systemic Juvenile Idiopathic Arthritis (sJIA) Still’s disease

systemic Juvenile Idiopathic Arthritis

 Also classified as

sJIA, Still’s disease

Research – Resources – Information

  • Review Article

    Is Still’s Disease an Auto-inflammatory Syndrome?

    Clinical Characteristics of sJIA

    sJIA represents 10–15% of all JIA, with a broad peak of onset between 0 and 5 years of age, with 2 years being the most common [3], and an equal sex ratio. It is called Still’s disease (AoSD) when it occurs in patients over the age of 16. AoSD is less common than sJIA but the disease features are the same, even severe arthritis occurs exceptionally. Therefore, sJIA and AoSD likely represent a continuum of the same disease entity [6].

    SJIA is defined by [1] the presence of arthritis in one or more joints associated with spiking fever (a typically daily high fever with spike in the evening) persisting for a minimum of 15 days, with at least one of the following manifestations: skin rash (evanescent, non-fixed erythematous rash that accompanies fever spikes), generalized lymphadenopathy, hepatomegaly and/or splenomegaly, or serositis (pleuritis or pericarditis).

    None of the clinical signs is specific to sJIA, especially at presentation, and differential diagnosis can be difficult (bacterial and viral infections, malignancy, and other rheumatic diseases). Moreover, arthritis may be absent at onset and can develop during disease course, usually progressing to a polyarticular and symmetrical involvement.

    The disease course can be highly variable. It can be monocyclic, polycyclic with relapses followed by intervals of remission, or unremitting, leading about half of the patients to a chronic destructive arthritis representing the major long-term problem.

    SJIA shows a strong association with macrophage activation syndrome (MAS), a form of reactive hemophagocytic lymphohistiocytosis (HLH), characterised by an uncontrolled activation of well-differentiated macrophages releasing a high amount of proinflammatory cytokines, particularly IL-18, which belongs to the IL-1 family. MAS is a severe, potentially life-threatening disorder, and clinically characterized by fever, hepatosplenomegaly, lymphadenopathy, neurologic dysfunction, and coagulopathy. Some studies suggest that up to 50% of sJIA patients might have occult MAS [7, 8]. Heterozygous mutations in genes involved in HLH have been described in some subsets of SoJIA patients and might play a role in the development of MAS [9]. Specific criteria for sJIA-associated MAS have been recently proposed [10]. Interestingly, MAS has been recently included as an individual group of autoinflammatory diseases in an updated classification proposed by Masters et al. [5].

    Taken from the International Journal of Inflammation
    Volume 2012 (2012), Article ID 480373, 5 pages


Video on YouTube

STILL’S Disease: Dr. Thomas Lehman Interview


As children head off to college, they become more aware of the role they play in maintaining their health. Prior to leaving for college, pediatricians discuss certain health care topics such as vaccines and management of chronic conditions, but it is also important that the students know their own medical histories and are prepared for the transition. Away from home and their usual source of care, students may have to wait for care in a crisis until medical records are made available. Students can be more prepared to manage their health while away from home with a few steps:

  • Make sure medical records are easily accessible by the college health service
  • Carry a current health insurance card and know about health insurance coverage
  • Talk about how to handle stress and the value of sleep
  • Purchase health care monitoring items such as thermometers

Students with special healthcare needs should consider these items and specifically what additional items they need to manage their health such as medical foods or monitoring equipment. Consider what systems impact your health while at home and how that care will be maintained in a new place.  : A Revised and Updated Directory for the Internet Age  by Icon Health Publications –  Paperback   – August 13, 2002  ( Amazon Books)


Additional Resources and Information

Living with Juvenile Arthritis: A Parent’s Guide by Kimberly Poston Miller  (Sep 10, 2013) Amazon Books

How Juvenile Arthritis Affects the Eyes  This article will give you  information about how juvenile arthritis can cause eye problems – including uveitis – and what can be done to protect your child’s eyes.
What is Juvenile Rheumatoid Arthritis?  Living with Arthritis Today Magazine
The information on this page is preserved from the former International Still’s Disease Foundation, which dissolved in 2017.