PERCIVAL DEGRAVA SAMPAIO BARROS
Índice h a partir de 2011
26
Projetos de Pesquisa
Unidades Organizacionais
Instituto Central, Hospital das Clínicas, Faculdade de Medicina - Médico
LIM/17 - Laboratório de Investigação em Reumatologia, Hospital das Clínicas, Faculdade de Medicina
LIM/17 - Laboratório de Investigação em Reumatologia, Hospital das Clínicas, Faculdade de Medicina
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- Registries in systemic sclerosis: a worldwide experience(2011) GALLUCCIO, Felice; WALKER, Ulrich A.; NIHTYANOVA, Svetlana; MOINZADEH, Pia; HUNZELMANN, Nicholas; KRIEG, Thomas; STEEN, Virginia; BARON, Murray; SAMPAIO-BARROS, Percival; KAYSER, Cristiane; NASH, Peter; DENTON, Chris P.; TYNDALL, Alan; MUELLER-LADNER, Ulf; MATUCCI-CERINIC, MarcoSSc is a multisystem disease characterized by an unpredictable course, high mortality and resistance to therapy. The complexity and severity of SSc is a growing burden on the health-care systems. As a result, researchers are seeking new therapeutic strategies for effectively managing these patients. Disease registries are used to support care management efforts for groups of patients with chronic diseases and are meaningful to capture and track key patient information to assist the physicians in managing patients. For these reasons, SSc surveys, research associations and consortiums are pivotal to conduct ongoing research and data collection to enhance disease knowledge and support research projects. Currently, there are several national SSc registries in the UK, Germany, USA, Canada, Brazil and Australia. There is also an international registry established by the European League Against Rheumatism scleroderma trial and research (EUSTAR) called minimal essential data set (MEDS) Online, which collects data from over 8000 patients from 92 centres worldwide, including 21 European centres and 9 centres outside Europe. By collecting, analysing and disseminating data on disease progression and patient responses to long-term disease management strategies, registries help to improve understanding of the disease and keep medical professionals up to date on the latest advances.
- Clinical and laboratory features of African-Brazilian patients with systemic sclerosis(2020) MENDES, Cristiane; VIANA, Vilma S. T.; PASOTO, Sandra G.; LEON, Elaine P.; BONFA, Eloisa; SAMPAIO-BARROS, Percival D.Objective African-Brazilians comprise a group of blacks and ""pardos."" As racial differences can be associated with distinct presentations, we evaluated the clinical and serological associations of African-Brazilians with systemic sclerosis (SSc). Methods Sera from 260 adult SSc patients (203 whites and 57 African-Brazilians) were evaluated. Patients with overlap syndromes were excluded. Clinical and demographic data were obtained from an electronic register database. Laboratory analysis included the following: anti-CENP-A/CENP-B, Scl70, RNA polymerase III, Ku, fibrillarin, Th/To, PM-Scl75, and PM-Scl100 by line immunoassay and anti-nuclear antibodies (ANA) by indirect immunofluorescence (IIF) on HEp-2 cells. Results African-Brazilian SSc patients presented shorter disease duration (12.8 +/- 6.5 vs. 15.9 +/- 8.1 years, p = 0.009), higher frequency of nucleolar ANA pattern (28% vs. 13%, p = 0.008), and lower frequencies of centromeric ANA pattern (14% vs. 29%, p = 0.026) and CENP-B (18% vs. 34%, p = 0.017), as well as an association with severe interstitial lung disease (58% vs. 43%; p = 0.044). Further comparison of ethnic groups according to subsets revealed that diffuse SSc African-Brazilian patients presented higher frequency of pulmonary hypertension (p = 0.017), heart involvement (p = 0.037), nucleolar ANA pattern (p = 0.036), anti-fibrillarin antibodies (p = 0.037), and higher mortality (48% vs. 19%; p = 0.009). A different pattern was observed for the limited subset with solely a lower frequency of esophageal involvement (p = 0.050) and centromeric ANA pattern (p = 0.049). Survival analysis showed that African-Brazilians had a higher mortality, when adjusted for age, gender, and clinical subset (RR 2.06, CI 95% 1.10-3.83, p = 0.023). Conclusion African-Brazilians have distinct characteristics according to clinical subset and an overall more severe SSc than whites, similar to the blacks from other countries.
