Clinical analysis of macrophage activation syndrome in pediatric patients with autoimmune diseases

Clinical Rheumatology

Volumn 31, Issue 8, 2012, Pages 1223-1230

  Lin, Cheng I ^a   Yu, Hsin Hui ^a   Lee, Jyh Hong ^a   Wang, Li Chieh ^a   Lin, Yu Tsan ^a   Yang, Yao Hsu ^a   Chiang, Bor Luen ^a  

^a NATIONAL TAIWAN UNIVERSITY HOSPITAL   (Taiwan)

Author keywords

Autoimmune associated hemophagocytosis (AAHS); Hemophagocytic lymphohistiocytosis (HLH); Macrophage activation syndrome (MAS); Systemic juvenile rheumatoid arthritis (sJRA)

Indexed keywords

ALANINE AMINOTRANSFERASE; ASPARTATE AMINOTRANSFERASE; BILIRUBIN; C REACTIVE PROTEIN; CORTICOSTEROID; CYCLOSPORIN A; D DIMER; ETANERCEPT; FERRITIN; FIBRINOGEN; IMMUNOGLOBULIN; LACTATE DEHYDROGENASE; LAMOTRIGINE; METHYLPREDNISOLONE; NONSTEROID ANTIINFLAMMATORY AGENT; PHENYTOIN; TRIACYLGLYCEROL;

ADENOVIRUS INFECTION; ADOLESCENT; ADULT; ANEMIA; ANTIPHOSPHOLIPID SYNDROME; ARTICLE; AUTOIMMUNE DISEASE; BONE MARROW; CENTRAL NERVOUS SYSTEM DISEASE; CHILD; CLINICAL FEATURE; CONSCIOUSNESS DISORDER; CORTICOSTEROID THERAPY; DRUG DOSE REDUCTION; DRUG EFFICACY; DRUG INDUCED DISEASE; DRUG MEGADOSE; DRUG PULSE THERAPY; DRUG TREATMENT FAILURE; EPSTEIN BARR VIRUS INFECTION; ERYTHROCYTE SEDIMENTATION RATE; ERYTHROPHAGOCYTOSIS; FEVER; GROUPS BY AGE; HEPATOSPLENOMEGALY; HUMAN; HYPOFIBRINOGENEMIA; IMMUNE DEFICIENCY; IMMUNOTHERAPY; INFLAMMATION; INTENSIVE CARE; JAUNDICE; LABORATORY TEST; LEUKOPENIA; LYMPHADENOPATHY; MACROPHAGE ACTIVATION SYNDROME; MAJOR CLINICAL STUDY; MEDICAL RECORD REVIEW; MIXED CONNECTIVE TISSUE DISEASE; MORTALITY; MULTIPLE ORGAN FAILURE; MYOPATHY; OPPORTUNISTIC INFECTION; PNEUMOCYSTIS JIROVECI INFECTION; PNEUMOCYSTOSIS; PRIORITY JOURNAL; RASH; RETROSPECTIVE STUDY; SCHOOL CHILD; SEIZURE; SYSTEMIC LUPUS ERYTHEMATOSUS; THROMBOCYTE COUNT; THROMBOCYTOPENIA; TREATMENT OUTCOME;

ADOLESCENT; ANTIPHOSPHOLIPID SYNDROME; ARTHRITIS, JUVENILE RHEUMATOID; AUTOIMMUNE DISEASES; CHILD; CYCLOSPORINE; DRUG THERAPY, COMBINATION; FEMALE; GLUCOCORTICOIDS; HUMANS; IMMUNOGLOBULINS, INTRAVENOUS; IMMUNOSUPPRESSIVE AGENTS; LUPUS ERYTHEMATOSUS, SYSTEMIC; MACROPHAGE ACTIVATION SYNDROME; MALE; METHYLPREDNISOLONE; RETROSPECTIVE STUDIES; TREATMENT OUTCOME;

EID: 84865986803     PISSN: 07703198     EISSN: 14349949     Source Type: Journal    
DOI: 10.1007/s10067-012-1998-0     Document Type: Article

References (42)

