Adult Onset Still’s Disease: A Review on Diagnostic Workup and Treatment Options

Abstract

Adult onset Still's disease (AOSD) is a rare systemic inflammatory disease of unknown etiology and pathogenesis that presents in 5 to 10% of patients as fever of unknown origin (FUO) accompanied by systemic manifestations. We report an interesting case of a 33-year-old African-American male who presented with one-month duration of FUO along with skin rash, sore throat, and arthralgia. After extensive workup, potential differential diagnoses were ruled out and the patient was diagnosed with AOSD based on the Yamaguchi criteria. The case history, incidence, pathogenesis, clinical manifestations, differential diagnoses, diagnostic workup, treatment modalities, and prognosis of AOSD are discussed in this case report.

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.…”

“…But his productive cough, oral ulceration, muscle cramp, weight loss (10.15%) indicate inflammatory process continued and release of inflammatory mediators. 10 Of all patients with Still's disease, 100% patient have high intermittent fever, 40-45% have hepatosplenomegaly 5,8,10 , 85% have a marked Leukocytosis, hepatomegaly, progressively rising erythrocytes sedimentation rate and ferritin level appears to correlate well with the activity of illness. 8,10,15 The underlying mechanism of this is probably continuation of inflammatory process.…”

“…10 Of all patients with Still's disease, 100% patient have high intermittent fever, 40-45% have hepatosplenomegaly 5,8,10 , 85% have a marked Leukocytosis, hepatomegaly, progressively rising erythrocytes sedimentation rate and ferritin level appears to correlate well with the activity of illness. 8,10,15 The underlying mechanism of this is probably continuation of inflammatory process. A hallmark of AOSD is neutrophil 10 and macrophage activation 15 possibly under the effects of the proinflammatory interleukin-18 (IL-18) signalling.…”

“…Clinically, most of the patients with AOSD present with fever, sore throat, arthralgia, arthritis, and/or skin rash but some patients may also have lymphadenopathy, hepatosplenomegaly and /or serositis. 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.…”

“…12 Serum ferritin levels are typically increased during disease flares and this finding is useful for diagnosis and monitoring of AOSD . 8,10,13,15 Elevated ferritin level is a nonspecific but common finding 8…”