticoide em dose imunossupressora que evoluiu com glaucoma, panuveíte por CMV, perda da acuidade visual e infecção bacteriana secundária.

Cytomegalovirus (CMV) retinitis is a rare disease which mainly affects patients with acquired immunodeficiency syndrome (AIDS). Nevertheless, other immunosuppressed patients, such as the organ transplant recipients, the ones using chemotherapy, patients with systemic lupus erythematosus (SLE) or in treatment with immunosuppressive drugs can also be attacked. The clinical characteristics are blurred vision, decrease of the visual acuity or visual field alterations, generally unilateral, with the possibility of retinal detachment. The visual loss is progressive, evolving in a variable rate until complete amaurosis of the attacked eye. The present case report describes a patient with severe leukocytoclastic vasculitis, submitted to corticosteroid therapy in immunosuppressive doses that evolved with glaucoma, panuveitis by CMV, loss of visual acuity and secondary bacterial infection.

Análise de prevalência e evolução das manifestações neuropsiquiátricas moderadas e graves em pacientes com lúpus eritematoso sistêmico internados no serviço de reumatologia do Hospital Geral de Fortaleza / Prevalence and follow-up analysis of evolution of severe and moderate neuropsychiatric manifestations in hospitalized systemic lupus erythematosus in the rheumatology service of the Hospital General de Fortaleza

OBJETIVO: Avaliar a prevalência e a resposta terapêutica das manifestações neuropsiquiátricas (MNP) moderadas ou graves em pacientes lúpicos internados no Hospital Geral de Fortaleza (HGF). MÉTODOS: Durante dois anos, 110 pacientes de internações consecutivas com lúpus eritematoso sistêmico, segundo os critérios do Colégio Americano de Reumatologia, foram avaliados para MNP moderadas ou graves. Nestes pacientes estudou-se: causa da internação, presença de auto-anticorpos, liquor, imagens radiológicas, análise retrospectiva dos prontuários e terapêutica utilizada. RESULTADOS: A prevalência de MNP foi de 16,4 por cento (18/110), em 17 mulheres e um homem, com idade média de 29 anos; por ocasião do internamento, 33 por cento (6/18) dos pacientes apresentavam apenas MNP. A ocorrência da primeira MNP foi de 11 por cento (2/18) antes do diagnóstico, 33 por cento (6/18) por ocasião do diagnóstico e 56 por cento (10/18) pós-diagnóstico. A mortalidade foi de 11 por cento (2/18). As principais MNP foram: convulsões e cefaléia (50 por cento), psicose (22 por cento), acidente vascular cerebral (17 por cento) e síncope e depressão maior (11 por cento). Resposta terapêutica: 28 por cento (5/18) pacientes responderam à corticoterapia oral, 17 por cento (3/18) realizaram pulso de metilprednisolona e 56 por cento (10/18) pulso de ciclofosfamida. CONCLUSÃO: A prevalência de MNP na população estudada encontra-se no limite inferior da literatura. Na ausência de estudos clínicos controlados, a abordagem destes pacientes é fundamentada em séries de casos e na experiência profissional do serviço.

OBJECTIVE: Evaluate the prevalence and therapy outcome of moderate and severe neuropsychiatric manifestations (NPM) in patients with systemic lupus erythematosus admitted at the Hospital Geral de Fortaleza. METHODS: During two years, 110 patients with consecutive hospitalizations suffering from systemic lupus erythematosus, according to the American College of Rheumatology criteria, were evaluated for moderate and severe NPM. The following parameters were studied in these patients: cause of admission, presence of auto-antibodies, cerebral spinal fluid, radiological imaging, retrospective study of medical records and treatment. RESULTS: The prevalence of NPM was 16.4 percent (18/110), being seventeen women and one man; the mean age was 29 years; during the hospitalization term, only 33 percent (6/18) of the patients had an NPM. The occurrence of the first NPM was in 11 percent (2/18) of the cases before diagnosis, in 33 percent (6/18) during diagnosis and in 56 percent (10/18) after diagnosis. The mortality rate was 11 percent (2/18). The most common NPM's were: seizures and headache (50 percent), psychosis (22 percent), cerebrovascular disease (17 percent), syncope and major depression (11 percent). Treatment outcome: 28 percent (5/18) of the patients responded to oral corticoids, 17 percent (3/18) had a pulse of methylprednisolone and 56 percent (10/18) were treated with cyclophosphamide. CONCLUSION: The prevalence of NPM in the patients of this study was on the lowest limit reported by the related literature. With no controlled clinical trials, the approach to these patients is based on case reports and the professional experience of the service.