1. Ramanan AV, Schneider R (2003) Macrophage activation syndrome -what's in a name! J Rheumatol 30(12):2513-2516
2. Hadchouel M, Prieur AM, Griscelli C (1985) Acute hemorrhagic, hepatic, and neurologic manifestations in juvenile rheumatoid arthritis: Possible relationship to drugs or infection. J Pediatr 106 (4):561-566
3. Kumakura S, Ishikura H, Kondo M, Murakawa Y, Masuda J, Kobayashi S (2004) Autoimmune-associated hemophagocytic syndrome. Mod Rheumatol 14(3):205-215
4. Deane S, Selmi C, Teuber SS, Gershwin ME (2010) Macrophage activation syndrome in autoimmune disease. Int Arch Allergy Immunol 153(2):109-120
5. Lin YT, Wang CT, Gershwin ME, Chiang BL (2011) The pathogenesis of oligoarticular/polyarticular vs systemic juvenile idiopathic arthritis. Autoimmun Rev 10(8):482-489
6. Villanueva J, Lee S, Giannini EH, Graham TB, Passo MH, Filipovich A, Grom AA (2005) Natural killer cell dysfunction is a distinguishing feature of systemic onset juvenile rheumatoid arthritis and macrophage activation syndrome. Arthritis Res Ther 7(1):R30-R37
7. Stepp SE, Dufourcq-Lagelouse R, Le Deist F, Bhawan S, Certain S, Mathew PA, Henter JI, Bennett M, Fischer A, de Saint BG, Kumar V (1999) Perforin gene defects in familial hemophagocytic lymphohistiocytosis. Science 286(5446):1957-1959
8. Hazen MM, Woodward AL, Hofmann I, Degar BA, Grom AA, Filipovich AH, Binstadt BA (2008) Mutations of the hemophagocytic lymphohistiocytosis- associated gene UNC13D in a patient with systemic juvenile idiopathic arthritis. Arthritis Rheum 58(2):567-570
9. Zhang K, Biroschak J, Glass DN, Thompson SD, Finkel T, Passo MH, Binstadt BA, Filipovich A, Grom AA (2008) Macrophage activation syndrome in patients with systemic juvenile idiopathic arthritis is associated with MUNC13-4 polymorphisms. Arthritis Rheum 58(9):2892-2896
10. Henter JI, Horne A, Arico M, Egeler RM, Filipovich AH, Imashuku S, Ladisch S, McClain J, Webb D, Winiarski J, Janka G (2007) HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 48 (2):124-131
11. Ravelli A, Magni-Manzoni S, Pistorio A, Besana C, Foti T, Ruperto N, Viola S, Martini A (2005) Preliminary diagnostic guidelines for macrophage activation syndrome complicating systemic juvenile idiopathic arthritis. J Pediatr 146(5):568-604
12. Hochberg MC (1997) Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 40(9):1725-1734
13. Gladman DD, Ibañez D, Urowitz MB (2002) Systemic lupus erythematosus disease activity index 2000. J Rheumatol 29 (2):288-291
14. Brewer EJ, Bass J, Baum J, Cassidy JT, Fink C, Jacobs J, Hanson V, Levinson JE, Schaller J, Stillman JS (1977) Current proposed revision of JRA Criteria. JRA Criteria Subcommittee of the Diagnostic and Therapeutic Criteria Committee of the American Rheumatism Section of The Arthritis Foundation. Arthritis Rheum 20 (suppl):195-199
15. Yao TC, Kuo ML, See LC, Ou LS, Lee WI, Chan CK, Huang JL (2006) RANTES and monocyte chemoattractant protein 1 as sensitive markers of disease activity in patients with juvenile rheumatoid arthritis: A six-year longitudinal study. Arthritis Rheum 54 (8):2585-2593
16. Kasukawa R, Sharp GC (1987) Preliminary diagnostic criteria for classification of mixed connective tissue disease. In: Kasukawa R, Sharp GC (eds) Mixed connective tissue disease and antinuclear antibodies. Elsevier, Amsterdam, pp 41-48
17. Lage LV, Caleiro MT, Carvalho JF (2010) Proposed disease activity criteria for mixed connective tissue disease. Lupus 19(2):223-224
18. Miyakis S, Lockshin MD, Atsumi T, Branch DW, Brey RL, Cervera R, Derksen RHWM, De Groot PG, Koike T, Meroni PL, Reber G, Shoenfeld Y, Tincani A, Vlachoyiannopoulos PG, Krilis SA (2006) International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost 4(2):295-306
19. Taylor FB Jr, Toh CH, Hoots WK, Wada H, Levi M (2001) Towards definition, clinical and laboratory criteria, and a scoring system for disseminated intravascular coagulation. Thromb Haemost 86(5):1327-1330
20. McCann LJ, Hasson N, Pilkington CA (2006) Macrophage activation syndrome as an early presentation of Lupus. J Rheumatol 33 (2):438-440
21. Kaneko K, Takahashi K, Fujiwara S, Maruyama T, Obinata K (1998) Kawasaki disease followed by haemophagocytic syndrome. Eur J Pediatr 157(7):610-611
22. Kobayashi I, Yamada M, Kawamura N, Kobayashi R, Okano M, Kobayashi K (2000) Platelet-specific hemophagocytosis in a patient with juvenile dermatomyositis. Acta Paediatr 89(5):617-619
23. Kato M, Sato S, Suzuki M, Oka H, Kaneko Y, Yasuoka H, Nojima T, Suwa A, Hirakata M, Ikeda Y (2004) A case of mixed connective tissue disease successfully treated for hemophagocytic syndrome with intermittent intravenous injection of cyclophosphamide. Jpn J Clin Immunol 27(5):345-349
