Fabricio Souza Neves

Cross-cultural adaptation of the Behçet’s Disease Current Activity Form (BDCAF) to Brazilian Portuguese language

The Behçet’s Disease Current Activity Form (BDCAF) is a clinical instrument used to assess the activity of Behçet’s disease (BD), which was originally developed in English. The aim of the present study was to perform a cross-cultural adaptation of the BDCAF to Brazilian Portuguese language and to evaluate its reliability in a population of Brazilian patients with BD. Brazilian Portuguese version of the BDCAF, named BR-BDCAF, was obtained according to established guidelines. Forty Brazilian patients with BD diagnosed according to the International Study Group for Behçet’s Disease criteria were assessed by two rheumatologists in independent sessions and submitted to the BR-BDCAF. Inter- and intraobserver agreement were then evaluated by kappa scores (values higher than 0.6 indicated good agreement). Good inter- and intraobserver agreements were achieved for the most common manifestations of BD: kappa scores higher than 0.6 were obtained for oral and genital ulcerations, skin lesions, and articular and general complaints. Moderate interobserver agreement was obtained for ocular activity (kappa 0.483) and fair interobserver agreement was obtained for gastrointestinal (kappa 0.322), major vessel (kappa 0.281), and central nervous system activity (kappa 0.304). BR-BDCAF was found to be a reliable instrument for the classic mucocutaneous and articular manifestations of BD and for general complaints, but complementary assessment is needed to evaluate specific visceral involvement for disease activity.

Spontaneous pneumomediastinum and dermatomyositis may be a not so rare association: report of a case and review of the literature

One patient with dermatomyositis (DM) who developed spontaneous pneumomediastinum (SPnM) is described. A review of 15 previously reported cases shows that this is a complication of DM but not of polymyositis. It occurs in young patients with recent onset of disease characterized by cutaneous vasculopathy, and patients treated with immunosuppressive drugs have, in general, better outcomes. We suggest that the onset of SPnM in DM patients must alert to possible vasculitic activity and that immunosuppressive agents with progressive tapering of corticosteroids are part of the most reasonable approach in these cases.

Is there a link between inflammation and abnormal lipoprotein profile in Sjögren's syndrome?

OBJECTIVES To investigate the lipoprotein profile of patients with primary Sjögrens syndrome (pSS) and its association with laboratory tests, including markers of inflammation. METHODS This is a cross-sectional study among patients with pSS and healthy controls. We analyzed the lipoprotein profile of 73 pSS patients compared to 65 healthy individuals in the control group. We further evaluated possible associations between dyslipidemia in pSS patients and laboratory parameters including: hypergammaglobulinemia, autoantibodies [antinuclear antibodies (ANA), rheumatoid factor (RF), anti-Ro, anti-La], and acute-phase reactants [C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR)]. RESULTS Patients and controls were comparable regarding the demographic variables. Lipoprotein profile was similar between pSS patients and controls: total cholesterol (204.0+/-43.39 versus 206.5+/-42.76 mg/mL, P=0.73), LDL fraction (131.6+/-37.38 versus 130.62+/-38.24 mg/dL, P=0.88) and HDL fraction (49.7+/-13.5 versus 51+/-11.5mg/dL, P=0.56), triglycerides (129.3+/-81.0 versus 116.8+/-53.5mg/dL, P=0.29). However, patients with pSS had a strong trend to present dyslipidemia when compared to healthy individuals (76.7% versus 61.5%, P=0.06). The presence of dyslipidemia in pSS was associated with increased ESR (44.05+/-28.07 versus 28.28+/-18.00, P=0.03), but not with other laboratory markers of the disease and inflammation. DISCUSSION/CONCLUSION pSS patients frequently present abnormal lipid profile, which are associated with higher levels of ESR. Thus, similar to other systemic inflammatory diseases such as rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE), lipid profile should be evaluated in pSS patients, with the aim of initiating specific therapeutic strategy for prevention of cardiovascular events.

Infliximab treatment in pachydermoperiostosis: a rare disease without an effective therapeutic option.

Hypertrophic osteoarthropathy (HOA) is characterized by periostitis of tubular bones, thickened skin, and digital clubbing. Its pathogenesis is unknown but an inflammatory factor and increased bone remodeling have been implicated. It is a very rare disease, usually diagnosed late with few therapeutic options. Bone and joint pains are secondary to periostitis and are usually difficult to control. Tumor necrosis factor-alpha is a cytokine that induces other inflammatory cytokine production, has an osteoclastogenic effect in different rheumatic diseases and probably also has an important role in periostitis and the systemic inflammatory manifestations in HOA. We describe the case of a patient with the primary form of HOA, who had refractory bone pain and arthritis that responded partially to infliximab treatment.

