Life-threatening complications of adult-onset Still's disease

Clinical Rheumatology

Volumn 33, Issue 3, 2014, Pages 305-314

  Efthimiou, Petros ^a,b   Kadavath, Sabeeda ^a   Mehta, Bella ^c  

^a LINCOLN MEDICAL AND MENTAL HEALTH CENTER   (United States)

^b WEILL CORNELL MEDICAL COLLEGE   (United States)

^c WESTCHESTER MEDICAL CENTER   (United States)

Author keywords

Adult onset still's disease; Complications; Etiology; Macrophage activation syndrome (MAS); Pathogenesis; Treatment

Indexed keywords

ABATACEPT; AMINOTRANSFERASE; BLOOD CLOTTING FACTOR 7; CELL ADHESION MOLECULE; COLONY STIMULATING FACTOR 1; CORTICOSTEROID; CYCLOSPORIN A; ETANERCEPT; FERRITIN; GAMMA INTERFERON; IMMUNOGLOBULIN; IMMUNOGLOBULIN G; IMMUNOSUPPRESSIVE AGENT; INTERLEUKIN 1; INTERLEUKIN 18; INTERLEUKIN 6; LIVER ENZYME; METHOTREXATE; NONSTEROID ANTIINFLAMMATORY AGENT; PENICILLAMINE; PLASMINOGEN; PROTHROMBIN; RECOMBINANT INTERLEUKIN 1 RECEPTOR BLOCKING AGENT; RHEUMATOID FACTOR; SALAZOSULFAPYRIDINE; TOCILIZUMAB; TRIACYLGLYCEROL; TUMOR NECROSIS FACTOR ALPHA; UNINDEXED DRUG; VON WILLEBRAND FACTOR CLEAVING PROTEINASE;

ADULT ONSET STILL DISEASE; ADULT RESPIRATORY DISTRESS SYNDROME; ARTHRITIS; BLOOD CLOTTING; BONE MARROW BIOPSY; CLINICAL FEATURE; CONNECTIVE TISSUE DISEASE; CYTOKINE PRODUCTION; DIFFUSE ALVEOLAR HEMORRHAGE; DISEASE ASSOCIATION; DISEASE CLASSIFICATION; DISSEMINATED INTRAVASCULAR CLOTTING; ERYTHROPHAGOCYTOSIS; FEVER; HEPATOMEGALY; HUMAN; HYPERTRANSAMINASEMIA; HYPERTRIGLYCERIDEMIA; HYPOFIBRINOGENEMIA; IMMUNOSUPPRESSIVE TREATMENT; KIDNEY FUNCTION TEST; LIVER FAILURE; LIVER FUNCTION TEST; LUNG ARTERY PRESSURE; LUNG HEMORRHAGE; MACROPHAGE ACTIVATION; MACROPHAGE ACTIVATION SYNDROME; MULTIPLE ORGAN FAILURE; MYOCARDITIS; PATHOGENESIS; PLASMAPHERESIS; PRIORITY JOURNAL; PULMONARY ARTERY OCCLUSION PRESSURE; PULMONARY HYPERTENSION; RASH; RESPIRATORY DISTRESS SYNDROME; REVIEW; SPLENECTOMY; THROMBOCYTOPENIA; THROMBOTIC THROMBOCYTOPENIC PURPURA;

DISSEMINATED INTRAVASCULAR COAGULATION; HUMANS; HYPERTENSION, PULMONARY; MACROPHAGE ACTIVATION SYNDROME; PURPURA, THROMBOTIC THROMBOCYTOPENIC; STILL'S DISEASE, ADULT-ONSET;

EID: 84896493293     PISSN: 07703198     EISSN: 14349949     Source Type: Journal    
DOI: 10.1007/s10067-014-2487-4     Document Type: Review

References (111)