- Systemic sclerosis induced by the use of cocaine: is there an association?(2019) ANDREUSSI, Rafael; SILVA, Lila Morena Bueno; SILVA, Henrique Carrico da; LUPPINO-ASSAD, Ana Paula; ANDRADE, Danieli Castro O.; SAMPAIO-BARROS, Percival D.The association between cocaine abuse and systemic sclerosis (SSc) is rarely described. Two new cases of this association are presented: two young adults, after using inhaled cocaine for a few years, were diagnosed with SSc. While a 24 year-old white female patient presented with diffuse SSc with multiple digital ulcers and scleroderma renal crisis (SRC), a 27 year-old male patient presented limited SSc with skin ulcers and digital gangrene, rapidly evolving to death due to massive intestinal hemorrhage. The authors performed a literature search and found only eight previously published cases. The clinical picture of these patients shows a predominance of vascular involvement, including multiple ulcers and SRC. There is no association with specific SSc autoantibodies. The concomitance of alcohol and other drugs abuse, as well as the presence of drug adulterers, complicates a clear understanding of the role of cocaine in SSc patients.
- Inflammatory myopathies overlapping with systemic sclerosis: a systematic review(2022) GONCALVES JUNIOR, Jucier; MUGII, Naoki; INAOKA, Pleiades Tiharu; SAMPAIO-BARROS, Percival Degrava; SHINJO, Samuel KatsuyukiWe performed a systematic review of the clinical manifestations and complementary exams of patients with myopathies and systemic sclerosis overlap syndrome (MyoSScOS). Systematic review from January 1976 to November 2021 according PRISMA protocol on three electronic databases: PubMed, Web of Science, and Scopus. Studies were analyzed based on the following eligibility criteria: at least one combination of the terms described in the search strategy appears in the title; written in English, Portuguese, or Spanish; and addresses MyoSScOS. Brief communications, reviews, studies that addressed myopathies in children, congress proceedings, monographs, and dissertations were excluded. Thirty-five articles were selected. MyoSScOS seems to be more common in women. It also commonly affects the esophagus and joints with symmetrical and bilateral muscle involvement, Raynaud's phenomenon, and impairment of forced vital capacity. Concerning SSc, the most common subtype was the diffuse form. Cardiovascular and pulmonary complications are an important cause of death. Anti-centromere, anti-PM/Scl, anti-Scl70, anti-RNA polymerase III, anti-Ku, and anti-RNP were more correlated with this entity, and muscle biopsies may present a more aggressive pattern. Electroneuromyography patterns are quite similar to those found in inflammatory myopathies. The absence of studies with robust methodologies and the large number of case reports and series make more robust statistical analyses such as meta-analyses unfeasible. The characterization of MyoSScOS is important for the formulation of therapeutic measures and specific treatments aiming at better quality of life and prognosis. Greater and better theoretical contributions are necessary to better characterize it.
