Macrophage activation syndrome complicating adult onset Still's disease: A single center case series and comparison with literature

Seminars in Arthritis and Rheumatism

Volumn 45, Issue 6, 2016, Pages 711-716

  Lenert, Aleksander ^a,b   Yao, Qingping ^a,c  

^a LERNER RESEARCH INSTITUTE   (United States)

^b UNIVERSITY OF KENTUCKY   (United States)

^c STONY BROOK UNIVERSITY MEDICAL CENTER   (United States)

Author keywords

Adult onset Still's disease; Anakinra; Cyclosporine; Hemophagocytosis; Macrophage activation syndrome; Treatment outcomes

Indexed keywords

CYCLOSPORIN; GLUCOCORTICOID; INTERLEUKIN 2 RECEPTOR; METHOTREXATE; RECOMBINANT INTERLEUKIN 1 RECEPTOR BLOCKING AGENT; SALAZOSULFAPYRIDINE; TUMOR NECROSIS FACTOR INHIBITOR; IMMUNOSUPPRESSIVE AGENT; INTERLEUKIN 1 RECEPTOR BLOCKING AGENT;

ADULT; ADULT ONSET STILL DISEASE; AGED; ARTHRALGIA; ARTICLE; BONE MARROW BIOPSY; CLINICAL ARTICLE; CLINICAL FEATURE; COMBINATION CHEMOTHERAPY; DISEASE ASSOCIATION; DISSEMINATED INTRAVASCULAR CLOTTING; ERYTHROCYTE SEDIMENTATION RATE; FEMALE; FEVER; FOLLOW UP; HUMAN; INFLAMMATION; KIDNEY FAILURE; LEUKOCYTOSIS; LIVER DYSFUNCTION; LUNG DISEASE; LYMPHADENOPATHY; MACROPHAGE ACTIVATION SYNDROME; MALE; MONOTHERAPY; PRIORITY JOURNAL; RETROSPECTIVE STUDY; SHOCK; SORE THROAT; COMPLICATION; DISSEMINATED INTRAVASCULAR COAGULATION; LIVER DISEASES; LUNG DISEASES; MIDDLE AGED; PATHOPHYSIOLOGY; RENAL INSUFFICIENCY; SEROSITIS; STILL'S DISEASE, ADULT-ONSET; YOUNG ADULT;

ADULT; CYCLOSPORINE; DISSEMINATED INTRAVASCULAR COAGULATION; FEMALE; GLUCOCORTICOIDS; HUMANS; IMMUNOSUPPRESSIVE AGENTS; INTERLEUKIN 1 RECEPTOR ANTAGONIST PROTEIN; LIVER DISEASES; LUNG DISEASES; MACROPHAGE ACTIVATION SYNDROME; MALE; MIDDLE AGED; RENAL INSUFFICIENCY; RETROSPECTIVE STUDIES; SEROSITIS; SHOCK; STILL'S DISEASE, ADULT-ONSET; YOUNG ADULT;

EID: 84949032766     PISSN: 00490172     EISSN: 1532866X     Source Type: Journal    
DOI: 10.1016/j.semarthrit.2015.11.002     Document Type: Article

References (52)