1. Jaqua NT, Finger D, Hawley JS. Adult-onset Still 's disease masquerading as sepsis in an asplenic active duty soldier. Case reports in medicine. 2012;2012:349521. PubMed PMID: 23251171. Pubmed Central PMCID: PMC3522482. Epub 2012/12/20. eng.
2. Duburcq T, Delannoy PY, Sivova N, Leroy O (2013) Adult onset Still's disease revealed by a myocarditis. Ann Fr Anesth Reanim 32(1):50-52, PubMed PMID: 23200908. Epub 2012/12/04. Myocardite revelant une maladie de Still de l'adulte. fre
3. Fautrel B (2008) Adult-onset Still disease. Best Pract Res Clin Rheumatol 22(5):773 -792, PubMed PMID: 19028363. Epub 2008/11/26. eng
4. Efthimiou P, Paik PK, Bielory L (2006) Diagnosis and management of adult onset Still's disease. Ann Rheum Dis 65(5):564-572, PubMed PMID: 16219707. Epub 2005/10/13. eng
5. Kontzias A, Efthimiou P (2008) Adult-onset Still's disease: pathogenesis, clinical manifestations and therapeutic advances. Drugs 68(3):319-337, PubMed PMID: 18257609. Epub 2008/02/09. eng
6. Kontzias A, Efthimiou P (2012) The use of Canakinumab, a novel IL-1beta long-acting inhibitor, in refractory adult-onset Still's disease. Semin Arthritis Rheum 42(2):201-205, PubMed PMID: 22512815. Epub 2012/04/20. eng
7. Yokota S, Tanaka T, Kishimoto T (2012) Efficacy, safety and tolerability of tocilizumab in patients with systemic juvenile idiopathic arthritis. Ther Adv Musculoskelet Dis 4(6):387-397, PubMed PMID: 23227116. Pubmed Central PMCID: PMC3512171. Epub 2012/12/12. eng
8. De Benedetti F, Brunner HI, Ruperto N, Kenwright A, Wright S, Calvo I et al (2012) Randomized trial of tocilizumab in systemic juvenile idiopathic arthritis. N Engl J Med 367(25):2385-2395, PubMed PMID: 23252525. Epub 2012/12/21. eng
9. Ogata A, Tanaka T (2012) Tocilizumab for the treatment of rheumatoid arthritis and other systemic autoimmune diseases: current perspectives and future directions. Int J Rheumatol 2012:946048, PubMed PMID: 22315615. Pubmed Central PMCID: PMC3270395. Epub 2012/02/09. en
10. Pouchot J, Sampalis JS, Beaudet F, Carette S, Decary F, Salusinsky- Sternbach M et al (1991) Adult Still's disease: manifestations, disease course, and outcome in 62 patients. Medicine (Baltimore) 70(2):118-136, PubMed PMID: 2005777. Epub 1991/03/01. eng
11. Ohta A, Yamaguchi M, Kaneoka H, Nagayoshi T, Hiida M (1987) Adult Still's disease: review of 228 cases from the literature. J Rheumatol 14(6):1139-1146, PubMed PMID: 3325642
12. Efthimiou P, Kontzias A, Ward CM, Ogden NS (2007) Adult-onset Still's disease: can recent advances in our understanding of its pathogenesis lead to targeted therapy? Nat Clin Pract Rheumatol 3(6):328-335, PubMed PMID: 17538564. Epub 2007/06/01. eng
13. Cagatay Y, Gul A, Cagatay A, Kamali S, Karadeniz A, InancMet al (2009) Adult-onset Still's disease. Int J Clin Pract 63(7):1050-1055, PubMed PMID: 17511792. Epub 2007/05/22. eng
14. Najam F, Fuchs HA (2001) Cardiac tamponade: a life-threatening complication of Still 's disease. J Clin Rheumatol 7(2):97-101, PubMed PMID: 17039104. Epub 2006/10/14. eng
15. Biron C, Chambellan A, Agard C, Denis M, Germaud P, Hamidou MA (2006) Acute respiratory failure revealing adult-onset Still's disease: diagnostic value of low glycosylated ferritin level. Clin Rheumatol 25(5):766- 768, PubMed PMID: 16283419. Epub 2005/11/12. eng
16. Bagnari V, Colina M, Ciancio G, Govoni M, Trotta F (2010) Adult-onset Still's disease. Rheumatol Int 30(7):855-862, PubMed PMID: 20020138. Epub 2009/12/19. eng
