Izaias Pereira da Costa

Catastrophic antiphospholipid syndrome during pregnancy and puerperium: maternal and fetal characteristics of 15 cases

Background: The catastrophic variant of the antiphospholipid syndrome (APS) is a life-threatening form of presentation of this syndrome that can be triggered by several factors. Aim: To describe the characteristics of patients who developed catastrophic APS triggered during pregnancy and puerperium. Methods: A review of the first 255 cases collected in the website-based “CAPS Registry” was undertaken. Three new and unpublished cases of catastrophic APS developed during pregnancy and puerperium were added. Results: Fifteen cases were identified. The mean (range) age was 27 (17–38) years. Most patients had a previous unsuccessful obstetric history. In 7 of 14 (50%) cases with available medical history, the catastrophic APS appeared during pregnancy, in 6 (43%) during the puerperium and in 1 (7%) after curettage for a fetal death. The main clinical and serological characteristics were similar to those patients with catastrophic APS triggered by other factors, except for a history of a higher prevalence of previous abortions (p<0.01). Several specific features were found, including the HELLP (haemolysis, elevated liver enzymes, low platelets) syndrome in 8 (53%) patients, placental infarctions in 4 (27%) patients, and pelvic vein thrombosis and myometrium thrombotic microangiopathy in 1 (7%) patient each. Mortality rate was high for the mothers (46%), and for the babies (54%). Conclusions: It is important to consider the possibility of the development of catastrophic APS in those patients with signs of HELLP syndrome and multiorgan failure during pregnancy or puerperium, especially in those patients with previous history of abortions and/or thrombosis.

Gender characterization in a large series of Brazilian patients with spondyloarthritis

An increasing number of women have been diagnosed with spondyloarthritis (SpA) in recent decades. While a few studies have analyzed gender as a prognostic factor of the disease, no studies have addressed this matter with a large number of patients in South America, which is a peculiar region due to its genetic heterogeneity. The aim of the present study was to analyze the influence of gender on disease patterns in a large cohort of Brazilian patients with SpA. A prospective study was carried out involving 1,505 patients [1,090 males (72.4%) and 415 females (27.6%)] classified as SpA according to the European Spondyloarthropaties Study Group criteria who attended at 29 reference centers for rheumatology in Brazil. Clinical and demographic variables were recorded and the following disease indices were administered: Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), Bath Ankylosing Spondylitis Functional Index (BASFI), Bath Ankylosing Spondylitis Radiologic Index (BASRI), Maastricht Ankylosing Spondylitis Enthesitis Score (MASES), and Ankylosing Spondylitis Quality of Life (ASQoL). Ankylosing spondylitis (AS) was the most frequent disease in the group (65.4%), followed by psoriatic arthritis (18.4%), undifferentiated SpA (6.7%), reactive arthritis (3.3%), arthritis associated to inflammatory bowel disease (3.2%), and juvenile SpA (2.9%). The male-to-female ratio was 2.6:1 for the whole group and 3.6:1 for AS. The females were older (p < 0.001) and reported shorter disease duration (p = 0.002) than the male patients. The female gender was positively associated to peripheral SpA (p < 0.001), upper limb arthritis (p < 0.001), dactylitis (p = 0.011), psoriasis (p < 0.001), nail involvement (p < 0.001), and family history of SpA (p = 0.045) and negatively associated to pure axial involvement (p < 0.001), lumbar inflammatory pain (p = 0.042), radiographic sacroiliitis (p < 0.001), and positive HLA-B27 (p = 0.001). The number of painful (p < 0.001) and swollen (p = 0.006) joints was significantly higher in the female gender, who also achieved higher BASDAI (p < 0.001), BASFI (p = 0.073, trend), MASES (p = 0.019), ASQoL (p = 0.014), and patient’s global assessment (p = 0.003) scores, whereas the use of nonsteroidal anti-inflammatory drugs (p < 0.001) and biological agents (p = 0.003) was less frequent in the female gender. Moreover, BASRI values were significantly lower in females (p < 0.001). The female gender comprised one third of SpA patients in this large cohort and exhibited more significant peripheral involvement and less functional disability, despite higher values in disease indices.

