MARCO FELIPE CASTRO DA SILVA
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- INVASIVE FUNGAL INFECTIONS SURVEY IN 852 CHILDHOOD-ONSET SYSTEMIC LUPUS ERYTHEMATOSUS: A MULTICENTER COHORT(2015) SILVA, M. F.; FERRIANI, M. P.; TERRERI, M. T.; PEREIRA, R. M.; MAGALHAES, C. S.; BONFA, E.; CAMPOS, L. M.; OKUDA, E. M.; APPENZELLER, S.; FERRIANI, V. P.; BARBOSA, C. M.; RAMOS, V. C.; LOTUFO, S.; SILVA, C. A.
article 10 Citação(ões) na Scopus PENILE AND SCROTUM SWELLING IN JUVENILE DERMATOMYOSITIS(2011) SALLUM, Adriana Maluf Elias; SILVA, Marco Felipe Castro; MICHELIN, Cintia Maria; DUARTE, Ricardo Jordao; BARONI, Ronaldo Hueb; AIKAWA, Nadia Emi; SILVA, Clovis ArturEdema is a well-known feature of juvenile dermatomyositis (JDM). However, to our knowledge localized penile and scrotum swelling was not previously reported. During a 27-year period, 5,506 patients were followed up at the Pediatric Rheumatology Unit of our University Hospital and 157 patients (2.9%) had JDM. One of them (0.6%) had concomitant localized penile and scrotum swelling. He had severe disease activity since he was 7-year-old, manifested by diffuse cutaneous vasculitis, recurrent localized edema (limbs or face) and only one episode of generalized edema. At the age of 10, he presented edema of the genitalia associated with mild skin erythema. Penis, scrotum and testicular ultrasound as well as magnetic resonance imaging showed skin edema without testicular involvement. He was taking prednisone, methotrexate, cyclosporin, hydroxychloroquine and thalidomide. Improvement of skin rash, penile and scrotum swelling was noticed only with rituximab therapy. No adverse event was observed during anti-CD20 infusions and after six months of follow up. Penile and scrotum edema was a rare manifestation of JDM which improved with anti-CD20 monoclonal antibody treatment.conferenceObject Digital vasculitis survey in 852 childhood-onset systemic lupus erythematosus patients: a multicenter cohort study(2015) SAKAMOTO, A. P.; SILVA, C. A.; SILVA, M. F. C.; LOPES, A. S.; FRAGA, M. M.; BONFA, E.; MAGALHAES, C. S.; PEREIRA, R. M. R.; LEN, C. A.; TERRERI, M. T.article 6 Citação(ões) na Scopus Laryngotracheal stenosis requiring emergency tracheostomy as the first manifestation of childhood-relapsing polychondritis(2013) BUSCATTI, Izabel M.; GIACOMIN, Maria Fernanda A.; SILVA, Marco Felipe C.; CAMPOS, Lucia M. A.; SALLUM, Adriana M. E.; SILVA, Clovis A.Relapsing polychondritis is a rare childhood disorder of unknown etiology, characterized by inflammatory; recurrent and destructive cartilage lesions. The chondritis could be widespread and involves generally laryngeal and auricular hyaline cartilages. We described a 9 years and 4 months old girl, who presented recurrent acute laryngotracheitis and laryngotracheal stenosis, which were the first manifestations of relapsing polychondritis, and was submitted to emergency tracheostomy. She also had ear condritis and arthritis, being treated with prednisolone and methotrexate. In conclusion, we reported a rare case of relapsing polychondritis that presented a life-threatening laryngo-tracheo-bronchial disorder requiring tracheostomy. We suggest that the diagnosis of relapsing polychondritis should be considered for patients who present recurrent acute laryngotracheitis with other types of condritis, as well as musculoskeletal manifestations.- Features of 847 Childhood-Onset Systemic Lupus Erythematosus Patients in Three Age Groups at Diagnosis: A Brazilian Multicenter Study(2016) GOMES, Roberta C.; SILVA, Marco F.; KOZU, Katia; BONFA, Eloisa; PEREIRA, Rosa M.; TERRERI, Maria T.; MAGALHAES, Claudia S.; SACCHETTI, Silvana B.; MARINI, Roberto; FRAGA, Melissa; CARVALHO, Luciana M.; BARBOSA, Cassia M.; CARNEIRO-SAMPAIO, Magda; SILVA, Clovis A.ObjectiveTo evaluate demographic data and clinical and laboratory features at disease diagnosis in 3 different age groups of childhood-onset systemic lupus erythematosus (SLE): group A, early-onset (<6 years); group B, school age (6 to <12 years); and group C, adolescent (12 to <18 years). MethodsThis was a Brazilian multicenter cohort retrospective study in 10 pediatric rheumatology centers, including 847 childhood-onset SLE patients. ResultsPatients were divided into 3 groups: group A with 39 patients (4%), group B with 395 patients (47%), and group C with 413 patients (49%). Of 39 childhood-onset SLE patients in group A, 3 (8%) were ages <2 years, 4 (10%) were 2 to <3 years, and 32 (82%) were 3 and <6 years. A total of 74 childhood-onset SLE patients were analyzed for C1q levels, and complete C1q deficiency was observed in 3 of 74 patients (4%), all in group A. Groups were similar regarding high frequencies of female sex, nephritis, neuropsychiatric involvement, Systemic Lupus Erythematosus Disease Activity Index 2000 score 8, autoantibody profile, elevated acute phase proteins, and low complement levels (P>0.05). However, the frequency of fever (78% versus 61% versus 47%; P<0.0001), hepatomegaly (42% versus 29% versus 14%; P<0.0001), splenomegaly (28% versus 12% versus 4%; P<0.0001), and discoid lupus (13% versus 4% versus 4%; P=0.020) was significantly higher in group A compared to groups B and C. The frequency of weight loss >2 kg (19% versus 28% versus 36%; P=0.017), photosensitivity (34% versus 41% versus 51%; P=0.006), leukopenia <4,000/mm(3) (14% versus 25% versus 30%; P=0.048), and lymphopenia <1,500/mm(3) (22% versus 41% versus 47%; P=0.011) was significantly lower in group A. ConclusionOur large multicenter study identified the finding that the initial appearance of childhood-onset SLE is characterized by comparable high frequency of internal organ involvement and some distinct clinical and laboratory features in early-onset and adolescent groups.