17. Silva CA, Silva CH, Robazzi TC, Lotito AP, Mendroni Junior A, Jacob CM et al (2004) Macrophage activation syndrome associated with systemic juvenile idiopathic arthritis. J Pediatr 80(6):517-522, PubMed PMID: 15622430. Epub 2004/12/29. Sindrome de ativacao macrofagica associada com artrite idiopatica juvenil sistemica. por
18. 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, PubMed PMID: 12563702. Epub 2003/02/04. eng
19. Sawar H, Espinoza LR, Gedalia A (2004) Macrophage activation syndrome and etanercept in children with systemic juvenile rheumatoid arthritis. J Rheumatol 31(3):623, author reply -4. PubMed PMID: 14994420. Epub 2004/03/03. eng
20. Tristano AG (2008) Macrophage activation syndrome: a frequent but under-diagnosed complication associated with rheumatic diseases. Medical science monitor : international medical journal of experimental and clinical research 14(3):RA27-RA36, PubMed PMID: 18301366. Epub 2008/02/28. eng
21. Durand M, Troyanov Y, Laflamme P,Gregoire G(2010)Macrophage activation syndrome treated with anakinra. J Rheumatol 37(4):879-880, PubMed PMID: 20360206. Epub 2010/04/03. eng
22. Arlet JB, D Leth, Marinho A, Amoura Z, Wechsler B, Papo T, et al. Reactive haemophagocytic syndrome in adult onset Still's disease: report of 6 patients and review of the literature. Ann Rheum Dis. 2006 Mar 15. PubMed PMID: 16540551.
23. Singh S, Samant R, Joshi VR (2008) Adult onset Still's disease: a study of 14 cases. Clin Rheumatol 27(1):35-39, PubMed PMID: 17701269. Epub 2007/08/19. eng
24. Sawhney S, Woo P, Murray KJ (2001) Macrophage activation syndrome: a potentially fatal complication of rheumatic disorders. Arch Dis Child 85(5):421-426, PubMed PMID: 11668110. Pubmed Central PMCID: 1718981. Epub 2001/10/23. eng
25. Davi S, Consolaro A, Guseinova D, Pistorio A, Ruperto N, Martini A et al (2011) An international consensus survey of diagnostic criteria for macrophage activation syndrome in systemic juvenile idiopathic arthritis. J Rheumatol 38(4):764-768, PubMed PMID: 21285158. Epub 2011/02/03. eng
26. de Saint Basile G, Fischer A, Dautzenberg MD, Durandy A, Le Deist F, Angles-Cano E et al (1985) Enhanced plasminogen-activator production by leukocytes in the human and murine Chediak-Higashi syndrome. Blood 65(5):1275-1281, PubMed PMID: 4039611. Epub 1985/05/01. eng
27. Ravelli A,Magni-Manzoni S, Pistorio A, Besana C, Foti T, Ruperto N et al (2005) Preliminary diagnostic guidelines for macrophage activation syndrome complicating systemic juvenile idiopathic arthritis. J Pediatr 146(5):598-604, PubMed PMID: 15870661. Epub 2005/05/05. eng
28. Fall N, Barnes M, Thornton S, Luyrink L, Olson J, Ilowite NT et al (2007) Gene expression profiling of peripheral blood from patients with untreated new-onset systemic juvenile idiopathic arthritis reveals molecular heterogeneity that may predict macrophage activation syndrome. Arthritis Rheum 56(11):3793-3804, PubMed PMID: 17968951. Epub 2007/10/31. eng
29. Bleesing J, Prada A, Siegel DM, Villanueva J, Olson J, Ilowite NT et al (2007) The diagnostic significance of soluble CD163 and soluble interleukin-2 receptor alpha-chain in macrophage activation syndrome and untreated new-onset systemic juvenile idiopathic arthritis. Arthritis Rheum 56(3):965-971, PubMed PMID: 17328073. Epub 2007/03/01. eng
30. 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, PubMed PMID: 17343315. Epub 2007/03/09. eng
31. Mazodier K, Marin V, Novick D, Farnarier C, Robitail S, Schleinitz N et al (2005) Severe imbalance of IL-18/IL-18BP in patients with secondary hemophagocytic syndrome. Blood 106(10):3483-3489, PubMed PMID: 16020503. Pubmed Central PMCID: 1895045. Epub 2005/07/16. eng
