MACROPHAGE ACTIVATION SYNDROME ASSOCIATED WITH ADULT ONSET STILL’S DISEASE

Authors

  • Madeeha Gulzar postgraduate trainee,General Medicine,SIHS,lhr
  • Ali Sabir postgraduate trainee,General Medicine,SIHS,lhr
  • Muhammad Afzal Hamdani MBBS,DIP CARD, MRCP,FCPS(Medicine), FCPS ( Rheumatology), Assistant Professor Medicine, SIHS
  • Asma Saadia MBBS,FCPS (Pathology), Assistant Professor Pathology, SIHS

Abstract

Macrophage 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

Author Biographies

Madeeha Gulzar, postgraduate trainee,General Medicine,SIHS,lhr

post graduate trainee,General Medicine,Lhr

Ali Sabir, postgraduate trainee,General Medicine,SIHS,lhr

postgraduate trainee,General Medicine,SIHS,lhr

Muhammad Afzal Hamdani, MBBS,DIP CARD, MRCP,FCPS(Medicine), FCPS ( Rheumatology), Assistant Professor Medicine, SIHS

MBBS,DIP CARD, MRCP,FCPS(Medicine), FCPS ( Rheumatology)        Assistant Professor Medicine, SIHS

Asma Saadia, MBBS,FCPS (Pathology), Assistant Professor Pathology, SIHS

MBBS,FCPS (Pathology),        Assistant Professor Pathology, SIHS 

References

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.

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Published

2018-03-16

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