Vnitr Lek 2009, 55(10):991-994

24-year old male with febricity, multi-organ dysfunction and fast progressing ARDS

J. Chvojka^*, A. Kroužecký, J. Raděj, R. Sýkora, T. Karvunidis, I. Novák, M. Matějovič

I. interní klinika Lékařské fakulty UK a FN Plzeň, přednosta doc. MUDr. Martin Matějovič, Ph.D.

A 24-year-old man presented with cough, sore throat, fever, maculopapulous exanthema, pericardial and pleural effusion. Despite extensive evaluation neither infectious, autoimmune, hematological nor oncological disorders were revealed. Broad spectrum antibiotic and subsequently corticosteroid treatment failed to resolve the symptoms. Multiorgan failure with rapid progress of acute respiratory distress syndrome and circulatory failure developed and patient died. Adult onset Still's disease (AOSD), a diagnosis considered in this patient, is a rare disease with unknown prevalence, pathogenesis and etiology. Clinically it is characterized by spiking fever, arthritis, rash, and impairment of multiple organs. There is no single diagnostic test for AOSD. Rather, the diagnosis is based on the clinical criteria and requires the exclusion of infectious, neoplastic, and other autoimmune diseases. Rarely the course of the disease can be rapidly progressive to death. Treatment includes the use of non-steroid antirheumatic drugs and corticosteroids. Limited data suggest that biological agents (e. g. anti-TNF-α, anti-IL-1), rituximab or intravenous immunoglobulins might be promising for the treatment of severe cases.

Keywords: fever; exanthema; multiorgan dysfunction; ferritin; adult onset Still's disease

Received: February 25, 2009; Accepted: April 10, 2009; Published: October 1, 2009  Show citation