32. El Karoui K, Karras A, Lebrun G, Charles P, Arlet J-B, Jacquot C, Orssaud C, Nochy D, Pouchot J (2009) Thrombotic microangiopathy and Purtscher-like retinopathy associated with adult-onset Still's disease: a role for glomerular vascular endothelial growth factor? Arthritis Rheum 61:1609-1613. doi:10.1002/art.24826
33. Mizrahi M, Ben-Chetrit E (2009) Relapsing macrophage activating syndrome in a 15-year-old girl with Still's disease: a case report. J Med Case Reports 3:138, PubMed PMID: 20062775. Pubmed Central PMCID: 2803809. Epub 2010/01/12. eng
34. Kelly A, Ramanan AV (2008) A case of macrophage activation syndrome successfully treated with anakinra. Nat Clin Pract Rheumatol 4(11):615-620, PubMed PMID: 18825135. Epub 2008/10/01. eng
35. 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, PubMed PMID: 21169853. Epub 2010/12/21. eng
36. Record JL, Beukelman T, Cron RQ (2011) Combination therapy of abatacept and anakinra in children with refractory systemic juvenile idiopathic arthritis: a retrospective case series. J Rheumatol 38(1): 180-181, PubMed PMID: 21196588. Epub 2011/01/05. eng
37. Pamuk ON, Pamuk GE, Usta U, Cakir N (2007) Hemophagocytic syndrome in one patient with adult-onset Still 's disease. Presentation with febrile neutropenia. Clin Rheumatol 26(5):797-800, PubMed PMID: 16550302. Epub 2006/03/22. eng
38. Nadia EA, Carvalho JF, Bonfa E, Lotito AP, Silva CA (2009) Macrophage activation syndrome associated with etanercept in a child with systemic onset juvenile idiopathic arthritis. The Israel Medical Association journal : IMAJ 11(10):635-636, PubMed PMID: 20077954. Epub 2010/01/19. eng
39. Stern A, Riley R, Buckley L (2001) Worsening of macrophage activation syndrome in a patient with adult onset Still's disease after initiation of etanercept therapy. J Clin Rheumatol 7(4):252-256, PubMed PMID: 17039144. Epub 2006/10/14. eng
40. Kobayashi M, Takahashi Y, Yamashita H, Kaneko H, Mimori A (2011) Benefit and a possible risk of tocilizumab therapy for adult-onset Still's disease accompanied by macrophage-activation syndrome. Modern rheumatology/the Japan Rheumatism Association 21(1):92-96, PubMed PMID: 20737186. Epub 2010/08/26. eng
41. Arai Y, Handa T, Mitani K (2004) Adult-onset Still disease presenting with disseminated intravascular coagulation. Rinsho Ketsueki 45(4):316-318
42. Aellen P, Raccaud O, Waldburger M, Chamot AM, Gerster JC (1991) Still's disease in adults with disseminated intravascular coagulation. Schweizerische Rundschau fur Medizin Praxis= Revue suisse de medecine Praxis 80(15):376-378, PubMed PMID: 2031109. Epub 1991/04/09. Maladie de Still de l'adulte avec coagulation intravasculaire disseminee (CIVD). fre
43. Park JH, Bae JH, Choi YS, Lee HS, Jun JB, Jung S et al (2004) Adult-onset Still's disease with disseminated intravascular coagulation and multiple organ dysfunctions dramatically treated with cyclosporine A. J Korean Med Sci 19(1):137-141, PubMed PMID: 14966357 (Pubitemid 38296291)
44. Sanada I, Kawano F, Tsukamoto A, Kiyokawa T, Shido T, Koga S (1997) Disseminated intravascular coagulation in a case of adult onset Still's disease]. [Rinsho ketsueki. The Japanese journal of clinical hematology 38(11):1194-1198, PubMed PMID: 9423337. Epub 1998/01/10. jpn
45. Colina M, Govoni M, Trotta F (2009) Fatal myocarditis in adult-onset Still disease with diffuse intravascular coagulation. Rheumatol Int 29(11):1355-1357, PubMed PMID: 19048255. Epub 2008/12/03. eng