- Severity and mortality of COVID-19 in patients with systemic sclerosis: a Brazilian multicenter study(2022) OLIVEIRA, Sandra Maximiano de; MARTINS, Lucas Victoria de Oliveira; LUPINO-ASSAD, Ana Paula; MEDEIROS-RIBEIRO, Ana Cristina; MORAES, Daniela Aparecida de; DEL-RIO, Ana Paula Toledo; OLIVEIRA, Maria Carolina; SAMPAIO-BARROS, Percival Degrava; KAYSER, CristianeIntroduction: COVID-19 may be associated with greater severity and mortality in patients with systemic sclerosis (SSc). The present study aimed to evaluate the prevalence, severity and mortality of COVID-19 in a Brazilian cohort of SSc patients. Methods: This multicenter, retrospective, observational study included 1,042 SSc patients followed in four centers of Sao Paulo between March 2020 and June 2021. Diagnosis of COVID-19 was established by proper positive RT-PCR testing or by highly suspicious infection. Patients were grouped into mild (outpatient setting treatment and no need for oxygen support) and moderate-to-severe (hospitalization and/or need for oxygen support) COVID-19. Results: Of the 1,042 SSc patients, 118 patients were diagnosed with COVID-19. Interstitial lung disease (SSc-ILD) was present in 65.6% of the total cohort and in 46.3% of SSc patients with COVID-19. There were 78 (66.1%) cases of mild COVID-19, and 40 (33.9%) cases of moderate-to-severe disease, with 6 (5.1%) deaths. By univariate analysis, pulmonary arterial hypertension (OR 9.50, p=0.006), SSc-ILD (OR 3.90, p=0.007), FVC <80% (OR 2.90, p=0.01), cardiac involvement (OR 5.53, p=0.003), and use of rituximab (OR 3.92, p=0.039), but not age, gender, comorbidities or use of corticosteroids, were predictors of worse outcome for COVID-19. Using multivariate analysis, only SSc-ILD was significantly associated to a higher risk of moderate-to-severe COVID-19 (OR 2.73, 95% CI 1.12-6.69, p=0.02). Forty percent of the patients remained with symptoms after presenting COVID-19, predominantly dyspnea and/or cough (17%). Conclusion: In this cohort of patients with SSc, those with SSc-ILD were highly impacted by COVID-19, with a higher risk of moderate-to-severe COVID-19 infection and death.
- Cross-cultural adaptation and validation of the Brazilian version of the Scleroderma Health Assessment Questionnaire (SHAQ)(2014) ROCHA, Luiza F.; MARANGONI, Roberta G.; SAMPAIO-BARROS, Percival D.; LEVY-NETO, Mauricio; YOSHINARI, Natalino H.; BONFA, Eloisa; STEEN, Virginia; KOWALSKI, Sergio C.The Scleroderma Health Assessment Questionnaire (SHAQ) is a feasible multisystem specific tool that has been extensively used as an additional assessment for systemic sclerosis (SSc). The aim of this study is to cross-culturally adapt and validate the Brazilian version of the SHAQ. Construct validity was assessed based on the correlations between SHAQ and both the Medical Outcomes Survey Short Form 36 version 2 (SF-36v2 (TM)) and the Health Assessment Questionnaire Disability Index (HAQ-DI). The correlation between the SHAQ and disease severity was assessed by Spearman's correlation coefficient. The reproducibility of the SHAQ was evaluated by the intraclass correlation coefficient (ICC). Among the 151 consecutive outpatients evaluated, 59 % had limited SSc subtype. The overall disease severity visual analog scale (VAS) of the SHAQ was statistically significantly correlated to HAQ-DI, pain VAS, and the SF-36v2 (TM) physical component summary score (r = 0.595, r = 0.612, and r = -0.582, respectively; p < 0.001). Further analysis of all SF-36v2 (TM) components revealed statistically significant correlations between overall disease severity VAS and bodily pain (r = -0.621, p < 0.001), vitality (r = -0.544, p < 0.001), physical function (r = -0.510, p < 0.001), and role limitation-physical dimensions (r = -0.505, p < 0.001). Moreover, digestive, pulmonary, and overall disease severity VASs were statistically significantly correlated to the number of organs involved (r = 0.178, p = 0.029; r = 0.214, p = 0.008; r = 0.282, p < 0.001). We also demonstrated high reproducibility for SHAQ (ICC = 0.757, 95 % confidence interval = 0.636-0.842). The Brazilian version of the SHAQ demonstrated both construct and discriminant validities as well as good reproducibility.
