Adult-onset Still’s disease: a report of 28 cases and review of the literature

Abstract

Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology. It is characterized by fever, skin rash, polyarthralgias or polyarthritis, sore throat, hepatosplenomegaly, lymphadenopathy, leukocytosis, liver enzyme elevation, and high serum level of ferritin. Several kinds of skin lesions have been reported in this condition. The aim of this study was to assess the clinical and laboratory aspects of 28 patients with AOSD in central Iran. According to the diagnostic criteria of AOSD, we identified 28 patients between 2002 and 2007. We intended to describe the clinical characteristics, treatment, and outcome of our patients with AOSD. Of 28 patients with AOSD, 21 (75%) were female, 7 (25%) were male. Fever (100%), sore throat (92%), Arthralgia (92%), dermatographism (92%), typical rash (85%) and arthritis (60%) were the most common findings. The mean values of laboratory findings were as follows; C-reactive protein (CRP) level of 14.4 mg/dl, erythrocyte sedimentation rate (ESR) of 91.5 mm/h, leukocyte count of 15744.4/microl. Abnormal levels of aspartate aminotransferase and alanine aminotransferase were observed in 25 (89%) patients. Twenty patients (71%) had high ferritin values (>500 ng/ml). The clinical characteristics were similar to previous series. A febrile polyarthritis was the most frequent presentation form. Dermatographism was frequently encountered phenomenon in our patients with AOSD. Being that dermatographism is a simple inducible skin reaction, along with its sensitivity in active disease, we suggest more controlled studies to validate accuracy and positive predictive value of it in convenient clinical setting in the diagnosis of AOSD and to consider including it in diagnostic criteria.

Adult-onset Still disease: a rare disorder with a potentially fatal outcome

“…DIC is one of the most dramatic events in AOSD and is a possible cause of death [2,18,20,22,28] although complete recovery in several patients has been reported [2,14,22,35,42,[45][46][47][48][49][50]. It can follow or be associated with reactive haematophagocytic syndrome (RHS), a further possible life-threatening complication in AODS [15,17,21,22,43,51]. This event is characterized by an uncontrolled and sustained activation of macrophages associated with impairment of cytotoxic function with a true cytokine storm [22].…”

Adult - onsetStill′s disease: A review

“…It occurs worldwide and has been reported from France, [5,6] Norway, [7] Japan, [8,9] Greece, [10] USA, [11] Taiwan, [12,13] Spain, [14] Korea, [15,16] China, [17,18] Turkey, [19,20] India [21,22] and Iran. [23] The disease affects young people and has a bimodal age distribution with two peaks -at 15-25 and 36-46 years of age. [24] It is generally considered a disorder of youth, [25] but there are several reports of new cases of AOSD in older people.…”

“…[24,26] The disease affects a slightly larger number of women as compared to men. [19,23] On the other hand, in some case series, male-to-female ratio was seen to be high. [4,6,21] …”

“…[67] Dermatographism is a frequently encountered phenomenon in patients with AOSD. [23] The onset of the disease is usually heralded by a sore throat and constitutional manifestations. Sore throat is known as a cardinal sign of AOSD and may be associated with odynophagia.…”

Adult Onset Still’s Disease-a Diagnosis of Exclusion

“…13 Other features of AOSD include lymphadenopathy, 14 hepatosplenomegaly, 15 pericarditis, pleuritis and central nervous system involvement. 16 Laboratory studies show marked ESR elevation and leukocytosis with predominance of neutrophils.…”