Синдром активации макрофагов у больных системным ювенильным артритом
https://doi.org/10.14412/1995-4484-2014-202-208
Аннотация
Синдром активации макрофагов (САМ) является одним из гистиоцитарных заболеваний, развивающихся из клеток макрофагального ряда, – гемофагоцитарным лимфогистиоцитозом (ГЛГ). Показано, что ревматиче- ские заболевания нередко сопровождаются развитием CАМ, наиболее часто – при системном варианте юве- нильного артрита (сЮА). Рассматриваются отдельные вопросы патогенеза с концепцией дефекта механиз- мов Т-клеточной цитотоксичности и снижения уровня активности естественных киллеров (NK), сопряжен- ных с мутацией в гене, кодирующем перфорин, – PRF1, а также гиперпродукции Т-лимфоцитами и гистио- цитами ряда цитокинов (интерлейкина 1β – ИЛ1β, интерферона γ, фактора некроза опухоли α, растворимо- го ИЛ2-рецептора), опосредованно ведущих к активации тканевых макрофагов и продукции провоспали- тельных цитокинов. Обсуждаются проблемы диагностики, связанные с низкой чувствительностью и специ- фичностью диагностических критериев гемофагоцитарного синдрома HLH 2010 г., опирающихся на молеку- лярную генетику и патоморфологическую диагностику ГЛГ. Представлены диагностические критерии син- дрома активации макрофагов (2012), разработанные для сЮА, с ведущим значением тромбоцитопении, ги- перферритинемии и патогистологических признаков гемофагоцитоза. Обращено внимание на необходи- мость диагностики субклинических и «мягких» форм САМ, выявления потенциальной группы риска и пре- дотвращения развития САМ. Рассмотрены вопросы дифференциальной диагностики, учитывая схожесть клинических проявлений с сЮА, терапевтической тактики с применением протокола HLH 2004 г., биологи- ческой терапии.
Об авторах
С. Р. Родионовская
ФГБУ «Научно- исследовательский институт им. В.А. Насоновой» РАМН, Москва, Россия
Россия
Россия
И. П. Никишина
ФГБУ «Научно- исследовательский институт им. В.А. Насоновой» РАМН, Москва, Россия
Россия
Россия
Список литературы
1. Grom AA. Macrophage activation syndrome. In: Textbook of pediatric rheumatology. 6th ed. Cassidy JT, Petty RE, Laxer R, Lindsley C, editors. Philadelphia: Saunders, Elsevier; 2011:674–81.
2. Sawhney S, Woo P, Murray KJ. Macrophage activation syndrome: A potentially fatal complication of rheumatic disorders. Arch Dis Child. 2001;85(5):421–6. DOI: http://dx.doi.org/10.1136/adc.85.5.421.
3. Ravelli A, Magni-Manzoni S, Pistorio A, et al. Preliminary diag-
4. nostic guidelines for macrophage activation syndrome complicat- ing systemic juvenile idiopathic arthritis. J Pediatr. 2005;146(5):598–04. DOI: http://dx.doi.org/10.1016/j.jpeds.2004.12.016.
5. Grom AA. Natural killer cell dysfunction: A common pathway in systemic onset juvenile rheumatoid arthritis, macrophage activa- tion syndrome, and hemophagocytic lymphohistiocytosis. Arthritis Rheum. 2004;50(3):689–98. DOI: http://dx.doi.org/10.1002/art.20198.
6. Hadchouel M, Prieur AM, Griscelli C. Acute hemorrhagic, hepat- ic, and neurologic manifestations in juvenile rheumatoid arthritis: possible relationship to drugs or infection. J Pediatr. 1985 Apr;106(4):561–6. DOI: http://dx.doi.org/10.1016/S0022- 3476(85)80072-X.
7. Stephan JL, Zeller J, Hubert P, et al. Macrophage activation syn- drome and rheumatic disease in childhood: a report of four new cases. Clin Exp Rheumatol. 1993 Jul–Aug;11(4):451–6.
8. Behrens EM, Beukelman T, Paessler M, et al. Occult macrophage activation syndrome in patients with systemic juvenile idiopathic arthritis. J Rheumatol. 2007;34(5):1133–8. Epub 2007 Mar 1.
9. Tristano AG. Macrophage activation syndrome: a frequent but under-diagnosed complication associated with rheumatic diseases. Med Sci Monit. 2008;14(3):RA27–36.
10. Avcin T, Tse SM, Schneider R, et al. Macrophage activation syn- drome as the presenting manifestation of rheumatic diseases in childhood. J Pediatr. 2006;148(5):683–6. DOI: http://dx.doi.org/10.1016/j.jpeds.2005.12.070.
