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Doctoral Thesis
DOI
10.11606/T.5.2013.tde-17102013-124203
Document
Author
Full name
Mariana Figueiroa Careta
E-mail
Institute/School/College
Knowledge Area
Date of Defense
Published
São Paulo, 2013
Supervisor
Committee
Romiti, Ricardo (President)
Nico, Marcello Menta Simonsen
Daldon, Patricia Erica Christofoletti
Sato, Emilia Inoue
Sato, Maria Notomi
Title in Portuguese
Estudo prospectivo para avaliar a evolução radiológica de 12 pacientes portadores de esclerodermia da face e perfil demográfico, manifestações clínicas e alterações laboratoriais de 34 casos
Keywords in Portuguese
Doenças do colágeno
Doenças do sistema imune
Encefalopatias/diagnóstico
Esclerodermia localizada
Esclerodermia localizada/epidemiologia
Estudos prospectivos
Face
Imagem por ressonância magnética
Investigação laboratorial
Manifestações neurológicas
Manifestações oculares
Neuroimagem
Seguimento
Sinais e sintomas
Abstract in Portuguese
Introdução: A esclerodermia é rara doença do tecido conectivo que se manifesta através da esclerose cutânea e variável acometimento sistêmico. Duas categorias de esclerodermia são conhecidas: esclerose sistêmica, caracterizada por esclerose cutânea e acometimento visceral e a esclerodermia localizada ou morfeia que classicamente apresenta evolução benigna e autolimitada, confinada a pele e/ou tecidos subjacentes. Estudos recentes demonstram que a forma localizada possa eventualmente apresentar acometimento de órgãos internos e morbidade variável. Objetivo: Neste estudo objetivamos determinar as características demográficas, a prevalência de manifestações sistêmicas e alterações laboratoriais, bem como a associação com doenças autoimunes, em pacientes com esclerodermia da face. Métodos: Pacientes com esclerodermia localizada, incluindo os casos de esclerodermia em golpe de sabre, síndrome de Parry-Romberg e morfeia em placas com acometimento facial, foram avaliados e submetidos à investigação neurológica, incluindo exame clínico neurológico e ressonância magnética de crânio, e avaliação oftalmológica. Após 3 anos, o subgrupo de pacientes disponível para seguimento foi ressubmetido à ressonância magnética. Resultados: Foram estudados 34 pacientes com esclerodermia localizada da face. Deste total, 64,7% apresentavam uma ou mais manifestações extracutâneas, sendo cefaleia a queixa mais frequente, encontrada em 61,8% dos pacientes. Dos 23 pacientes submetidos à avaliação neurológica, 56,5% apresentaram alterações neurológicas possivelmente associadas à esclerodermia. Alterações à ressonância magnética foram observadas em 50% dos casos. O achado mais frequente foi a presença de lesões parenquimatosas com alteração de sinal em 50% dos pacientes. Dos pacientes que apresentavam alteração neurológica, 80% também apresentavam alguma alteração à ressonância magnética. Doze pacientes foram ressubmetidos a novo exame após 3 anos. Em todos os pacientes os achados de imagem se mantiveram inalterados. Durante esse intervalo de 3 anos, 25% dos pacientes apresentaram sinais de atividade da esclerodermia. Quanto à avaliação oftalmológica, 67,9% dos pacientes avaliados apresentaram alteração, sendo os achados mais frequentes a ocorrência de alterações orbiculares da esclerodermia (20,6%) e xeroftalmia (10,7%). Conclusão: Pacientes com esclerodermia localizada da face apresentam alta prevalência de alterações neurológicas e oftalmológicas. Baseado nestes achados, sugerimos que todos os casos de esclerodermia localizada da face devam ser detalhadamente examinados quanto à presença de alterações sistêmicas
Title in English
Prospective study to evaluate the radiological evolution of 12 patients with localized scleroderma of the face and the demographic, clinical and laboratory findings of 34 cases
Keywords in English
Brain diseases/diagnosis
Collagen diseases
Eye manifestations
Face
Follow-up studies
Immune system diseases
Laboratory research
Magnetic resonance imaging
Neuroimaging
Neurologic manifestations
Prospective studies
Scleroderma localized
Scleroderma localized/epidemiology
Signs and symptoms
Abstract in English
Introduction: Scleroderma is a rare connective tissue disease that manifests as skin sclerosis and variable systemic involvement. Two categories of scleroderma are known: systemic sclerosis, characterized by cutaneous sclerosis and visceral involvement and localized scleroderma or morphea which classically presents benign evolution and selflimited, confined to the skin and / or underlying tissue. Recent studies show that the localized form may possibly course with involvement of internal organs and variable morbidity. Objective: This study aimed to determine the demographic characteristics, the prevalence of systemic manifestations and laboratory findings, as well as the association with autoimmune diseases, in patients with scleroderma of the face. Methods: Patients with localized scleroderma, including cases of scleroderma en coup de sabre, Parry-Romberg syndrome and morphea plaque with facial involvement were evaluated and underwent neurological examination, including neurologic examination and magnetic resonance imaging, and ophthalmology evaluation. After 3 years, the subgroup of patients available for follow-up was subjected again to MRI. Results: We studied 34 patients with localized scleroderma of the face. Of this total, 64,7% had one or more extracutaneous manifestation, headache being the most frequent complaint found in 61,8% of patients. Of the 23 patients undergoing neurological evaluation, 56,5% had neurological changes possibly associated with scleroderma. MRI changes were observed in 50% of cases. The most frequent was the presence of parenchymal lesions with signal alteration in 50% of patients. Of the patients who had neurological deficits, 80% also had a change to MRI. Twelve patients were subjected again to another MRI scan after 3 years. In all patients, imaging findings were unchanged. During this interval of 3 years, 25% of patients showed signs of activity of scleroderma. As for ophthalmologic evaluation, 67,9% of patients showed abnormalities, with the most frequent findings being the occurrence of orbicular changes of scleroderma (20.6%) and xerophthalmia (10.7%). Conclusion: Patients with localized scleroderma face have a high prevalence of neurological and ophthalmological changes. Based on these findings, we suggest that all cases of localized scleroderma of the face should be thoroughly examined for the presence of systemic changes
 
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Publishing Date
2013-10-21
 
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