Caracterização da resposta imune em modelo experimental de esclerodermia induzida por colágeno tipo V / Humoral immune response characterization of the type V collagen induced scleroderma experimental model

Os modelos experimentais reproduzem doenças que acometem seres humanos, sendo de extrema importância porque possibilitam o estudo da patogênese e abordagem terapêutica dessas enfermidades. Nesse grupo inclui-se o modelo experimental de esclerodermia induzida pela imunização de coelhos com colágeno V humano provocando alterações histológicas (pele, pulmão e rim) similares àquelas observadas em humanos. As doenças auto-imunes têm sua etiologia desconhecida e são particularmente caracterizadas pela presença de auto-anticorpos no soro. Nesse aspecto, 90 a 95% dos pacientes com esclerodermia apresentam algum auto-anticorpo contra antígenos intracelulares (proteínas nucleolares RNA polimerase I, II e III, Scl-70, centriolares ou golginas) ou da matriz extracelular (colágeno). O presente estudo tem como objetivo avaliar a resposta imunológica nos animais do modelo experimental de esclerodermia. Para tanto, o soro dos animais foram testados quanto à presença de auto-anticorpos e de outros fatores imunológicos séricos que indicassem um processo imunológico ativo paralelo às lesões teciduais em desenvolvimento e incluiu: pesquisa de anticorpos anticolágenos V, III e I, imunocomplexos circulantes, fator reumatóide, níveis de complemento, fatores antinucleares (FAN) por imunofluorescência indireta em células HEp-2 e caracterização dos antígenos alvos por immunoblot. A análise da resposta imune revelou que as alterações histológicas do pulmão, rim e pele se desenvolviam com o aumento dos níveis séricos de anticorpos anti-colágeno V. Além disso, todos os animais imunizados com Col V apresentavam anticorpos para outros tipos de colágeno. Esses animais desenvolveram uma marcante resposta imunológica a antígenos intracelulares com 100% de positividade para o FAN e presença de imunocomplexos nos soros obtidos 30, 75 e 120 dias pós-imunização quando comparados a dois grupos controles (albumina e adjuvante completo de Freud). Todos os animais do grupo Col V apresentaram...

Experimental models for human diseases are of utmost importance, since they allow the study of their pathogenesis and therapeutic approach. In this group we can include the experimental model of collagen V-induced scleroderma, in rabbits, with histological alterations (skin, lung and kidney) similar to those observed in humans. Auto-immune diseases have an unknown etiology, and are characterized by the presence of auto-antibodies in serum. In this aspect, 90%-95% of the patients with scleroderma present some kind of auto-antibody against intracellular antigens (RNA polymerase I, II and III nucleoproteins, Scl-70, centriolar or golgins) or to components of the extracellular matrix (collagen). This study aims to assess the immune response of the animal subjects in a scleroderma experimental model. The animals sera were tested for auto-antibodies and other serum immunological factors that would point to an active immunological process, parallel to the developing tissue lesions, including anti-collagen V, III and I antibodies, circulating immune complexes, rheumatoid factor, complement levels, antinuclear antibodies (ANA) by indirect immunofluorescence in HEp-2 (IFI) cells, and characterization of target antigens with an immunoblot. The analysis of the immune response in the studied animals revealed that histological alterations in lungs, kidneys and skin developed with an increase of the serum levels of anti-collagen V antibodies. Furthermore, all the Col Vimmunized animals presented antibodies against other types of collagen as well. These animals also developed a strong immune response against intracellular antigens, being 100% positive for ANA, showing also immune complexes in sera obtained 30, 75 and 120 days after immunization with Col V, when compared to the control groups (albumin and Freunds complete adjuvant). All the animals from the Col V group showed a cytoplasmic pattern at the IFI, Golgi simile and, in 10% of the cases, additional reactivity...