Juvenile idiopathic arthritis: Symptoms of rheumatism, arthritis in children?

Juvenile idiopathic arthritis: Symptoms of rheumatism, arthritis in children?
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The most common long-term rheumatic disease of childhood is called: Juvenile idiopathic arthritis. Cause unknown. Treatment is necessary and very important.


Juvenile idiopathic arthritis is one of the most common long-term rheumatic inflammatory diseases of childhood, but also one of the most significant chronic problems in children.

Wondering what the long name means?

Juvenile = intervening childhood (juvenile). More precisely, this term refers to diseases related to the childhood period, which arose before the age of 16.

Idiopathic = a disease that has an unknown cause (idiopathic).

Arthritis = a general term for inflammatory joint disease (arthritis).

The disease is also called JIA.

A more precise definition of the child forms of arthritis that:
These are each at least one joint arthritis,
which occurs before the age of 16 years of age
lasting at least six weeks
with unknown cause

The basis of the disease is autoimmune inflammation. Basically, this inflammation is caused by the child's immunity, which does not work properly and attacks the body's own cells, tissues

The disease is said to be one of the most common chronic rheumatic diseases of childhood. There are approximately 2 - 20 cases per 100,000 children. Girls are affected 2 to 3 times more often.

Due to the link to childhood, it is also possible to find an indication that JIA has two main peaks in the start, namely: 
in children under 3 years of age
and the second  
in children at puberty

Seven forms of juvenile idiopathic arthritis in the table

Juvenile idiopathic arthritis is not a single disease but combines several subtypes. And they are divided into 7 forms.

Classification of juvenile idiopathic arthritis was modified in 2011 by the International League Against Rheumatism - ILAR International League of Associations for Rheumatology.

7 forms of juvenile idiopathic arthritis in the table

Form Description
Oligoarticular JIA
  • oligoarthritis
  • up to 6 months of the course of the disease, a maximum of 4 joints are affected
  • about 30% of cases
  • mostly children aged 1 to 6 years
    • often only 1 inflamed joint
  • persistent oligoarticular JIA 
    • if the course continues by affecting a maximum of 4 joints
  • extended oligoarticular JIA
    • inflammation spreads to 5 or more joints
  • has potentially serious complications
  • frequent delays in diagnosis
  • children cannot describe their difficulties
    • joint pain or morning stiffness
  • objectively often seen
    • curvature
    • fatigue
    • refusal to walk, run
    • it gets up worse in the morning
    • after moving - the difficulties subside
  • may have extra-articular complications
    • Inflammation of the eye
Polyarticular JIA RF positive
  • already during the first 6 months of the disease5 or more joints are affected
  • positive rheumatoid factor present - RF present
  • polyarticular juvenile rheumatoid arthritis with positive rheumatoid factor
  • a related type with rheumatoid arthritis in adulthood
  • rare form uncommon, only 5% of cases
  • mostly in adolescent girls at puberty
  • mainly affects small joints
    • symmetrical arthritis, the same joints on both sides of the body
    • later also larger joints, knees, ankles
  • rheumatic nodes
  • complications include
    • vasculitis 
    • lung damage
    • uveitis does not occur - inflammation of the iris
  • more aggressive course and worse prognosis
  • early detection and early treatment are very important!
Polyarticular  RF negative form
  • polyarticular juvenile rheumatoid arthritis negative for RF factor
  • also affecting 5 or more joints
  • rheumatoid factor is not present
  • 15 - 20% of cases
  • at any age
  • in both sexes
  • any joint
System JIA
  • older name than Still's disease
  • is characterized by a systemic origin
  • fever, even above 39 ° C,  precedes or accompanies the joint inflammation
    • lasts at least 2 weeks
  • inflammation of one but also more joints
  • is complicated by the association of difficulties such as:
    • sowing of skin rashes - trunk and upper limbs
    • generalized (whole body) enlargement of lymph nodes - lymphadenopathy
    • enlargement of the liver (hepatomegaly) and spleen (splenomegaly)
    • tachycardia - fast heart beat
    • pericarditis - inflammation of the pericardium
    • inflammation of the iris of the eye
  • overall interferes with the development and growth of the child - growth disorders - shortening of the limb
  • alleviation of difficulties in about half of the cases
  • the other half deterioration of the course and progression of the condition
Arthritis associated with enthesitis
  • involvement of tendons (enthesia), tendonitis - enthesitis
  • mostly in boys older than 6 years - up to 7 times more often
  • the presence of the HLAB27 antigen
  • associated diseases in the family - ankylosing spondylitis, non-specific intestinal inflammation and other
  • any tendons, especially the Achilles tendon, leg tendons, knee apples
  • mostly inflammation of the larger joints of the lower limbs, knees, hips and ankles
  • involvement of the spinal joints and the sacroiliac joint are also associated
  • inflammatory pain of the back, lower back, in the sitting area and lumbar joint
  • a similar picture of ankylosing spondylitis
  • also referred to as spondyloarthropathy
  • acute uveitis - inflammation of the iris - may also be associated
  • in some cases it recedes
  • in others it spreads to affect the spine
Psoriatic arthritis
  • joint damage associated with psoriasis - psoriasis
  • frequent swelling of the fingers - dactylitis - reminiscent of sausages, sausage type of swelling
  • nail damage
  • skin redness to the formation of scales - map-like in certain areas - elbows, hairy part and others
  • more in preschool girls and in the 10th year of life
  • most often small joints of the hand and foot + knee
  • often eye inflammation
  • You can read more in a separate article on Health: Psoriatic arthritis
Undifferentiated arthritis
  • indicates the type of arthritis
    • meet criteria of  2 or more forms simultaneously
    • does not meet the criteria of either