- Clinical and laboratory profile of juvenile-onset systemic sclerosis in a Brazilian cohort(2019) SAMPAIO-BARROS, Percival D.; BORTOLUZZO, Adriana B.; RIO, Ana Paula T. Del; LUPPINO-ASSAD, Ana Paula; ANDRADE, Danieli C. O.; MARQUES-NETO, Joao FranciscoObjective: To characterize the clinical and laboratory profile of juvenile-onset compared to adult-onset systemic sclerosis in a large Brazilian cohort. Methods: Retrospective analysis of a cohort of 1016 systemic sclerosis patients followed at the Scleroderma Outpatient Clinic from two referral university centers in Brazil. Patients were classified as systemic sclerosis according to the 1980 American College of Rhaumatology (ACR) criteria. Juvenile-onset systemic sclerosis was defined if age at onset was Results: Thirty-one (3.1%) patients were classified as juvenile-onset systemic sclerosis. These patients were predominantly females (90.3%), Caucasians (71.0%), and presented diffuse systemic sclerosis (51.6%), with mean age at onset of 12.71 years. Compared to the adult-onset patients, juvenile onset was associated with diffuse systemic sclerosis (p < 0.001), calcinosis (p < 0.001), myositis (p = 0.050), and lower frequency of interstitial lung disease (p = 0.050), pulmonary hypertension (p = 0.035), and esophageal (p = 0.005) involvement. Conclusion: Juvenile-onset systemic sclerosis characterized a distinct clinical pattern in this large series of systemic sclerosis patients, since it was predominantly associated with diffuse systemic sclerosis without significant organ involvement.
- Is exposure to environmental factors associated with a characteristic clinical and laboratory profile in systemic sclerosis? A retrospective analysis(2021) AGUILA, Lisbeth A.; SILVA, Henrique Carrico da; MEDEIROS-RIBEIRO, Ana Cristina; BUNJES, Bruna Giusto; LUPPINO-ASSAD, Ana Paula; SAMPAIO-BARROS, Percival D.To identify environmental factors (EF) in a large cohort of patients with systemic sclerosis (SSc) analyzing their clinical and laboratory presentation. A cohort of consecutive patients attended at a single Brazilian SSc outpatient clinic was analyzed regarding EF. Data were analyzed according to clinical, demographic and laboratory characteristics, as well as SSc subtype. In a cohort of 662 patients, 70 (10.6%) had known previous exposure to EF, predominantly organic solvents (51.4%), silica (20%), silicone (12.9%) and pesticides (11.4%). In the SSc cohort, patients with EF had a significantly higher frequency of male gender (p < 0.01), African-Brazilian ethnicity (p = 0.01), myopathy (p = 0.02), and pigmentary disorders (p = 0.04), with shorter disease duration (p = 0.01). When SSc subtypes were analyzed separately, there was positive association with male gender in limited (p < 0.01) and diffuse (p < 0.01) SSc, as well as African-Brazilian ethnicity (p = 0.04), severe interstitial lung disease (p < 0.01), myopathy (p = 0.02) and SD pattern at nailfold capillaroscopy (p = 0.01) in limited SSc, and negative association with esophageal hypomotility (p < 0.01) and ANA positivity (p = 0.02) in diffuse SSc. Multiple regression analyses showed that myopathy was independently associated with previous exposure to EF (OR = 2.09; 95% CI 1.15-3.82), especially silica exposure (OR = 3.09; 95% CI 1.67-5.73). This study showed that SSc patients with previous exposure to EF may have some specific clinical characteristics, mainly a higher frequency of myopathy, also showing more severe ILD, preferably in male and African-Brazilian patients, associated with a lower frequency of ANA positivity.
