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Abstract Objectives To compare the impact of COVID-19 on the clinical status and psychological distress of patients with immune-mediated rheumatic disease (IMRD) caused by SARS-CoV-2 infection with that of noninfected IMRD controls during a 6-month follow-up period. Methods The ReumaCoV Brazil is a longitudinal study designed to follow IMRD patients for 6 months after COVID-19 (patients) compared with IMRD patients without COVID-19 (controls). Clinical data, disease activity measurements and current treatments regarding IMRD and COVID-19 outcomes were evaluated in all patients. Disease activity was assessed through validated tools at inclusion and at 3 and 6 months post-COVID-19. Fatigue, using FACIT-F (Functional Assessment of Chronic Illness Therapy) and psychological distress, using DASS 21 (Depression, Anxiety and Stress Scale − 21 Items), used to evaluated psychological distress, were evaluated at 6 months after COVID-19 in both groups. The significance level was set as p

Abstract Background Patients with immune-mediated rheumatic diseases (IMRDs) have been prioritized for COVID-19 vaccination to mitigate the infection severity risks. Patients with rheumatoid arthritis (RA) are at a high risk of severe COVID-19 outcomes, especially those under immunosuppression or with associated comorbidities. However, few studies have assessed the safety of the COVID-19 vaccine in patients with RA. Objective To evaluate the safety of vaccines against SARS-CoV-2 in patients with RA. Methods This data are from the study “Safety and Efficacy on COVID-19 Vaccine in Rheumatic Diseases,” a Brazilian multicentric prospective phase IV study to evaluate COVID-19 vaccine in IMRDs in Brazil. Adverse events (AEs) in patients with RA of all centers were assessed after two doses of ChAdOx1 (Oxford/AstraZeneca) or CoronaVac (Sinovac/Butantan). Stratification of postvaccination AEs was performed using a diary, filled out daily and returned at the end of 28 days for each dose. Results A total of 188 patients with RA were include, 90% female. CoronaVac was used in 109 patients and ChAdOx1 in 79. Only mild AEs were observed, mainly after the first dose. The most common AEs after the first dose were pain at the injection (46,7%), headache (39,4%), arthralgia (39,4%), myalgia (30,5%) and fatigue (26,6%), and ChAdOx1 had a higher frequency of pain at the injection (66% vs 32 %, p

Abstract Background There is a remarkable variability in the frequency of HLA-B27 positivity in patients with spondyloarthritis (SpA), which may be associated with different clinical presentations worldwide. However, there is a lack of data considering ethnicity and sex on the evaluation of the main clinical and prognostic outcomes in mixed-race populations. The aim of this study was to evaluate the frequency of HLA-B27 and its correlation with disease parameters in a large population of patients from the Brazilian Registry of Spondyloarthritis (RBE). Methods The RBE is a multicenter, observational, prospective cohort that enrolled patients with SpA from 46 centers representing all five geographic regions of Brazil. The inclusion criteria were as follow: (1) diagnosis of axSpA by an expert rheumatologist; (2) age ≥18 years; (3) classification according to ASAS axial. The following data were collected via a standardized protocol: demographic data, disease parameters and treatment historical. Results A total of 1096 patients were included, with 73.4% HLA-B27 positivity and a mean age of 44.4 (±13.2) years. Positive HLA-B27 was significantly associated with male sex, earlier age at disease onset and diagnosis, uveitis, and family history of SpA. Conversely, negative HLA-B27 was associated with psoriasis, higher peripheral involvement and disease activity, worse quality of life and mobility. Conclusions Our data showed that HLA-B27 positivity was associated with a classic axSpA pattern quite similar to that of Caucasian axSpA patients around the world. Furthermore, its absence was associated with peripheral manifestations and worse outcomes, suggesting a relevant phenotypic difference in a highly miscegenated population.

