Welcome to Francis Academic Press

International Journal of Frontiers in Medicine, 2024, 6(2); doi: 10.25236/IJFM.2024.060205.

Case study of tocilizumab in the treatment of macrophage activation syndrome secondary to systemic lupus erythematosus

Author(s)

Zhimin Wang, Caixia Sun, Minghua Xu

Corresponding Author:
Zhimin Wang
Affiliation(s)

The Affiliated Hospital of Hebei University, Baoding, China

Abstract

Macrophage activation syndrome (MAS) is a serious complication of autoimmune diseases, with a potentially fatal risk, belonging to secondary hemophagocytic lymphohistiocytosis (HLH), often secondary to juvenile idiopathic arthritis (sJIA), adult still disease (ASOD), and can also be secondary to systemic lupus erythematosus (SLE), etc., SLE combined with MAS has a very high mortality rate, and the clinical manifestations are not easy to control high fever, pancytopenia, hepatosplenic lymphadenopathy, coagulation dysfunction, etc. Because SLE patients can have a variety of clinical manifestations, MAS is often difficult to identify, some patients are critically ill, and traditional glucocorticoids, immunosuppressants and other treatments are often difficult to control the disease, and the treatment of cytokines is gradually tried. This article reports a case of refractory SLE complicated with MAS successfully controlled by tocilizumab, and summarizes and analyzes the literature to explore the diagnosis and treatment experience of SLE complicated with MAS.

Keywords

Tocilizumab; systemic lupus erythematosus; macrophage activation syndrome

Cite This Paper

Zhimin Wang, Caixia Sun, Minghua Xu. Case study of tocilizumab in the treatment of macrophage activation syndrome secondary to systemic lupus erythematosus. International Journal of Frontiers in Medicine (2024), Vol. 6, Issue 2: 32-37. https://doi.org/10.25236/IJFM.2024.060205.

References

[1] Wafa A, Hicham H, Naoufal R, et al. Clinical spectrum and therapeutic management of systemic lupus erythematosus-associated macrophage activation syndrome: a study of 20 Moroccan adult patients. Clin. Rheumatol. 2022, 41(7): 2021-2033. 

[2] Vilaiyuk S, Sirachainan N, Wanitkun S, et al. Recurrent macrophage activation syndrome as the primary manifestation in systemic lupus erythematosus and the benefit of serial ferritin measurements: a case-based review. Clin. Rheumatol. 2013, 32: 899-904. 

[3] Janka GE, Lehmberg K. Hemophagocytic syndromes--an update. Blood. Rev. 2014, 28(4): 135-142.

[4] Wafa A, Hicham H, Naoufal R, et al. Clinical spectrum and therapeutic management of systemic lupus erythematosus-associated macrophage activation syndrome: a study of 20 Moroccan adult patients. Clin Rheumatol. 2022 Jul; 41(7):2021-2033. 

[5] Gerstein M, Borgia RE, Dominguez D, et al. Predicting Macrophage Activation Syndrome in Childhood-onset Systemic Lupus Erythematosus Patients at Diagnosis. J Rheumatol. 2021 Sep; 48(9): 1450-1457.

[6] Henderson LA, Cron RQ. Macrophage Activation Syndrome and Secondary Hemophagocytic Lymphohistiocytosis in Childhood Inflammatory Disorders: Diagnosis and Management. Paediatr Drugs. 2020 Feb; 22(1):29-44.

[7] De Benedetti F, Grom AA, Brogan PA, et al. Efficacy and safety of emapalumab in macrophage activation syndrome. Ann Rheum Dis. 2023 Jun;82(6):857-865. 

[8] Henter JI, Horne A, Aricó M, et al. HLH -2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistio cytosis. Pediatr. Blood. Cancer. 2007, 48(2): 124-131.

[9] Aziz A, Castaneda EE, Ahmad N, et al. Exploring Macrophage Activation Syndrome Secondary to Systemic Lupus Erythematosus in Adults: A Systematic Review of the Literature. Cureus. 2021, 13(10): 18822.

[10] Ramos-Casals M, Brito-Zerón P, López-Guillermo A, et al. Adult haemophagocytic syndrome. Lancet. 2014, 383(9927): 1503-1516. 

[11] Nakagishi Y, Shimizu M, Kasai K, et al. Successful therapy of macrophage activation syndrome with dexamethasone palmitate. Mod. Rheumatol. 2016, 26: 617-620.

[12] Gouda W, Alsaqabi F, Moshrif A, et al. Macrophage activation syndrome triggered by systemic lupus erythematosus flare: successful treatment with a combination of dexamethasone sodium phosphate, intravenous immunoglobulin, and cyclosporine: a case report. J. Med. Case. Rep. 2021, 15(1): 497. 

[13] Miettunen PM, Narendran A, Jayanthan A, et al. Successful treatment of severe paediatric rheumatic disease-associated macrophage activation syndrome with interleukin-1 inhibition following conventional immunosuppressive therapy: case series with 12 patients. Rheumatology (Oxford), 2011, 50(2): 417-419. 

[14] Kübler L, Bittmann I, Kuipers JG. Macrophage activation syndrome triggered by active systemic lupus erythematosus: Successful treatment by interleukin-1 inhibition (anakinra). Z Rheumatol. 2020, 79(10): 1040-1045.

[15] Shakoory B, Carcillo JA, Chatham WW, et al. Interleukin-1 Receptor Blockade Is Associated With Reduced Mortality in Sepsis Patients With Features of Macrophage Activation Syndrome: Reanalysis of a Prior Phase III Trial. Crit. Care. Med. 2016, 44(2): 275-281. 

[16] Schulert GS, Minoia F, Bohnsack J, et al. Effect of Biologic Therapy on Clinical and Laboratory Features of Macrophage Activation Syndrome Associated With Systemic Juvenile Idiopathic Arthritis. Arthritis. Care. Res. (Hoboken). 2018, 70(3): 409-419. 

[17] Chang ES, Yu HH, Wu CE, et al. Acute respiratory distress syndrome associated with macrophage activation syndrome in systemic lupus erythematosus: A case report and literature review. Medicine (Baltimore). 2022, 101(5): 28612.

[18] Al-Qaaneh AM, Al-Ghamdi FH. Tocilizumab prescribing criteria for COVID-19 patients. Hum Vaccin Immunother. 2021, 17(4): 1128. 

[19] Iwai A, Naniwa T, Tamechika S, et al. Short-term add-on tocilizumab and intravenous cyclophosphamide exhibited a remission-inducing effect in a patient with systemic lupus erythematosus with refractory multiorgan involvements including massive pericarditis and glomerulonephritis. Mod. Rheumatol. 2017, 27(3): 529-532. 

[20] Inokuchi S, Mitoma H, Kawano S, et al. Activation of caspase-1 is mediated by stimulation of interferon genes and NLR family pyrin domain containing 3 in monocytes of active systemic lupus erythematosus. Clin. Exp. Rheumatol. 2022, 40(3): 522-531. 

[21] Schulert GS, Cron RQ. The genetics of macrophage activation syndrome. Genes Immun. 2020 May; 21(3): 169-181.

[22] Tang S, Li S, Zheng S, et al. Understanding of cytokines and targeted therapy in macrophage activation syndrome. Semin. Arthritis. Rheum. 2021, 51(1): 198-210.