Team Name: Origin of sJIA, aka “Still Swole.”
Region: Origin Story
Base Article: Still GF. On a Form of Chronic Joint Disease in Children. Med Chir Trans. 1897;80:47-60.9. PMID: 20896907
Authors: Montefiore Medical Center Pediatric Rheumatology Fellowship. Malki Peskin, MD; 3rd year Pediatric Rheumatology fellow; Jessica Perfertto, MD; 2nd year Pediatric Rheumatology fellow; Faith-Andrea Ikalina, MD; 2nd year Med-Peds Rheumatology fellow; Samar Sohail, MD; 1st year Pediatric Rheumatology fellow.
Team Overview
Back in the 1800s, when pathologists measured lymph nodes by comparing them to nut sizes, pediatricians age-stratified patients based on tooth-eruption, and rheumatologic treatment included electric currents, doctors knew that children could get rheumatoid arthritis (RA), a chronic, progressive, enlargement of joints that led to damage and limitation. What was not known was that chronic arthritis in childhood could also be associated with extra-articular manifestations and represented an entirely different entity. If only children weren’t historically looked at as little adults, maybe little Alice C would not have remained hospitalized and bed-bound with fever for 2 years. Perhaps little Jane R could have received anakinra sooner, shrinking her lymph nodes from hazelnuts to pine nuts.
Dr. Still first characterized in 1897 what was later to become known as Still’s disease and what is now known as systemic JIA (sJIA). He described a case-series of 22 patients at the Hospital for Sick Children in England and contrasted this entity with its cousin RA. Unlike RA patients with mostly small joint involvement, these children typically had painless swelling of the knees and wrists, as well as earlier involvement of the C-spine. If you listened closely, he reported “creaking” of the tendons or cartilage but “never any bony grating”. He also noted that the female predominance of RA was not seen in this new disease, with boys and girls affected almost equally. Extra-articular manifestations included fevers, lymphadenopathy, splenomegaly, serositis, and anemia. Fevers were either high and intermittent, or continuous mild pyrexia. Dr. Still stressed the impressive splenomegaly and the hazelnut-sized lymphadenopathy as a major distinction. Serositis was also described as “adherent pericardium” and was noted in some symptomatic children, but was otherwise found on autopsy.
Implications
Dr. Still clearly demonstrated that there are marked clinical and pathological differences between adult RA and this new and distinct disease in children. We can also thank him for starting the complicated classification schema for JIA as he also described a different disease that appeared “identical in every respect” to adult RA, likely an early description of what is now known as polyarticular JIA.
Fortunately, as we continue to learn about sJIA, our knowledge no longer comes from autopsies as it did in 25% of Dr. Still’s patients. This point in and of itself should sway you to understand just how revolutionary this description truly was. Noticing that these patients seemed different was the first step towards the development of life-altering therapies which now save sJIA patients on a daily basis.
Despite the medical advances in the 127 years since Dr. Still’s revolutionary publication, children are often still considered little adults in the world of rheumatology research. In rheumatology, we constantly strive to improve our evidence base to enhance our understanding and management of diseases, despite being a realm of rare diseases. The challenges and limitations are even more pronounced in pediatrics. We inherently assume similarity in most of our diseases with their adult counterparts and therefore extrapolate data from adult drug trials to treat our children. Pharmacokinetic and safety differences aside, if pediatric rheumatic diseases differ in more ways than temporal relation to dental eruption as astutely noted by Dr. Still, it is imperative that we continue to work towards trials and observational studies in our pediatric population rather than extrapolations.
Chances in the Tournament
We are still the underdogs in this tournament given that we remain the only pediatric team. However, this revolutionary article’s eloquent Victorian descriptions of this new disease process marked the turning point at which patients with systemic JIA and by extension, the still-eponymous adult-onset Still’s disease, could be researched and treated differently. Dr. Still was a visionary as he was the first to realize that rheumatic diseases differ in children compared to adults in more than just age, though these children were not paid heed until after the 1940s.
Let’s do better than our Victorian counterparts – this is your chance to vote for little Alice C, Jane R, and their modern counterparts. Let’s continue Dr. Still’s efforts to work towards a world where we recognize that children need dedicated, targeted research to continue to improve outcomes. Just as it’s shocking to hear of children with JIA wasting away in a hospital bed for years or being examined in the morgue, hopefully as we continue research in pediatric rheumatology, the current state of affairs for children with rheumatic diseases will be shocking to readers 127 years from now.
Back to the full list of scouting reports.
References
- Still GF. On a Form of Chronic Joint Disease in Children. Med Chir Trans. 1897;80:47-60.9.
- Newton S, Maher D. A brief history of still’s disease. Still’s disease, the 411. https://sdthe411.com/2020/12/10/a-brief-history-of-stills-disease/. Published December 10, 2020. Accessed January 11, 2023.
- Schaller, J. The History of Pediatric Rheumatology. Pediatr Res. 2005;58: 997–1007.