Objective: To evaluate the humoral response to and impact of SARS-CoV-2 vaccination in patients with systemic lupus erythematosus in a multicenter cohort design. Methods: Data for this analysis were obtained from the Study of Safety, Effectiveness and Duration of Immunity after Vaccination against SARS-CoV-2 in Patients with Immune-Mediated Inflammatory Diseases (SAFER), a prospective, multicenter, phase IV, real-world study conducted across different regions of Brazil from June/2021 to March/2024. Patients aged >18 years with systemic lupus erythematosus (SLE) who received any one of the SARS-CoV-2 vaccines approved by the Brazilian health regulatory agency (CoronaVac [inactivated SARS-CoV-2 vaccine], ChAdOx-1 [AstraZeneca], or BNT162b2 [Pfizer-BioNTech]) were included. Immunogenicity was assessed in pre- and post-vaccination blood samples, and patients were monitored in person and remotely for the occurrence and severity of COVID-19. Results: Two hundred and thirty-five patients with SLE who had completed their vaccination schedules (two doses + booster dose) were included in this study. Most patients were female (89.3%) and had low disease activity or were in remission (72.4%); the majority were also on some form of immunosuppressive therapy (58.1%). One hundred and sixteen patients received two doses of CoronaVac followed by one dose of BNT162b2 (Pfizer-BioNTech) vaccine, eighty-seven received two doses of ChAdOx1-S (AstraZeneca) followed by one dose of BNT162b2 (Pfizer-BioNTech) vaccine, and thirty-two received three doses of BNT162b2 (Pfizer-BioNTech) vaccine. Twenty-eight cases of COVID-19, none meeting criteria for severe COVID-19, were recorded in patients with respiratory symptoms after the second dose of a SARS-CoV-2 vaccine. Regarding immunogenicity, an increase in seroconversion rate was observed following consecutive vaccine doses, with no difference between vaccination schedules, reaching 97.57% seropositivity after a booster dose. The geometric mean IgG titers differed between the different vaccination schedules after the first and the second vaccine dose, being lowest for the CoronaVac-based schedule, but titers were similar after the administration of a booster dose. Conclusion: In patients with SLE, SARS-CoV-2 vaccines are immunogenic, inducing a robust humoral response. No severe outcomes associated with death or hospitalization were found in the evaluated patient sample. Complete vaccination schedules including a booster dose induced higher humoral responses than incomplete schedules, especially in patients initially immunized with an inactivated virus vaccine schedule and those with a suboptimal humoral response.

Background/Objectives: The effectiveness of COVID-19 vaccine in patients with immune-mediated inflammatory diseases (IMID) depends on the underlying disease, immunosuppression degree and the vaccine regimens. We evaluate the safety and immunogenicity of different COVID-19 vaccine schedules. Methods: The SAFER study: “Safety and effectiveness of the COVID-19 Vaccine in Rheumatic Disease”, is a Brazilian multicentric prospective observational phase IV study in the real-life. Data were analyzed after 2 or 3 doses of COVID-19 vaccines: adenoviral vectored vaccine (ChAdOx1 nCoV-19, Astrazeneca), mRNA vaccine (BNT162b2, Pfizer–BioNTech) or inactivated SARS-COV-2 vaccine (CoronaVac, Sinovac Biotech). IgG antibody against SARS-CoV-2 spike (IgG-S) receptor-binding domain level were quantified at baseline (T1) and 28 days after the first (T2), 2nd (T3) and 3rd (T4) doses by chemiluminescence (SARS-CoV-2-IgG-II Quant-assay, Abbott-Laboratories). Results: 721 patients with IMID were included in the analysis. The median titers of IgG-S (BAU/mL) increased progressively over the times: at baseline was 6.26 (5.41–7.24), T2: 73.01 (61.53–86.62), T3: 200.0 (174.36–229.41) and T4: 904.92 (800.49–1022.97). The multivariate linear regression showed that greater IgG-S titers were associated with pre-exposure to COVID-19 (p < 0.001) and BNT162b2 booster vaccine (p < 0.001). Rituximab and immunosuppressant drugs were independent factors for low titers (p = 0.002, p < 0.001, respectively). No serious adverse event was reported. Conclusions: All platforms were safe and induced an increase in IgG-S antibodies. COVID-19 pre-exposure and BNT162b2 booster regimens were predictors of higher humoral immune responses, which is relevant in immunosuppressed populations. Immunosuppressants (mainly rituximab) predicted the lowest antibodies.

