MACROPHAGE ACTIVATION SYNDROME ASSOCIATED WITH ADULT ONSET STILL’S DISEASE
AbstractMacrophage activation syndrome (MAS) is a potentially lethal complication of chronic rheumatological conditions like ankylosing spondylitis, rheumatoid arthritis, and adult-onset Still’s disease (AOSD). It is a multisystem inflammatory syndrome caused by immense cytokine release from activated lymphocytes and macrophages. We give an account of the incidence of a twenty years old Asian girl suffering from non-remitting fever and an evanescent rash for last ten weeks. Physical examination and laboratory work-up suggested high grade fever, pancytopenia, hyperferritinemia, hypertriglyceridemia, hypofibrinogenemia and impaired liver function. Bone marrow biopsy was also done. It showed active hemophagocytosis. She was diagnosed as a case of Macrophage Activation Syndrome associated with Adult Onset Still’s disease. She was treated with high dose steroids and cyclosporine and recovered completely.Keywords: Adult Onset Still’s disease, Macrophage Activation Syndrome, hemophagocytosis, Bone Marrow Biopsy
Henter JI, Home A, Arico M, Eqeler RM, Filipovich AH, Imashuku S, et al. HLH‐2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2017;48(2):124–31.
Deane S, Selmi C, Teuber SS, Gershwin ME. Macrophage Activation Syndrome in Autoimmune Disease. Int Arch Allergy Immunol 2017;153(2):109–20.
Maeshima K, Ishii K, Iwakura M, Akamine M, Hamasaki H, Abe I, et al. Adult-onset Still’s disease with macrophage activation syndrome successfully treated with a combination of methotrexate and etanercept. Mod Rheumatol 2012;22(1):137–41.
Owlia M, Soleimani H, Mortazavizadeh M. Macrophage activation syndrome (MAS) and thrombotic thrombocytopenic purpura (TTP)-: Are they from a single spectrum? J Indian Acad Clin Med 2005;6(4).
Park JH, Bae JH, Choi YS, Lee HS, Jun JB, Jung S, et al. Adult-onset Still's disease with disseminated intravascular coagulation and multiple organ dysfunctions dramatically treated with cyclosporine A. J Korean Med Sci 2004;19(1):137–41.
Avčin T, Tse SM, Schneider R, Ngan B, Silverman ED. Macrophage activation syndrome as the presenting manifestation of rheumatic diseases in childhood. J Pediatr 2006;148(5):683–6.
Gorelik M, Fall N, Altaye M, Barnes MG, Thompson SD, Grom AA, et al. Follistatin-like protein 1 and the ferritin/erythrocyte sedimentation rate ratio are potential biomarkers for dysregulated gene expression and macrophage activation syndrome in systemic juvenile idiopathic arthritis. J Rheumatol 2013;40(7):1191–9.
Ravelli A, Minoia F, Davì S, Horne A, Bovis F, Pistorio A, et al. 2016 Classification Criteria for Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis. A European League Against Rheumatism/American College of Rheumatology/Paediatric Rheumatology International Trials Organisation Collaborative Initiative. Ann Rheum Dis 2016;75(3):481–9.
Osugi Y, Hara J, Tagawa S, Takai K, Hosoi G, Matsuda Y, et al. Cytokine production regulating Th1 and Th2 cytokines in hemophagocytic lymphohistiocytosis. Blood 1997;89(11):4100–3.
Schulert GS, Grom AA. Macrophage activation syndrome and cytokine-directed therapies. Best Pract Res Clin Rheumatol 2014;28(2):277–92.
Arlet JB, Le TH, Marinho A, Amoura Z, Wechsler B, Papo T, et al. Reactive haemophagocytic syndrome in adult-onset Still’s disease: a report of six patients and a review of the literature. Ann Rheum Dis 2006;65(12):1596–601.
Hot A, Toh ML, Coppéré B, Perard L, Madoux MH, Mausservey C, et al. Reactive hemophagocytic syndrome in adult-onset Still disease: clinical features and long-term outcome: a case-control study of 8 patients. Medicine (Baltimore) 2010;89(1):37–46.
Nordström D, Knight A, Luukkainen R, van Vollenhoven R, Rantalaiho V, Kajalainen A, et al. Beneficial effect of interleukin 1 inhibition with anakinra in adult-onset Still’s disease. An open, randomized, multicenter study. J Rheumatol 2012;39(10):2008–11.
Quartier P, Allantaz F, Cimaz R, Pillet P, Messiaen C, Bardin C, et al. A multicentre, randomised, double-blind, placebo-controlled trial with the interleukin-1 receptor antagonist anakinra in patients with systemic-onset juvenile idiopathic arthritis (ANAJIS trial). Ann Rheum Dis 2011;70(5):747–54.
Canna S, Frankovich J, Higgins G, Narkewicz MR, Nash SR, Hollister JR, et al. Acute hepatitis in three patients with systemic juvenile idiopathic arthritis taking interleukin-1 receptor antagonist. Pediatr Rheumatol Online J 2009;7(1):21.