46. Yokoyama M, Suwa A, Shinozawa T, Fujii T,Mimori T, Akizuki M et al (1995) A case of adult onset Still's disease complicated with adult respiratory distress syndrome and disseminated intravascular coagulation. Nihon Rinsho Men'eki Gakkai kaishi=Japanese journal of clinical immunology 18(2):207-214, PubMed PMID: 7553055. Epub 1995/04/01. jpn
47. Kotter I, Wacker A, Koch S, Henes J, Richter C, Engel A et al (2007) Anakinra in patients with treatment-resistant adult-onset Still's disease: four case reports with serial cytokine measurements and a review of the literature. Semin Arthritis Rheum 37(3):189-197, PubMed PMID: 17583775. Epub 2007/06/23. eng
48. Mori T, Tanigawa M, Iwasaki E, Tamaki S, Ono T, Wada H et al (1993) Cyclosporine therapy of adult onset Still's disease with disseminated intravascular coagulation]. [Rinsho ketsueki. The Japanese journal of clinical hematology 34(2):147-152, PubMed PMID: 8492412. Epub 1993/02/01. jpn
49. Matsumoto K, Nagashima T, Takatori S, Kawahara Y, Yagi M, Iwamoto M et al (2009) Glucocorticoid and cyclosporine refractory adult onset Still's disease successfully treated with tocilizumab. Clin Rheumatol 28(4):485- 487, PubMed PMID: 19184270. Epub 2009/02/03. eng
50. Perez MG, Rodwig FR Jr (2003) Chronic relapsing thrombotic thrombocytopenic purpura in adult onset Still's disease. South Med J 96(1):46-49, PubMed PMID: 12602713. Epub 2003/02/27. eng
51. Gopal M, Cohn CD, McEntire MR, Alperin JB (2009) Thrombotic thrombocytopenic purpura and adult onset Still's disease. Am J Med Sci 337(5):373-376, PubMed PMID: 19322066. Epub 2009/03/27. eng
52. Zheng XL, Kaufman RM, Goodnough LT, Sadler JE (2004) Effect of plasma exchange on plasma ADAMTS13 metalloprotease activity, inhibitor level, and clinical outcome in patients with idiopathic and nonidiopathic thrombotic thrombocytopenic purpura. Blood 103(11):4043-4049, PubMed PMID: 14982878. Epub 2004/02/ 26. eng
53. Masuyama A, Kobayashi H, Kobayashi Y, Yokoe I, Sugimura Y, Maniwa K et al (2013) A case of adult-onset Still's disease complicated by thrombotic thrombocytopenic purpura with retinal microangiopathy and rapidly fatal cerebral edema.Modern rheumatology/ the Japan RheumatismAssociation 23(2):379-385, PubMed PMID: 22623015. Epub 2012/05/25. eng
54. Tandon NN, Rock G, Jamieson GA(1994) Anti-CD36 antibodies in thrombotic thrombocytopenic purpura. Br J Haematol 88(4):816-825, PubMed PMID: 7529543. Epub 1994/12/01. eng
55. Furlan M, Robles R, Galbusera M, Remuzzi G, Kyrle PA, Brenner B et al (1998) von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome. N Engl J Med 339(22):1578-1584, PubMed PMID: 9828245. Epub 1998/11/26. eng
56. Sayarlioglu M, Sayarlioglu H, Ozkaya M, Balakan O, Ucar MA (2008) Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome and adult onset Still's disease: case report and review of the literature. Modern rheumatology/the Japan Rheumatism Association 18(4):403-406, PubMed PMID: 18427722. Epub 2008/04/23. eng
57. Ruggenenti P, Noris M, Remuzzi G (2001) Thrombotic microangiopathy, hemolytic uremic syndrome, and thrombotic thrombocytopenic purpura. Kidney Int 60(3):831-846, PubMed PMID: 11532079. Epub 2001/09/05. eng
58. Onundarson PT, Rowe JM, Heal JM, Francis CW (1992) Response to plasma exchange and splenectomy in thrombotic thrombocytopenic purpura. A 10-year experience at a single institution. Arch Intern Med 152(4):791-796
