DOI: 10.12890/2024_005026
Macrophage activation syndrome in a patient with systemic lupus erythematous triggered by tuberculous meningitis
Authors
naowais@hotmail.co.uk
Keywords
Hemophagocytic lymphohistiocytosis (HLH), macrophage activation syndrome (MAS), systemic lupus erythematosus (SLE), National Early Warning Score (NEWS)Abstract
Background: Hemophagocytic lymphohistiocytosis (HLH), is characterized by systemic uncontrolled inflammation resulting from immune dysregulation secondary to various triggers, including genetics, infections, autoimmune diseases, and malignancies. Macrophage activation syndrome (MAS) is an immune dysregulation phenomenon, in which an underlying rheumatological disease is present. We report a rare, interesting case of a middle-aged female, with a systemic lupus erythematosus (SLE) flare complicated by macrophage activation syndrome (MAS), in which tuberculous meningitis (TBM) was the identified trigger.
Case report: A 48-year-old female with an African ethnic background, diagnosed with SLE, presented with a 2-week history of high-grade fever, cough, and worsening arthralgia. She was initially admitted with suspected SLE flare secondary to community acquired pneumonia and was started on broad-spectrum antibiotics. Later during her hospital stay, she underwent extensive workup because of her clinical condition, as she had continuous spikes of fever, progressive cytopenia, accompanied with acute confusion state despite treatment. She was ultimately diagnosed with SLE flare complicated by MAS secondary to TBM in view of persistent fever, neuropsychiatric symptoms, hyperferritinemia and hypertriglyceridemia, supported by bone marrow aspiration findings, a tuberculosis (TB) positive contact and supporting cerebrospinal fluid analysis suggestive of TBM. She was immediately started on pulse doses of steroids and anti-tuberculosis medications in which significant clinical improvement was observed. She was discharged home, with continuity of care done at the outpatient department in addition to continuity of physical rehabilitation.
Conclusion: Given the wide range of clinical presentations and similarities between SLE and the life-threatening MAS, it is essential to maintain a high level of suspicion to establish the diagnosis and promptly start the necessary treatment to ensure better clinical and survival outcomes.
References
- Aringer M, Costenbader K, Daikh D, Brinks R, Mosca M, Ramsey-Goldman R, et al. 2019 European League Against Rheumatism/American College of Rheumatology classification criteria for systemic lupus erythematosus. Ann Rheum Dis 2019;78:1151-1159.
- Ameer MA, Chaudhry H, Mushtaq J, Khan OS, Babar M, Hashim T, Zet al. An Overview of Systemic Lupus Erythematosus (SLE) Pathogenesis, Classification, and Management. Cureus 2022;14:e30330.
- Wijetunga WMUA, Satarasinghe RL, Dayananda BM, Darshani GK. Unusual acute lupus hemophagocytic syndrome - a test of diagnostic criteria: a case report. J Med Case Rep 2017;11:185.
- Awasthi S, Upreti S. Macrophage activation syndrome in a patient with systemic lupus erythematosus (SLE) and dual viremia. J Community Hosp Intern Med Perspect 2020;10:470-474.
- Filipovich A, McClain K, Grom A. Histiocytic disorders: recent insights into pathophysiology and practical guidelines. Biol Blood Marrow Transplant 2010;16:S82-89.
- Szulawski R, Kourlas PJ, Antonchak M. Macrophage Activation Syndrome (MAS) in a Recently Released Prisoner with Systemic Lupus Erythematosus (SLE). Am J Case Rep 2018;19:734-738.
- Henter JI, Horne A, Aricó M, Egeler RM, Filipovich AH, Imashuku S, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2007;48:124-131.
- Janarthanan M, Antony T, Mohan R, Premkumar S, Pasupathy U. Disseminated tuberculosis with macrophage activation syndrome in a child with lupus nephritis. Sudan J Paediatr 2021;21:190-194.
- Granata G, Didona D, Stifano G, Feola A, Granata M. Macrophage Activation Syndrome as Onset of Systemic Lupus Erythematosus: A Case Report and a Review of the Literature. Case Rep Med 2015;2015:294041.
- Torres Jiménez A, Solís Vallejo E, Zeferino Cruz M, Céspedes Cruz A, Sánchez Jara B. Macrophage activation syndrome as the initial manifestation of severe juvenile onset systemic lupus erythematosus. Favorable response to cyclophosphamide. Reumatol Clin 2014;10:331-335.