Reinforcing a medical hypothesis with a new question: is there a subgroup of shrinking lungs syndrome that is induced by pleurisy in systemic lupus erythematosus and is this subgroup marked by anti-Ro/SSA?

Shrinking lungs syndrome (SLS) is a rare entity associated with autoimmune diseases and its underlying pathogenesis is still unclear. We describe a series of seven consecutive cases of SLS in systemic lupus erythematosus, all of them with serositis and six (85.7%) with anti-Ro/SSA antibodies. Our findings reinforce the hypothesis that SLS may be, in some cases, a consequence of diaphragmatic restriction due to pleuritic pain, and we suggest anti-Ro/SSA as a marker of this subgroup of SLS.

Síndrome de Behçet: à procura de evidências

Behcets syndrome (BS) is an inflammatory disorder, currently known as a singular model of vasculitis, characterized by recurrent oral and genital ulcers, uveitis and skin lesions. Blindness is frequent, resulting from repeated attacks of uveitis. Genetic susceptibility and environmental factors can be associated with this syndrome. International diagnostic criteria, established in 1990, and the development of disease activity measurement criteria may allow worldwide standardization of clinical trials. This article reviews epidemiological and clinical data, the current hypothesis about causation and pathophysiology and presents a discussion about therapeutic interventions. It includes classic immunossupressive drugs and new biologic agents, and points the need for new evidenced based information about BS treatment.

Behçet’s disease: clinical value of the video capsule endoscopy for small intestine examination

We described a pilot study using Video capsule endoscopy (VCE) aiming the assessment of small bowel in Behçet’s disease (BD). Ten patients with BD and abdominal complaints underwent VCE with Given Pillcam SB (Given Imaging Ltd., Yoqneam). VCE revealed small bowel lesions in all ten subjects. Five patients had active symptoms of BD while five others had clinically inactive disease. Jejune was the most frequently affected GI segment being involved in eight cases, four from each group. We suggest that small bowel involvement may be frequent in BD patients, even in the absence of oral ulcerations.

Adaptação transcultural da versão simplificada (s) do Behçet's Disease Current Activity Form (BDCAF) e comparação do desempenho das versões brasileiras dos dois instrumentos de avaliação da atividade da Doença de Behçet: BR-BDCAF e BR-BDCAF(s)

OBJECTIVE: Behcets Disease Current Activity Form (BDCAF), an instrument for assessing activity of Behcets Disease (BD), was originally produced in English. Its cross-cultural adaptation to Brazilian Portuguese language generated a version called BR-BDCAF. Recently, a simplified model of this protocol (with 12 items) was developed by the International Society for Behcets Disease (ISBD) to generate an index called BDAI (Behcets Disease Activity Index). For this reason, in this work we intended to create the simplified model of the Brazilian version, measure their reliability and evaluate the validity of the results of both instruments, comparing their performances. METHODS: The simplified version of BR-BDCAF was called BR-BDCAF(s). Both protocols were applied in 25 BD patients. Reliability of BR-BDCAF(s) was evaluated through intra- and interobserver agreement according to kappa statistic. Sensitivity (Se), specificity (Sp) and accuracy of both protocols on defining BD active cases were evaluated by comparing the rheumatologist expert clinical judgement to each protocol, and the best cut-off points were established for each instrument by the ROC curve (receive-operator characteristic). RESULTS AND CONCLUSIONS: BR-BDCAF(s) showed good reliability on items relating to mucocutaneous and articular manifestations of BD. The best cut-off points for the definition of active cases were: higher than four with BR-BDCAF (Se = 80.0%, Sp = 86.7%) and higher than one with BR-BDCAF( s) (Se = 70.0%, Sp = 86.7%). Accuracy of both protocols was similar, suggesting that both can be used as tools to assess activity of DB.

Mielopatia torácica por calcificação do ligamento amarelo cursando com hiperproteinorraquia e resposta à corticoterapia: relato de caso

Calcification and ossification of the ligamentum flavum or of the posterior longitudinal ligament are causes of compressive myelopathy, more frequent in the lower thoracic levels, and extremely rare in Western populations. Surgical decompression is the only therapy, but the disease is usually progressive, and its recurrence after surgery is common. Inflammatory mediators might play a role in the progression of compressive myelopathy, but, to our knowledge, the therapeutic approach involving anti-inflammatory agents has never been tried before. We report a case of compressive myelopathy due to calcification of the ligamentum flavum, in which hyperproteinorachia and response to steroid therapy have been observed. Those data have not been published before and might provide new ideas for the disease understanding.

Síndrome de Behçet e policondrite recidivante: síndrome MAGIC

We describe a 30 years old man with typical features of both Behcets syndrome and relapsing polychondritis but neither complete classification criteria. The term MAGIC syndrome (Mouth And Genital ulcers with Inflamed Cartilage) has been used to describe similarly affected patients. We present a brief review of previously reported cases.