59. Sumida K, Ubara Y, Hoshino J, Suwabe T, Hiramatsu R, Hasegawa E et al (2010) Etanercept-refractory adult-onset Still's disease with thrombotic thrombocytopenic purpura successfully treated with tocilizumab. Clin Rheumatol 29(10):1191-1194, PubMed PMID: 20225049. Epub 2010/03/13. eng
60. Cheema GS, Quismorio FP Jr (1999) Pulmonary involvement in adult-onset Still's disease. Curr Opin Pulm Med 5(5):305-309, PubMed PMID: 10461535
61. Sari I, Birlik M, Binicier O,Akar S, Yilmaz E, Onen F et al (2009) A case of adult-onset Still's disease complicated with diffuse alveolar hemorrhage. J Korean Med Sci 24(1):155-157, PubMed PMID: 19270830. Pubmed Central PMCID: PMC2650997. Epub 2009/03/ 10. eng
62. Efthimiou P, Georgy S (2006) Pathogenesis and management of adult-onset Still's disease. Semin Arthritis Rheum 36(3):144-152, PubMed PMID: 16949136. Epub 2006/09/05. eng
63. Collard HR, Schwarz MI (2004) Diffuse alveolar hemorrhage. Clin Chest Med 25(3):583-592, vii. PubMed PMID: 15331194. Epub 2004/08/28. eng
64. Schwarz MI, Fontenot AP (2004) Drug-induced diffuse alveolar hemorrhage syndromes and vasculitis. Clin Chest Med 25(1):133-140, PubMed PMID: 15062605. Epub 2004/04/06. eng
65. Yamaguchi M, Ohta A, Tsunematsu T, Kasukawa R, Mizushima Y, Kashiwagi H et al (1992) Preliminary criteria for classification of adult Still's disease. J Rheumatol 19(3):424-430, PubMed PMID: 1578458
66. Campos M, Schiopu E (2012) Pulmonary arterial hypertension in adult-onset Still 's disease: rapid response to anakinra. Case reports in rheumatology 2012:537613, PubMed PMID: 22973530. Pubmed Central PMCID: PMC3437612. Epub 2012/09/14. eng
67. Zen A, Yamashita N, Ueda M, Asakawa Y, Yoshikawa Y, Funai T et al (1990) A case of adult Still's disease with pulmonary hypertension. Ryumachi 30(1):45-52 (Pubitemid 20214460)
68. Chen CH, Chen HA, Wang HP, Liao HT, Chou CT, Huang DF (2006) Pulmonary arterial hypertension in autoimmune diseases: an analysis of 19 cases from a medical center in northern Taiwan. Journal of microbiology, immunology, and infection = Wei mian yu gan ran za zhi 39(2):162-168
69. Mubashir E, Ahmed MM, Hayat S, Heldmann M, Berney SM (2007) Pulmonary hypertension in a patient with adult-onset Stills disease. Clin Rheumatol 26(8):1359- 1361, PubMed PMID: 16871352. Epub 2006/07/28. eng
70. Thakare M, Habibi S, Agrawal S, Narsimulu G (2013) Pulmonary arterial hypertension complicating adult-onset Still's disease. Clin Rheumatol 32(Suppl 1):S1-S2, PubMed PMID: 19669855. Epub 2009/08/12. eng
71. Chatterjee S (2011) Pulmonary hypertension in systemic sclerosis. Semin Arthritis Rheum 41(1):19-37, PubMed PMID: 21047671. Epub 2010/11/05. eng
72. Sanchez O, Humbert M, Sitbon O, Jais X, Simonneau G (2010) Guidelines for the diagnosis and treatment of pulmonary hypertension. Rev Mal Respir 27(2):141-150
73. Hoeper MM (2002) Pulmonary hypertension in collagen vascular disease. Eur Respir J 19(3):571-576, PubMed PMID: 11936539. Epub 2002/04/09. eng
74. Ngian GS, Stevens W, Prior D, Gabbay E, Roddy J, Tran A et al (2012) Predictors of mortality in connective tissue disease-associated pulmonary arterial hypertension: a cohort study. Arthritis Res Ther 14(5):R213, PubMed PMID: 23039366. Pubmed Central PMCID: PMC3580525. Epub 2012/10/09. Eng
75. Sanchez O, Humbert M, Sitbon O, Simonneau G (1999) Treatment of pulmonary hypertension secondary to connective tissue diseases. Thorax 54(3):273-277, PubMed PMID: 10325906. Pubmed Central PMCID: PMC1745447. Epub 1999/05/18. eng
76. Badesch DB, Hill NS, Burgess G, Rubin LJ, Barst RJ, Galie N et al (2007) Sildenafil for pulmonary arterial hypertension associated with connective tissue disease. J Rheumatol 34(12):2417-2422, PubMed PMID: 17985403. Epub 2007/11/07. eng