Breakthrough COVID-19 (occurring in fully vaccinated people) has been described. Data on its characteristics among immune-mediated rheumatic disease (IMRD) patients are scarce. This study describes breakthrough COVID-19 occurring in IMRD patients participating in the SAFER-study, a Brazilian multicentric cohort evaluating the safety, effectiveness, and immunogenicity of SARS-CoV-2 vaccines in patients with autoimmune diseases. A descriptive analysis of the population and a binary logistic regression model were performed to evaluate the predictors of COVID-19-related hospitalization. A p-value < 0.05 was significant. The included 160 patients were predominantly females (83.1%), with a mean (SD) age of 40.23 (13.19) years. The patients received two (19%), three (70%), or four (11%) vaccine doses. The initial two-dose series was mainly with ChAdOx1 (Oxford/AstraZeneca) (58%) or BBIBP-CorV (Sinopharm-Beijing) (34%). The first booster (n = 150) was with BNT162b2 (BioNtech/Fosun Pharma/Pfizer) (63%) or ChAdOx1 (29%). The second booster (n = 112) was with BNT162b2 (40%) or ChAdOx1 (26%). The COVID-19 hospitalization rate was 17.5%. IMRD moderate/high activity (OR: 5.84; CI: 1.9–18.5; p = 0.002) and treatment with corticosteroids (OR: 2.94; CI: 1.02–8.49; p = 0.0043) were associated with higher odds of hospitalization, while increasing the number of vaccine doses was protective (OR: 0.37; CI: 0.15–0.9; p = 0.032). These findings, along with previous reassuring results about the safety of the COVID-19 vaccines, argue in favor of booster vaccination in IMRD patients.

Abstract Objectives To evaluate the disease activity before and after COVID-19 and risk factors associated with outcomes, including hospitalization, intensive care unit (ICU) admission, mechanical ventilation (MV) and death in patients with spondylarthritis (SpA). Methods ReumaCoV Brazil is a multicenter prospective cohort of immune-mediated rheumatic diseases (IMRD) patients with COVID-19 (case group), compared to a control group of IMRD patients without COVID-19. SpA patients enrolled were grouped as axial SpA (axSpA), psoriatic arthritis (PsA) and enteropathic arthritis, according to usual classification criteria. Results 353 SpA patients were included, of whom 229 (64.9%) were axSpA, 118 (33.4%) PsA and 6 enteropathic arthritis (1.7%). No significant difference was observed in disease activity before the study inclusion comparing cases and controls, as well no worsening of disease activity after COVID-19. The risk factors associated with hospitalization were age over 60 years (OR = 3.71; 95% CI 1.62–8.47, p = 0.001); one or more comorbidities (OR = 2.28; 95% CI 1.02–5.08, p = 0.001) and leflunomide treatment (OR = 4.46; 95% CI 1.33–24.9, p = 0.008). Not having comorbidities (OR = 0.11; 95% CI 0.02–0.50, p = 0.001) played a protective role for hospitalization. In multivariate analysis, leflunomide treatment (OR = 8.69; CI = 95% 1.41–53.64; p = 0.023) was associated with hospitalization; teleconsultation (OR = 0.14; CI = 95% 0.03–0.71; p = 0.01) and no comorbidities (OR = 0.14; CI = 95% 0.02–0.76; p = 0.02) remained at final model as protective factor. Conclusions Our results showed no association between pre-COVID disease activity or that SARS-CoV-2 infection could trigger disease activity in patients with SpA. Teleconsultation and no comorbidities were associated with a lower hospitalization risk. Leflunomide remained significantly associated with higher risk of hospitalization after multiple adjustments.

Abstract Sjogren's syndrome (SS) is an autoimmune disease characterized by lymphocytic infiltration of the exocrine glands and other organs, associated with sicca syndrome but also with systemic involvement with varying degrees of severity. Despite their importance, some systemic manifestations, mainly liver, gastrointestinal, and pancreatic are not routinely evaluated. To address these manifestations, the Sjögren's Syndrome Committee of the Brazilian Society of Rheumatology conducted a broad systematic review of the literature on studies investigating prevalence and diagnosis of these symptoms in Sjogren´s patients and made recommendations based on the findings. Agreement between the experts was achieved using the Delphi method. This is the second part of this guideline, providing 6 recommendations for liver, gastrointestinal, and pancreatic care of SS patients.

