FERNANDO HENRIQUE CARLOS DE SOUZA
Projetos de Pesquisa
Unidades Organizacionais
P ICHC, 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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Resultados de Busca
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bookPart Miopatias autoimunes sistêmicas(2021) SOUZA, Fernando Henrique Carlos de; MIOSSI, Renata; SHINJO, Samuel KatsuyukibookPart Cuidados pré-imunossupressão(2021) LOPES, Michelle Remião Ugolini; PROENçA, Adriana Coracini Tonacio de; SOUZA, Fernando Henrique Carlos de; MORAES, Júlio César Bertacini de- Brachio-cervical inflammatory myopathy associated with systemic sclerosis. Case series and review of literature(2021) ARAUJO, C. S. R.; MIOSSI, R.; SOUZA, F. H. C. de; COSTA, M. D.; SILVA, A. M. S. da; CAMPOS, E. D.; ZANOTELI, E.; SHINJO, S. K.This study was aimed at describing a case series of brachio-cervical inflammatory myopathy (BCIM) associated with systemic sclerosis (SSc), due to its rarity and limited coverage in published data Another aim was to provide a literature review. We reported four cases of BCIM-SSc from our tertiary center. In addition, we researched the literature and found six articles featuring 17 patients who fit this phenotype. We pooled all cases and reported their features. Most patients were female and had limited SSc, and the median time of BCIM presentation was three years after SSc diagnosis. Asymmetric muscle involvement, scapular winging, dropped head, axial weakness, camptocormia, dysphagia, and dermatomyositis stigmas were common features. All patients had esophageal involvement. Most had positive antinuclear antibody results, a scleroderma pattern in their capillaroscopy images, elevated serum creatine phosphokinase. myopathic electrophysiology, and muscle involvement in magnetic resonance imaging. Muscle histopathological findings varied widely, but in general all showed the presence of lymphoid infiltrates, muscle atrophy. increased MHC-I expression, MAC deposits, vasculopathy, and muscle fiber necrosis. The response to immunosuppressive therapy was highly irregular. BCLM-SSc is a rare disorder that shares many similar phenotypes among the described cases. but has a highly heterogeneous response to treatment. At present. more data on the physiopathology, clinical features. and treatment is still needed.
- The relevance of anti-Jo-1 autoantibodies in patients with definite dermatomyositis(2021) ANDRADE, Vanessa Posener de; SOUZA, Fernando Henrique Carlos De; PINTO, Gustavo Luiz Behrens; SHINJO, Samuel KatsuyukiBackground To assess the prevalence and clinical relevance of anti-Jo-1 autoantibodies in a representative sample of patients with definite dermatomyositis (DM). Methods This retrospective cohort study took place from 2005 to 2020 and assessed 118 adult patients from a tertiary center who were diagnosed with definite DM. A commercial kit was used to detect anti-Jo-1 autoantibodies. Results The presence of anti-Jo-1 autoantibodies was observed in 10 out of 118 (8.5%) patients with definite DM. The following variables were comparable between individuals with and without anti-Jo-1 autoantibodies: age at diagnosis, sex, ethnicity, disease duration, follow-up period, recurrence rate, complete clinical response, death rate, and cancer incidence. There was no difference in clinical features between groups, except for an increased prevalence of ""mechanic's hands,"" joint involvement, and lung disease, as well as a reduced occurrence of skin findings in patients positive for anti-Jo-1 autoantibodies. No anti-Jo-1-positive patients went into remission; they required greater use of glucocorticoids and immunosuppressive drugs. Conclusions Anti-Jo-1 positivity was found in 8.5% of patients with definite DM. This autoantibody was associated with an antisynthetase syndrome phenotype and might predict clinical outcomes in patients with definite DM.