Abstract
Adult onset Still’s disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology. Systemic onset juvenile idiopathic arthritis (SoJIA) is the preferred nomenclature of Still’s disease. Strong association with so-called macrophage activation syndrome (MAS) may provide a clue to the understanding of the distinctive pathogenetic features of SoJIA. MAS is a severe, potentially life-threatening complication characterized by the excessive activation of well-differentiated macrophages. It is more appropriately named autoimmune disease associated reactive hemophagocytic lymphohistiocytosis (ReHLH), a subset of a histiocytic disorder: class II histiocytosis hemophagocytic lymphohistiocytosis (HLH). The relation of SoJIA with HLH is still under debate. We propose that MAS, HLH, SoJIA, and AOSD are indeed the same disease, in different clinical presentations that may be classified based on severity and laboratory findings, but with essentially the same physiopathogenesis. We propose that the case described by Hong & Lee (Rheumatol Int 2008) was actually an AOSD-associated MAS/RHS/ReHLH fulminant disease.
References
Efthimiou P, Paik PK, Bielory L (2006) Diagnosis and management of adult onset Still’s disease. Ann Rheum Dis 65:564–572
Ramanan AV, Grom AA (2005) Does systemic-onset juvenile idiopathic arthritis belong under juvenile idiopathic arthritis? Rheumatology 44:1350–1353
Sawhney S, Woo P, Murray KJ (2001) Macrophage activation syndrome: a potentially fatal complication of rheumatic disorders. Arch Dis Child 85:421–426
Arlet JB, Huong DLT, Marinho A, Amoura Z, Wechsler B, Papo T, Piette JC (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
Janka G, Zur Stadt U (2005) Familial and acquired hemophagocytic lymphohistiocytosis. Hematology Am Soc Hematol Educ Program. 82–88
Janka GE, Schneider EM (2004) Modern management of children with haemophagocytic lymphohistiocytosis. Br J Haematol 124:4–14
Petty RE, Southwood TR, Manners P, Baum J, Glass DN, Goldenberg J, He X, Maldonado-Cocco J, Orozco-Alcala J, Prieur A-M, Suarez-Almazor ME, Woo P (2004) International league of associations for rheumatology classification of juvenile idiopathic arthritis: second revision, Edmonton, 2001. J Rheumatol 31:390–392
Stepp SE, Dufourcq-Lagelouse R, Le Deist F, Bhawan S, Certain S, Mathew PA, Henter J-I, Bennett M, Fischer A, de Saint Basile G, Kumar V (1999) Perforin gene defects in familial hemophagocytic lymphohistiocytosis. Science 286:1957–1959
Wulffraat NM, Rijkers GT, Elst E, Brooimans R, Kuis W (2003) Reduced perforin expression in systemic onset juvenile idiopathic arthritis is restored by autologous stem-cell transplantation. Rheumatology 42:375–379
Hong YH, Lee CK (2008) A case of adult onset Still’s disease with systemic inflammatory response syndrome complicated by fatal status epilepticus. Rheumatol Int 28:931–933
Conflicts of interest statement
There are not potential sources of conflicts of interest.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Félix, F.H.C., Leal, L.K.A.M. & Fontenele, J.B. Cloak and dagger: the case for adult onset still disease and hemophagocytic lymphohistiocytosis. Rheumatol Int 29, 973–974 (2009). https://doi.org/10.1007/s00296-008-0825-z
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00296-008-0825-z