Abstract
We present a patient who had adult-onset Still’s disease (AOSD) complicated by thrombotic thrombocytopenic purpura (TTP) that resulted in retinal microangiopathy and rapidly fatal cerebral edema. The patient was a 37-year-old male who developed fever, eruption, arthritis and hepatic dysfunction, that, based on close examination, was diagnosed as AOSD. Despite treatment with corticosteroids, the patient developed acute visual field defect, neurological deterioration including convulsions and impaired consciousness, as well as acute renal failure that ultimately resulted in death. Pathological examination of autopsy specimens revealed multiple fibrin thrombi disseminated in small vessels of the brain and kidney, which was consistent with TTP, along with marked cerebral edema. Although TTP has rarely been reported in association with AOSD, awareness of the possible coexistence of these two diseases is important for diagnosis and treatment.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
Reference
Cush JJ, Medsqer TA Jr, Christy WC, Herbert DC, Coopserstein LA. Adult-onset Still’s disease: clinical course and outcome. Arthritis Rheum. 1987;30:186–94.
Yamaguchi M, Ohta A, Tsunematu T, Kasukawa R, Mizushima Y, Kashiwagi H, et al. Preliminary criteria for classification of adult Still’s disease. J Rheumatol. 1992;19:424–30.
Kanno N, Kaneko M, Yatomi Y. Japanese Ministry of Health, Labour and Welfare DIC diagnostic criteria. Rinsho Byori. 2011;Suppl 147:31–5.
Masson C, Myhal D, Menard H, Lussier A. Purpura thrombotique thrombopenique fatal chez une patiente atteinte d’une maladie de Still de L’adulte. Rev Rhum Mal Osteoartic. 1986;53:389–91.
Boki KA, Tsirantonaki MJ, Markakis K, Moutsopoulos HM. Thrombotic thrombocytopenic purpura in adult Still’s disease. J Rheumatol. 1996;23:385–7.
Portoles J, de Tomas E, Espinosa A, Gallego E, Nieva GS, Blanco J. Thrombotic thrombocytopenic purpura and acute renal failure in adult Still’s disease. Nephrol Dial Transpl. 1997;12:1471–3.
Diamond JR. Hemolytic uremic syndrome/thrombotic thrombocytopenic purpura (HUS/TTP) complicating adult Still’s disease: remission induced with intravenous immunoglobulin G. J Nephrol. 1997;10:253–7.
Kuo HL, Huang DF, Lee AF. Thrombotic microangiopathy in a patient with adult onset Still’s disease. J Clin Rheumatol. 2002;8:276–80.
Domingues RB, da Gama AM, Caser EB, Musso C, Santos MC. Disseminated cerebral thrombotic microangiopathy in a patient with adult’s still disease. Arq Neuropsiquiatr. 2003;61:259–61.
Perez MG, Rodwig FR Jr. Chronic relapsing thrombotic thrombocytopenic purpura in adult onset Still’s disease. South Med J. 2003;96:46–9.
Quéméneur T, Noel LH, Kyndt X, Droz D, Fleury D, et al. Thrombotic microangiopathy in adult Still’s disease. Scand J Rheumatol. 2005;34:399–403.
Hirata S, Okamoto H, Ohta S, Kobashigawa T, Uesato M, Kawaguchi Y, et al. Deficient activity of von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura in the setting of adult-onset Still’s disease. Rheumatology (Oxford). 2006;45:1046–7.
Robert V, Eszto P, Perrotez JL, Galzin M, Poussel J-F. Treatment by plasmapheresis of a thrombotic thrombocytopenic purpura associated to a Still’s disease: a case report. Ann Fr Anesth Reanim. 2006;25:532–4.
Wang H-P, Chen H-A, Chen C-H, Liao H-T, Huang D-F. Adult Still’s disease patient developed thrombotic microangiopathy with diffuse digital gangrene. Scand J Rheumatol. 2007;36:76–8.
Okwuosa TM, Lee EW, Starosta M, Chohan S, Volkov S, Flicker M, et al. Purtscher-like retinopathy in a patient with adult-onset Still’s disease and concurrent thrombotic thrombocytopenic purpura. Arthritis Rheum. 2007;57:182–5.
Sayarlioglu M, Sayarlioglu H, Ozkaya M, Balakan O, Ucar MA. Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome and adult onset Still’s disease: case report and review of the literature. Mod Rheumatol. 2008;18:403–6.
Salamon L, Salamon T, Morovic-Vergles J. Thrombotic microangiopathy in adult-onset Still’s disease: case report and review of the literature. Wien Klin Wochenschr. 2009;121:583–8.
El Karoui K, Karras A, Lebrun G, Charles P, Arlet JB, Jacquot C, et al. Thrombotic microangiopathy and Purtscher-like retinopathy associated with adult-onset Still’s disease: a role for glomerular vascular endothelial growth factor? Arthritis Rheum. 2009;61:1609–13.
Yamada R, Nozawa K, Yoshimine T, Takasaki Y, Ogawa H, Takamori K, et al. A case of thrombotic thrombocytopenia purpura associated with systemic lupus erythematosus: diagnostic utility of ADAMTS-13 activity. Autoimmune Dis. 2011;483642.
Agrawal A, Mckibbin M. Purtscher’s retinopathy: epidemiology, clinical features and outcome. Br J Ophthalmol. 2007;91:1456–9.
Acknowledgments
The authors gratefully acknowledge the contributions of Prof. M. Harigai at Department of Pharmacovigilance, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University.
Conflict of interest
None.
Author information
Authors and Affiliations
Corresponding author
About this article
Cite this article
Masuyama, A., Kobayashi, H., Kobayashi, Y. et al. A case of adult-onset Still’s disease complicated by thrombotic thrombocytopenic purpura with retinal microangiopathy and rapidly fatal cerebral edema. Mod Rheumatol 23, 379–385 (2013). https://doi.org/10.1007/s10165-012-0650-9
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10165-012-0650-9