Adult onset Still’s disease: a comparison of two clinical cases

Abstract

Still’s disease is a disease of unknown etiology that was identified for the first time in 1897 by George Still who noted the association of fever, arthralgias and cutaneous rash in a group of 22 children. In 1971, Bywaters noticed that this symptomatology could also be found in adult patients. High fever, arthralgias, a diffuse cutaneous rash with sore throat, increased spleen volume and lymph nodes are the clinical manifestations of this disease. However, it shows high variability in its clinical presentation (monocyclic, polycyclic and chronic forms). Blood tests show high levels of white blood cells, increased ferritin levels, and negative autoantibodies and rheumatoid factor. We examined 2 clinical cases that strongly suggested adult Still’s disease: a 38- year old woman and a 30-year old man. The woman came to our attention because of the following symptomatology: fever for more than eleven days before hospitalization and polyarthralgias. Blood tests showed high inflammatory markers (ferritin >35,000), low platelets and increased white blood cells. A diffuse rash and oral aphthae were then observed. The second case presented the following symptoms: fever (38.4°C), cutaneous rash, polyarthralgias, and sore throat. Blood tests showed high levels of inflammatory markers and blood cell count showed an increase in neutrophil levels. Abdominal ultrasonography showed hepatosplenomegaly. Concentrating on these elements allowed us to formulate the diagnosis of adult Still’s disease, both patients showing a highly suggestive clinical profile. Yamaguchi’s criteria were satisfied and different diseases could be excluded according to the pharmacological response to anti-inflammatory drugs. This strengthened our diagnostic hypothesis and allowed us to focus our attention on this unknown disease, often unrecognized because of the problems of diagnosis. In fact, the clinical characters are initially aspecific (sore throat and fever). Furthermore, there is a large inter-individual variability during the disease course that could lead to misunderstanding. However, satisfaction of Yamagouchi’s criteria and the pharmacological response to corticosteroid drugs suggested adult Still’s disease.