A Case of Fever of Unknown Origin with a Course of Hemophagocytic Syndrome and Fulminant Hepatitis: Adult Onset Still's Disease

Yıl 2009, Cilt: 14 Sayı: 2, 156 - 159, 01.04.2009

Emrullah Solmazgül Nurittin Ardıç Özkan Sayan Alev Erikçi Hakan Çermik Haldun Uluutku Selim Nalbant Yaşar Küçükardalı

Öz

Adult onset Still's disease (AOSD) is a systemic inflammatory disease of unknown etiology and pathology characterised by polyarthritis, intermittent high fever and typical skin rashes. On rare occasions AOSD may manifest itself as hemophagocytic syndrome (HPS) or fulminant hepatitis. In this paper we describe a 23-year-old male patient pathologically determined to have HPS and fulminant hepatitis, and whom we diagnosed with AOSD while being followed up for fever of unknown origin and investigated for fever, hepatosplenomegaly and pancytopenia, and on whom we performed splenectomy during the diagnosis and treatment stage. In the light of knowledge in the literature and the fatal course followed by our case we are of the opinion that AOSD follows a more fatal course in the presence of HPS or fulminant hepatitis, and that aggressive treatment will be appropriate in such cases.

Anahtar Kelimeler

-

Hemofagositik Sendrom ve Fulminan Hepatit ile Seyreden Nedeni Bilinmeyen Ateş Olgusu: Erişkin Still Hastalığı

Öz

Erişkin Still hastalığı (ESH), etiyoloji ve patogenezi bilinmeyen, poliartrit, intermittan yüksek ateş ve tipik cilt döküntüleri ile karakterize sistemik inflamatuar bir hastalıktır. ESH çok ender olarak hemofagositik sendrom (HFS) veya fulminan hepatit olarak kendini gösterebilir. Biz bu yazıda, nedeni bilinmeyen ateş olarak takip edilip, ateş, hepatosplenomegali, pansitopeni ile araştırılırken HFS olduğu patolojik olarak saptanan ve ESH tanısı koyduğumuz, tanı ve tedavi aşamasında splenektomi uyguladığımız 23 yaşında bir erkek hastayı sunduk. Literatür bilgileri ve olgumuzun mortal seyretmesi ışığında HFS veya fulminan hepatit varlığında ESH daha mortal seyrettiğinden, agresif tedavi uygulanmasınının uygun olacağı kanaatindeyiz.

Anahtar Kelimeler

Erişkin Still hastalığı, hemofagositik sendrom, fulminan hepatit, tedavi

Kaynakça

• Kadar J, Petrovicz E. Adult-onset Still’s disease. Best Pract Res Clin Rheumatol 2004; 18: 663-676.
• Yamaguchi M, Ohta A, Tsunematsu T, et al. Preliminary criteria for classification of adult Still’s disease. J Rheumatol 1992; 19: 424-430.
• Larroche C, Mouthon L. Pathogenesis of hemophagocytic syndrome (HPS). Autoimmun Rev 2004; 3: 69-75.
• Hisano C, Nakamura M, Mayumi T, et al. A case of hemophagocytic syndrome manifesting adult Still’s disease and acute hepatitis. Nihon Rinsho Meneki Gakkai Kaishi 1995; 18: 256-264.
• Ogata A, Kitano M, Yamanaka J, et al. Interleukin 18 and hepatocyte growth factor in fulminant hepatic failure of adult onset Still's disease. J Rheumatol 2003; 30: 1093-1096.
• Regoli G, Trotta M, Agostino I. Differential diagnosis of fever of unknown origin. A case of adult onset Still disease. Minerva Med 1998; 89: 11-13.
• Ishikawa M, Masumoto T, Oguni T, et al. A case of hemophagocytic syndrome with severe liver injury manifestating adult Still's disease. Nihon Rinsho Meneki Gakkai Kaishi 1997; 20: 447-452.
• Dino O, Provenzano G, Giannuoli G, et al. Fulminant hepatic failure in adult onset Still's disease. J Rheumatol 1996; 23: 784-785.
• Tsuda H. Hemophagocytic syndrome (HPS) in children and adults. Int J Hematol 1997; 65: 215-226.
• Min JK, Cho CS, Kim HY, Oh EJ. Bone marrow findings in patients with adult Still’s disease. Scand J Rheumatol 2003; 32: 119-121.
• Kucukardali Y, Oncul O, Cavuslu S, et al. (fever of Unknown Origin Study Group). The spectrum of diseases causing fever of unknown origin in Turkey: a multicenter study. Int J Infect Dis 2008; 12: 71-79.