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

Abstract

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.

Keywords

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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

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