Fever of unknown origin: a review of 20 patients with Adult-onset Still's disease

Abstract

In this study we aimed to investigate the findings in patients with adult-onset Still's disease (AOSD) admitted with fever of unknown origin (FUO) during the last 18 years in our unit, in order to discover the ratio of such patients to all patients with FUO during the same period, and to determine the clinical features of AOSD in FUO. The number and the aetiologies of the patients with FUO diagnosed between 1984 and 2001, and the clinical features of those with AOSD, were taken from the patient files. The diagnosis of AOSD was reanalysed according to the diagnostic criteria of Cush et al. [11]. The presumed diagnoses before a diagnosis of AOSD was established were also noted. The chi(2) and Fisher's exact tests were used for statistical analysis. We studied 130 patients with a diagnosis of FUO, 36 (28%) of whom had collagen vascular diseases. Of these 36 patients, 20 (56%, 12 female, 8 male, mean age 34 years, range 16-65) had AOSD. Clinical and laboratory findings were as follows: fever (100%), arthralgia (90%), rash (85%), sore throat (75%), arthritis (65%), myalgia (60%), splenomegaly (40%), hepatomegaly (25%), lymphadenopathy (15%), anaemia (65%), neutrophilic leukocytosis (90%), increased erythrocyte sedimentation rate (100%), elevated transaminase levels (65%), a negative RF (100%), and a negative FANA (80%). Antibiotics had been prescribed in 18 (90%) of cases. The presumed infectious diagnoses were streptococcal tonsillitis/pharyngitis (50%), infective endocarditis (four patients), sepsis (two patients) and acute bacterial meningitis (two patients). The presumed non-infectious diagnoses were acute rheumatic fever (three patients), seronegative rheumatoid arthritis (two patients) and polymyositis (two patients). Sixteen patients were followed for a mean duration of 30 months (range 2-59). A remission was obtained with indomethacin in three cases (19%), and with prednisolone in the remainder. Relapse was detected in three cases (19%). AOSD is one of the most frequent aetiologies of FUO. During the diagnostic course of a patient with FUO, a maculopapular rash and/or arthralgia and/or sore throat should raise the suspicion of AOSD. Because the disease has heterogeneous clinical findings, certain bacterial infections (e.g. streptococcal pharyngitis and sepsis) are generally considered and the prescribing of antibiotics is common.

Chronic Fever with Severe Weakness - A Case Report

“…3 Rapid diagnoses of AOSD is difficult because of non-specific clinical and laboratory features. 2,8 Our patient had high fever, productive cough Leukocytosis, progressively rising erythrocytes sedimentation rate, hepatomegaly and increased ferritin level confirm the diagnosis of AOSD. 8,10,13,15 The clinical course of AOSD is heterogeneous, with patients falling into one of three clinical patterns.…”

“…8 The fever, typically higher than 39°C, starts suddenly and could present as FUO alone. 2,6,7 Our patient present with Extreme fatigue with accompanying waves of high fevers that rise daily to 102 0 F (39 0 C) or even higher and rapidly return to normal levels or below. Fever spikes often occur at approximately the same time every day.…”

“…10,15 The laboratory investigations can show an increased systemic inflammatory response 12 , such as high white blood cell count (usually 10,000 to 15,000 with more than 80% granulocytes), thrombocytosis, low hemoglobin, ESR and significant increase in serum ferritin level. 2,6,9 ESR level could be >100 mm in some cases. A high level of ferritin seems to be characteristic of AOSD and is seen in nearly 70% of cases.…”

“…8,10,13,15 The clinical course of AOSD is heterogeneous, with patients falling into one of three clinical patterns. 2,4,8,11,15 (1)Monocyclic or self-limiting pattern, which has a single episode of systemic disease of variable duration followed by complete remission. (2)Polycyclic or intermittent pattern, where 2 or more episodes of systemic disease are separated by symptomfree remission period lasting for a minimum of 2 months.…”

An Adult Onset Still's Disease with Normal Ferritin Level

“…[6] Laboratuvar bulguları ise eritrosit sedimentasyon hızı yüksekliği, nötrofil hakim lökositoz, hipergamma-globülinemi, anti-nükleer antikor (ANA) negatifliği, romatoid faktör (RF) negatifliği ve akut faz cevabı ile açıklanamayacak derecede yüksek serum ferritin düzeyidir. [7] Tanı diğer etiyolojiler dışlanarak konulur.…”