Qualidade de vida relacionada à saúde em pacientes com lúpus eritematoso sistêmico no Centro-Oeste do Brasil

INTRODUCTION/OBJECTIVE: Evaluate the quality of life (QOL) of women with systemic lupus erythematosus (SLE) and the association between QOL domains, measured by the World Health Organization Quality of Life Group (WHOQOL-100) assessment instrument, and disease activity. PATIENTS AND METHODS: A cross-sectional analytic study involving 95 patients with SLE, according to the American College of Rheumatology (ACR) criteria, aged 20-49 years, attended at the University Center of Universidade Federal de Mato Grosso do Sul, Campo Grande, MS. We used the following instruments: demographic and clinical data collection form and WHOQOL-100 instrument. Evaluation of disease activity was performed by SLEDAI. On statistical analysis, we applied Students t-test, ANOVA, and Pearsons correlation. RESULTS: Group-case: 79 female with mild to moderate SLE activity (SLEDAI = 1-10) and severe activity (SLEDAI > 11). Control group: 16 female with inactive SLE (SLEDAI = 0). All domains of WHOQOL-100 proved to be affected, with significant difference (P < 0.005) between the case and control groups in which there was a better perception of QOL in the domain Spirituality and worst perception of QOL in the domain Environment. When confronting QOL domains with education, we detected a significant difference (P < 0.05) in the Physical, Psychological, Level of independence, and Environment domains. The comparison between QOL and disease activity was significant (P < 0.005) in three domains: Physical, Psychological, and Environment. CONCLUSION: SLE with intense activity determines a worse QOL condition than inactive SLE. The assessment of QOL enables the knowledge of disease and treatment impact in a contextualized way, which can render more appropriate and more comprehensive interventions.

Leishmaniasis during anti-tumor necrosis factor therapy: Report of 4 cases and review of the literature (additional 28 cases)

OBJECTIVE To describe the development of 4 new cases of leishmaniasis in patients receiving anti-tumor necrosis factor-α (anti-TNF) agents and review the pertinent literature. METHODS Chart review of the 4 cases and MEDLINE search for additional reported cases. RESULTS All reported cases, including ours, came from endemic areas. The infection was detected on an average of 23.5 months after the initiation of anti-TNF therapy. The majority of cases had the classical clinical presentation. The biological therapy was suspended in 21 cases. The results were successful for leishmaniasis therapy in all cases. In 10 cases it was possible to reintroduce anti-TNF agents. On follow-up it was observed that there was an infection relapse in 3 cases. CONCLUSIONS The present study shows that leishmaniasis, in its several clinical forms, should be included in the differential diagnosis of possible infections involving patients under use of aTNF therapy. Endemic disease under geographic expansion, easy international displacement and intense human migratory flows certainly represents a risk of this infection in an increasing universe of people which includes the immunosuppressed patients. Cutaneous lesions, prolonged fever, splenomegaly, and pancytopenias, the main clinical-laboratory findings of leishmaniasis, can also be present in autoimmune rheumatic disease, thus leading to delayed diagnosis and treatment of the parasitic disease. The diagnosis depends basically on a high suspicion index, being confirmed with the identification of the protozoan. The classic treatment of the infection when instituted is associated with complete recovery. It is important to point out that all cases reported so far had either originated from or been recently in regions regarded as endemic of leishmaniasis.

Ethnic Influence in Clinical and Functional Measures of Brazilian Patients with Spondyloarthritis

Objective. Spondyloarthritides (SpA) can present different disease spectra according to ethnic background. The Brazilian Registry of Spondyloarthritis (RBE) is a nationwide registry that comprises a large databank on clinical, functional, and treatment data on Brazilian patients with SpA. The aim of our study was to analyze the influence of ethnic background in SpA disease patterns in a large series of Brazilian patients. Methods. A common protocol of investigation was prospectively applied to 1318 SpA patients in 29 centers distributed through the main geographical regions in Brazil. The group comprised whites (65%), African Brazilians (31.3%), and people of mixed origins (3.7%). Clinical and demographic variables and various disease index scores were compiled. Ankylosing spondylitis (AS) was the most frequent disease in the group (65.1%); others were psoriatic arthritis (18.3%), undifferentiated SpA (6.8%), enteropathic arthritis (3.7%), and reactive arthritis (3.4%). Results. White patients were significantly associated with psoriasis (p = 0.002), positive HLA-B27 (p = 0.014), and use of corticosteroids (p < 0.0001). Hip involvement (p = 0.02), axial inflammatory pain (p = 0.04), and radiographic sacroiliitis (p = 0.025) were associated with African Brazilian descent. Sex distribution, family history, and presence of peripheral arthritis, uveitis, dactylitis, urethritis, and inflammatory bowel disease were similar in the 3 groups, as well as age at disease onset, time from first symptom until diagnosis, and use of anti-tumor necrosis factor-α agents (p > 0.05). Schober test and thoracic expansion were similar in the 3 groups, whereas African Brazilians had higher Maastricht Ankylosing Spondylitis Enthesitis Scores (p = 0.005) and decreased lateral lumbar flexion (p = 0.003), while whites had a higher occiput-to-wall distance (p = 0.02). African Brazilians reported a worse patient global assessment of disease (p = 0.011). Other index scores and prevalence of work incapacity were similar in the 3 groups, although African Brazilians had worse performance in the Ankylosing Spondylitis Quality of Life questionnaire (p < 0.001). Conclusion. Ethnic background is associated with distinct clinical aspects of SpA in Brazilian patients. African Brazilian patients with SpA have a poorer quality of life and report worse disease compared to whites.