77. Girgis RE, Frost AE, Hill NS, Horn EM, Langleben D,McLaughlin VV et al (2007) Selective endothelin A receptor antagonism with sitaxsentan for pulmonary arterial hypertension associated with connective tissue disease. Ann Rheum Dis 66(11):1467-1472, PubMed PMID: 17472992. Pubmed Central PMCID: PMC2111639. Epub 2007/05/03. eng
78. Denton CP, Humbert M, Rubin L, Black CM (2006) Bosentan treatment for pulmonary arterial hypertension related to connective tissue disease: a subgroup analysis of the pivotal clinical trials and their open-label extensions. Ann Rheum Dis 65(10):1336-1340, PubMed PMID: 16793845. Pubmed Central PMCID: PMC1798307. Epub 2006/06/24. eng
79. Oudiz RJ, Schilz RJ, Barst RJ, Galie N, Rich S, Rubin LJ et al (2004) Treprostinil, a prostacyclin analogue, in pulmonary arterial hypertension associated with connective tissue disease. Chest 126(2):420-427, PubMed PMID: 15302727. Epub 2004/08/11. eng
80. Badesch DB, Tapson VF, McGoon MD, Brundage BH, Rubin LJ, Wigley FM et al (2000) Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease. A randomized, controlled trial. Ann Intern Med 132(6):425-434 (Pubitemid 30149039)
81. Franchini S, Dagna L, Salvo F, Aiello P, Baldissera E, Sabbadini MG (2010) Efficacy of traditional and biologic agents in different clinical phenotypes of adult-onset Still's disease. Arthritis Rheum 62(8):2530-2535, PubMed PMID: 20506370. Epub 2010/05/28. eng
82. Fitzgerald AA, Leclercq SA, Yan A, Homik JE, Dinarello CA (2005) Rapid responses to anakinra in patients with refractory adult-onset Still 's disease. Arthritis Rheum 52(6):1794-1803, PubMed PMID: 15934079 (Pubitemid 40852888)
83. Kishimoto T (1989) The biology of interleukin-6. Blood 74(1):1-10, PubMed PMID: 2473791. Epub 1989/07/01. eng
84. Akira S, Taga T, Kishimoto T (1993) Interleukin-6 in biology and medicine. Adv Immunol 54:1-78, PubMed PMID: 8379461. Epub 1993/01/01. eng
85. Kishimoto T (2005) Interleukin-6: from basic science to medicine - 40 years in immunology. Annu Rev Immunol 23:1-21, Pub Med PMID: 15771564. Epub 2005/03/18. eng
86. Chen DY, Lan JL, Hsieh TY et al (2004) Clinical manifestations, disease course, and complications of adult-onset Still's disease in Taiwan. J Formos Med Assoc 103:844-852
87. Pouchot J, Sampalis JS, Beaudet F et al (1991) Adult Still's disease: manifestations, disease course, and outcome in 62 patients. Medicine 70:118-136
88. Andres E, Kurtz JE, Perrin AE et al (2003) Retrospective monocentric study of 17 patients with adult Still's disease, with special focus on liver abnormalities. Hepatogastroenterology 50:192-195 (Pubitemid 36182966)
89. Ishikawa M, Masumoto T, Oguni T, Hyoudou N, Michitaka K, Horiike N, Onji M (1997) A case of hemophagocytic syndrome with severe liver injury manifestating adult Still's disease. Nihon Rinsho Meneki Gakkai Kaishi 20:447-452
90. Walker NJ, Zurier RB (2002) Liver abnormalities in rheumatic diseases. Clin Liver Dis 6:933-946 (Pubitemid 36026456)
91. Arlet JB, Le TH, Marinho A et al (2006) Reactive haemophagocytic syndrome in adult-onset Still's disease: a report of six patients and a review of the literature. Ann Rheum Dis 65:1596-1601 (Pubitemid 44799671)
92. Ohta A, Yamaguchi M, Kaneoka H, Nagayoshi T, Hiida M (1987) Adult Still's disease: Review of228 cases from the literature. J Rheumatol 14:1139
93. Wouters JMGW, van Rijswijk MH, van de Putte LBA (1985) Adult onset Still's disease in the elderly: a report of two cases. J Rheumatol 12:1791
