Téma:  Case Studies

Adult form of still´s disease with refraction pleuritis without significant hyperferitinemia

Veronika Csibová, Michal Rastislav Piják, Štefan Hrušovský, Martin Demeš, Štefánia Moricová

The authors present a case report of a 46-year-old female patient with adult Still´s disease with predominant mass exudative polyserositis in clinical picture. Diagnosis of Still´s disease was determined on basis of Yamaguchi, Cush and Fautrel criteria after eliminating infectious, oncological and other diseases with similar clinical picture. Polyserositis present at the onset of the disease needed repeated evacuated pleural punctions and pericardial fenestration. Clinical manifestations receded after corticoid therapy detraction of which was recurrently linked with clinical relapse without manifestation of serositis but with subtle maculopapulous exanthema. After added metotrexate the patient was in clinical remission for several months. After infection of respiratory tract, relapse developed again, with the picture of bilateral pleural exudates. After increasing dose of corticosteroids and adding cyclosporine the mass pleural exudates persisted though other clinical and lab manifestations retreated. We have never read about the similar case. The case report deserves attention due to uniqueness of the disease without significant hyperferitinemia that is considered pathognomonic for this disease and resistance of pleuritis to standard immunosuppressive therapy. Uniqueness of the disease is also in atypical cells population in the bone marrow.

Ročník 2009  Témy časopisu Internal medicine 7 / 2009

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