Objective Some patients with rheumatic diseases might be at higher risk for coronavirus disease 2019 (COVID‐19) acute respiratory distress syndrome (ARDS). We aimed to develop a prediction model for COVID‐19 ARDS in this population and to create a simple risk score calculator for use in clinical settings. Methods Data were derived from the COVID‐19 Global Rheumatology Alliance Registry from March 24, 2020, to May 12, 2021. Seven machine learning classifiers were trained on ARDS outcomes using 83 variables obtained at COVID‐19 diagnosis. Predictive performance was assessed in a US test set and was validated in patients from four countries with independent registries using area under the curve (AUC), accuracy, sensitivity, and specificity. A simple risk score calculator was developed using a regression model incorporating the most influential predictors from the best performing classifier. Results The study included 8633 patients from 74 countries, of whom 523 (6%) had ARDS. Gradient boosting had the highest mean AUC (0.78; 95% confidence interval [CI]: 0.67‐0.88) and was considered the top performing classifier. Ten predictors were identified as key risk factors and were included in a regression model. The regression model that predicted ARDS with 71% (95% CI: 61%‐83%) sensitivity in the test set, and with sensitivities ranging from 61% to 80% in countries with independent registries, was used to develop the risk score calculator. Conclusion We were able to predict ARDS with good sensitivity using information readily available at COVID‐19 diagnosis. The proposed risk score calculator has the potential to guide risk stratification for treatments, such as monoclonal antibodies, that have potential to reduce COVID‐19 disease progression.

Leprosy is an infectious and contagious disease of slow evolution, triggered by Mycobacterium leprae. Arthritis is its third most common manifestation, after cutaneous and peripheral nerve involvement. Since musculoskeletal symptoms may be the initial presentation of the disease, it is important for health professionals to recognize its rheumatic manifestations for early diagnosis and appropriate treatment, especially in endemic areas. In addition, cases of leprosy have increased globally, notably in patients undergoing treatment with TNF-α blockers and due to the increase in migration and travel of people from developing countries to developed countries. This review proposes to discuss the main scenarios of mimicry of different rheumatic diseases by leprosy, as well as the role of immunosuppressive drugs used in rheumatology practice in the treatment of reactional states and in the risk of developing the infection.

Abstract Sjogren's Syndrome (SS) is an autoimmune disease characterized by lymphocytic infiltration of the exocrine glands and other organs, associated with sicca syndrome but also with systemic involvement with varying degrees of severity. Despite their importance, these systemic manifestations are not routinely evaluated and there is no homogenous approach to their diagnosis or evaluation. To close this gap, a panel of experts from the Brazilian Society of Rheumatology conducted a systematic review and meta-analysis on the identification of epidemiologic and clinical features of these manifestations and made recommendations based on the findings. Agreement between the experts was achieved using the Delphi method. The first part of this guideline summarizes the most important topics, and 11 recommendations are provided for the articular, pulmonary, and renal care of SS patients.

Abstract Background Patients using immunosuppressive drugs may have unfavorable results after infections. However, there is a lack of information regarding COVID-19 in these patients, especially in patients with rheumatoid arthritis (RA). Therefore, the aim of this study was to evaluate the risk factors associated with COVID-19 hospitalizations in patients with RA. Methods This multicenter, prospective cohort study is within the ReumaCoV Brazil registry and included 489 patients with RA. In this context, 269 patients who tested positive for COVID-19 were compared to 220 patients who tested negative for COVID-19 (control group). All patient data were collected from the Research Electronic Data Capture database. Results The participants were predominantly female (90.6%) with a mean age of 53 ± 12 years. Of the patients with COVID-19, 54 (20.1%) required hospitalization. After multiple adjustments, the final regression model showed that heart disease (OR = 4.61, 95% CI 1.06–20.02. P

Abstract Objective To provide guidelines on the coronavirus disease 2019 (COVID-19) vaccination in patients with immune-mediated rheumatic diseases (IMRD) to rheumatologists considering specific scenarios of the daily practice based on the shared-making decision (SMD) process. Methods A task force was constituted by 24 rheumatologists (panel members), with clinical and research expertise in immunizations and infectious diseases in immunocompromised patients, endorsed by the Brazilian Society of Rheumatology (BSR), to develop guidelines for COVID-19 vaccination in patients with IMRD. A consensus was built through the Delphi method and involved four rounds of anonymous voting, where five options were used to determine the level of agreement (LOA), based on the Likert Scale: (1) strongly disagree; (2) disagree, (3) neither agree nor disagree (neutral); (4) agree; and (5) strongly agree. Nineteen questions were addressed and discussed via teleconference to formulate the answers. In order to identify the relevant data on COVID-19 vaccines, a search with standardized descriptors and synonyms was performed on September 10th, 2021, of the MEDLINE, EMBASE, Cochrane Central Register of Controlled Trials, ClinicalTrials.gov, and LILACS to identify studies of interest. We used the Newcastle–Ottawa Scale to assess the quality of nonrandomized studies. Results All the nineteen questions-answers (Q&A) were approved by the BSR Task Force with more than 80% of panelists voting options 4—agree—and 5—strongly agree—, and a consensus was reached. These Guidelines were focused in SMD on the most appropriate timing for IMRD patients to get vaccinated to reach the adequate covid-19 vaccination response. Conclusion These guidelines were developed by a BSR Task Force with a high LOA among panelists, based on the literature review of published studies and expert opinion for COVID-19 vaccination in IMRD patients. Noteworthy, in the pandemic period, up to the time of the review and the consensus process for this document, high-quality evidence was scarce. Thus, it is not a substitute for clinical judgment.

