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Este relato objetiva descrever um caso de COVID-19 em paciente do sexo feminino, 48 anos, portadora do diagnóstico de Esclerose Sistêmica forma difusa (ESd) há 14 meses, associada à fibrose pulmonar, em uso de micofenolato de mofetil (MMF). A paciente foi internada no hospital com história de tosse e febre há uma semana. Após 4 dias, testou positivo para COVID-19 e recebeu alta após 11 dias de internação. Prevê-se que os imunossupressores predispõem a um maior risco de infecções e consequente maior agressão ao organismo. Este trabalho objetivou avaliar essa relação, tendo em vista que a paciente sobreviveu à infecção por COVID-19 e não apresentou uma forma grave da doença. Embora este relato descreva evolução favorável de um paciente, ressalta-se a relevância da realização de futuros estudos para avaliar efeitos da imunossupressão na COVID-19.

Background: The current subclassification of systemic sclerosis into cutaneous subtypes does not fully capture the heterogeneity of the disease. We aimed to compare the performances of stratification into LeRoy's cutaneous subtypes versus stratification by autoantibody status in systemic sclerosis. Methods: For this cohort study, we assessed people with systemic sclerosis in the multicentre international European Scleroderma Trials and Research (EUSTAR) database. Individuals positive for systemic-sclerosis autoantibodies of two specificities were excluded, and remaining individuals were classified by cutaneous subtype, according to their systemic sclerosis-specific autoantibodies, or both. We assessed the performance of each model to predict overall survival, progression-free survival, disease progression, and different organ involvement. The three models were compared by use of the area under the curve (AUC) of the receiver operating characteristic and the net reclassification improvement (NRI). Missing data were imputed. Findings: We assessed the database on July 26, 2019. Of 16 939 patients assessed for eligibility, 10 711 patients were included: 1647 (15·4%) of 10 709 were male, 9062 (84·6%) were female, mean age was 54·4 (SD 13·8) years, and mean disease duration was 7·9 (SD 8·2) years. Information regarding cutaneous subtype was available for 10 176 participants and antibody data were available for 9643 participants. In the prognostic analysis, there was no difference in AUC for overall survival (0·82, 95% CI 0·81–0·84 for cutaneous only vs 0·84, 0·82–0·85 for antibody only vs 0·84, 0·83–0·86 for combined) or for progression-free survival (0·70, 0·69–0·71 vs 0·71, 0·70–0·72 vs 0·71, 0·70–0·72). However, at 4 years the NRI showed substantial improvement for the antibody-only model compared with the cutaneous-only model in prediction of overall survival (0·57, 0·46–0·71 for antibody only vs 0·29, 0·19–0·39 for cutaneous only) and disease progression (0·36, 0·29–0·46 vs 0·21, 0·14–0·28). The antibody-only model did better than the cutaneous-only model in predicting renal crisis (AUC 0·72, 0·70–0·74 for antibody only vs 0·66, 0·64–0·69 for cutaneous only) and lung fibrosis leading to restrictive lung function (AUC 0·76, 0·75–0·77 vs 0·71, 0·70–0·72). The combined model improved the prediction of digital ulcers and elevated systolic pulmonary artery pressure, but did poorly for cardiac involvement. Interpretation: The autoantibody-only model outperforms cutaneous-only subsetting for risk stratifying people with systemic sclerosis in the EUSTAR cohort. Physicians should be aware of these findings at the time of decision making for patient management. Funding: World Scleroderma Foundation.