11. Pringe A, Trail L, Ruperto N, et al. Macrophage activation syn- drome in juvenile systemic lupus erythematosus: an under-recog- nized complication. Lupus. 2007;16(8):587–92. DOI: http://dx.doi.org/10.1177/0961203307079078.
12. Stephan JL, Kone-Paut I, Galambrun C, et al. Reactive haemophagocytic syndrome in children with inflammatory disor- ders. A retrospective study of 24 patients. Rheumatology. 2001;40(11):1285–92. DOI: http://dx.doi.org/10.1093/rheumatol- ogy/40.11.1285.
13. Silverman ED, Miller JJ, Bernstein B, Shafai T.I. Consumption coagulopathy associated with systemic juvenile rheumatoid arthri- tis. J Pediatr. 1983 Dec;103(6):872–6. DOI: http://dx.doi.org/10.1016/S0022-3476(83)80704-5.
14. Cuende E, Vesga JC, Perez LB, et al. Macrophage activation syndrome as the initial manifestation of systemic onset juvenile idiopathic arthritis. Clin Exp Rheumatol. 2001 Nov–Dec;19(6):764–5.
15. Davies SV, Dean JD, Wardrop CA, Jones JH. Epstein-Barr virus- associated haemophagocytic syndrome in a patient with juvenile chronic arthritis. Br J Rheumatol. 1994;33(5):495–7. DOI: http://dx.doi.org/10.1093/rheumatology/33.5.495.
16. Jacobs JC, Goin LJ, Hanissian AS, et al. Consumption coagu- lopathy associated with gold therapy for juvenile rheumatoid arthritis. J Pediatr. 1984 Oct;105(4):674–5. DOI: http://dx.doi.org/10.1016/S0022-3476(84)80450-3.
17. Ravelli A, Caria MC, Buratti S, et al. Methotrexate as a possible trigger of macrophage activation syndrome in systemic juvenile idiopathic arthritis. J Rheumatol. 2001 Apr;28(4):865–7.
18. Eraso R, Gedalia A, Espinosa LR. Methotrexate as a possible trig- ger of macrophage activation syndrome. J Rheumatol. 2002;29(5):1104–5.
19. Sterba G, Rodriguez G, Sifontes S, Vigilanza P. Macrophage acti- vation syndrome due to methotrexate in a 12 year old boy with dermatomyositis. J Rheumatol. 2004;31(5):1014–5.
20. Lau G, Kwan C, Chong SM. The 3-week sulphasalazine syndrome strikes again. Forensic Sci Int. 2001;122(2–3):79–84. DOI: http://dx.doi.org/10.1016/S0379-0738(01)00476-5.
21. Athreya BH. Is macrophage activation syndrome is a new entity? Clin Exp Rheumatol. 2002;20(2):121–3.
22. Clementi R, Emi L, Maccario R, et al. Adult onset and atypical presentation of hemophagocytic lymphohistiocytosis in siblings carrying PRF1 mutations. Blood. 2002 Sep 15;100(6):2266–7. DOI: http://dx.doi.org/10.1182/blood-2002-04-1030.
23. Filipovich HA. Hemophagocytic lymphohistiocytosis. Immunol Allergy Clin N Am. 2002;22:281–300. DOI: http://dx.doi.org/10.1016/S0889-8561(01)00009-1.
24. Stepp SE, Dufourcq-Lagelouse R, Le Deist F, et al. Perforin gene defects in familial hemophagocytic lymphohistiocytosis. Science. 1999 Dec 3;286(5446):1957–9. DOI: http://dx.doi.org/10.1126/science.286.5446.1957.
25. Vastert SJ, van Wijk R, D’Urbano LE, et al. Mutations in the per- forin gene can be linked to macrophage activation syndrome in patients with systemic onset juvenile idiopathic arthritis. Rheumatology (Oxford). 2010 Mar;49(3):441–9. DOI: http://dx.doi.org/10.1093/rheumatology/kep418.
26. Feldmann J, Callebaut I, Raposo G, et al. MUNC13-4 is essential for cytolytic granules fusion and is mutated in a form of familial hemophagocytic lymphohistiocytosis (FHL3). Cell. 2003;115(4):461–73. DOI: http://dx.doi.org/10.1016/S0092- 8674(03)00855-9.
27. Menasche G, Pastural E, Feldman J, et al. Mutations in Rab27a cause Griscelli syndrome associated with haemophagocytic syn- drome. Nat Genet. 2000;25(2):173–6. DOI: http://dx.doi.org/10.1038/76024.
