SJIA: Systemic Juvenile Idiopathic Arthritis

What are the symptoms of SJIA?

The symptoms of SJIA come and go in short-term flares. These are the first and most common symptoms of SJIA and often cause:^1,5,6,7

Fever that lasts longer than 2 weeks, reoccurs daily (most often in the late afternoon/evening) and then rapidly returns to normal

Skin rash, red or salmon-pink in color, that often appears on the torso, arms and legs during periods of fever

Arthritis, which causes swollen and painful joints, most often the knees, wrists and ankles

Enlarged internal organs such as lymph nodes, liver, and spleen

If SJIA is poorly controlled, it can cause painful and lasting damage to various parts of the body and seriously affect a child’s quality of life. The earlier SJIA is recognized and treated, the better the outcome is likely to be.

References

1. Arthritis Foundation. Systemic Juvenile Idiopathic Arthritis.  Available from: https://www.arthritis.org/diseases/systemic-juvenile-idiopathic-arthritis (accessed May 2020)
2. Dinarello CA. Blocking IL-1 in systemic inflammation. J Exp Med. 2005;201(9):1355–9.
3. Woo P. Nat Clin Pract Rheumatol. 2006;2:28–34.
4. Behrens EM, Beukelman T, Gallo L et al. J Rheumatol. 2008;35(2):343–8.
5. De Benedetti F, Schneider R. In: Cassidy JT, Laxer RM, Petty RE, Lindsley CB, eds. Textbook of Pediatric Rheumatology. 6th ed. Philadelphia, PA: Saunders Elsevier;2011;236–47.
6. Ravelli A, Martini A. Lancet 2007;369:767–78.
7. Frosch M, Roth J. Rheumatology. 2008;47(2):121–5.
8. Sawhney S, Woo P, Murray K. Arch Dis Child . 2001;85:421–6.
9. Mayo Clinic. Amyloidosis. 2020. Available from: https://www.mayoclinic.org/diseases-conditions/amyloidosis/symptoms-causes/syc-20353178 (accessed May 2020)
10. R. Gurion, T. J. A. Lehman, L. N. Moorthy. International Journal of Inflammation. 2012; Article ID 271569: 1.
11. Ramanan AV, Grom AA. Rheumatology (Oxford) 2005;44(11):1350–3;44(11):1350–3.
12. Beukelman T, Patkar NM, Saag KG, et al. Arthritis Care & Research. 2011; 63(4):465–482.
13. Dewitt EM, Kimura Y, Beukelman T, et al. Arthritis Care & Research. 2012;64(7):1001–1010.
14. Mellins ED, Macaubas C, Grom AA. Nat Rev Rheumatol. 2011;7(7):416–26.
15. Singh-Grewal D, Schneider R, Bayer N et al. Arthritis Rheum. 2006;54:1595–1601.

If your child is displaying any of the symptoms of SJIA, it is important you speak to your doctor as soon as possible and obtain an accurate diagnosis. Click here for guidance on finding a specialist pediatric doctor and what you need to know to prepare for your first appointment with an SJIA expert.

How is SJIA diagnosed?

Correct diagnosis of SJIA has improved greatly in recent years. Most often, children presenting with symptoms of SJIA to their family doctor will be referred to a pediatric rheumatologist. Knowledgeable in autoinflammatory diseases, this specialist doctor is best placed to diagnose SJIA.

In addition to assessing a child’s symptoms (e.g. high fever for at least two weeks, joint pain and inflammation for at least six weeks), the rheumatologist will conduct a comprehensive physical exam. X-rays or ultrasound are often used to detect joint, lung or heart inflammation, while a blood test may be necessary to diagnose SJIA.¹

Real life stories from people with rare disease 

Living with a rare autoinflammatory disease is different from one person to the next. Watch this series of videos below to find out how patients and their families share their experiences of living with SJIA, FMF and other autoinflammatory conditions.

A Day in a Rare Life - SJIA

Download our eBook I’m Rare or Living with Recurrent Fever eBook to learn more from the experiences of others diagnosed with SJIA and a what it means to live with a rare periodic fever syndrome like SJIA

What is life like once SJIA is diagnosed ?

