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DOI: 10.1016/j.rcreue.2021.07.001
Macrophage activation syndrome as a result of rheumatological diseases: Report of 4 cases
Síndrome de activación macrofágica como desenlace de enfermedades reumatológicas: reporte de 4 casos
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Lina María Saldarriaga Riveraa,b,
Corresponding author
vasculitisreumato@gmail.com

Corresponding author.
, Nicolás Sánchez-Ramíreza, Laura Rodríguez-Condea, Diana Paola Tejedor-Restrepoa
a Internal Medicine Research Group, School of Medicine, Technological University of Pereira, Pereira, Colombia
b Service of Rheumatology, Clínica los Rosales, Pereira, Colombia
Received 23 June 2019. Accepted 21 April 2020
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Table 1. Clinical, serological and treatment characteristics of the patients.
Table 2. Parodi diagnostic criteria for macrophage activation syndrome.
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Abstract

Macrophage activation syndrome (MAS) is a serious complication of several rheumatic disorders, among which are the systemic juvenile idiopathic arthritis, Still’s disease and systemic lupus erythematosus. This syndrome is part of the Acquired Haemophagocytic Lymphohistiocytoses, and is a potentially fatal disease, with difficulty in its identification and a lack of consensus regarding its management. A series of cases are described of patients with macrophage activation syndrome, explaining their diagnostic process, their relationship with rheumatic diseases, their monitoring and treatment, as well as the results of different management schemes.

Keywords:
Macrophage activation syndrome
Haemophagocytic lymphohistiocytosis
Systemic lupus erythematosus
Antiphospholipid syndrome
Rheumatic diseases
Arthritis
Resumen

El síndrome de activación macrofágica (SAM) es una grave complicación de varias entidades reumáticas entre las que se encuentran la artritis idiopática juvenil sistémica, enfermedad de Still y lupus eritematoso sistémico. Este síndrome forma parte de las linfohistiocitosis hemofagocíticas adquiridas y constituye una enfermedad potencialmente mortal, con dificultad en su identificación y carencia de consensos en cuanto a su manejo. Describimos una serie de casos de pacientes con SAM, exponiendo su proceso diagnóstico, su relación con las enfermedades reumáticas de base, su seguimiento y tratamiento, así como los resultados de diferentes esquemas de manejo.

Palabras clave:
Síndrome de activación macrofágica
Linfohistiocitosis hemofagocítica
Lupus eritematoso sistémico
Síndrome antifosfolípido
Enfermedades reumáticas
Artritis

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