The causes of juvenile idiopathic arthritis are unknownHowever, a multifactorial effect is expected.


Several factors are involved in the creation of the JIA. The trigger is a combination of genetic predisposition and environmental - external factors, the influence of the external environment.

Behind the problem is an inflammatory reaction that damages the joints and their surroundings. The autoimmune component means that it is a diseased immune response that attacks the body's own cells.

The joint contains cartilage, which has a damping meaning and protects the bone of the joint. There is a thin layer of synovial fluid on the cartilage, made up of sons.

Synovial fluid = lubricant, reduces friction.

The basis of damage to the inflamed synovium - synovitis.

This is the cause of increased fluid formation in the joint, as well as an increase in the synovial layer. Excessive pathological tissue is formed - pannus. The cartilage is gradually destroyed, which is caused by damage to the bone near the joint.

Inflammation then manifests as:

  1. pain - pain
  2. swelling - tumor
  3. local temperature rise - calor
  4. redness  - rubor
  5. dysfunction - lactic function

Risk factors for JIA

  • genetic predisposition
  • familial occurrence - not direct inheritance, but an increased presumption if rheumatic diseases occur in the family
  • presence of HLA antigens
  • infection - virus, bacteria, outbreak after infection
  • hormonal changes
  • stress and long-term mental overload
  • injury
  • drugs - some drugs
  • dysregulation of the autonomic nervous system

The risk of developing JIA is up to 12 times higher in the case of familial occurrence.


Symptoms of juvenile idiopathic arthritis are characterized by the presence of joint damage. Only one joint can be affected, but also several joints of the human body.

The various subtypes preferably affect small and small joints, such as the arms or legs. Others, on the other hand, have large joints, ankles, knees, or hips.

When one and at most 4 joints are affected, it is an oligoarticular disease and 5 or more are polyarticular diseases. 

Forms of JIA with the main characteristics are listed in the table.

Inflammatory joint disease has its typical symptoms.

As already mentioned, inflammation is manifested by:
1. pain
2. swelling 
3. increase in temperature in the area of ​​inflammation
4. reddening of the skin above the inflamed joint 
5. impaired joint function

The general symptoms of arthritis range between these 5 manifestations, with varying variability, intensity, and combination.

