EJMCR |

Case Report

Volume: 6 | Issue: 2 | Published: Feb 28, 2022 | Pages: 27 - 32 | DOI: 10.24911/ejmcr/173-1608028385

Macrophage activation syndrome in a young adult patient with a background of systemic lupus erythematosus: a case report

Authors: Chi Chun Wan , Teresa Doherty

Article Info

Authors

Chi Chun Wan

Barking Havering Redbridge University Trust, United Kingdom

Teresa Doherty

Barking Havering Redbridge University Trust, Untied Kingdom.

Publication History

Received: December 15, 2020

Accepted: November 22, 2021

Published: February 28, 2022

Abstract

Background: Macrophage activation syndrome (MAS) is a rare but potentially life-threatening condition associated with systemic lupus erythematosus (SLE), as well as several other autoimmune conditions. Case Presentation: We present a case of MAS in a 30-year-old patient with a background of SLE presenting with persistent fever and hemodynamic instability. Broad-spectrum antibiotics were initially started for possible sepsis of unknown origin, but a lack of response or evidence of an infection source led to further laboratory investigations and additional diagnostic imaging. Hyperferritinemia, a low fibrinogen, hypertriglyceridemia, and pancytopenia raised the suspicion of MAS. Echocardiography revealed a pericardial effusion requiring pericardiocentesis. With rapid disease progression, multiorgan failure ensued, and shortly after a high dose, methylprednisolone was commenced; the patient's condition worsened and she died after a cardiac arrest. Conclusion: This case highlights the challenges involved in diagnosing MAS and in differentiating it from more common presentations such as sepsis or autoimmune disease flares, and demonstrates the possible rapid disease progression. Therefore, early recognition of certain clinical features and laboratory markers are essential to enable early diagnosis, commencement of immunosuppressive treatment, and to ultimately improve outcomes in such critically unwell patients.

Keywords: Macrophage Activation Syndrome, Systemic Lupus Erythematosus, Rare Rheumatology Diseases, Diagnosis, Ferritin, Mortality, Abstract, green

Pubmed Style

Chi Chun Wan, Teresa Doherty. Macrophage activation syndrome in a young adult patient with a background of systemic lupus erythematosus: a case report. EJMCR. 2022; 28 (February 2022): 27-32. doi:10.24911/ejmcr/173-1608028385

Web Style

Chi Chun Wan, Teresa Doherty. Macrophage activation syndrome in a young adult patient with a background of systemic lupus erythematosus: a case report. https://ejmcr.com/articles/1280 [Access: December 25, 2024]. doi:10.24911/ejmcr/173-1608028385

AMA (American Medical Association) Style

Vancouver/ICMJE Style

Chi Chun Wan, Teresa Doherty. Macrophage activation syndrome in a young adult patient with a background of systemic lupus erythematosus: a case report. EJMCR. (2022), [cited December 25, 2024]; 28 (February 2022): 27-32. doi:10.24911/ejmcr/173-1608028385

Harvard Style

Chi Chun Wan, Teresa Doherty (2022) Macrophage activation syndrome in a young adult patient with a background of systemic lupus erythematosus: a case report. EJMCR, 28 (February 2022): 27-32. doi:10.24911/ejmcr/173-1608028385

Chicago Style

Chi Chun Wan, Teresa Doherty. "Macrophage activation syndrome in a young adult patient with a background of systemic lupus erythematosus: a case report." 28 (2022), 27-32. doi:10.24911/ejmcr/173-1608028385

MLA (The Modern Language Association) Style

Chi Chun Wan, Teresa Doherty. "Macrophage activation syndrome in a young adult patient with a background of systemic lupus erythematosus: a case report." 28.February 2022 (2022), 27-32. Print. doi:10.24911/ejmcr/173-1608028385

APA (American Psychological Association) Style

Chi Chun Wan, Teresa Doherty (2022) Macrophage activation syndrome in a young adult patient with a background of systemic lupus erythematosus: a case report. , 28 (February 2022), 27-32. doi:10.24911/ejmcr/173-1608028385