28. Barbosa MD, Nguyen QA, Tchernev VT, et al. Identification of the homologous beige and ChediakHigashi syndrome genes (LYST). Nature. 1996 Jul 18;382(6588):262–5. DOI: http://dx.doi.org/10.1038/382262a0.
29. Coffey AJ, Brooksbank RA, Brandau O, et al. Host response to EBV infection in X-linked lymphoproliferative disease results from mutations in an SH2-domain encoding gene. Nat Genet. 1998 Oct;20(2):129–35. DOI: http://dx.doi.org/10.1038/2424.
30. Arico M, Danesino C, Pende D, Moretta L. Pathogenesis of haemophagocytic lymphohistiocytosis. Br J Haematol. 2001;114(4):761–9. DOI: http://dx.doi.org/10.1046/j.1365- 2141.2001.02936.x.
31. Menasche G, Feldmann J, Fischer A, de Saint Basile G. Primary hemophagocytic syndromes point to a direct link between lympho- cyte cytotoxicity and homeostasis. Immunol Rev. 2005 Feb;203:165–79. DOI: http://dx.doi.org/10.1111/j.0105- 2896.2005.00224.x.
32. Shimizu M, Yokoyama T, Yamada K, et al. Distinct cytokine pro- files of systemic-onset juvenile idiopathic arthritis-associated macrophage activation syndrome with particular emphasis on the role of interleukin-18 in its pathogenesis. Rheumatology (Oxford). 2010;49(9):1645–53. DOI: http://dx.doi.org/10.1093/rheumatol- ogy/keq133.
33. Jordan MB, Hildeman D, Kappler J, Marrack P. An animal model of hemophagocytic lymphohistiocytosis (HLH): CD8+ T cells and interferon gamma are essential for the disorder. Blood. 2004 Aug 1;104(3):735–43. DOI: http://dx.doi.org/10.1182/blood-2003-10- 3413.
34. Kristiansen M, Graversen JH, Jacobsen C, et al. Identification of the hemoglobin scavenger receptor. Nature. 2001 Jan 11;409(6817):198–201. DOI: http://dx.doi.org/10.1038/35051594.
35. Schaer DJ, Schleiffenbaum B, Kurrer M, et al. Soluble hemoglo- bin-haptoglobin scavenger receptor CD163 as a lineage-specific marker in the reactive hemophagocytic syndrome. Eur J Haemotol. 2005;74(1):6–10. DOI: http://dx.doi.org/10.1111/j.1600- 0609.2004.00318.x.
36. Bleesing J, Prada A, Villanueva J, et al. The diagnostic significance of soluble CD163 and soluble IL2Rα chains in macrophage activa- tion syndrome and untreated new onset systemic juvenile idiopath- ic arthritis. Arthritis Rheum. 2007 Mar;56(3):965–71. DOI: http://dx.doi.org/10.1002/art.22416.
37. Fall N, Barnes M, Thornton S, et al. Gene expression profiling in peripheral blood in untreated new onset systemic juvenile idiopathic arthritis reveals molecular heterogeneity that may predict macrophage activation syndrome. Arthritis Rheum. 2007 Nov;56(11):3793–804. DOI: http://dx.doi.org/10.1002/art.22981.
38. Komp DM, Mcnamara J, Buckley P. Elevated soluble interleukin- 2 receptor in childhood hemophagocytic histiocytic syndromes. Blood. 1989;73(8):2128–32.
39. Moller HJ, Aerts H, Gronbaek H, et al. Soluble CD163: a marker molecule for monocyte/macrophage activity in disease. Scand J Clin Lab Invest Suppl. 2002;237:29–33. DOI: http://dx.doi.org/10.1097/BOR.0b013e32825a6a79.
40. Kelly A, Ramanan AV. Recognition and management of macrophage activation syndrome in juvenile arthritis. Curr Opin Rheumatol. 2007;19(5):477–81. DOI: http://dx.doi.org/10.1097/BOR.0b013e32825a6a79.
41. Ravelli A, Magni-Manzoni S, Pistorio A, et al. Preliminary diag- nostic guidelines for macrophage activation syndrome complicat- ing systemic juvenile idiopathic arthritis. J Pediatr. 2005;146(5):598–04. DOI: http://dx.doi.org/10.1016/j.jpeds.2004.12.016.
42. Davi S, Consolaro A, Guseinova D, et al. An international con- sensus survey of diagnostic criteria for macrophage activation syn- drome in systemic juvenile idiopathic arthritis. J Rheumatol. 2011 Apr;38(4):764–8. DOI: http://dx.doi.org/10.3899/jrheum.100996.