Neurological manifestations in Baggio-Yoshinari Syndrome (Brazilian Lyme disease-like syndrome)

INTRODUCAO: A doenca de Lyme (DL) e uma doenca de picada de carrapato, causado pela espiroqueta Borrelia burgdorferi sensu lato, transmitida por carrapatos do complexo Ixodes ricinus, que promove multiplas manifestacoes clinicas sistemicas. No Brasil, uma sindrome diferente e descrita e mimetiza sintomas de DL, mas tambem se manifesta com alta frequencia de episodios recorrentes e manifestacoes alergicas e imunologicas. E transmitida pelo carrapato Amblyomma cajennense e o agente etiologico e uma espiroqueta nao cultivavel de forma atipica. Devido a essas particularidades, esta zoonose emergente tem sido denominada sindrome brasileira semelhante a doenca de Lyme ou sindrome de Baggio-Yoshinari (SBY). OBJETIVO: Descrever o espectro da manifestacao neurologica da SBY. PACIENTES: Foram analisados 30 pacientes com SBY e sintomas neurologicos. RESULTADOS: A media de idade dos pacientes foi de 34,2 ± 13,3 anos (6 a 63 anos); 20 eram mulheres e 10 homens. Um alto numero de episodios recorrentes (73,6%) e disturbios psiquiatricos e psicossociais graves (20%) foram caracteristicas tipicas. Eritema migrans similar ao visto em hemisferio norte foi identificado em 43,3% dos pacientes no inicio da doenca. A recorrencia das lesoes cutâneas diminuiu com a progressao da doenca. Sintomas articulares (artrite) aconteceram em aproximadamente metade dos pacientes com SBY no inicio e durante o episodio de recidiva. CONCLUSOES: A SBY e considerada uma nova doenca transmitida por carrapato no Brasil que difere da classica DL observada no hemisferio norte. A SBY reproduz sintomas neurologicos observados na DL, exceto pela presenca adicional de recorrencia de episodios e uma tendencia de causar manifestacoes neurologicas cronicas e articulares.

Glucocorticoid-induced tumor necrosis factor receptor expression in patients with cervical human papillomavirus infection

INTRODUCTION The progression of human papillomavirus (HPV) infection in the anogenital tract has been associated with the involvement of cells with regulatory properties. Evidence has shown that glucocorticoid-induced tumor necrosis factor receptor (GITR) is an important surface molecule for the characterization of these cells and proposes that GITR ligand may constitute a rational treatment for many cancer types. We aimed to detect the presence of GITR and CD25 in cervical stroma cells with and without pathological changes or HPV infection to better understand the immune response in the infected tissue microenvironment. METHODS We subjected 49 paraffin-embedded cervical tissue samples to HPV DNA detection and histopathological analysis, and subsequently immunohistochemistry to detect GITR and CD25 in lymphocytes. RESULTS We observed that 76.9% of all samples with high GITR expression were HPV-positive regardless of histopathological findings. High GITR expression (77.8%) was predominant in samples with ≥ 1,000 RLU/PCB. Of the HPV-positive samples negative for intraepithelial lesion and malignancy, 62.5% had high GITR expression. High GITR expression was observed in both carcinoma and high-grade squamous intraepithelial lesion (HSIL) samples (p = 0.16). CD25 was present in great quantities in all samples. CONCLUSIONS The predominance of high GITR expression in samples with high viral load that were classified as HSIL and carcinoma suggests that GITR+ cells can exhibit regulatory properties and may contribute to the progression of HPV-induced cervical neoplasia, emphasizing the importance of GITR as a potential target for immune therapy of cervical cancer and as a disease evolution biomarker.

Pesquisa de anticorpos anti-Borrelia e anti-Babesia em soro de crianças com manifestações clínicas e epidemiologia compatíveis com a doença de Lyme-Simile no Estado de Mato Grosso do Sul

