Cargando…

Connective tissue disease with macrophage activation syndrome: A case report

Macrophage activation syndrome (MAS), or secondary hemophagocytic lymphocytosis (sHLH), is a rare systemic inflammatory response syndrome that is fatal. Adult patients lack clear criteria for diagnosis and treatment, primarily derived from guidelines and protocols for treating family hemophagocytic...

Descripción completa

Detalles Bibliográficos
Autores principales: Chen, Qu, Zhang, Qiushuang, Wang, Xuebin
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Lippincott Williams & Wilkins 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9794242/
https://www.ncbi.nlm.nih.gov/pubmed/36595872
http://dx.doi.org/10.1097/MD.0000000000032426
_version_ 1784859994065207296
author Chen, Qu
Zhang, Qiushuang
Wang, Xuebin
author_facet Chen, Qu
Zhang, Qiushuang
Wang, Xuebin
author_sort Chen, Qu
collection PubMed
description Macrophage activation syndrome (MAS), or secondary hemophagocytic lymphocytosis (sHLH), is a rare systemic inflammatory response syndrome that is fatal. Adult patients lack clear criteria for diagnosis and treatment, primarily derived from guidelines and protocols for treating family hemophagocytic lymphocytosis and systemic juvenile idiopathic arthritis (sJIA)-related MAS in children or from retrospective case reports. As a subtype of sHLH, MAS has a clinical presentation like sHLH, but treatment varies. Herein, we report the case of a 40-year-old female with MAS caused by a connective tissue disease. PATIENT CONCERNS: The patient presented to the Rheumatology and Immunology Clinic with recurrent fever and rash, and MAS was confirmed after a series of examinations. The patient had no significant effect after treatment with JAK inhibitors, but after the use of the IL-6 inhibitor tocilizumab, the fever and rash were significantly reduced, and laboratory indicators returned to normal levels. DIAGNOSIS: Considering the patient’s condition and laboratory test results, we judged that the patient had connective tissue disease with MAS. INTERVENTIONS: We gave sequential treatment of tocilizumab. OUTCOMES: ALL indicators are mostly back to normal when the patient was monitored at the outpatient clinic. LESSONS: MAS/HLH lacks clear criteria for diagnosis or treatment in adult patients and is extremely difficult to distinguish from bacterial sepsis or other systemic inflammatory response syndromes. Consequently, early diagnosis and treatment are indispensable for enhancing patient survival.
format Online
Article
Text
id pubmed-9794242
institution National Center for Biotechnology Information
language English
publishDate 2022
publisher Lippincott Williams & Wilkins
record_format MEDLINE/PubMed
spelling pubmed-97942422022-12-28 Connective tissue disease with macrophage activation syndrome: A case report Chen, Qu Zhang, Qiushuang Wang, Xuebin Medicine (Baltimore) 3600 Macrophage activation syndrome (MAS), or secondary hemophagocytic lymphocytosis (sHLH), is a rare systemic inflammatory response syndrome that is fatal. Adult patients lack clear criteria for diagnosis and treatment, primarily derived from guidelines and protocols for treating family hemophagocytic lymphocytosis and systemic juvenile idiopathic arthritis (sJIA)-related MAS in children or from retrospective case reports. As a subtype of sHLH, MAS has a clinical presentation like sHLH, but treatment varies. Herein, we report the case of a 40-year-old female with MAS caused by a connective tissue disease. PATIENT CONCERNS: The patient presented to the Rheumatology and Immunology Clinic with recurrent fever and rash, and MAS was confirmed after a series of examinations. The patient had no significant effect after treatment with JAK inhibitors, but after the use of the IL-6 inhibitor tocilizumab, the fever and rash were significantly reduced, and laboratory indicators returned to normal levels. DIAGNOSIS: Considering the patient’s condition and laboratory test results, we judged that the patient had connective tissue disease with MAS. INTERVENTIONS: We gave sequential treatment of tocilizumab. OUTCOMES: ALL indicators are mostly back to normal when the patient was monitored at the outpatient clinic. LESSONS: MAS/HLH lacks clear criteria for diagnosis or treatment in adult patients and is extremely difficult to distinguish from bacterial sepsis or other systemic inflammatory response syndromes. Consequently, early diagnosis and treatment are indispensable for enhancing patient survival. Lippincott Williams & Wilkins 2022-12-23 /pmc/articles/PMC9794242/ /pubmed/36595872 http://dx.doi.org/10.1097/MD.0000000000032426 Text en Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc. https://creativecommons.org/licenses/by/4.0/This is an open access article distributed under the Creative Commons Attribution License 4.0 (CCBY) (https://creativecommons.org/licenses/by/4.0/) , which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle 3600
Chen, Qu
Zhang, Qiushuang
Wang, Xuebin
Connective tissue disease with macrophage activation syndrome: A case report
title Connective tissue disease with macrophage activation syndrome: A case report
title_full Connective tissue disease with macrophage activation syndrome: A case report
title_fullStr Connective tissue disease with macrophage activation syndrome: A case report
title_full_unstemmed Connective tissue disease with macrophage activation syndrome: A case report
title_short Connective tissue disease with macrophage activation syndrome: A case report
title_sort connective tissue disease with macrophage activation syndrome: a case report
topic 3600
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9794242/
https://www.ncbi.nlm.nih.gov/pubmed/36595872
http://dx.doi.org/10.1097/MD.0000000000032426
work_keys_str_mv AT chenqu connectivetissuediseasewithmacrophageactivationsyndromeacasereport
AT zhangqiushuang connectivetissuediseasewithmacrophageactivationsyndromeacasereport
AT wangxuebin connectivetissuediseasewithmacrophageactivationsyndromeacasereport