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What is juvenile idiopathic arthritis (JIA)?
Juvenile idiopathic arthritis is a disease with chronic evolution determined by persistent inflammation at the joint level accompanied by pain, swelling and limitation of mobility. The term “idiopathic” signifies the fact that the exact cause that determines the appearance of joint pathological changes is not known, and “juvenile” attributes this condition children under the age of 16.
Juvenile idiopathic arthritis is the most common form of joint inflammation in children and includes the following clinical subtypes:
- Oligoarthritis involving damage to a maximum of 4 joints
- Polyarthritis involving at least 5 joints
- The systemic form whose evolution includes joint inflammation, fever, feeling of fatigue and skin rashes
- Arthritis associated with enthesitis (inflammation at the level of the tendon insertion on the bone)
- Psoriatic arthritis involves joint inflammation and a scaly rash.
It is currently considered that juvenile idiopathic arthritis is part of the category autoimmune diseases characterized by the existence of an aberrant response of the immune system directed against the body’s own cells.
Learn more about autoimmune diseases | What are they and what exactly triggers them?
Other rheumatic diseases in children
Juvenile dermatomyositis is an inflammatory autoimmune disease whose evolution involves:
- Fatigue
- Muscle weakness manifested by difficulties climbing the stairs or getting out of bed
- Joint pains
- Joint stiffness
- Periorbital edema (swelling of the eyelids)
- Heliotrope rash (purple-pink discoloration of the eyelids)
- Malar rash (redness of the cheeks).
In certain situations, the evolution of the disease may involve the appearance of nodules of increased consistency (hard to the touch) consisting of calcium deposits, these calcinosis lesions being difficult to treat due to the risk of ulcers that it associates.
Systemic lupus erythematosus it is an autoimmune condition with multi-organ manifestation, in the course of which both the joints and the skin, the renal system and the central nervous system can be affected. The incidence of this pathology in the European pediatric population is approximately 1:2,500 children, approximately 15% of the total number of cases being diagnosed before the age of 18.
The skin lesions specific to SLE are typical and have the appearance of butterfly wings, being located on the nose and cheeks in 33-50% of lupus patients.
Other symptoms associated with SLE in children include:
- Alopecia (hair loss)
- Febrile syndrome
- Anorexia (lack of appetite)
- Unintentional weight loss
- Raynaud’s syndrome (blue color of the fingers caused by exposure to cold)
- Swelling of the joints
- myalgia
- Anemia
- The tendency to easy bruising with minimal trauma
- DISORDERS
- Edema in the lower limbs.
Juvenile scleroderma is an autoimmune inflammatory disease that mainly affects female children up to the age of 16. Depending on the associated clinical manifestations, juvenile scleroderma can be localized or systemic, but only 10% of the total number of pediatric patients diagnosed with this disease have the generalized form.
Localized or linear scleroderma involves pathological changes of the skin in the form of spots (morphea) or hardened bands (linear scleroderma), of the tissues underlying the affected integumentary area, uveitis and arthritis. Systemic scleroderma involves Raynaud’s phenomenon, fingertip ulcers, joint swelling and pain, calcinosis and clinical manifestations due to multiorgan damage (lungs, digestive and circulatory system).
Causes and risk factors in juvenile idiopathic arthritis
The exact cause of juvenile idiopathic arthritis is not known however, it is currently considered that the main risk factors involved are represented by genetic predisposition and exposure to infectious environmental factors.
Juvenile idiopathic arthritis – symptoms
The clinical manifestations of JIA vary depending on the type of juvenile arthritis and are mainly represented by:
- Persistent febrile syndrome
- Uveitis (inflammation of the membrane that covers the eyeball uvea)
- Photosensitivity (painful sensitivity of the eyes to light)
- Inflammation and joint stiffness with limitation of its mobility
- Rashes associated especially with febrile pustules
- Scaly plaques on elbows and knees
- Myalgia (muscle pain)
- Inflammation of the insertion points of the tendons on the bones
- Hepatomegaly (increase in size of the liver)
- Splenomegaly
- Lymphadenopathy (swelling of the lymph nodes)
- Pericarditis (heart damage due to inflammation of the sac that surrounds the heart)
- Pleuritis (inflammation of the pleural sheets that cover the lungs).
Juvenile idiopathic arthritis – analysis and diagnosis
The diagnosis of juvenile idiopathic arthritis is established by the rheumatologist with the help of the information provided by the anamnesis and clinical examination of the patient and those obtained after carrying out certain investigations which may include:
- Serum determination of rheumatoid factor
- Determination of AAN antinuclear antibodies
- Identification of HLA-B27, a positive cell marker in approximately 80% of patients with JIA that associates enthesitis
- Imaging investigations such as joint ultrasounds and nuclear magnetic resonance that allow the assessment of the degree of joint damage, but also the long-term monitoring of patients under treatment.
Liver and kidney functional investigations they are made to assess the extent of damage to the internal organs and can be recommended by the attending physician along with non-specific inflammation tests:
- ESR
- C-reactive protein (CRP)
- Blood count – to evaluate the general health of the patient and the potential toxicity of the applied treatment.
Treatment of juvenile idiopathic arthritis
The main therapeutic objectives in the case of juvenile idiopathic arthritis are represented by keeping inflammatory processes under control, reducing pain discomfort and joint stiffness, preventing the occurrence of joint deformations, maintaining and improving the degree of mobility of the pediatric patient with the assurance of normal growth and development processes and the long-term improvement of the patient’s quality of life.
The main classes of drugs used in JIA therapy are represented by non-steroidal anti-inflammatory drugs, analgesics, DMARDs (non-biological therapy), biological therapy and corticosteroids.
Physical therapy reduces the pain discomfort of the patient, improving at the same time the degree of mobility of the affected joints, strengthens the muscles and prevents injuries of the locomotor apparatus that may occur during the practice of physical activities.
Complications of AIJ
The unfavorable evolution of children with idiopathic arthritis can be associated with complications represented by:
- Growth and development retardation
- Eye damage
- Severe joint deformity
- Irreversible joint destruction that requires endoprosthesis.
Can juvenile idiopathic arthritis be prevented?
Unfortunately juvenile idiopathic arthritis cannot be prevented or avoidedbut certain changes in the little one’s lifestyle that include swimming, walking or cycling can improve the general condition of the child by reducing pain discomfort at the joint level.
References:
- Juvenile idiopathic arthritisAndrea T. Borchers, Carlo Selmi, Gurtej Cheema, Carl L. Keen, Yehuda Shoenfeld, M. Eric Gershwin
- Juvenile Idiopathic Arthritis: Diagnosis and TreatmentGabriella Giancane, Alessandro Consolaro, Stefano Lanni, Sergio Davì, Benedetta Schiappapietra, Angelo Ravelli
- Medical Treatment of Juvenile Idiopathic ArthritisPhilip J. Hashkes, Ronald M. Laxer
- Systemic juvenile idiopathic arthritisAlberto Martini