1. Dhote R., Simon J., Papo T., Detournay B., Sailler L., Andre M.H., et al. Reactive hemophagocytic syndrome in adult systemic disease: report of twenty six cases and literature review. Arthritis Rheum 2003, 49:633-639. 10.1002/art.11368.
2. Efthimiou P., Kadavath S., Mehta B. Life-threatening complications of adult-onset Still's disease. Clin Rheumatol 2014, 33:305-314. 10.1007/s10067-014-2487-4.
3. Schulert G.S., Grom A.A. Pathogenesis of macrophage activation syndrome and potential for cytokine-directed therapies. Ann Rev Med 2015, 66:145-159. 10.1146/annurev-med-061813-012806.
4. Kumakura S., Ishikura H., Kondo M., Murakawa Y., Masuda J., Kobayashi S. Autoimmune-associated hemophagocytic syndrome. Mod Rheumatol 2004, 14:205-215. 10.1007/s10165-004-0293-6.
5. Hot A., Toh M.L., Coppéré B., Perard L., Madoux M.H., 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 2010, 89:37-46. 10.1097/MD.0b013e3181caf100.
6. Jordan M.B., Allen C.E., Weitzman S., Filipovich A.H., McClain K.L. How I treat hemophagocytic lymphohistiocytosis. Blood 2011, 118:4041-4052. 10.1182/blood-2011-03-278127.
7. Arlet J.B., Le T.H., 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, 65:1596-1601. 10.1136/ard.2005.046904.
8. Rosário C., Zandman-Goddard G., Meyron-Holtz E.G., D'Cruz D.P., Shoenfeld Y. The hyperferritinemic syndrome: macrophage activation syndrome, Still's disease, septic shock and catastrophic antiphospholipid syndrome. BMC Med 2013, 11:185. 10.1186/1741-7015-11-185.
9. Ravelli A., Magni-Manzoni S., Pistorio A., Besana C., Foti T., Ruperto N., et al. Preliminary diagnostic guidelines for macrophage activation syndrome complicating systemic juvenile idiopathic arthritis. J Pediatr 2005, 146:598-604. 10.1016/j.jpeds.2004.12.016.
10. Davì S., Minoia F., Pistorio A., Horne A., Consolaro A., Rosina S., et al. Performance of current guidelines for diagnosis of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis. Arthritis Rheum 2014, 66:2871-2880. 10.1002/art.38769.
11. Yamaguchi M., Ohta A., Tsunematsu T., Kasukawa R., Mizushima Y., Kashiwagi H., et al. Preliminary criteria for classification of adult Still's disease. J Rheumatol 1992, 19:424-430.
12. Fautrel B., Zing E., Golmard J.L., Le Moel G., Bissery A., Rioux C., et al. Proposal for a new set of classification criteria for adult-onset still disease. Medicine 2002, 81:194-200.
13. Mehta B.M., Hashkes P.J., Avery R., Deal C.L. A 21-year-old man with Still's disease with fever, rash, and pancytopenia. Arthritis Care Res 2010, 62:575-579. 10.1002/acr.20001.
14. Taylor F.B., Toh C.H., Hoots W.K., Wada H., Levi M. Towards definition, clinical and laboratory criteria, and a scoring system for disseminated intravascular coagulation. Thromb Haemost 2001, 86:1327-1330.
15. Atteritano M., David A., Bagnato G., Beninati C., Frisina A., Iaria C., et al. Haemophagocytic syndrome in rheumatic patients. A systematic review. Eur Rev Med Pharmacol Sci 2012, 16:1414-1424.
16. Zaiontz C. Real Statistics software. Available at: Accessed 13.01.15. http://www.real-statistics.com.
17. Sarkar R.N., Bhattacharya R., Bhattacharyya K., Paul R., Mullick O.S. Adult onset Still's disease with persistent skin lesions complicated by secondary hemophagocytic lymphohistiocytosis. Int J Rheum Dis 2014, 17:118-121. 10.1111/1756-185x.12170.
18. Yanai H., Moriyama S., Hirowatari Y., Kaneko H. Very low density lipoprotein, chylomicron, and lipoprotein (a) are more useful to detect the development of macrophage activation syndrome in adult-onset Still's disease as compared with cytokines and triglyceride. Am J Hematol 2013, 88:828-830. 10.1002/ajh.23517.
