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Abordagem terapêutica na dermatomiosite
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Abstract(s)
A Dermatomiosite é uma doença autoimune multissistémica rara, classificada como
miopatia inflamatória idiopática. As suas principais características incluem manifestações
cutâneas e inflamação das fibras musculares, que se traduz numa fraqueza dos músculos
proximais, em especial dos músculos dos ombros, bacia e coxas.
Apresenta uma incidência estimada nos adultos, de aproximadamente 1 a 10 casos novos
por milhão de habitantes/ano, exibindo dois picos de ocorrência: na infância, dos 5 aos 15 anos,
e nos adultos, dos 40 aos 60 anos.
São reconhecidos seis subtipos da patologia, como dermatomiosite primária idiopática,
dermatomiosite amiopática, dermatomiosite juvenil, dermatomiosite associada a outras
doenças do tecido conjuntivo, dermatomiosite associada a neoplasia e dermatomiosite
induzida por fámacos.
O foco desta dissertação assenta na abordagem da atual terapêutica da dermatomiosite,
que por se tratar de uma doença de origem autoimune, não existe uma cura, pelo que o seu
tratamento incide no alívio dos sintomas e na manutenção das capacidades do paciente.
Tendo em vista a obtenção da máxima eficácia no tratamento, a combinação de
terapêuticas farmacológicas tópicas e sistémicas com terapias não farmacológicas e
complementares, como fisioterapia, dieta e suplementação, fazem parte de um regime ideal. O
tratamento farmacológico baseia-se na utilização de corticosteróides, e, caso a resposta não
seja satisfatória, é instituída a segunda linha terapêutica, que compreende essencialmente
agentes imunossupressores e poupadores de corticosteróides. A terapia com imunoglobulina
humana normal intravenosa deve ser considerada em pacientes que não toleram a terapêutica
imunossupressora pelos efeitos adversos que esta acarreta, bem como em pacientes cujos
agentes citotóxicos são contra-indicados. Já o envolvimento cutâneo assenta sobretudo num
regime de tratamento baseado na fotoproteção e na terapêutica tópica anti-inflamatória.
Todavia, o surgimento de fármacos promissores, principalmente de componente biológica,
requer um maior desenvolvimento ao nível da sua investigação de forma a serem incluídos num
regime terapêutico seguro e eficaz.
Dermatomyositis is a rare autoimmune and multisystemic disease classified as an idiopathic inflammatory myopathy. The main characteristics of this disease include cutaneous manifestations and muscle fiber inflammation, which translate in proximal muscle weakness, especially for the shoulders, thigh, and pelvis muscles. This disease presents a estimates incidence in adults of approximately 1 to 10 new cases per million of habitants yearly, exhibiting two spikes: one in childhood from 5 to 15 years of age, and one in adults from 40 to 60 years of age. Six pathologic subtypes are recognized, primary idiopathic dermatomyositis, amyopathic dermatomyositis, juvenile dermatomyositis, dermatomyositis associated with a connective tissue disease, dermatomyositis associated with a malignancy and drug-induced dermatomyositis. The focus for this thesis is on the current approach to dermatomyositis therapeutic. The fact that it is a disease with autoimmune origin makes it so that there is no cure, so current treatment focus on relieving symptoms and maintaining patient capacity. With the end goal of obtaining the maximum efficiency in the treatment, the combination of topical and systemic pharmacological therapeutics with complementary and nonpharmacological therapeutics, like physiotherapy, dieting and supplementation, make for an ideal treatment course. The pharmacological treatment is based mostly on the utilization of corticosteroids and, when the response to this is not satisfactory, a second line of treatment is used. This secondary line of treatment is based essentially in immunosuppressant agents and corticosteroids savers. The therapy with human normal immunoglobulin for intravenous administration should only be considered in a patient who does not tolerate immunosuppressant therapeutics because of adverse effects this therapeutic may cause, as well as patients who have cytotoxic agents contraindicated. The treatment regime for cutaneous manifestations of this disease is based in photoprotections and anti-inflammatory topic therapeutics.
Dermatomyositis is a rare autoimmune and multisystemic disease classified as an idiopathic inflammatory myopathy. The main characteristics of this disease include cutaneous manifestations and muscle fiber inflammation, which translate in proximal muscle weakness, especially for the shoulders, thigh, and pelvis muscles. This disease presents a estimates incidence in adults of approximately 1 to 10 new cases per million of habitants yearly, exhibiting two spikes: one in childhood from 5 to 15 years of age, and one in adults from 40 to 60 years of age. Six pathologic subtypes are recognized, primary idiopathic dermatomyositis, amyopathic dermatomyositis, juvenile dermatomyositis, dermatomyositis associated with a connective tissue disease, dermatomyositis associated with a malignancy and drug-induced dermatomyositis. The focus for this thesis is on the current approach to dermatomyositis therapeutic. The fact that it is a disease with autoimmune origin makes it so that there is no cure, so current treatment focus on relieving symptoms and maintaining patient capacity. With the end goal of obtaining the maximum efficiency in the treatment, the combination of topical and systemic pharmacological therapeutics with complementary and nonpharmacological therapeutics, like physiotherapy, dieting and supplementation, make for an ideal treatment course. The pharmacological treatment is based mostly on the utilization of corticosteroids and, when the response to this is not satisfactory, a second line of treatment is used. This secondary line of treatment is based essentially in immunosuppressant agents and corticosteroids savers. The therapy with human normal immunoglobulin for intravenous administration should only be considered in a patient who does not tolerate immunosuppressant therapeutics because of adverse effects this therapeutic may cause, as well as patients who have cytotoxic agents contraindicated. The treatment regime for cutaneous manifestations of this disease is based in photoprotections and anti-inflammatory topic therapeutics.
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Keywords
Dermatomiosite Autoimune Envolvimento muscular e cutâneo Terapêutica