Cargando…

Acute respiratory distress syndrome associated with macrophage activation syndrome in systemic lupus erythematosus: A case report and literature review

RATIONALE: Previous treatment for macrophage activation syndrome (MAS) includes high-dose intravenous methylprednisolone along with intravenous immunoglobulin G. If MAS worsened, second-line therapy consisted of anakinra; if the disease remained refractory, third-line therapy with etoposide was cons...

Descripción completa

Detalles Bibliográficos
Autores principales: Chang, En-Shuo, Yu, Han-Hua, Wu, Chiao-En, Chan, Tien-Ming
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Lippincott Williams & Wilkins 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8812624/
https://www.ncbi.nlm.nih.gov/pubmed/35119005
http://dx.doi.org/10.1097/MD.0000000000028612
_version_ 1784644693923987456
author Chang, En-Shuo
Yu, Han-Hua
Wu, Chiao-En
Chan, Tien-Ming
author_facet Chang, En-Shuo
Yu, Han-Hua
Wu, Chiao-En
Chan, Tien-Ming
author_sort Chang, En-Shuo
collection PubMed
description RATIONALE: Previous treatment for macrophage activation syndrome (MAS) includes high-dose intravenous methylprednisolone along with intravenous immunoglobulin G. If MAS worsened, second-line therapy consisted of anakinra; if the disease remained refractory, third-line therapy with etoposide was considered. In addition, cyclosporine A plays a role in early MAS and in preventing recurrence. Some studies have reported the use of cytokine-targeting agents other than anakinra, such as canakinumab, tocilizumab, abatacept, and tofacitinib. PATIENT CONCERNS: The patient with systemic lupus erythematosus (SLE) had an uncommon combination of intermittent fever, hyperferritinemia, hypertriglyceridemia, jaundice, and significantly abnormal liver function test results. The patient reported a history of daily fever of 38 to 39°C, painful oral ulcer, anorexia, abdominal bloating, diarrhea, and malar rash progression for 2 weeks, and jaundice, tea-colored urine, and clay-colored stool for 1 week preceding hospital admission. DIAGNOSIS: SLE flareups in the patient were initially suspected. However, the final diagnosis was acute respiratory distress syndrome (ARDS) associated with MAS. INTERVENTIONS: The treatment included disease-modifying antirheumatic drugs (DMARDs), such as azathioprine, and titrated steroid doses of methylprednisolone (40 mg q8 h) and dexamethasone (15 mg q8 h), after the patient had ARDS and was intubated. Dose-adjusted monotherapy with dexamethasone was found to be effective; this may be attributed to some DMARDs being unsuitable for cytokine storms, that is, some DMARDs may cause complications in cytokine storms. OUTCOMES: After dexamethasone 15 mg q8 h treatment, the patient's fever subsided within 2 days, and liver function became normal within 3 weeks. The patient regularly attended scheduled outpatient follow-up visits after discharge. After 2 years, the patient reported no symptoms or signs of SLE with 2 mg/d oral dexamethasone. LESSONS: Early diagnosis of MAS and dexamethasone treatment for MAS with ARDS appear to be crucial for these patients.
format Online
Article
Text
id pubmed-8812624
institution National Center for Biotechnology Information
language English
publishDate 2022
publisher Lippincott Williams & Wilkins
record_format MEDLINE/PubMed
spelling pubmed-88126242022-02-18 Acute respiratory distress syndrome associated with macrophage activation syndrome in systemic lupus erythematosus: A case report and literature review Chang, En-Shuo Yu, Han-Hua Wu, Chiao-En Chan, Tien-Ming Medicine (Baltimore) 3600 RATIONALE: Previous treatment for macrophage activation syndrome (MAS) includes high-dose intravenous methylprednisolone along with intravenous immunoglobulin G. If MAS worsened, second-line therapy consisted of anakinra; if the disease remained refractory, third-line therapy with etoposide was considered. In addition, cyclosporine A plays a role in early MAS and in preventing recurrence. Some