19. Orr J., Bury Y., Hudson M., Masson S. Liver transplantation for acute liver failure caused by macrophage activation syndrome. Transpl Int 2013, 26:e105-e108. 10.1111/tri.12176.
20. Banse C., Vittecoq O., Benhamou Y., Gauthier-Prieur M., Lequerré T., Lévesque H. Reactive macrophage activation syndrome possibly triggered by canakinumab in a patient with adult-onset Still's disease. Joint Bone Spine 2013, 80:653-655. 10.1016/j.jbspin.2013.04.011.
21. Zhang X.H., Han Y.M., Wang W.W., Cheng H., Zhu K.J. Hemophagocytic syndrome secondary to adult-onset Still's disease but very similar to lymphoma. Int J Clin Exp Pathol 2012, 5:377-381.
22. 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, 22:137-141. 10.1007/s10165-011-0477-9.
23. Loh N.K., Lucas M., Fernandez S., Prentice D. Successful treatment of macrophage activation syndrome complicating adult Still disease with anakinra. Inter Med J 2012, 42:1358-1362. 10.1111/imj.12002.
24. Fujii K., Kitamura Y., Osugi Y., Koyama Y., Ota T. A case of adult-onset Still's disease complicated by hemophagocytic syndrome and interstitial pneumonia with pneumomediastinum/recurrent pneumothorax. Int J Rheum Dis 2012, 15:e60-e62. 10.1111/j.1756-185X.2011.01691.x.
25. Burgi U., Mendez A., Hasler P., Hullstrung H.D. Hemophagocytic syndrome in adult-onset Still's disease (AOSD): a must for biologics?-case report and brief review of the literature. Rheumatol Int 2012, 32:3269-3272. 10.1007/s00296-010-1497-z.
26. Kobayashi M., Takahashi Y., Yamashita H., Kaneko H., Mimori A. Benefit and a possible risk of tocilizumab therapy for adult-onset Still's disease accompanied by macrophage-activation syndrome. Mod Rheumatol 2011, 21:92-96. 10.1007/s10165-010-0348-9.
27. Gorelik M., Torok K.S., Kietz D.A., Hirsch R. Hypocomplementemia associated with macrophage activation syndrome in systemic juvenile idiopathic arthritis and adult onset Still's disease: 3 cases. J Rheumatol 2011, 38:396-397. 10.3899/jrheum.100833.
28. Besma Ben Dhaou, Hmaidi F.D., Fatma Boussema Sonia Ketari, Jamoussi Lilia, Baili Samir, Kochbati Ouahida, et al. Le syndrome d'activation macrophagique: à propos de 4 observations. Tunis Med 2011, 89:70-75.
29. Yeh H.M., Liu M.F., Chang K.K., Liu S.M., Chen C.H. Adult-onset Still's disease complicated with hemophagocytic syndrome. J Formos Med Assoc 2010, 109:85-88.
30. Kaneko K., Kaburaki M., Muraoka S., Tanaka N., Yamamoto T., Kusunoki Y., et al. Exacerbation of adult-onset Still's disease, possibly related to elevation of serum tumor necrosis factor-alpha after etanercept administration. Int J Rheum Dis 2010, 13:e67-e69. 10.1111/j.1756-185X.2010.01544.x.
31. Yoshizaki A., Kawakami A., Aramaki T., Ida H., Eguchi K. Preferential recovery by an intensive initial therapy from hemophagocytic syndrome complicated with adult onset Still's disease presenting as agranulocytosis and hypercytokinemia. Clin Exp Rheumatol 2008, 26:383.
32. Gianella S., Schaer D.J., Schwarz U., Kurrer M., Heppner F.L., Fehr J., et al. Retinal microangiopathy and rapidly fatal cerebral edema in a patient with adult-onset Still's disease and concurrent macrophage activation syndrome. Am J Hematol 2008, 83:424-427. 10.1002/ajh.21084.
33. Fukaya S., Yasuda S., Hashimoto T., Oku K., Kataoka H., Horita T., et al. Clinical features of haemophagocytic syndrome in patients with systemic autoimmune diseases: analysis of 30 cases. Rheumatology 2008, 47:1686-1691. 10.1093/rheumatology/ken342.
