Case Report
Copyright ©The Author(s) 2019. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Nov 26, 2019; 7(22): 3859-3865
Published online Nov 26, 2019. doi: 10.12998/wjcc.v7.i22.3859
Respiratory failure and macrophage activation syndrome as an onset of systemic lupus erythematosus: A case report
Juan Sun, Jian-Wen Wang, Rui Wang, Hao Zhang, Jian Sun
Juan Sun, Jian-Wen Wang, Hao Zhang, Jian Sun, Department of Nephrology and Rheumatology, The Third Xiangya Hospital, Central South University, Changsha 410013, Hunan Province, China
Rui Wang, Department of Gastroenterology, The Third Xiangya Hospital, Central South University, Changsha 410013, Hunan Province, China
Author contributions: Sun J was responsible for collecting the materials and drafting the manuscript. Wang JW and Zhang H provided important intellectual content, reviewed the literature and contributed to drafting the manuscript. Wang R polished the language in the manuscript and performed diseases consultation. Sun J contributed to interpreting the clinical materials, designing the manuscript, revising and modifying the manuscript critically for important intellectual content, and offered valuable suggestions for improving the manuscript. All authors gave final approval for the version to be submitted.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare that they have no conflict of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Corresponding author: Jian Sun, MD, Doctor, Professor, Department of Nephrology and Rheumatology, The Third Xiangya Hospital, Central South University, 138 Tongzipo Road, Changsha 410013, Hunan Province, China. 600959@csu.edu.cn
Telephone: +86-0731-88618210
Received: April 22, 2019
Peer-review started: April 23, 2019
First decision: September 9, 2019
Revised: October 9, 2019
Accepted: October 15, 2019
Article in press: October 15, 2019
Published online: November 26, 2019
Abstract
BACKGROUND

Macrophage activation syndrome (MAS) is defined as a specific secondary hemophagocytic lymphohistiocytosis that refers particularly to those triggered by autoimmune diseases. MAS is a rare and highly lethal complication of systemic lupus erythematosus (SLE), which can be associated with, or mimic, disease flare. However, the data regarding the clinical course, management and outcome of SLE with MAS is limited, especially in adults. Lack of clinical recognition of the disease often leads to poor prognosis.

CASE SUMMARY

We report a 36-year-old Chinese woman without relevant past medical history who was admitted to hospital with a 6-d history of jaundice and a high fever of 39.4°C lasting one day. Abdominal magnetic resonance imaging excluded obstructive jaundice, no infection was identified and empiric superior antibiotic treatment (meropenem) showed no clinical improvement. However, newly emerged pancytopenia and respiratory failure endangered the patient’s life. Autoimmune work-up finally led to the diagnosis of SLE, which initially presented as MAS and manifested respiratory failure, although neither bone marrow biopsy nor lymph node biopsy showed hemophagocytosis. To our knowledge, such a scenario has never been reported in detail before. The patient had a favorable reaction to combination treatment with corticosteroid and cyclosporine A and has been in clinical remission during the 1-year follow up period.

CONCLUSION

Respiratory failure and MAS can be an onset of SLE. Early diagnosis and appropriate treatment are extremely important for a better prognosis.

Keywords: Systemic lupus erythematosus, Macrophage activation syndrome, Respiratory failure, Case report

Core tip: We report a 36-year-old Chinese woman diagnosed with systemic lupus erythematosus who initially presented with macrophage activation syndrome (MAS) and manifested respiratory failure. The administration of corticosteroid and cyclosporine A improved her respiratory depression along with all other symptoms. The clinical characteristics of MAS show great heterogeneity, and sufficient knowledge of these syndromes and early diagnosis are essential to improve prognosis. Corticosteroids are the mainstay of initial treatment for MAS.