Typical joint symptoms:

  • joint stiffness
    • characteristically especially in the morning after waking up and during inactivity
    • passes in about 30-60 minutes 
  • swelling of the joints - joints
  • knuckle ache
  • sensitivity in the area of ​​joints and tendon attachments
  • reddening of the skin above the joint
  • warm skin over the joint
  • warming up and moving relieves difficulties
  • limitation of joint mobility - curvature, inability to stand up, and mobility disorders

In young children who are not yet able to express their subjective difficulties, such as pain or weakness, it is possible to observe outward bleeding or rejection of normal physical activities.

Impairment and curvature occurred without injury.

A child can be observed even higher fatigability, overall fatigue, weakness, and listlessness. It may complain of pain in feet and hands, and after a long walk and manipulation with hands, in the morning he difficult to get up from bed.

In JIA, the occurrence of a growth disorder is also a possibility. Inflammation affecting the proximal area of ​​the growth cleft can cause two conditions =  asymmetric limb growth.

  1. stimulation of the growth cleft by inflammation = the limb on the side of inflammation will be longer
  2. growth arrest by premature closure of the growth gap = the limb on the inflammatory side will be shorter.

Particular attention should be paid to disability:

  • temporomandibular joint - TMK - sleep - sled joint
    • joint pain
    • movement limitation - a problem with eating
    • sledge deflection in unilateral disability
    • restriction of sledge growth - negative effect on tooth growth
  • atlantoaxial joint - the connection between the skull and the spine
    • the inflammatory process causes changes in the cervical vertebrae
    • the risk is the oppression of the spinal cord
    • pain in the cervical spine and head
    • stiff neck

Out-of-joint manifestations of JIA

Plus, joint manifestations don't have to be the only ones.

In autoimmune rheumatic diseases, it is common for non-joint disorders to be associated. These can affect different parts of the human body.

Eyes and vision problems

A common example is the eyes, but also the heart and the cardiovascular system or the digestive system.

In some forms, it is common for eye inflammation to occur, such as uveitis, which is inflammation of the iris, cornea - keratitis. Iridocyclitis - inflammation of the anterior segment of the eye, and therefore the iris and the wrinkled body of the eye.

The eyes and conjunctiva are red, irritated, the eyes itch. In severe cases, there can be a risk of eye damage and even blindness. 

Heart and blood vessels

Another complication may be pericarditis, ie pericarditis or myocarditis, which is inflammation of the heart muscle. Inflammation of the blood vessels is dangerous - vasculitis or lung damage.

The digestive system also ...

Possible problems related to the digestive system. Then it may be a non-specific inflammation of the intestine, but also an enlargement of the liver

Lymph nodes

We cannot forget about the enlargement of the lymph nodes, which can also be in the whole-body form as generalized lymphadenopathy. Plus enlargement of the spleen.

And what about leather?

In the case of systemic JIA, skin sowing occurs on the skin in the torso and upper limbs.

The psoriatic form is characterized by the occurrence of maple skin reds with the formation of white scales that peel off.

Rheumatism is also characterized by the formation of rheumatic nodules, which are clearly visible on the outside.

The overall difficulties are a matter of course

In addition, there are general symptoms during the course of the disease, for example:

  • fatigue
  • increased fatigue
  • exhaustion
  • muscle weakness
  • increase in body temperature to fever
  • sweating, night sweats
  • anorexia and weight loss
  • mood disorders, anxiety to depression
  • the pain was reported as pain in the whole body, joints, muscles, as in a viral infection
  • slow growth and failure to thrive


Diagnosis, in the juvenile form, is performed by a pediatrician and a pediatric rheumatologist. Possibly specialists from other fields (ophthalmologist, cardiologist, pulmonologist, etc.)

The anamnesis is significant. Plus, rheumatological examination, blood, and urine collection for laboratory examination and evaluation of inflammatory and RF parameters are added, as well as normal blood counts and others.