- Initial Benchmarking of the Quality of Medical Care of Children and Adolescents with Lupus(2014) ZAAL, Ahmad I.; APPENZELLER, Simone; HARRIS, Julia G.; SILVA, Marco F.; LEE, Jiha; CENTEVILLE, Maraisa; LIU, HaiMei; PENDL, Joshua D.; HUGGINS, Jennifer L.; JOHNSON, Anne; SILVA, Clovis A.; BRUNNER, Hermine
- Chronic Spontaneous Urticaria: A Survey of 852 Cases of Childhood-Onset Systemic Lupus Erythematosus(2015) FERRIANI, Mariana Paes Leme; SILVA, Marco Felipe Castro; PEREIRA, Rosa Maria Rodrigues; TERRERI, Maria Teresa; MAGALHAES, Claudia Saad; BONFA, Eloisa; PASTORINO, Antonio C.; SANTOS, Maria Carolina dos; APPENZELLER, Simone; FERRIANI, Virginia Paes Leme; LEN, Claudio Arnaldo; SALLUM, Adriana Maluf Elias; LIBORIO, Jonatas; CASTRO, Tania Caroline Monteiro de; SILVA, Clovis ArturBackground: Data regarding the prevalence of chronic spontaneous urticaria (CSU) in childhood-onset systemic lupus erythematosus (cSLE) patients and possible associated factors are limited to a few case reports. The objectives of this study were to assess CSU in a large cSLE population, in order to evaluate the demographic data, clinical manifestations, disease activity/damage, laboratory abnormalities and treatment. Methods: A retrospective multicenter cohort study (Brazilian cSLE group) was performed in 10 Pediatric Rheumatology services and included 852 cSLE patients. CSU was diagnosed according to the guidelines of the European Academy of Allergy and Clinical Immunology, the Global Allergy and Asthma European Network, the European Dermatology Forum and the World Allergy Organization. Patients with CSU (evaluated at urticaria diagnosis) and patients without CSU (evaluated at the last visit) were assessed for lupus clinical/laboratory features and treatment. Results: CSU was observed in 10/852 cSLE patients (1.17%). The median of cSLE duration at urticaria diagnosis was 0 (-3 to 5) years. Comparison of cSLE patients with and without CSU revealed a greater frequency of constitutional symptoms (40 vs. 8%, p = 0.006), reticuloendothelial system involvement (30 vs. 3%, p = 0.003), mucocutaneous (90 vs. 28%, p < 0.0001) and musculoskeletal manifestations (50 vs. 6%, p < 0.0001) and methylprednisolone pulse therapy use (60 vs. 9%, p < 0.0001) in the former group. The frequency of immunosuppressive treatment was lower in patients with CSU (p = 0.018). The median SLE Disease Activity Index 2000 (12 vs. 2, p < 0.0001) and erythrocyte sedimentation rate (40 vs. 19 mm/1st hour, p = 0.024), was higher in patients with CSU. Conclusions: To our knowledge, this is the first study with evidence that CSU may be linked to cSLE. We also demonstrated that this particular skin manifestation occurs predominantly at disease onset and is associated with lupus moderate/high disease activity without major organ involvement. (C) 2015 S. Karger AG, Basel
- Invasive aspergillosis: a severe infection in juvenile systemic lupus erythematosus patients(2012) SILVA, M. F.; RIBEIRO, A. S.; FIOROT, F. J.; AIKAWA, N. E.; LOTITO, A. P.; CAMPOS, L. M.; MAUAD, T.; SILVA, C. A.Infections are an important cause of morbidity and mortality in juvenile systemic lupus erythematosus (JSLE). Among them, invasive aspergillosis (IA), which is usually related to immunosuppressed patients, has been rarely reported in JSLE. From 1983 to 2011, 5604 patients were followed at our institution and 283 (5%) met the American College of Rheumatology (ACR) classification criteria for SLE. Six (2.1%) of our JSLE patients had IA. One of them was previously reported and five will be described herein. Four of them were female. The median age at JSLE diagnosis was 12 years (8-16) and the median interval between diagnosis of JSLE and IA was 6 months (1-38). All had pulmonary involvement and three of them had systemic involvement. The median Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) was 19 (7-22). Diagnosis of IA was performed