Camurati-Engelmann disease: case report

Objetivos: Fornecer informações sobre a doença de Camurati-Engelmann, também conhecida como Displasia Diafisária Progressiva, possibilitando o seu diagnóstico pelo profissional de saúde. Método: Relato de caso associado a revisão da literatura científica sobre o tema. Discussão: Doença rara, de herança autossômica dominante, caracterizada por hiperostose progressiva com envolvimento das diáfises dos ossos. Os sintomas mais comuns são dores em extremidades, alteração da deambulação, fatigalibidade e fraqueza muscular. Seu diagnóstico é clínico-radiológico. O tratamento consiste basicamente na administração de glicocorticóides e anti-inflamatórios não esteroidais para melhorar os sintomas clínicos como dor e fadiga.Goals: To provide information about Camurati-Engelmann disease, also known as progressive diaphyseal dysplasia, allowing its diagnosis by a healthcare professional. Methods: A case report associated with literature review. Discussion: It is a rare, autosomal dominant type, disease characterized by progressive hyperostosis involving the diaphysis of bones. The most commom clinical symptoms are pain in extremities, waddling gait, fatigability and muscle weakness. Its diagnosis may be determined by clinical and radiological data. Treatment involves basically glucocorticosteroids and non-steroid anti-inflammatory administration to promote improvement in clinical symptoms such as pain and fatigue

Intestinal microsporidiosis: a hidden risk in rheumatic disease patients undergoing anti-tumor necrosis factor therapy combined with disease-modifying anti-rheumatic drugs?

OBJECTIVE: Immunosuppressed patients are at risk of microsporidiosis, and this parasitosis has an increased rate of dissemination in this population. Our objective was to evaluate the presence of microsporidiosis and other intestinal parasites in rheumatic disease patients undergoing anti-tumor necrosis factor/disease-modifying anti-rheumatic drug treatment. METHODS: Ninety-eight patients (47 with rheumatoid arthritis, 31 with ankylosing spondylitis and 11 with psoriatic arthritis) and 92 healthy control patients were enrolled in the study. Three stool samples and cultures were collected from each subject. RESULTS: The frequency of microsporidia was significantly higher in rheumatic disease patients than in control subjects (36 vs. 4%, respectively; p<0.0001), as well as in those with rheumatic diseases (32 vs. 4%, respectively; p<0.0001), ankylosing spondylitis (45 vs. 4%, respectively; p<0.0001) and psoriatic arthritis (40 vs. 4%, respectively; p<0.0001), despite a similar social-economic class distribution in both the patient and control groups (p = 0.1153). Of note, concomitant fecal leukocytes were observed in the majority of the microsporidia-positive patients (79.5%). Approximately 80% of the patients had gastrointestinal symptoms, such as diarrhea (26%), abdominal pain (31%) and weight loss (5%), although the frequencies of these symptoms were comparable in patients with and without this infection (p&gt;0.05). Rheumatoid arthritis, ankylosing spondylitis and psoriatic arthritis disease activity parameters were comparable in both groups (p&gt;0.05). The duration of anti-tumor necrosis factor/disease-modifying anti-rheumatic drugs and glucocorticoid use were also similar in both groups. CONCLUSION: We have documented that microsporidiosis with intestinal mucosa disruption is frequent in patients undergoing concomitant anti-tumor necrosis factor/disease-modifying anti-rheumatic drug therapy. Impaired host defenses due to the combination of the underlying disease and the immunosuppressive therapy is the most likely explanation for this finding, and this increased susceptibility reinforces the need for the investigation of microsporidia and implementation of treatment strategies in this population.FAPESPCNPQFederico FoundationWyet

High levels of immunosuppression are related to unfavourable outcomes in hospitalised patients with rheumatic diseases and COVID-19 : first results of ReumaCoV Brasil registry

Objectives To evaluate risk factors associated with unfavourable outcomes: emergency care, hospitalisation, admission to intensive care unit (ICU), mechanical ventilation and death in patients with immune-mediated rheumatic disease (IMRD) and COVID-19. Methods Analysis of the first 8 weeks of observational multicentre prospective cohort study (ReumaCoV Brasil register). Patients with IMRD and COVID-19 according to the Ministry of Health criteria were classified as eligible for the study. Results 334 participants were enrolled, a majority of them women, with a median age of 45 years; systemic lupus erythematosus (32.9%) was the most frequent IMRD. Emergency care was required in 160 patients, 33.0% were hospitalised, 15.0% were admitted to the ICU and 10.5% underwent mechanical ventilation; 28 patients (8.4%) died. In the multivariate adjustment model for emergency care, diabetes (prevalence ratio, PR 1.38; 95% CI 1.11 to 1.73; p=0.004), kidney disease (PR 1.36; 95% CI 1.05 to 1.77; p=0.020), oral glucocorticoids (GC) (PR 1.49; 95% CI 1.21 to 1.85; p50 years (PR 1.89; 95% CI 1.26 to 2.85; p=0.002), no use of tumour necrosis factor inhibitor (TNFi) (PR 2.51;95% CI 1.16 to 5.45; p=0.004) and methylprednisolone pulse therapy (PR 2.50; 95% CI 1.59 to 3.92; p<0.001); for ICU admission, oral GC (PR 2.24; 95% CI 1.36 to 3.71; p<0.001) and pulse therapy with methylprednisolone (PR 1.65; 95% CI 1.00 to 2.68; p<0.043); the two variables associated with death were pulse therapy with methylprednisolone or cyclophosphamide (PR 2.86; 95% CI 1.59 to 5.14; p<0.018). Conclusions Age >50 years and immunosuppression with GC and cyclophosphamide were associated with unfavourable outcomes of COVID-19. Treatment with TNFi may have been protective, perhaps leading to the COVID-19 inflammatory process

Evolução do centro de dispensação de medicação de alto custo em São José do Rio Preto, Estado de São Paulo: Relato de experiência.