SUMMARY INTRODUCTION Systemic sclerosis (SSC) is an autoimmune disorder that affects several organs of unknown etiology, characterized by vascular damage and fibrosis of the skin and organs. Among the organs involved are the esophagus and the lung. OBJECTIVES To relate the profile of changes in esophageal electromanometry (EM), the profile of skin involvement, interstitial pneumopathy (ILD), and esophageal symptoms in SSC patients. METHODS This is an observational, cross-sectional study carried out at the SSC outpatient clinic of the Hospital de Clínicas of the Federal University of Uberlândia. After approval by the Ethics Committee and signed the terms of consent, 50 patients were initially enrolled, from 04/12/2014 to 06/25/2015. They were submitted to the usual investigations according to the clinical picture. The statistical analysis was descriptive in percentage, means, and standard deviation. The Chi-square test was used to evaluate the relationship between EM, high-resolution tomography, and esophageal symptoms. RESULTS 91.9% of the patients had some manometric alterations. 37.8% had involvement of the esophageal body and lower esophageal sphincter. 37.8% had ILD. 24.3% presented the diffuse form of SSC. No association was found between manometric changes and clinical manifestations (cutaneous, pulmonary, and gastrointestinal symptoms). CONCLUSION The present study confirms that esophageal motility alterations detected by EM are frequent in SSC patients, but may not be related to cutaneous extension involvement, the presence of ILD, or the gastrointestinal complaints of patients. RESUMO INTRODUÇÃO A esclerose sistêmica (ES) é uma doença autoimune que afeta vários órgãos de etiologia desconhecida, caracterizada por dano vascular e fibrose da pele e órgãos. Entre os órgãos envolvidos estão o esôfago e o pulmão. OBJETIVOS Relacionar o perfil das alterações na eletromanometria (ME), o perfil de acometimento da pele, a pneumopatia intersticial (PI) e os sintomas esofágicos em pacientes com ES. MÉTODO Trata-se de um estudo observacional, transversal, realizado no ambulatório de SSC do Hospital das Clínicas da Universidade Federal de Uberlândia. Após aprovação pelo Comitê de Ética e assinatura dos termos de consentimento, 50 pacientes foram inicialmente convidados, de 04/12/2014 a 25/06/2015. Eles foram submetidos às investigações usuais de acordo com o quadro clínico. A análise estatística foi descritiva em porcentagem, média e desvio padrão. O teste Qui-quadrado foi utilizado para avaliar a relação entre ME, tomografia de alta resolução e sintomas esofágicos. RESULTADOS 91,9% dos pacientes apresentaram alterações manométricas. 37,8% tinham envolvimento do corpo esofágico e do esfíncter esofágico inferior. 37,8% tinham IP. 24,3% apresentaram a forma difusa da ES. Não há associação entre alterações manométricas e manifestações clínicas (sintomas cutâneos, pulmonares e gastrointestinais). CONCLUSÃO O presente estudo confirma que as alterações da motilidade esofágica detectadas pela EM são frequentes em pacientes com SSC, mas podem não estar relacionadas ao envolvimento cutâneo, à de DPI ou às queixas gastrointestinais dos pacientes.

Gastrointestinal (GI) involvement is almost universal in patients with systemic sclerosis (SSc) and is associated with significant disease-related morbidity and mortality.1 The entire GI tract can be involved and other disease features (eg, low mood, terminal organ failure and functional hand impairment) can result in significant nutritional impairment. Severe GI involvement has been reported to occur in ~10% of patients with SSc and often occurs early in the course of the disease.2 However, identification of patients at high risk of clinically significant weight loss is extremely challenging, including from the high prevalence of GI symptoms in patients with SSc. Therefore, there is a need to understand high-risk patients including potentially modifiable risk factors, with a view to early intervention strategies. Against this background, the aim of this study was to examine potential clinical risk factors of significant weight loss in patients with SSc. We performed an analysis of patients with SSc enrolled in the multinational, longitudinal European League Against Rheumatism (EULAR) Scleroderma Trials and Research (EUSTAR) database. In our study, we defined significant weight loss as 4.5 kg and/or least 5% of their body weight at 5 months onwards.3 Patients with a recorded second visit after 3 months and before 12 months were included in the analysis. We adopted a pragmatic approach (relevant to clinical practice) in …

Introdução A Esclerose Sistêmica (ES) é uma desordem autoimune que atinge vários sistemas e órgãos, de etiologia ainda desconhecida, caracterizada por dano vascular e por fibrose de pele e de órgãos. Entre os órgãos envolvidos estão o esôfago e o pulmão. Objetivos Relacionar o perfil das alterações da manometria esofágica(ME), com o tipo de envolvimento cutâneo, com a pneumopatia intersticial(PI) bem como com os sintomas esofágicos nos pacientes com ES. Método Trata-se de um estudo observacional, transversal, realizado no ambulatório de ES do Hospital de Clínicas da Universidade Federal de Uberlândia. Após aprovação no Comitê de Ética e assinados os termos de consentimento, foram incluídos inicialmente 50 pacientes, entre os dias 04/12/2014 a 25/06/2015. Os mesmos foram submetidos às investigações habituais para o quadro e conforme queixas clínicas. A análise estatística foi descritiva em percentual, médias e desvio padrão. Para avaliar a relação entre ME, tomografia de alta resolução e sintomas esofágicos foi utilizado o teste Qui-quadrado. Resultados 91,9% dos pacientes apresentavam alguma alteração manométrica. 37,8% apresentavam envolvimento do corpo esofágico e do esfíncter inferior do esôfago. 37,8% apresentavam PI. 24,3% apresentavam a forma difusa da ES. Conclusão Este estudo não demonstrou um padrão de distribuição de alterações do esôfago relacionado ao tipo de envolvimento cutâneo. Não houve relação de um padrão manométrico específico com a presença de PI. Nem mesmo a presença de sintomas clínicos gastrointestinais se relacionou com um padrão cutâneo ou com alterações manométricas do esôfago ou com a presença de PI. Palavras chaves: Esclerose sistêmica, Esôfago, Doenças pulmonares intersticiais Introduction Systemic Sclerosis (SS) is an autoimmune disorder that affects several systems and organs, of unknown etiology, characterized by vascular damage and fibrosis of the skin and organs. Among the organs involved are the esophagus and the lung. Objectives To relate the profile of changes in esophageal manometry (EM), the type of skin involvement, interstitial lung disease (ILD) as well as esophageal symptoms in SS patients. Method This is an observational, cross-sectional study carried out at the outpatient clinic of the Hospital of the Federal University of Uberlândia. After approval by the Ethics Committee and signed the terms of consent, 50 patients were initially included, between 04/12/2014 to 06/25/2015. They were submitted to the usual investigations for the condition and according to clinical complaints. The statistical analysis was descriptive in percentage, means and standard deviation. The Chi-square test was used to evaluate the relationship between EM, high resolution tomography and oesophageal symptoms. Results 91.9% of the patients had some manometric alterations. 37.8% had involvement of the esophageal body and lower esophageal sphincter. 37.8% presented ILD. 24.3% presented the diffuse form of SS. Conclusion This study did not demonstrate a pattern of distribution of esophageal changes related to the type of cutaneous involvement. There was no relation of a specific manometric standard with the presence of ILD. Not even the presence of gastrointestinal clinical symptoms was associated with a cutaneous pattern or with manometric changes of the esophagus or with the presence of ILD. Key words: Systemic sclerosis, Esophagus, Interstitial lung disease. Dissertação (Mestrado)