94. Esdaile JM, Tannenbaum H (1980) Hawkins adult Still's disease. Am J Med 68:825
95. Tesser JRP, Pisko EJ, Hartz JW, Weinblatt ME (1982) Chronic liver disease and Still' s disease. Arthritis Rheum 25:579
96. Dino O, Provenzano G, Giannuoli E, Sciarrino E, Pouyet M, Pagliaro L (1992) Fulminant hepatic failure in adult onset Still's disease. J Rheum 23:784-785
97. McFarlane M, Harth M, Wall WJ (1997) Liver transplant in adult Still's disease. J Rheum 24:2038-2041 (Pubitemid 27431941)
98. Cush JJ, Medsger TA Jr, Christy WC, Herbert DC, Cooperstein LA (1987) Adult-onset Still's disease. Clinical course and outcome. Arthritis Rheum 30(2):186-194, PubMed PMID: 3827959 (Pubitemid 17060774)
99. Reginato AJ, Schumacher HR Jr, Baker DG, O'Connor CR, Ferreiros J (1987) Adult onset Still's disease: experience in 23 patients and literature review with emphasis on organ failure. Semin Arthritis Rheum 17(1):39-57, PubMed PMID: 3306931 (Pubitemid 17135784)
100. Taillan B, Fuzibet JG, Vinti H, Castela J, Ferrari E, Pesce A et al (1990) Non-infectious mitral endocarditis during Still's disease in adults. A case with histologic study of the endocardium. Ann Med Interne (Paris) 14(1):78-79 (Pubitemid 20111087)
101. Cabane J, Michon A, Ziza JM, Bourgeois P, Bletry O, Godeau P et al (1990) Comparison of long term evolution of adult onset and juvenile onset Still's disease, both followed up for more than 10 years. Ann Rheum Dis 49(5):283-285, PubMed PMID: 2344206 (Pubitemid 20168782)
102. Sayarlioglu M, Ucmak H, Ozkaya M, Ucar MA, Sayarlioglu H (2007) Leflunomide and methotrexate combination in refractory adult-onset Still's disease. Ann Pharmacother 41(7):1319-1320, PubMed PMID: 17535840. Epub 2007/05/31. eng
103. Cefle A (2005) Leflunomide and azathioprine combination in refractory adult-onset Still's disease. Ann Pharmacother 39(4):764-767, PubMed PMID: 15741418
104. Meaux-Ruault N, Magy N, Gil H, Dupond JL (2003) Efficacy of thalidomide in refractory adult Still's disease: a new case report. Clin Exp Rheumatol 21(2):272, PubMed PMID: 12747295 (Pubitemid 36519584)
105. Pouchot J, Arlet J-B (2012) Best Pract Res Clin Rheumatol 26:477-87
106. Efthimiou P P, O. Rilonacept treatment of refractory adult onset Stills disease. Arthritis & Rheumatism. 2009 October 2009; 60.
107. Kraetsch HGAC, Kalden JR, Manger B (2002) Successful treatment of a small cohort of patients with adult onset of Still's disease with infliximab: first experiences. Ann Rheum Dis 61(9):859-860
108. Yang DH, Chang DM, Lai JH, Kuo SY, Ho TY, Lin KM et al (2008) Etanercept as a rescue agent in patient with adult onset Still's disease complicated with congestive heart failure. Rheumatol Int 29(1):95-98, PubMed PMID: 18500457. Epub 2008/05/27. eng
109. Benucci M, Li GF, Del Rosso A, Manfredi M (2005) Adalimumab (anti-TNF-alpha) therapy to improve the clinical course of adult-onset Still's disease: the first case report. Clin Exp Rheumatol 23(5): 733, PubMed PMID: 16173267 (Pubitemid 41300733)
110. Iwamoto M, Nara H, Hirata D, Minota S, Nishimoto N, Yoshizaki K (2002) Humanized monoclonal anti-interleukin-6 receptor antibody for treatment of intractable adult-onset Still's disease. Arthritis and rheumatism 46(12):3388-3389, PubMed PMID: 12483747 (Pubitemid 35453552)
111. Ahmadi-Simab K, Lamprecht P, Jankowiak C, Gross WL (2006) Successful treatment of refractory adult onset Still's disease with rituximab. Ann Rheum Dis 65(8):1117-1118, PubMed PMID: 16837497. Pubmed Central PMCID: 1798247. Epub 2006/07/14. eng