Macrophage activation syndrome: early diagnosis is key

Butsabong Lerkvaleekul, Soamarat Vilaiyuk Division of Rheumatology, Department of Pediatrics, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand Abstract: Macrophage activation syndrome (MAS) is a life-threatening condition, and it is a subset of hemophagocytic lymphohis...

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Autores principales:	Lerkvaleekul B, Vilaiyuk S
Formato:	article
Lenguaje:	EN
Publicado:	Dove Medical Press 2018
Materias:	Hemophagocytic lymphohistiocytosis Systemic juvenile idiopathic arthritis Systemic lupus erythematosus Kawasaki disease Autoimmune diseases Early diagnosis Diseases of the musculoskeletal system RC925-935
Acceso en línea:	https://doaj.org/article/e3c2fd07d46843b0ad95d4025b883514
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spelling	oai:doaj.org-article:e3c2fd07d46843b0ad95d4025b8835142021-12-02T02:13:28ZMacrophage activation syndrome: early diagnosis is key1179-156Xhttps://doaj.org/article/e3c2fd07d46843b0ad95d4025b8835142018-08-01T00:00:00Zhttps://www.dovepress.com/macrophage-activation-syndrome-early-diagnosis-is-key-peer-reviewed-article-OARRRhttps://doaj.org/toc/1179-156XButsabong Lerkvaleekul, Soamarat Vilaiyuk Division of Rheumatology, Department of Pediatrics, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand Abstract: Macrophage activation syndrome (MAS) is a life-threatening condition, and it is a subset of hemophagocytic lymphohistiocytosis (HLH). The clinical features include a persistent high-grade fever, hepatosplenomegaly, lymphadenopathy, hemorrhagic manifestations, and a sepsis-like condition. From the clinical features, it is usually difficult to differentiate between a true sepsis, disease flare-ups, or MAS. Although the laboratory abnormalities are similar to those of a disseminated intravascular coagulation, which shows pancytopenia, coagulopathy, hypofibrinogenemia, and an elevated d-dimer test, it can also be a late stage of MAS. Currently, MAS is still underrecognized and usually results in delayed in diagnosis, which leads to high morbidity and mortality. This literature review was conducted in the context of the clinical manifestations and the laboratory abnormalities in MAS, which might provide some clues for an early diagnosis. The best ways for an early recognition and a satisfactory diagnosis were based on the relative changes in the overall parameters from the baseline, together with a thorough and continuous physical examination for these kinds of patients. At present, diagnostic criteria have been proposed for HLH, MAS-associated systemic juvenile idiopathic arthritis, and an MAS-associated systemic lupus erythematosus. Therefore, selecting the proper diagnostic criteria for use is essential because not all of the criteria are suitable for every autoimmune disease. Keywords: hemophagocytic lymphohistiocytosis, systemic juvenile idiopathic arthritis, systemic lupus erythematosus, Kawasaki disease, autoimmune diseases, early diagnosisLerkvaleekul BVilaiyuk SDove Medical PressarticleHemophagocytic lymphohistiocytosisSystemic juvenile idiopathic arthritisSystemic lupus erythematosusKawasaki diseaseAutoimmune diseasesEarly diagnosisDiseases of the musculoskeletal systemRC925-935ENOpen Access Rheumatology: Research and Reviews, Vol Volume 10, Pp 117-128 (2018)
institution	DOAJ
collection	DOAJ
language	EN
topic	Hemophagocytic lymphohistiocytosis Systemic juvenile idiopathic arthritis Systemic lupus erythematosus Kawasaki disease Autoimmune diseases Early diagnosis Diseases of the musculoskeletal system RC925-935
spellingShingle	Hemophagocytic lymphohistiocytosis Systemic juvenile idiopathic arthritis Systemic lupus erythematosus Kawasaki disease Autoimmune diseases Early diagnosis Diseases of the musculoskeletal system RC925-935 Lerkvaleekul B Vilaiyuk S Macrophage activation syndrome: early diagnosis is key
description	Butsabong Lerkvaleekul, Soamarat Vilaiyuk Division of Rheumatology, Department of Pediatrics, Faculty of Medicine Ramathibodi Hospital, Mahidol University, Bangkok, Thailand Abstract: Macrophage activation syndrome (MAS) is a life-threatening condition, and it is a subset of hemophagocytic lymphohistiocytosis (HLH). The clinical features include a persistent high-grade fever, hepatosplenomegaly, lymphadenopathy, hemorrhagic manifestations, and a sepsis-like condition. From the clinical features, it is usually difficult to differentiate between a true sepsis, disease flare-ups, or MAS. Although the laboratory abnormalities are similar to those of a disseminated intravascular coagulation, which shows pancytopenia, coagulopathy, hypofibrinogenemia, and an elevated d-dimer test, it can also be a late stage of MAS. Currently, MAS is still underrecognized and usually results in delayed in diagnosis, which leads to high morbidity and mortality. This literature review was conducted in the context of the clinical manifestations and the laboratory abnormalities in MAS, which might provide some clues for an early diagnosis. The best ways for an early recognition and a satisfactory diagnosis were based on the relative changes in the overall parameters from the baseline, together with a thorough and continuous physical examination for these kinds of patients. At present, diagnostic criteria have been proposed for HLH, MAS-associated systemic juvenile idiopathic arthritis, and an MAS-associated systemic lupus erythematosus. Therefore, selecting the proper diagnostic criteria for use is essential because not all of the criteria are suitable for every autoimmune disease. Keywords: hemophagocytic lymphohistiocytosis, systemic juvenile idiopathic arthritis, systemic lupus erythematosus, Kawasaki disease, autoimmune diseases, early diagnosis
format	article
author	Lerkvaleekul B Vilaiyuk S
author_facet	Lerkvaleekul B Vilaiyuk S
author_sort	Lerkvaleekul B
title	Macrophage activation syndrome: early diagnosis is key
title_short	Macrophage activation syndrome: early diagnosis is key
title_full	Macrophage activation syndrome: early diagnosis is key
title_fullStr	Macrophage activation syndrome: early diagnosis is key
title_full_unstemmed	Macrophage activation syndrome: early diagnosis is key
title_sort	macrophage activation syndrome: early diagnosis is key
publisher	Dove Medical Press
publishDate	2018
url	https://doaj.org/article/e3c2fd07d46843b0ad95d4025b883514
work_keys_str_mv	AT lerkvaleekulb macrophageactivationsyndromeearlydiagnosisiskey AT vilaiyuks macrophageactivationsyndromeearlydiagnosisiskey
_version_	1718402625492221952

Macrophage activation syndrome: early diagnosis is key

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