34. Pamuk O.N., Pamuk G.E., Usta U., Cakir N. Hemophagocytic syndrome in one patient with adult-onset Still's disease. Presentation with febrile neutropenia. Clin Rheumatol 2007, 26:797-800. 10.1007/s10067-006-0238-x.
35. Hamidou M., Boutoille D., Masseau A., Garand R., Raffi F. Adult-onset Still disease with hemophagocytic syndrome treated with cyclosporine. Press Med 2005, 34:1634-1636.
36. Park J.H., Bae J.H., Choi Y.S., Lee H.S., Jun J.B., 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, 19:137-141.
37. Arai Y., Handa T., Mitani K. Adult-onset Still disease presenting with disseminated intravascular coagulation. Rinsho Ketsueki 2004, 45:316-318.
38. Hirayama Y., Sakamaki S., Tsuji Y., Sagawa T., Nakajima T., Matsunaga T., et al. Adult-onset Still's disease accompanied by hemophagocytic syndrome at onset. Rinsho Ketsueki 2002, 43:97-101.
39. Susuki J., Nawata M., Koike M., Wada R., Sekigawa I., Iida N., et al. Two cases of reactive hemophagocytic syndrome: a patient with adult-onset Still's disease and a patient with herpes zoster and autoimmune abnormalities. Mod Rheumatol 2001, 11:336-339. 10.3109/s10165-001-8066-y.
40. Stern A., Riley R., Buckley L. Worsening of macrophage activation syndrome in a patient with adult onset Still's disease after initiation of etanercept therapy. J Clin Rheumatol 2001, 7:252-256.
41. Nishida T., Suzuki K., Kuwada N., Nakamura Y., Motoyoshi K., Kamakura K. Hemophagocytic syndrome and adult Still's disease associated with meningoencephalitis and unconsciousness. Intern Med 2001, 40:1037-1040.
42. Takeshita A., Takeuchi T., Nakagawa A., Tsuda Y., Fukuda A., Nariyama K., et al. Adult onset Still's disease with hemophagocytic syndrome and severe liver dysfunction. Hepatol Res 2000, 17:139-144.
43. Kumakura S., Ishikura H., Munemasa S., Adachi T., Murakawa Y., Kobayashi S. Adult onset Still's disease associated hemophagocytosis. J Rheumatol 1997, 24:1645-1648.
44. Coffernils M., Soupart A., Pradier O., Feremans W., Nève P., Decaux G. Hyperferritinemia in adult onset Still's disease and the hemophagocytic syndrome. J Rheumatol 1992, 19:1425-1427.
45. Machaczka M., Klimkowska M. Bone marrow assessment in the diagnosis of acquired hemophagocytic lymphohistiocytosis in adults. Am J Clin Pathol 2015, 143:308-310. 10.1309/ajcpuk8tli2mlyoq.
46. Durand M., Troyanov Y., Laflamme P., Gregoire G. Macrophage activation syndrome treated with anakinra. J Rheumatol 2010, 37:879-880. 10.3899/jrheum.091046.
47. Mouy R., Stephan J.L., Pillet P., Haddad E., Hubert P., Prieur A.M. Efficacy of cyclosporine A in the treatment of macrophage activation syndrome in juvenile arthritis: report of five cases. J Pediatr 1996, 129:750-754.
48. Ravelli A., De Benedetti F., Viola S., Martini A. Macrophage activation syndrome in systemic juvenile rheumatoid arthritis successfully treated with cyclosporine. J Pediat 1996, 128:275-278.
49. Kelly A., Ramanan A.V. A case of macrophage activation syndrome successfully treated with anakinra. Nat Clin Pract Rheumatol 2008, 4:615-620. 10.1038/ncprheum0919.
50. Miettunen P.M., Narendran A., Jayanthan A., Behrens E.M., Cron R.Q. 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 2011, 50:417-419. 10.1093/rheumatology/keq218.
51. Hoffman G.S., Kerr G.S., Leavitt R.Y., Hallahan C.W., Lebovics R.S., Travis W.D., et al. Wegener granulomatosis: an analysis of 158 patients. Ann Inter Med 1992, 116:488-498.
52. Lenert A., Lenert P. Current and emerging treatment options for ANCA-associated vasculitis: potential role of belimumab and other BAFF/APRIL targeting agents. Drug Des Dev Ther 2015, 9:333-347. 10.2147/dddt.s67264.