studies have reported the use of cytokine-targeting agents other than anakinra, such as canakinumab, tocilizumab, abatacept, and tofacitinib. PATIENT CONCERNS: The patient with systemic lupus erythematosus (SLE) had an uncommon combination of intermittent fever, hyperferritinemia, hypertriglyceridemia, jaundice, and significantly abnormal liver function test results. The patient reported a history of daily fever of 38 to 39°C, painful oral ulcer, anorexia, abdominal bloating, diarrhea, and malar rash progression for 2 weeks, and jaundice, tea-colored urine, and clay-colored stool for 1 week preceding hospital admission. DIAGNOSIS: SLE flareups in the patient were initially suspected. However, the final diagnosis was acute respiratory distress syndrome (ARDS) associated with MAS. INTERVENTIONS: The treatment included disease-modifying antirheumatic drugs (DMARDs), such as azathioprine, and titrated steroid doses of methylprednisolone (40 mg q8 h) and dexamethasone (15 mg q8 h), after the patient had ARDS and was intubated. Dose-adjusted monotherapy with dexamethasone was found to be effective; this may be attributed to some DMARDs being unsuitable for cytokine storms, that is, some DMARDs may cause complications in cytokine storms. OUTCOMES: After dexamethasone 15 mg q8 h treatment, the patient's fever subsided within 2 days, and liver function became normal within 3 weeks. The patient regularly attended scheduled outpatient follow-up visits after discharge. After 2 years, the patient reported no symptoms or signs of SLE with 2 mg/d oral dexamethasone. LESSONS: Early diagnosis of MAS and dexamethasone treatment for MAS with ARDS appear to be crucial for these patients. Lippincott Williams & Wilkins 2022-02-04 /pmc/articles/PMC8812624/ /pubmed/35119005 http://dx.doi.org/10.1097/MD.0000000000028612 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), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. http://creativecommons.org/licenses/by/4.0 (https://creativecommons.org/licenses/by/4.0/)
spellingShingle 3600
Chang, En-Shuo
Yu, Han-Hua
Wu, Chiao-En
Chan, Tien-Ming
Acute respiratory distress syndrome associated with macrophage activation syndrome in systemic lupus erythematosus: A case report and literature review
title Acute respiratory distress syndrome associated with macrophage activation syndrome in systemic lupus erythematosus: A case report and literature review
title_full Acute respiratory distress syndrome associated with macrophage activation syndrome in systemic lupus erythematosus: A case report and literature review
title_fullStr Acute respiratory distress syndrome associated with macrophage activation syndrome in systemic lupus erythematosus: A case report and literature review
title_full_unstemmed Acute respiratory distress syndrome associated with macrophage activation syndrome in systemic lupus erythematosus: A case report and literature review
title_short Acute respiratory distress syndrome associated with macrophage activation syndrome in systemic lupus erythematosus: A case report and literature review
title_sort acute respiratory distress syndrome associated with macrophage activation syndrome in systemic lupus erythematosus: a case report and literature review
topic 3600
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8812624/
https://www.ncbi.nlm.nih.gov/pubmed/35119005
http://dx.doi.org/10.1097/MD.0000000000028612
work_keys_str_mv AT changenshuo acuterespiratorydistresssyndromeassociatedwithmacrophageactivationsyndromeinsystemiclupuserythematosusacasereportandliteraturereview
AT yuhanhua acuterespiratorydistresssyndromeassociatedwithmacrophageactivationsyndromeinsystemiclupuserythematosusacasereportandliteraturereview
AT wuchiaoen acuterespiratorydistresssyndromeassociatedwithmacrophageactivationsyndromeinsystemiclupuserythematosusacasereportandliteraturereview
AT chantienming acuterespiratorydistresssyndromeassociatedwithmacrophageactivationsyndromeinsystemiclupuserythematosusacasereportandliteraturereview