24. Maruyama J, Inokuma S (2010) Cytokine profiles of macrophage activation syndrome associated with rheumatic diseases. J Rheumatol 37(5):967-973
25. Stéphan JL, Koné-Paut I, Galambrun C, Mouy R, Bader-Meunier B, Prieur AM (2001) Reactive haemophagocytic syndrome in children with inflammatory disorders. A retrospective study of 24 patients. Rheumatology (Oxford) 40(11):1285-1292
26. Yu HH, Lee JH, Wang LC, Lin YT, Yang YH, Chiang BL (2006) Neuropsychiatric manifestations in pediatric systemic lupus erythematosus: A 20-year study. Lupus 15(10):651-657
27. Behrens EM, Beukelman T, Paessler M, Cron RQ (2007) Occult macrophage activation syndrome in patients with systemic juvenile idiopathic arthritis. J Rheumatol 34(5):1133-1138
28. Grom AA (2010) Macrophage activation syndrome. In: Cassidy JT, Petty RE, Laxer R, Lindsay C (eds) Textbook of pediatric rheumatology, vol 45, 6th edn. Saunders, Philadelphia, pp 674-681
29. Bleesing J, Prada A, Siegel DM, Villanueva J, Olson J, Ilowite NT, Brunner HI, Griffin T, Graham TB, Sherry DD, Passo MH, Ramanan AV, Filipovich A, Grom AA (2007) The diagnostic significance of soluble CD163 and soluble interleukin-2 receptor α-chain in macrophage activation syndrome and untreated new-onset systemic juvenile idiopathic arthritis. Arthritis Rheum 56(3):965-971
30. Shigemura T, Yamazaki T, Hara Y, Ou JN, Stevens AM, Ochs HD, Koike K, Agematsu K (2011) Monitoring serum IL-18 levels is useful for treatment of a patient with systemic juvenile idiopathic arthritis complicated by macrophage activation syndrome. Pediatr Rheumatol Online J 9(1):15
31. Fukaya S, Yasuda S, Hashimoto T, Oku K, Kataoka H, Horita T, Atsumi T, Koike T (2008) Clinical features of haemophagocytic syndrome in patients with systemic autoimmune diseases: Analysis of 30 cases. Rheumatology (Oxford) 47(11):1686-1691
32. Parodi A, Davì S, Pringe AB, Pistorio A, Ruperto N, Magni-Manzoni S, Miettunen P, Bader-Meunier B, Espada G, Sterba G, Ozen S, Wright D, Magalhães CS, Khubchandani R, Michels H, Woo P, Iglesias A, Guseinova D, Bracaglia C, Hayward K, Wouters C, Grom A, Vivarelli M, Fischer A, Breda L, Martini A, Ravelli A (2009) Macrophage activation syndrome in juvenile systemic lupus erythematosus: A multinational multicenter study of thirty-eight patients. Arthritis Rheum 60(11):3388-3399
33. Chen HH, Kuo HC, Wang L, Yu HR, Shen JM, Hwang KP, Yang KD (2007) Childhood macrophage activation syndrome differs from infection-associated hemophagocytosis syndrome in etiology and outcome in Taiwan. J Microbiol Immunol Infect 40(3):265-271
34. Singh S, Chandrakasan S, Ahluwalia J, Suri D, Rawat A, Ahmed N, Das R, Sachdeva M, Varma N (2011) Macrophage activation syndrome in children with systemic onset juvenile idiopathic arthritis: Clinical experience from northwest India. Rheumatol Int (epub). doi:10.1007/s00296-010-1711-z
35. Hot A, Madoux MH, Viard JP, Coppéré B, Ninet J (2008) Successful treatment of cytomegalovirus-associated hemophagocytic syndrome by intravenous immunoglobulins. Am J Hematol 83 (2):159-162
36. Katz U, Achiron A, Sherer Y, Shoenfeld Y (2007) Safety of intravenous immunoglobulin (IVIG) therapy. Autoimmun Rev 6 (4):257-259
37. Ravelli A, De Benedetti F, Viola S, Martini A (1996) Macrophage activation syndrome in systemic juvenile rheumatoid arthritis successfully treated with cyclosporine. J Pediatr 128(2):275-278
38. Ramanan AV, Schneider R (2003) Macrophage activation syndrome following initiation of etanercept in a child with systemic onset juvenile rheumatoid arthritis. J Rheumatol 30(2):401-403
39. Bruck N, Suttorp M, Kabus M, Heubner G, Gahr M, Pessler F (2011) Rapid and sustained remission of systemic juvenile idiopathic arthritis-associated macrophage activation syndrome through treatment with anakinra and corticosteroids. J Clin Rheumatol 17(1):23-27
40. Sawhney S, Woo P, Murray KJ (2001) Macrophage activation syndrome: A potentially fatal complication of rheumatic disorders. Arch Dis Child 85(5):421-426
41. Pinto L, Kagalwala F, Singh S, Balakrishnan C, Prabhu SV, Khodaiji S (2007) Macrophage activation syndrome: Experience from a tertiary referral centre. J Assoc Physicians India 55:185-187
42. Kaito K, Kobayashi M, Katayama T, Otsubo H, Ogasawara Y, Sekita T, Saeki A, Sakamoto M, Nishiwaki K, Masuoka H, Shimada T, Yoshida M, Hosoya T (1997) Prognostic factors of hemophagocytic syndrome in adults: Analysis of 34 cases. Eur J Haematol 59 (4):247-253