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Púrpura de Henoch-Schönlein : uma entidade benigna? : reflexão a propósito de um caso com envolvimento renal
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A púrpura de Henoch-Schönlein (PHS) é a vasculite mais frequente em idade pediátrica. É particularmente frequente em rapazes de raça caucasiana e durante os meses frios. Apesar da etiopatogénese ser desconhecida, sabe-se que as imunoglobulinas do tipo A1 (IgA1) têm um papel preponderante na doença, observando-se deposição da molécula nos vasos envolvidos.
Apresenta-se como púrpura e restante clínica variável, consoante os tecidos afetados – envolvendo mais frequentemente as articulações, trato gastrointestinal e rins. O diagnóstico da doença é clínico. Alguns exames complementares de diagnóstico podem ajudar no diagnóstico diferencial de outras vasculites.
A melhor estratégia terapêutica não é consensual. Em apresentações de menor gravidade, a terapêutica é de suporte; em apresentações mais severas - abdominal e renal - são utilizados corticoesteróides. É maioritariamente uma doença benigna, autolimitada, com remissão completa; contudo, quando há envolvimento renal - glomerulonefrite ou síndrome nefrótica - existe maior risco de desenvolvimento de doença renal crónica. É importante o follow-up nestas crianças.
É apresentado o caso clínico de uma criança de 10 anos do sexo masculino, que recorreu ao serviço de urgência por púrpura nos membros inferiores, com progressão para face, tronco e membros superiores. Nos membros inferiores evoluiu de forma exuberante, com formação de bolhas. Desenvolveu uma síndrome nefrótica e nefrítica e necessitou de um internamento prolongado para compensação clínica e otimização terapêutica. Dois dias após a alta, foi reinternado por recidiva da síndrome nefrótica, com edema acentuado na face e membros inferiores, no contexto de uma celulite no dorso dos pés secundária às lesões purpúrica. Foi iniciada antibioticoterapia endovenosa e desbridamento das lesões, tendo alta ao fim de dois dias de internamento.
Este caso permite refletir sobre a benignidade da doença uma vez que condicionou um internamento prolongado, necessidade de corticoterapia e acompanhamento em consulta de nefrologia pediátrica.
Henoch-Schönlein Purpura (HSP) is the most frequent vasculitis in pediatric age occurring particularly in Caucasian males and during cold months. Though its pathology is not well known, type A1 immunoglobulins (IgA1) play an important role, with visible deposits on the blood vessels involved. It is presented as purpura and other clinical features depending on affected tissues. It usually affects joints, the gastrointestinal system and kidneys. The diagnosis is based on clinical manifestations; further analysis and exams, as justified, may help in the differential diagnosis of other vasculitis. There is not a consensus on the best therapeutic strategy. Usually, mild presentation is controlled with support therapy; for more serious presentations with abdominal pain and kidney involvement, corticosteroids are required. On most cases, it is a benign, self-limited disease with full remission; however, when there is kidney involvement, namely glomerulonephritis or nephrotic syndrome, the risk of progression to chronic kidney disease is higher. It is important to follow these children in a long term basis. A clinical case is presented: a 10-year old boy goes to the emergency room (ER) with purpura in the lower limbs, which progressed to the face, body and upper limbs; skin blebs developed in the lower limbs. The child developed nephrotic and nephritic syndromes and a prolonged hospital-stay was required for clinical stabilization. Two days after discharge, he sought medical care due to worsening edema in the face and lower limbs, and inflammatory signs on skin lesions on the dorsum of both feet. Intravenous antibiotics and debriding of lesions were necessary; he was discharged after 2 days. The clinical scenario, described above, allows for a reflection on the so-called benign nature of the disease considering the long hospital-stay, corticotherapy and long term follow-up required.
Henoch-Schönlein Purpura (HSP) is the most frequent vasculitis in pediatric age occurring particularly in Caucasian males and during cold months. Though its pathology is not well known, type A1 immunoglobulins (IgA1) play an important role, with visible deposits on the blood vessels involved. It is presented as purpura and other clinical features depending on affected tissues. It usually affects joints, the gastrointestinal system and kidneys. The diagnosis is based on clinical manifestations; further analysis and exams, as justified, may help in the differential diagnosis of other vasculitis. There is not a consensus on the best therapeutic strategy. Usually, mild presentation is controlled with support therapy; for more serious presentations with abdominal pain and kidney involvement, corticosteroids are required. On most cases, it is a benign, self-limited disease with full remission; however, when there is kidney involvement, namely glomerulonephritis or nephrotic syndrome, the risk of progression to chronic kidney disease is higher. It is important to follow these children in a long term basis. A clinical case is presented: a 10-year old boy goes to the emergency room (ER) with purpura in the lower limbs, which progressed to the face, body and upper limbs; skin blebs developed in the lower limbs. The child developed nephrotic and nephritic syndromes and a prolonged hospital-stay was required for clinical stabilization. Two days after discharge, he sought medical care due to worsening edema in the face and lower limbs, and inflammatory signs on skin lesions on the dorsum of both feet. Intravenous antibiotics and debriding of lesions were necessary; he was discharged after 2 days. The clinical scenario, described above, allows for a reflection on the so-called benign nature of the disease considering the long hospital-stay, corticotherapy and long term follow-up required.
Descrição
Trabalho Final do Curso de Mestrado Integrado em Medicina, Faculdade de Medicina, Universidade de Lisboa, 2018
Palavras-chave
Púrpura de Henoch-Schönlein Biópsia renal Glomerulonefrite Síndrome nefrótica Corticoterapia Pediatria