RESUMO
Hemophagocytic lymphohistiocytosis (HLH) is a rare genetic hyperinflammatory syndrome that occurs early in life. Macrophage activation syndrome (MAS) usually refers to a secondary form of HLH associated with autoimmunity, although there are other causes of secondary HLH, such as infections and malignancy. In this article, we reviewed the concepts, epidemiology, clinical and laboratory features, diagnosis, differential diagnosis, prognosis, and treatment of HLH and MAS. We also reviewed the presence of MAS in the most common autoimmune diseases that affect children. Both are severe diseases that require prompt diagnosis and treatment to avoid morbidity and mortality.
Assuntos
Doenças Autoimunes , Linfo-Histiocitose Hemofagocítica , Síndrome de Ativação Macrofágica , Criança , Humanos , Síndrome de Ativação Macrofágica/diagnóstico , Síndrome de Ativação Macrofágica/etiologia , Linfo-Histiocitose Hemofagocítica/diagnóstico , Linfo-Histiocitose Hemofagocítica/complicações , Doenças Autoimunes/complicações , Diagnóstico DiferencialRESUMO
Abstract Hemophagocytic lymphohistiocytosis (HLH) is a rare genetic hyperinflammatory syndrome that occurs early in life. Macrophage activation syndrome (MAS) usually refers to a secondary form of HLH associated with autoimmunity, although there are other causes of secondary HLH, such as infections and malignancy. In this article, we reviewed the concepts, epidemiology, clinical and laboratory features, diagnosis, differential diagnosis, prognosis, and treatment of HLH and MAS. We also reviewed the presence of MAS in the most common autoimmune diseases that affect children. Both are severe diseases that require prompt diagnosis and treatment to avoid morbidity and mortality.
Assuntos
Humanos , Masculino , Criança , Artrite Juvenil/diagnóstico , Sinovite/diagnóstico , Articulação do Joelho/patologia , Artrite Juvenil/complicações , Sinovite/complicações , Sinovite/tratamento farmacológico , Biópsia , Imageamento por Ressonância Magnética , Metotrexato/uso terapêutico , Ultrassonografia , Antirreumáticos/uso terapêutico , Articulação do Joelho/diagnóstico por imagemAssuntos
Artrite Juvenil/diagnóstico , Articulação do Joelho/patologia , Sinovite/diagnóstico , Antirreumáticos/uso terapêutico , Artrite Juvenil/complicações , Biópsia , Criança , Humanos , Articulação do Joelho/diagnóstico por imagem , Imageamento por Ressonância Magnética , Masculino , Metotrexato/uso terapêutico , Sinovite/complicações , Sinovite/tratamento farmacológico , UltrassonografiaRESUMO
Riziform bodies are structures formed by fibrin and cells that can be found in the synovial fluid or attached to the synovium, and have this denomination due to its rice grain-like appearance. They have already been described in several diseases such as tuberculous arthritis, rheumatoid arthritis, and rarely in juvenile idiopathic arthritis (JIA). This is the case of a boy with a 4-month course of chronic monoarthritis of the left knee, with family history of sarcoidosis in which diagnostic investigation showed the presence of these riziform bodies in the synovial biopsy. Diagnostic investigation ruled out sarcoidosis, tuberculosis and malignancies, establishing the diagnosis of JIA. Our objective was to describe what we believe is the 9th case reported on the presence of riziform bodies in JIA, which are probably underdiagnosed, and should be considered mainly in cases of severe arthritis of difficult medical treatment.