O diabetes mellitus tipo 2 (DM2) é uma doença crônica de alta prevalência no mundo, a qual é causadora de importante morbimortalidade devido às suas complicações sistêmicas. Dentre elas, inclui-se as manifestações musculoesqueléticas caracterizadas pelo comprometimento ósseo e de tecido mole periarticular. Relata-se o caso de paciente do sexo feminino de 48 anos com DM2 e hipertensa há 12 anos, a mesma foi encaminhada por meio de um projeto de pesquisa ao serviço de reumatologia. No atendimento médico, relata um quadro de dor crônica com início em 2015, principalmente da mão esquerda com parestesia em punho. Além disso, relata que o dedo anelar da mesma mão se torna rígido necessitando deslocá-lo para voltar a sua posição normal. Pelos achados de exame físico e de imagem constatou-se um diagnóstico de tendinite de Quervain, dedo em gatilho e síndrome do túnel do carpo. Espera-se com esse relato conscientizar outros profissionais da real importância do tema. Torna-se imprescindível a exploração de queixas musculoesqueléticas objetivando um diagnóstico precoce, evitando um comprometimento na qualidade de vida de pacientes semelhantes a esse.

Abstract Background Primary Sjögren’s syndrome (pSS) is a systemic immune-mediated disease whose main characteristic is exocrine gland inflammation and, subsequent reduction in tear and saliva production. A delayed diagnosis is common due to the nonspecific clinical manifestations of disease. The aim of the present study was to develop recommendations for the diagnosis of glandular manifestations of pSS based on evidence and expert opinion. Main body of the abstract We conducted a systematic literature review to retrieve the best evidence available on the accuracy of diagnostic tests for pSS. We also held two in-person meetings with experts (rheumatologists, pathologists, ophthalmologists and dentists) to establish their level of agreement using the Delphi method. Ultimately, we generated 18 recommendations that aim to facilitate the diagnosis of the glandular manifestations of pSS. Conclusion The diagnosis of glandular manifestations of pSS is complex and multidisciplinary. It requires specific knowledge in the field of ophthalmology, immunology, pathology and imaging, making it compulsory for the rheumatologist to work with professionals from these different areas in order to improve accuracy and early diagnosis. Glandular dysfunction tests, ANA, RF, Anti-Ro, protein electrophoresis, urinalysis, blood count, C-Reactive protein, complement, testing for syphilis and viruses (HCV, HIV) and SGUS should be investigated when dryness or systemic manifestation are present. Minor salivary gland biopsy is recommended for all anti-Ro negative or incomplete criteria cases.