It can be uncomfortable learning that your child has an incurable disease like SJIA. For them it will mean limitations in their day-to-day activities, disruption to their overall development and upset to their emotional wellbeing and quality of life. Find more information and advice on the day-to-day management of SJIA here or follow the inspiring stories of other children and their families here.

How is SJIA treated?

There is no cure for SJIA, but there are treatments which can reduce the severity of the symptoms and even make remission possible.¹ Doctors are so much better equipped to help people with SJIA than they were 20 years ago.

• NSAIDs (non-steroidal anti-inflammatory drugs) can reduce the pain, inflammation and stiffness caused by SJIA
• Corticosteroids lower inflammation
• Biologics may be prescribed¹ to target and block inflammation-causing substances in the body

Exercise is an important part of the SJIA treatment process, as it will help build muscle strength, increase energy and reduce pain.

Good communication with doctors and specialists will help provide a better understanding of the diagnosis and the treatment options available.

What effect does diet have on SJIA ?

Making sure your child maintains a healthy diet is even more important when they have been diagnosed with SJIA. 

Some children affected by SJIA may experience a suppressed appetite, especially during flares. This could be as a result of the disease itself or perhaps a side-effect of a medication they are taking. When a child feels sick and is experiencing symptoms such as joint pain, fatigue, fever or stomach pain, they may find eating difficult. It could also be physically difficult for them to eat, as arthritis in their hands may make using utensils much harder.

Sometimes, though, medications could increase their appetite, meaning they feel hungry a lot of the time and perhaps eat more unhealthy food than they should.¹
 

How can I make sure my child eats healthily?

All this can make it hard to ensure your child is getting all of the nutrients they need in order to grow and be healthy. But there are steps you can take to make food less of a struggle for you and your child. A dietician or nutritional expert can work with you and your child to figure out ways to combat any food-related issues. This could be through behavioural changes such as altering mealtime routines, or it could be through dietary changes and suggestions on types of food to buy and cook.

It could also involve working with the dietician to educate your child about their diet and why it’s important to eat certain foods and get the nutrients they need to help their body grow strong and ready to deal with their flares.
 

Are there any foods which can alleviate SJIA symptoms?

There is currently no evidence to support a special diet for children with SJIA and simply following a balanced diet is recommended. But there has been research into foods which may have a beneficial effect on arthritis symptoms, through fighting inflammation, strengthening bones and supporting the immune system:

• Oily fish such as salmon, tuna and mackerel, as these are high in omega-3 - for children, it might be an idea to make fishcakes, fish pie or have fish toppings for baked potatoes.
• Olive oil – this “good fat” could be added to all sorts of dishes as a topping, dip or simply to cook in.
• Berries – add to yoghurts or make ice lollies with these to give your child a boost in antioxidants and vitamins.
• Vegetables high in Vitamin K such as broccoli, spinach, lettuce and kale – less “childfriendly” foods like these could be whizzed up in a healthy smoothie.
• Citrus fruits that are high in Vitamin C – pop a tasty orange into their lunch bag as an easy snack.
• Nuts including walnuts, pine nuts, pistachios and almonds – a handful of these could make a great snack during the day or before dinner time.
• Beans such as pinto, black, red kidney and garbanzo – why not incorporate into their favourite pasta dishes with tomato sauce.^2-3

These are just some suggestions, but simply doing what you can to help them keep a balanced diet and ensuring that they are receiving the right medical treatment are the most important ways to help them stay healthy with their condition.

References

1. Kidsgetarthritistoo.org. (2018). Juvenile Arthritis Diet | Nutritional Therapy | Arthritis Today. [online] Available at: http://www.kidsgetarthritistoo.org/living-with-ja/daily-life/healthy-eating/juvenile-arthritis-nutrition.php
2. Kidsgetarthritistoo.org. (2018). Juvenile Arthritis | Diet | Kids Get Arthritis Too. [online] Available at: http://www.kidsgetarthritistoo.org/kids-and-teens/teens/every-day-with-ja/healthy-eating-ja-diet.php
3. Jia.org.uk. (2018). Juvenile Idiopathic Arthritis. [online] Available at: https://www.jia.org.uk/diet-and-jia