A ocorrencia de manifestacoes clinicas e laboratoriais semelhantes as encontradas na doenca de Lyme e da coinfeccao com a babesiose ja foi demonstrada em trabalhos anteriores em adultos, porem nao existem estudos desta natureza em criancas. OBJETIVO: Caracterizar o perfil clinico-epidemiologico da sindrome de Lyme-Simile em criancas do Estado de Mato Grosso do Sul e avaliar a prevalencia dos anticorpos anti-Borrelia burgdorferi e anti-Babesia bovis no soro de pacientes que preencheram os criterios estabelecidos. METODOS: 100 pacientes entre 9 meses e 16 anos de idade foram submetidos a pesquisa dos anticorpos pela tecnica de ELISA e a soroprevalencia foi comparada com um grupo-controle. RESULTADOS: Positividade para anticorpos anti-B. burgdorferi ocorreu em 27% dos pacientes com suspeita clinica, sendo 17% IgM e 12% IgG. As manifestacoes articulares ocorreram em 21 pacientes, as manifestacoes cutâneas em tres pacientes e as manifestacoes neurologicas em tres pacientes. A prevalencia de anticorpos contra B. burgdorferi nos pacientes do grupo-controle foi de 15% (p < 0,05). Os anticorpos anti-B. bovis estiveram presentes em 24% dos pacientes suspeitos e em 3% do grupo-controle (p < 0,05). Concomitância dos anticorpos ocorreu em 10 pacientes com suspeita clinica e em nenhum controle. CONCLUSAO: Os resultados deste estudo evidenciam a etiologia de carater infeccioso e reacional das manifestacoes clinicas encontradas nos pacientes, podendo caracterizar uma sindrome, que possivelmente ocorra na presenca de multiplos microrganismos e esta relacionada com carrapatos. Foi encontrada frequencia superior de anticorpos contra Borrelia e Babesia nos pacientes em relacao aos controles. Embora pequena, essa diferenca foi estatisticamente significante. Este achado nao atesta que o quadro clinico dos pacientes seja causado por especies de Borrelia ou Babesia, mas levantam a possibilidade de participacao de algum agente infeccioso que possa estar relacionado a esses microrganismos. Os estudos devem prosseguir para melhorar a delineacao desta sindrome em nosso meio, assim como isolar o agente etiologico.

Artrite enteropática no Brasil: dados do registro brasileiro de espondiloartrites

UNLABELLED Inflammatory bowel diseases (Crohns disease and ulcerative rectocolitis) have extraintestinal manifestations 25% of the patients, with the most common one being the enteropathic arthritis. METHODS Prospective, observational, multicenter study with patients from 29 reference centers participating in the Brazilian Registry of Spondyloarthritis (RBE), which incorporates the RESPONDIA (Ibero-American Registry of Spondyloarthritis) group. Demographic and clinical data were collected from 1472 patients and standardized questionnaires for the assessment of axial mobility, quality of life, enthesitic involvement, disease activity and functional capacity were applied. Laboratory and radiographic examinations were performed. The aim of this study is to compare the clinical, epidemiological, genetic, imaging, treatment and prognosis characteristics of patients with enteropathic arthritis with other types of spondyloarthritis in a large Brazilian cohort. RESULTS A total of 3.2% of patients were classified as having enteroarthritis, 2.5% had spondylitis and 0.7%, arthritis (peripheral predominance). The subgroup of individuals with enteroarthritis had a higher prevalence in women (P < 0.001), lower incidence of inflammatory axial pain (P < 0.001) and enthesitis (P = 0.004). HLA-B27 was less frequent in the group with enteroarthritis (P = 0.001), even when considering only those with the pure axial form. There was a lower prevalence of radiographic sacroiliitis (P = 0.009) and lower radiographic score (BASRI) (P = 0.006) when compared to patients with other types of spondyloarthritis. They also used more corticosteroids (P < 0.001) and sulfasalazine (P < 0.001) and less non-steroidal anti-inflammatory drugs (P < 0.001) and methotrexate (P = 0.001). CONCLUSION There were differences between patients with enteroarthritis and other types of spondyloarthritis, especially higher prevalence of females, lower frequency of HLA-B27, associated with less severe axial involvement.

Recommendations of the Brazilian Society of Rheumatology for the diagnosis and treatment of chikungunya fever. Part 2 - Treatment

Chikungunya fever has become an important public health problem in countries where epidemics occur because half of the cases progress to chronic, persistent and debilitating arthritis. Literature data on specific therapies at the various phases of arthropathy caused by chikungunya virus (CHIKV) infection are limited, lacking quality randomized trials assessing the efficacies of different therapies. There are a few studies on the treatment of musculoskeletal manifestations of chikungunya fever, but these studies have important methodological limitations. The data currently available preclude conclusions favorable or contrary to specific therapies, or an adequate comparison between the different drugs used. The objective of this study was to develop recommendations for the treatment of chikungunya fever in Brazil. A literature review was performed via evidence-based selection of articles in the databases Medline, SciELO, PubMed and Embase and conference proceedings abstracts, in addition to expert opinions to support decision-making in defining recommendations. The Delphi method was used to define the degrees of agreement in 2 face-to-face meetings and several online voting rounds. This study is part 2 of the Recommendations of the Brazilian Society of Rheumatology (Sociedade Brasileira de Reumatologia - SBR) for the Diagnosis and Treatment of chikungunya fever and specifically addresses treatment.