Abstract Introduction Antiphospholipid antibodies (aPL) are described in individuals with leprosy without the clinical features of antiphospholipid antibody syndrome (APS), a condition involving thromboembolic phenomena. We have described the persistence of these antibodies for over 5 years in patients with leprosy after specific treatment. Objectives To determine whether epidemiological, clinical and immunological factors played a role in the long-term persistence of aPL antibodies in leprosy patients after multidrug therapy (MDT) had finished. Methods The study sample consisted of 38 patients with a diagnosis of leprosy being followed up at the Dermatology and Venereology Outpatient Department at the Alfredo da Matta Foundation (FUAM) in Manaus, AM. ELISA was used to detect anticardiolipin (aCL) and anti-β2 glycoprotein I (anti-β2GPI) antibodies. Patients were reassessed on average of 5 years after specific treatment for the disease (MDT) had been completed. Results Persistence of aPL antibodies among the 38 leprosy patients was 84% (32/38), and all had the IgM isotype. Mean age was 48.1 ± 15.9 years, and 23 (72.0%) were male. The lepromatous form (LL) of leprosy was the most common (n = 16, 50%). Reactional episodes were observed in three patients (9.4%). Eighteen (47.37%) were still taking medication (prednisone and/or thalidomide). Mean IgM levels were 64 U/mL for aCL and 62 U/mL for anti-β2GPI. In the multivariate binary logistic regression the following variables showed a significant association: age (p = 0.045, OR = 0.91 and CI 95% 0.82–0.98), LL clinical presention (p = 0.034; OR = 0.02 and CI 95% = 0.0–0.76) and bacterial index (p = 0.044; OR = 2.74 and CI 95% = 1.03–7.33). We did not find association between prednisone or thalidomide doses and positivity for aPL (p = 0.504 and p = 0.670, respectively). No differences in the variables vascular thrombosis, pregnancy morbidity, diabetes, smoking and alcoholism were found between aPL-positive and aPL-negative patients. Conclusion Persistence of positivity for aPL antibodies was influenced by age, clinical presentation and bacterial index. However, further studies are needed to elucidate the reason for this persistence, the role played by aPL antibodies in the disease and the B cell lineages responsible for generation of these antibodies.

Abstract Background The presence of enthesitis is associated with higher disease activity, more disability and incapacity to work and a poorer quality of life in spondyloarthritis (SpA). There is currently no consensus on which clinical score should be used to assess enthesitis in SpA. The objective of the present work was to compare the correlation of three enthesitis indices (MASES, SPARCC and LEI) with measures of disease activity and function in a heterogeneous population of patients with axial and peripheral SpA. Methods A cross-sectional study was conducted in three Brazilian public university hospitals; patients fulfilling ASAS classification criteria for peripheral or axial SpA were recruited and measures of disease activity and function were collected and correlated to three enthesitis indices: MASES, SPARCC and LEI using Spearman’s Correlation index. ROC curves were used to determine if the the enthesitis indices were useful to discriminate patients with active disease from those with inactive disease. Results Two hundred four patients were included, 71.1% (N = 145) fulfilled ASAS criteria for axial SpA and 28.9% (N = 59) for peripheral SpA. In axial SpA, MASES performed better than LEI (p = 0.018) and equal to SPARCC (p = 0.212) regarding correlation with disease activity (BASDAI) and function (BASFI). In peripheral SpA, only MASES had a weak but statistical significant correlation with DAS28-ESR (rs 0.310 p = 0.05) and MASES had better correlation with functional measures (HAQ) than SPARCC (p = 0.034). Conclusion In this sample composed of SpA patients with high coexistence of axial and peripheral features, MASES showed statistical significant correlation with measures of disease activity and function in both axial and peripheral SpA.

Abstract Hepatitis B infection is a global health issue. When considering patients with rheumatic diseases, this is no different. By using immunosuppressant drugs, such as DMARDs and biologics, viral reactivation is possible, leading to serious consequences on the patient. We report 3 cases of association between ankylosing spondylitis and hepatitis B with the use of immunosuppressant drugs. Case 1 was a patient with previous HBV infection using DMARD. Cases 2 and 3 were patients chronically infected by HBV during immunosuppressant therapy. The management of HBV infection during immunosuppressant therapy is challenging and needs multidisciplinary support.

RESUMOAs recomendações propostas pela Comissão de Síndrome de Sjögren da Sociedade Brasileira de Reumatologia para tratamento da síndrome de Sjögren foram baseadas em uma revisão sistemática da literatura nas bases de dados Medline (PubMed) e Cochrane até outubro de 2014 e opinião de especialistas na ausência de artigos sobre o assunto. Foram incluídos 131 artigos classificados de acordo com Oxford & Grade. Essas recomendações foram elaboradas com o objetivo de orientar o manejo adequado e facilitar o acesso aos tratamentos para aqueles pacientes com adequada indicação de recebê-los, considerando o contexto socioeconômico brasileiro e os medicamentos disponíveis no país.