Introdução: Em 2010 foi instituído o Centro de Dispensação de Medicação de Alto Custo (CEDMAC) em parceria com a Universidade de São Paulo, Secretaria de Estado da Saúde de São Paulo, o Departamento Regional de Saúde e a Fundação Faculdade Regional de Medicina (FUNFARME), com sede no Hospital de Base, localizado na cidade de São José do Rio Preto, interior do Estado de São Paulo, com o objetivo de tratar pacientes com doenças inflamatórias autoimunes e outras, no início, exclusivamente da reumatologia e, posteriormente, ampliando os atendimentos para outras especialidades. Em decorrência do número crescente de pacientes acompanhados no CEDMAC, foi implantado um estudo de farmacoeconomia, com objetivo de viabilizar os recursos públicos disponíveis, principalmente por meio da otimização de doses, possibilitando assim tratar pacientes cujas patologias não estão contempladas nos protocolos clínicos e diretrizes terapêuticas (PCDT) estabelecidos pelo Ministério da Saúde, além de também realizar devolução de sobras de medicamentos para o Componente Especializado de Assistência Farmacêutica, revertendo em tratamento para outros pacientes contemplados pelo PCDT e economia financeira ao Governo do Estado. Na literatura nacional há poucos estudos publicados que tratam de centros de dispensação de medicação de alto custo, por isso este estudo se torna relevante para contribuir com a estruturação e operação de outros centros similares. Objetivos: Descrever a evolução do CEDMAC da FUNFARME, no período de 2010 a 2017, apresentando a evolução do serviço e o estudo de farmacoeconomia realizado nos anos de 2015 a 2017. Métodos: Estudo empírico descritivo transversal, com abordagem metodológica qualitativa e quantitativa, cuja unidade de análise é o CEDMAC. O estudo de farmacoeconomia foi realizado por meio da quantificação financeira obtida através da otimização de doses. Resultados: Entre os anos de 2010 a 2015 houve uma média de 894 atendimentos médicos e de enfermagem. A partir de 2015 até 2017, os números de atendimentos realizados, de medicamentos administrados e de pacientes tratados tiveram aumentos de, respectivamente, 764,7%, 243,6% e 173,9%. O quadro de funcionários duplicou, passando de 7, em 2010, para 14, em 2017. O serviço obteve uma economia de R$ 535.323,71 com a otimização de doses de medicamentos entre os anos de 2015 a 2017. As especialidades médicas mais acompanhadas no centro foram reumatologia, gastroenterologia e proctologia e os medicamentos mais administrados foram adalimumabe e infliximabe, ambos Bloqueadores do Fator de Necrose Tumoral. Conclusão: A implantação do CEDMAC resultou como importante estratégia para o fortalecimento das políticas de assistência farmacêutica no uso racional de medicamentos, preenchendo uma lacuna importante de conhecimento e de experiência na estruturação do centro e otimização dos recursos financeiros. Os Resultados obtidos demonstram-se relevantes para contribuir com a estruturação e operação de outros centros similares

Piomiosite associada ao diabetes mellitus e cirrose hepática

A piomiosite é uma infecção primária do músculo esquelético mais comum nos países tropicais. Adultos que desenvolvem a doença apresentam, na maioria dos casos, comorbidades associadas que comprometem o sistema imunológico, entre elas diabetes mellitus e cirrose hepátic

Trombose de veia jugular em paciente com anticorpo anticardiolipina e lúpus eritematoso sistêmico

Os autores relatam o caso de uma paciente com 19 anos de idade, portadora de lúpus eritematoso sistêmico, que apresentou edema súbito e progressivo em membro superior esquerdo e dor e circulação colateral visível em região supraclavicular esquerda. Foi realizado diagnóstico de trombose venosa em veia jugular externa esquerda por meio do dúplex scan. Nos exames laboratoriais, evidenciou-se a presença de anticorpo anticardiolipina

Management of Enthesitis in Patients With Psoriatic Arthritis: An Updated Literature Review Informing the 2021 GRAPPA Treatment Recommendations.

OBJECTIVE: Enthesitis is a key pathological and clinical feature of psoriatic arthritis (PsA) in children and adults. Enthesitis is typically assessed clinically using several validated enthesitis scoring systems that have been used in clinical trials. Enthesitis treatment response has been reported as change in the total enthesitis score or the proportion of patients who achieved complete resolution. The majority of trials in PsA did not require patients to have enthesitis at study entry since enthesitis was evaluated only as a secondary outcome. Despite the inherent limitations of the clinical assessment of enthesitis, imaging of the entheses using ultrasound or magnetic resonance imaging has rarely been used in clinical trials to assess response to treatment of enthesitis. This systematic review summarizes existing evidence regarding pharmaceutical and nonpharmaceutical interventions for enthesitis in patients with PsA to facilitate an evidence-based update of the Group for Research and Assessment in Psoriasis and Psoriatic Arthritis (GRAPPA) treatment recommendations for PsA. METHODS: We performed a systematic literature review to identify 41 randomized clinical trials that reported enthesitis treatment response in patients with PsA. For each intervention, the response effect size was summarized and the quality of evidence was graded. Recommendations were then formulated for the various pharmacological and nonpharmacological therapies. RESULTS: We included 41 randomized clinical trials in our review and graded each intervention. CONCLUSION: Several classes of systemic conventional and advanced therapies and local measures were recommended for active enthesitis in patients with PsA