In the initial phase, hospitalization of the child is sometimes necessary, especially in younger children and in the case of acute worsening and association of fever.

Performs differential diagnostics and determination of the exact cause of difficulties.

Important are also imaging methods such as X-ray, CT, MRI, USG. Furthermore, arthroscopy or synovial biopsy.

Delay in diagnosis = delay in treatment,
and this may mean temporary disability or permanent disability.


The course of the disease is characterized by variability and individual characteristics. Of course, the appropriate form of the subtype is then determined according to the manifestations of the disease.

While juvenile idiopathic arthritis is a disease that occurs before 16 years of age. Sometimes in children under 3 years, sometimes in older and adolescent children.

Psoriatic arthritis and RF positive polyarticular JIA with predominance in girls. In contrast, arthritis with enthesitis in boys.

In systemic JIA, it is common for fever and other general problems to occur first. Alternatively, they take place together

Only one joint can be affected, but also several joints of the body. these are either the small joints of the hand and foot or large joints such as the ankle, knee, hip. On one side or on both sides.

It is similar to the degree of risk of complications.

It cannot be said that every child will have the same course and the same rate of progression or severity.

Young children are unable to express their difficulties and parents may notice increased fatigue or refusal to move and bend.

It is a long-term disease that can have a slow onset but is also severe with severe pain and associated non-joint difficulties.

Rheumatism means stretching, dragging, so it is a long-term disease that has a progressive character. Especially in case of neglect of treatment.

We can also observe two stages of the disease, namely remission, and relapse.

Remission = a period of relief: relatively no arthritis when no treatment is needed or only maintenance treatment is needed.

Relapse = the phase of recurrence of the disease, after a period of rest that has lasted for at least 6 months.

Early detection of JIA and early treatment help alleviate the progression, ie the progression of the disease, alleviate the difficulties and avoid late complications.

How it is treated: Juvenile idiopathic arthritis

How is Juvenile Idiopathic Arthritis treated? Medication, biological treatment and lifestyle

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Interesting resources

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  • Prakken, B; Albani, S; Martini, A (18 June 2011). "Juvenile idiopathic arthritis". Lancet377 (9783): 2138–49. 
  • Giancane, Gabriella; Consolaro, Alessandro; Lanni, Stefano; Davì, Sergio; Schiappapietra, Benedetta; Ravelli, Angelo (12 August 2016). "Juvenile Idiopathic Arthritis: Diagnosis and Treatment". Rheumatology and Therapy3 (2): 187–207
  • Hemke, Robert; Nusman, Charlotte M.; van der Heijde, Désirée M. F. M.; Doria, Andrea S.; Kuijpers, Taco W.; Maas, Mario; van Rossum, Marion A. J. (14 August 2014). "Frequency of joint involvement in juvenile idiopathic arthritis during a 5-year follow-up of newly diagnosed patients: implications for MR imaging as outcome measure". Rheumatology International35 (2): 351–357. 
  • Sen, Ethan S.; Dick, Andrew D.; Ramanan, Athimalaipet V. (31 March 2015). "Uveitis associated with juvenile idiopathic arthritis". Nature Reviews Rheumatology11 (6): 338–348.
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  • Burnham, Jon M.; Shults, Justine; Dubner, Sarah E.; Sembhi, Harjeet; Zemel, Babette S.; Leonard, Mary B. (August 2008). "Bone density, structure, and strength in juvenile idiopathic arthritis: Importance of disease severity and muscle deficits". Arthritis & Rheumatism58 (8): 2518–2527. 
  • Prahalad S, Glass DN (2002). "Is juvenile rheumatoid arthritis/juvenile idiopathic arthritis different from rheumatoid arthritis?". Arthritis Research4 (Suppl 3): 303–310.
  • Hinks A, Cobb J, Marion MC, Prahalad S, Sudman M, Bowes J, et al. (June 2013). "Dense genotyping of immune-related disease regions identifies 14 new susceptibility loci for juvenile idiopathic arthritis". Nature Genetics45 (6): 664–9
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