A silicose é a pneumoconiose mais frequente, decorrente da inalação de sílica ou de poeiras minerais contendo silicatos, caracterizada principalmente pela fibrose pulmonar de caráter irreversível. Está associada com o desenvolvimento de outras doenças, incluindo tuberculose pulmonar, câncer pulmonar e doenças autoimunes. A doença do tecido conjuntivo posterior à exposição à sílica ocorre geralmente após 15 anos do início da exposição. A síndrome de Erasmus refere-se ao desenvolvimento de esclerose sistêmica em indivíduos previamente expostos à sílica e que apresentavam ou não silicose. Os autores relatam dois casos de pacientes com diagnóstico de silicose que desenvolveram esclerose sistêmica.

A hanseníase apresenta acometimento cutâneo e neurológico característicos; entretanto, as manifestações reumáticas são relativamente comuns sendo, em alguns pacientes, a queixa inicial. O presente relato de caso descreve uma paciente do sexo feminino, com hanseníase borderline, cuja manifestação inicial foi poliartrite simétrica, lesões cutâneas ulceradas em membros inferiores e manifestações sistêmicas simulando doença reumática. Os autores enfatizam a importância do diagnóstico diferencial do comprometimento sistêmico, articular e cutâneo na hanseníase com as doenças reumáticas.Leprosy is a systemic disease with predominant involvement of skin and nerves; articular manifestations are common and, in some cases, represent the initial complaint. We describe the case of a female patient with borderline leprosy, which manifested, initially, with symmetric polyarthritis, cutaneous ulcerative lesions on the lower limbs, and systemic manifestations mimicking rheumatic disease. The authors emphasize the importance of the differential diagnosis of the systemic, joint and, cutaneous involvement of leprosy with rheumatic diseases.

O lúpus eritematoso sistêmico (LES) é uma doença inflamatória multissistêmica, na qual as infecções são responsáveis por altos índices de morbimortalidade. Os usos de corticóide e imunossupressores contribuem para o aumento das infecções. Embora as bactérias sejam os agentes mais comuns, grande variedade de patógenos tem sido relatada. Este artigo descreve um caso de LES em um menino de 15 anos com criptococose disseminada (sistema nervoso central, pulmão e rim) e encefalite tuberculosa presumível. A coexistência de infecção por Cryptococcus e LES é descrita na literatura, mas a associação desta com encefalite tuberculosa é incomum. O risco potencial de infecções em pacientes lúpicos imunossuprimidos deve alertar o médico a adotar estratégias diagnósticas e terapêuticas precoces visando ao espectro ampliado de possíveis patógenos.Systemic lupus erythematosus (SLE) is a multisystem inflammatory disease, in which infection is responsible for high rates of mortality. The use of corticosteroids and immunosuppressive therapy contributes to this high incidence of infections. Although bacteria are the most common agents, a wide variety of pathogens has been reported. This article reports a case of SLE in a 15 years-old boy with disseminated criptococosis (central nervous system, lungs and kidneys) and presumptive tuberculous encephalitis. The coexistence of infection by Cryptococcus and SLE is described in the literature, but the combination of this with tuberculous encephalitis is uncommon. The potential risk of infection in immunosuppressed SLE patients should alert the physician to adopt early diagnostic and therapeutic strategies aiming at an extended spectrum of pathogens.

Os autores relatam um caso de artrite reumatóide, com 20 anos de evolução, que desenvolveu neutropenia e esplenomegalia em uso de 20 mg de metotrexato, que persistiu mesmo com sua substituição pela ciclosporina A. Apresentou infecções de orofaringe, pele e trato urinário. Após afastar doenças hematológicas, o diagnóstico foi de síndrome de Felty. Realizou-se o tratamento com antibioticoterapia, fator estimulador de colônia de granulócitos e macrófagos, pulsoterapia com metilprednisolona e leflunomida. Evoluiu inicialmente com melhora clínica e laboratorial. Após 2 meses de uso do leflunomida, foi admitida em pronto-socorro com quadro de sepse, ocorrendo o óbito em poucas horasThe authors describe the case of a 42 years-old female patient with rheumatoid arthritis WITH A 20 years OF FOLLOW-UP. The patient was using METHOTREXATE (20 mg/WEEK); she developed neutropenia and splenomegaly that persisted despite changing medication for cYclosporine A. She then developed oropharyngeal, skin and urinary tract infections. After excluding for hematological affections, she was diagnosed as presenting Felty's syndrome. She was started on antibiotics while receiving also granulocyte-macrophage colony stimulating factor, pulsed methylprednisolone, andleflunomide. Two moths after the initiation ofleflunomide, she was admitted to an emergency hospital unit with septic shock that resulted in her death in a few hours