- Distal radius and tibia bone microarchitecture impairment in female patients with diffuse systemic sclerosis(2019) SAMPAIO-BARROS, M. M.; ALVARENGA, J. C.; TAKAYAMA, L.; ASSAD, A. P. L.; SAMPAIO-BARROS, P. D.; PEREIRA, R. M. R.A Summary Radius and tibia bone microarchitecture, analyzed through a high-resolution peripheral quantitative computed tomography, were significantly impaired in female patients with diffuse systemic sclerosis compared with healthy controls. Acroosteolysis, quality of life-grip strength, hand disability, and disease duration were significantly associated with this bone deterioration. Introduction The effect of diffuse systemic sclerosis (dSSc) on the bone is not completely understood. The objective of this study was to analyze the volumetric bone mineral density (vBMD), microarchitecture, and biomechanical parameters at the distal radius and tibia using high-resolution peripheral quantitative computed tomography (HR-pQCT, XtremeCT) in female patients with dSSc and identify clinical and laboratory variables associated with these parameters. Methods Thirty-eight women with dSSc and 76 healthy controls were submitted to HR-pQCT at the distal radius and tibia. Clinical and laboratory findings, bone mineral density(BMD), nailfold capillaroscopy (NC), total passive range of motion(ROM), and quality of life (health assessment questionnaire-HAQ) were associated with HR-pQCT (Scanco Medical AG, Bruttisellen, Switzerland) parameters. Multiple linear regression models adjusted for clinical and laboratory variables, ROM and HAQ, were performed. Results Density, microarchitecture, and biomechanical parameters at the distal radius and tibia were significantly impaired in dSSc patients compared with healthy controls (p < 0.001). Multiple linear regression models showed that lower trabecular density (Tb.vBMD) (radius R-2 = 0.561, p = 0.002; and tibia R-2 = 0.533, p = 0.005), and lower trabecular number (Tb.N) (tibia R-2 = 0.533, p = 0.005) were significantly associated with acroosteolysis. Higher trabecular separation (Tb.Sp) was associated with disease duration and higher HAQ-grip strength (radius R-2 = 0.489, p = 0.013), while cortical density (Ct.vBMD) was associated with ROM (radius R-2 = 0.294, p = 0.002). Conclusion Bone microarchitecture in patients with dSSc, analyzed through HR-pQCT, showed impairment of trabecular and cortical bone at distal radius and tibia. Variables associated with hand involvement (acroosteolysis, quality of life-grip strength, and ROM) and disease duration may be considered prognostic factors of this bone impairment.
- Symptomatic fractures in systemic sclerosis: A case-control study(2023) SAMPAIO-BARROS, Marilia M.; BORTOLUZZO, Adriana B.; SILVA, Henrique Carrico da; LUPPINO-ASSAD, Ana Paula; PEREIRA, Rosa Maria R.; SAMPAIO-BARROS, Percival D.This case-control study analyzed risk factors for symptomatic fractures in a group of 52 patients with systemic sclerosis compared with a group of 104 patients without fractures, matched for sex and age, who were attended at a single systemic sclerosis outpatient clinic from 2010 to 2020. Fractures affected predominantly vertebral (65.4%), rib (13.5%), and hip (7.7%) joints, while the mean age of fracture was 55.3 +/- 9.5 years. Age at disease onset, age at diagnosis, disease duration, age at menarche, and age at menopause were similar in both groups, and 58.9% of the patients were menopausal at the time of the fracture. The presence of fractures had a significant association with densitometric osteoporosis (p < 0.001), lower weight (p = 0.032), and bone mineral index (p = 0.044), anti-RNA polymerase III (p = 0.040), use of corticosteroids (p = 0.019), and bisphosphonates (p < 0.001), as well as with densitometric T-scores of lumbar spine (p < 0.001), femoral neck (p = 0.025), and total hip (p = 0.013). Multivariate analysis showed that the variables significantly associated with fractures were high doses of corticosteroids (odds ratio = 4.10; 95% confidence interval = 1.290-13.090; p = 0.017), bisphosphonates (odds ratio = 3.91; 95% confidence interval = 1.699-8.984; p = 0.001), negative anti-Scl70 (OR = 0.34; 95% confidence interval = 0.124-0.943; p = 0.038), and lumbar T-score (odds ratio = 0.39; 95% confidence interval = 0.034-0.460; p = 0.010). In conclusion, symptomatic fractures were associated predominantly with lower bone mineral density of lumbar spine and use of high doses of corticosteroids and bisphosphonates in this cohort.