43. Grom AA, Villanueva J, Lee S, et al. Natural killer cell dysfunction in patients with systemic-onset juvenile rheumatoid arthritis and macrophage activation syndrome. J Pediatr. 2003 Mar;142(3):292–6. DOI: http://dx.doi.org/10.1067/mpd.2003.110.
44. Mouy R, Stephan JL, Pillet P, et al. Efficacy of cyclosporine A in the treatment of macrophage activation syndrome in juvenile arthritis: report of five cases. J Pediatr. 1996 Nov;129(5):750–4. DOI: http://dx.doi.org/10.1016/S0022-3476(96)70160-9.
45. Henter JI, Horne A, Arico M, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2007 Feb;48(2):124–31. DOI: http://dx.doi.org/10.1002/pbc.21039.
46. Henter JI, Samuelsson-Horne A, Egeler RM, et al. Treatment of hemophagocytic lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantation. Blood. 2002;100:2367–73. DOI: http://dx.doi.org/10.1182/blood-2002- 01-0172.
47. Coca A, Bundy KW, Marston Bet Huggins J, Looney RJ. Macrophage activation syndrome: serological markers and treat- ment with anti-thymocyte globulin. Clin Immunol. 2009 Jul;132(1):10–8. DOI: http://dx.doi.org/10.1016/j.clim.2009.02.005.
48. Ramanan AV, Schneider R. Macrophage activation syndrome fol- lowing initiation of etanercept in a child with systemic onset juve- nile rheumatoid arthritis. J Rheumatol. 2003 Feb;30(2):401–3.
49. Lurati A, Teruzzi B, Salmaso A. Macrophage activation syndrome during anti-IL-1 receptor therapy (anakinra) in a patient affected by systemic onset idiopathic juvenile arthritis. Paed Rheumatol Online. 2005;(3):79–85.
50. Kessler E, VoraS, Verbsky J. Risk of significant cytopenias after treatment with tocilizumab in systemic juvenile arthritis patients with a history of macrophage activation syndrome. Pediatr Rheumatol Online J. 2012 Aug 29;10(1):30. DOI: 10.1186/1546- 0096-10-30.
51. De Benedetti F, Brunner H, Ruperto N, et al. Randomized trial of tocilizumab in systemic juvenile idiopathic arthritis. N Engl J Med. 2012 Dec 20;367(25):2385–95. DOI: http://dx.doi.org/10.1056/NEJMoa1112802.
52. Kelly A, Ramanan AV. A case of macrophage activation syndrome successfully treated with anakinra. Nat Clin Pract Rheumatol. 2008;4(11):615–20. DOI: 10.1038/ncprheum0919. Epub 2008 Sep 30.
53. Durand M, Troyanov Y, Laflamme P. Macrophage activation syn- drome treated with anakinra. J Rheumatol. 2010;37(4):879–80. DOI: 10.3899/jrheum.091046.
54. Behrens EM, Kreiger PA, Cherian S, Cron RQ. Interleukin 1 receptor antagonist to treat cytophagic histiocytic panniculitis with secondary hemophagocytic lymphohistiocytosis. J Rheumatol. 2006 Oct;33(10):2081–4.
55. Miettunen PM, Narendran A, Jayanthan A, et al. Successful treat- ment of severe paediatric rheumatic disease-associated macrophage activation syndrome with interleukin-1 inhibition fol- lowing conventional immunosuppressive therapy: case series with 12 patients. Rheumatology (Oxford). 2011;50(2):417–9. DOI: 10.1093/rheumatology/keq218. Epub 2010 Aug 7.
56. Balamuth NJ, Nichols KE, Paessler M, Teachey DT. Use of ritux- imab in cinjunction with immunosuppressive chemotherapy for EBV-associated hemophagocytic lymphohistiocytosis. J Pediatr Hematol Oncol. 2007 Aug;29(8):56–73. DOI: http://dx.doi.org/10.1097/MPH.0b013e3180f61be3.
Рецензия
Для цитирования:
Родионовская С.Р., Никишина И.П. Синдром активации макрофагов у больных системным ювенильным артритом. Научно-практическая ревматология. 2014;52(2):202-208. https://doi.org/10.14412/1995-4484-2014-202-208
For citation:
Rodionovskaya S.R., Nikishina I.P. MACROPHAGE ACTIVATION SYNDROME IN PATIENTS WITH SYSTEMIC JUVENILE ARTHRITIS. Rheumatology Science and Practice. 2014;52(2):202-208. (In Russ.) https://doi.org/10.14412/1995-4484-2014-202-208
Просмотров:
9268
Послать статью по эл. почте 