by isolation of Aspergillus spp., two in bronchoalveolar lavage culture and by way of autopsy in the others. All of them were treated with corticosteroids and/or immunosuppressive drugs at IA diagnosis (azathioprine and/or intravenous cyclophosphamide). They all required treatment in the pediatric intensive care unit with mechanical ventilation and antifungal therapy (fluconazole, amphotericin B, itraconazole and/or voriconazole); nonetheless, none of them survived. In conclusion, this was the first report that evaluated the prevalence of IA in a large population of JSLE patients from a tertiary pediatric hospital, and clearly showed the severity of the outcome, especially in patients with active disease and treated with immunosuppressive agents. This study reinforces the importance of early diagnosis and treatment with certain antifungals, especially in critically ill patients. Lupus (2012) 21, 1011-1016.
conferenceObject Features of 847 Childhood-Onset Systemic Lupus Erythematousus Patients in Three Age-Related Groups at Diagnosis: A Brazilian Multicenter Study(2015) SILVA, Clovis A.; SR., Roberta C. Gomes; SILVA, Marco F.; KOZU, Katia T.; BONFA, Eloisa; PEREIRA, Rosa M. R.; TERRERI, Maria Teresa; SR., Claudia S. Magalhaes; SR., Silvana B. Sacchetti; SR., Roberto Marini; FRAGA, Melissa; SR., Luciana M. Carvalho; BARBOSA, Cassia M.; SR., Magda Carneiro-Sampaio- A Multicenter Study of Invasive Fungal Infections in Patients with Childhood-onset Systemic Lupus Erythematosus(2015) SILVA, Marco F.; FERRIANI, Mariana P.; TERRERI, Maria T.; PEREIRA, Rosa M.; MAGALHAES, Claudia S.; BONFA, Eloisa; CAMPOS, Lucia M.; OKUDA, Eunice M.; APPENZELLER, Simone; FERRIANI, Virginia P.; BARBOSA, Cassia M.; RAMOS, Valeria C.; LOTUFO, Simone; SILVA, Clovis A.Objective. To study the prevalence, risk factors, and mortality of invasive fungal infections (IFI) in patients with childhood-onset systemic lupus erythematosus (cSLE). Methods. A retrospective multicenter cohort study was performed in 852 patients with cSLE from 10 pediatric rheumatology services. An investigator meeting was held and all participants received database training. IFI were diagnosed according to the European Organization for Research and Treatment of Cancer/Invasive Fungal Infections Cooperative Group and the National Institute of Allergy and Infectious Diseases Mycoses Study Group Consensus Group criteria (proven, probable, and possible). Also evaluated were demographic, clinical, and laboratory data, and disease activity [SLE Disease Activity Index 2000 (SLEDAI-2K)], cumulative damage (Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index), treatment, and outcomes. Results. IFI were observed in 33/852 patients (3.9%) with cSLE. Proven IFI was diagnosed in 22 patients with cSLE, probable IFI in 5, and possible IFI in 6. Types of IFI were candidiasis (20), aspergillosis (9), cryptococcosis (2), and 1 each disseminated histoplasmosis and paracoccidioidomycosis. The median of disease duration was lower (1.0 vs 4.7 yrs, p < 0.0001) with a higher current SLEDAI-2K [19.5 (0-44) vs 2 (0-45), p < 0.0001] and current prednisone (PRED) dose [50 (10-60) vs 10 (2-90) mg/day, p < 0.0001] in patients with IFI compared with those without IFI. The frequency of death was higher in the former group (51% vs 6%, p < 0.0001). Logistic regression analysis revealed that SLEDAI-2K (OR 1.108, 95% CI 1.057-1.163, p < 0.0001), current PRED dose (OR 1.046, 95% CI 1.021-1.071, p < 0.0001), and disease duration (OR 0.984, 95% CI 0.969-0.998, p = 0.030) were independent risk factors for IFI (R-2 Nagelkerke 0.425). Conclusion. To our knowledge, this is the first study to characterize IFI in patients with cSLE. We identified that disease activity and current glucocorticoid use were the main risk factors for these life-threatening infections, mainly in the first years of disease course, with a high rate of fatal outcome.