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Abstract(s)
Introdução: A Esclerose Sistémica é uma patologia rara caracterizada por alterações
imunológicas, vasculares e fibróticas da pele e de órgãos internos. É uma doença de
origem autoimune, que pode ter um curso crónico e progressivo e que apresenta taxas de
incapacidade significativas.
Métodos: Realização de um estudo transversal, retrospetivo e de caráter observacional de
doentes com o diagnóstico de Esclerose Sistémica (ICD9 – 7101), seguidos na Unidade de
Reumatologia do Hospital Amato Lusitano (Castelo Branco). Foram avaliadas as seguintes
variáveis: características epidemiológicas da amostra, idade do diagnóstico e o tempo até
ao diagnóstico, as diferentes classificações em subgrupos, o padrão de anticorpos e
parâmetros laboratoriais para a inflamação, a manifestação inicial, as manifestações
cutâneas, vasculares e articulares e a terapêutica em curso. Após a colheita de todos os
dados foi realizada uma análise descritiva, justificável pela reduzida amostra do estudo.
Resultados: A amostra era constituída por 24 doentes (10 com a forma cutânea limitada, 6
com a forma cutânea difusa, 7 com Pré-esclerodermia e 1 com Esclerose Sistémica sem
escleroderma). 22 pacientes eram do sexo feminino. A média de idade do diagnóstico foi
de 55,5 anos.
O padrão de anticorpos era liderado pelos anticorpos anti-nucleares (95,7%), sendo que
destes, os anticorpos anti-centrómero e anti-Scl-70 apresentavam-se como mais
prevalentes no perfil imunológico da doença.
As manifestações clínicas estudadas nesta amostra foram o envolvimento cutâneo,
vascular e articular. A manifestação mais comum foi o fenómeno de Raynaud (75%),
seguida do espessamento cutâneo (45,8%) e do envolvimento articular (41,7%). Como
primeira manifestação da doença, existiu também uma predominância do fenómeno de
Raynaud, sendo este o sintoma inicial em mais de metade dos pacientes (66,7%).
A terapêutica vasodilatadora foi a mais frequentemente usada (62,5%), nomeadamente a
Pentoxifilina (41,7%). O tratamento “imunossupressor” foi prescrito a 29,1% dos pacientes
desta amostra e a maioria destes realizava Hidroxicloroquina, atendendo ao envolvimento
articular. Os corticosteróides e os anti-inflamatórios não esteróides foram usados em ½ e
¼ dos indivíduos da amostra, respetivamente.
Conclusão: Verificou-se que cada vez é mais importante prestar atenção a característicaschave como: um perfil de auto-anticorpos específico ou o aparecimento isolado do fenómeno de Raynaud, para que seja mais fácil fazer o diagnóstico precoce de doentes com
Esclerose Sistémica.
Também o tratamento destes doentes assenta na gestão das diferentes manifestações, pelo
que a escolha da prescrição medicamentosa se baseava não só nas recomendações atuais,
mas também na experiência do clínico.
Introduction: Systemic Sclerosis is a rare disease characterized by immunological, vascular and fibrotic changes of the skin and internal organs. It is a disease with an autoimmune condition, which can have a chronic and progressive course and has significant disability rates. Methods: Cross-sectional, retrospective and observational study of patient with the diagnosis of Systemic Sclerosis (ICD9 - 7101), followed at the Rheumatology Unit of Hospital Amato Lusitano (Castelo Branco). The following variables were evaluated: epidemiological characteristics of the sample, age of diagnosis and time to diagnosis, different subgroup classifications, the pattern of antibodies and laboratory parameters for inflammation, initial manifestation, cutaneous, vascular and joint manifestations and the ongoing therapy. After collecting all data, a descriptive analysis was performed, justified by the small sample of the study. Results: The sample consisted of 24 patients (10 with limited cutaneous form, 6 with diffuse cutaneous form, 7 with pre-scleroderma and 1 with systemic sclerosis without scleroderma). 22 patients were female. The average age of diagnosis was 55.5 years. The antibody pattern was led by antinuclear antibodies. (95.7%), of which anticentromere and anti-Scl-70 were more prevalent in the immune profile of the disease. The clinical manifestations studied were cutaneous, vascular and articular involvement. The most common manifestation was Raynaud's phenomenon (75%), followed by skin thickening (45.8%) and joint involvement (41.7%). As first manifestation of the disease, there was also a predominance of Raynaud's phenomenon, which was the initial symptom in more than half of the patients (66.7%). Vasodilator therapy was the most frequently used (62.5%), namely Pentoxifylline (41.7%). “Immunosuppressive” treatment was prescribed to 29.1% of the patients in this sample, most of them undergoing Hydroxychloroquine, given to articular involvement. Corticosteroids and non-steroidal anti-inflammatory drugs were used in ½ and ¼ individuals of the sample, respectively. Conclusion: It was found that it is becoming increasingly important to pay attention to key features such as a specific autoantibody profile or the isolated appearance of the Raynaud Phenomenon, to make even easier early diagnosis of Systemic Sclerosis in patients. The treatment of these patients was also based on the management of the different manifestations, so the choice of drug prescription was based not only on current recommendations, but also on the experience of the clinician.
Introduction: Systemic Sclerosis is a rare disease characterized by immunological, vascular and fibrotic changes of the skin and internal organs. It is a disease with an autoimmune condition, which can have a chronic and progressive course and has significant disability rates. Methods: Cross-sectional, retrospective and observational study of patient with the diagnosis of Systemic Sclerosis (ICD9 - 7101), followed at the Rheumatology Unit of Hospital Amato Lusitano (Castelo Branco). The following variables were evaluated: epidemiological characteristics of the sample, age of diagnosis and time to diagnosis, different subgroup classifications, the pattern of antibodies and laboratory parameters for inflammation, initial manifestation, cutaneous, vascular and joint manifestations and the ongoing therapy. After collecting all data, a descriptive analysis was performed, justified by the small sample of the study. Results: The sample consisted of 24 patients (10 with limited cutaneous form, 6 with diffuse cutaneous form, 7 with pre-scleroderma and 1 with systemic sclerosis without scleroderma). 22 patients were female. The average age of diagnosis was 55.5 years. The antibody pattern was led by antinuclear antibodies. (95.7%), of which anticentromere and anti-Scl-70 were more prevalent in the immune profile of the disease. The clinical manifestations studied were cutaneous, vascular and articular involvement. The most common manifestation was Raynaud's phenomenon (75%), followed by skin thickening (45.8%) and joint involvement (41.7%). As first manifestation of the disease, there was also a predominance of Raynaud's phenomenon, which was the initial symptom in more than half of the patients (66.7%). Vasodilator therapy was the most frequently used (62.5%), namely Pentoxifylline (41.7%). “Immunosuppressive” treatment was prescribed to 29.1% of the patients in this sample, most of them undergoing Hydroxychloroquine, given to articular involvement. Corticosteroids and non-steroidal anti-inflammatory drugs were used in ½ and ¼ individuals of the sample, respectively. Conclusion: It was found that it is becoming increasingly important to pay attention to key features such as a specific autoantibody profile or the isolated appearance of the Raynaud Phenomenon, to make even easier early diagnosis of Systemic Sclerosis in patients. The treatment of these patients was also based on the management of the different manifestations, so the choice of drug prescription was based not only on current recommendations, but also on the experience of the clinician.
Description
Keywords
Doença Rara Esclerose Sistémica Fenómeno de Raynaud Perfil Imunológico Reumatologia