Title:
Adult Still's Disease in Spain: Description of New Cases, and Systematic Review

Authors:
Alende-Castro V, Perez-Garcia C, Sande D, Brea Aparicio R, Baz Lomba A, Fernandez Villar J, Valle B, Fernández Morales M, Perez Fernandez S, Velo-García A, Rodriguez Garcia JC, Spain

Abstract:

Background: Adult Still's disease is an inflammatory and very uncommon disorder. While there is a significant body of literatura in children, little is known about this disease in adult population. 

Objectives: to summarize the scientific evidence adult Still's disease in Spain and describe new cases.

Methods:  Patients with diagnosis of Still's disease in our regional hospital were included. Systematic review: We conducted a search of the Pubmed/Medline database. The search identified 75 articles, of which 15 were selected for extraction and data analysis. 60 studies were excluded because they failed to match the main criteria.

Results:  We present 12 new cases of adult Still's disease with a mean age of 39,3 years. The most frequent form of presentation was fever, arthritis or arthralgia and typical rash. None of the patients died. In this systematic review we identified suitable for inclusion 97 additional cases with a mean age of 38,8 years. A potential exposure risk was found in 3 patients. Five patients presented complications and none of the patients died.

Conclusions:  This is the first study to report on an adult Still's disease case series in Galicia. It shows that in this region, the disease affects young population -even in the absence of risk factors- and is linked to a low mortality. The most frequent form of presentation was fever, typical rash and arthritis or arthralgia with complications in some cases. Usually, it is necessary a clinical suspicion to do the diagnosis.