Os autores alertam para o múltiplo espectro de apresentação clínica da hanseníase, destacando as lesões cutâneas necrosantes e manifestações articulares simulando doenças reumáticas, confundindo o diagnóstico e o tratamento correto. Nesses aspectos, são relatados dois casos do sexo feminino com hanseníase virchowiana e dimorfa cujas manifestações iniciais foram de lesões eritêmato-violáceas, necróticas e ulceradas, livedo reticular, oligoartrite, poliartralgias, mialgias e edema de membros inferiores, que mimetizaram algumas doenças reumáticas como o lúpus eritematoso sistêmico e a vasculite sistêmica (poliarterite nodosa).Leprosy has a large spectrum of clinical manifestations, including necrotizing skin lesions and joint complaints that sometimes mimic a primary rheumatic disease, confounding the correct diagnosis and treatment. Herein, the authors report two cases of women with leprosy, respectively virchowian and dimorphic forms, presenting initially with purple-reddish skin lesions, evolving with necrosis and ulceration, livedo reticularis, joint pain, oligoarthritis, myalgia, and leg edema. The differential diagnosis with systemic lupus erythematosus (SLE) and systemic vasculitis such as polyarteritis nodosa (PAN) is discussed.

Na hanseníase, lesões ósseas por invasão direta do bacilo são de baixa incidência, e os achados radiológicos destas lesões são de osteomielite aguda ou crônica similares aos de outras infecções granulomatosas. As lesões ósseas mais comuns são devidas ao trauma e a infecção secundária nos tecidos denervados. Radiograficamente, são observados vários graus de reabsorção das extremidades distais envolvendo mãos e pés, ocasionando perda dos dígitos e osteoartropatia neuropática de pequenas articulações. Os achados radiológicos são semelhantes às outras condições nas quais há déficit sensorial, como esclerodermia, siringomielia e diabetes mellitus. A ultra-sonografia (US) e a ressonância magnética (RM) podem ser úteis na avaliação do comprometimento dos nervos periféricos auxiliando no diagnóstico das neurites, abscessos e no diagnóstico diferencial das síndromes compressivas.In leprosy, bone lesions due direct invasion of bacilli are low in incidence and these lesions exhibit radiologic findings of acute and chronic osteomyelitis similar to those of other granulomatous infectious agents. The more common bone lesions are those due to injurious effects of trauma and infection imposed upon denervated tissues. Radiographycally are seen various degrees of reabsorption of the extremities involving hands and feet with the loss of digits and disorganizing arthropathies in small joints. The radiologic appearance are similar to other conditions in which there is sensory impairment like scleroderma, syringomielia and diabetes mellitus. The ultrasonography and magnetic resonance imaging (MRI) can be helpful in evaluation of the involvement of the peripheral nerves helping in the diagnosis of the neuritis, abscess and differential diagnosis in compressive syndromes.

Os antiinflamatórios não-esteroidais (AINEs) orais são eficazes no alívio da dor na osteoartrite (OA), porém têm o risco de efeitos adversos sistêmicos. Para diminuir a toxicidade é necessário reduzir a concentração plasmática uma vez que os efeitos adversos são dose-dependentes. Os AINEs de uso tópico, em teoria, alcançam altas concentrações nos tecidos-alvo com concentrações plasmáticas baixas, sendo portanto uma alternativa terapêutica, especialmente para pacientes de risco. Nossa revisão dos ensaios clínicos randomizados mostrou que os AINEs tópicos foram mais efetivos que o placebo e tão eficazes quanto os AINEs orais. Porém, o pequeno número de ensaios e falhas na metodologia nos impossibilita de fazer conclusões definitivas, sendo necessários estudos bem desenhados e de maior duração.Oral non-steroidal anti-inflammatories drugs (NSAIDs) are effective in pain relief in osteoarthritis (OA), but are also associated with risks of adverse systemic side effects. In order to reduce its toxicity, it would be desirable to decrease the plasmatic levels of NSAIDs since those side effects are dose-dependent. Topical NSAIDs would be a therapeutic alternative in theory, because while they reach high levels in local tissues, they also produce low plasmatic levels. Our review analyzed clinical randomized and shown that topical NSAIDs were superior to placebo and have similar efficacy to oral NSAIDs, but due a few number of trials and methodological weaknesses we can't make definitive conclusions. Further well designed, long term studies are required.