Objective.To determine the prevalence of psoriatic arthritis (PsA) in a large cohort of Brazilian patients with psoriasis (PsO) being seen at dermatology centers.Methods.A multicenter study was conducted in 4 university dermatology clinics. In each center, consecutive patients with confirmed diagnoses of PsO were evaluated by a rheumatologist. Individuals were classified as having PsA according to the ClASsification criteria for Psoriatic ARthritis (CASPAR). Laboratory tests and radiographs were performed, as needed, based on the clinical judgment of the rheumatologist.Results.A total of 524 patients with PsO were evaluated. The mean age was 48.5 ± 14.5 years, 50% were women, and the mean PsO duration was 15.4 ± 11.7 years. A diagnosis of PsA was documented in 175 patients (33%), of whom 49% were newly identified by the rheumatologist. Most individuals with PsA (72%) had peripheral involvement, 11% had isolated axial involvement, and 17% had both peripheral and axial involvement. Dactylitis occurred in 20% and clinical enthesitis in 30% of the patients. Laboratory and/or radiograph tests were necessary for a definitive diagnosis of PsA in 42 of 175 individuals (24%).Conclusion.In our study, one-third of Brazilian patients with PsO, followed in dermatology settings, were diagnosed with PsA by a rheumatologist. Almost half of subjects with PsA had no previous diagnosis. A collaboration between dermatologists and rheumatologists is greatly needed to establish earlier PsA diagnoses and adequate multidisciplinary management.

Background Psoriatic Arthritis (PsA) may affect up to 40% of patients with psoriasis (PsO). Usually PsO precedes PsA for many years, though PsA is frequently not identified in dermatology clinics. Diagnosis of PsA is based on clinical recognition of arthritis, enthesitis and spondylitis. Objectives To determine the prevalence of PsA in a large cohort of Brazilian patients with PsO attending different dermatology reference centers. Methods A multicenter study was conducted in four universitary dermatology clinics, from January to March 2011. In each center, consecutive patients with a confirmed diagnosis of PsO were evaluated by a rheumatologist. Following detailed musculoskeletal anamnesis and physical examination, subjects were classified as having: isolated PsO, osteoarthritis (OA) and/or chronic myofascial pain (CMP) syndrome, PsA (CASPAR criteria 1 ). Laboratory and x-Ray tests were performed, as needed, according to the rheumatologist clinical judgment. Results A total of 524 PsO patients were evaluated. Mean age was 48.5 ± 14.5yrs, 50% were females and PsO mean duration was 15.4±11.7yrs. The vast majority (79%) had plaque psoriasis and 57.8% required systemic treatment. Isolated PsO was the diagnosis in 45% of patients, whereas 22% manifested OA and/or CMP. A definitive diagnosis of PsA was documented in 175 patients (33%), of which 49% were newly identified by the rheumatologist. Lab and/or x-Ray tests were necessary for the diagnosis of PsA in 42/175 individuals. In 38/175 subjects, PsA was associated with OA and/or CMP. Most PsA patients (72%) had peripheral, 11% axial and 17% both peripheral and axial involvement. Dactilitis occurred in 20% and clinical enthesitis in 30%. Remarkably, PsA patients were older (51 vs 47yrs, p 0.015), had more nail involvement (59 vs 47%, p 0.008), were less likely to be on any systemic treatment (42 vs 56%, p 0.01) and were more frequently using biologic drugs (21 vs 5%, p>0.001) compared with those without PsA. Conclusions We have demonstrated a similar worldwide prevalence of 1/3 of PsA among Brazilian patients with PsO. The identification of new PsA diagnosis by a rheumatologist in half of patients, previously unrecognized at the dermatology setting, points out to the need for shared care between dermatologists and rheumatologists in order to establish earlier PsA diagnosis and adequate multidisciplinary management. References Taylor W, Gladman D, Helliwell P et al. Classification criteria for psoriatic arthritis: development of new criteria from a large international study. Arthritis Rheum 2006; 54:2665–73 Disclosure of Interest None Declared