Juvenile Idiopathic Arthritis (JIA): Causes, Symptoms, and Treatment

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What is juvenile idiopathic arthritis (JIA)?

Juvenile idiopathic arthritis is a chronically progressive disease characterized by chronic inflammation at the joint level along with pain, swelling and limitation of movement. The word “idiopathic” indicates that the exact cause that determines the appearance of pathological changes is not known, and “juvenile” refers to this condition. children under the age of 16.

Juvenile idiopathic arthritis is the most common form of inflammation in children and includes the following clinical subtypes:

• Oligoarthritis involving damage to up to 4 joints
• Polyarthritis involving at least 5 joints
• The systemic form in which evolution involves joint swelling, fever, feeling tired and skin rashes
• Arthritis associated with enthesitis (inflammation at the point of tendon insertion on the bone)
• Psoriatic arthritis includes joint inflammation and a scaly rash.

Juvenile idiopathic arthritis is currently considered part of the category autoimmune diseases characterized by an abnormal response of the immune system directed against the body’s own cells.

Learn more about it autoimmune diseases | What are they and what exactly motivates them?

Other rheumatic diseases in children

Juvenile dermatomyositis It is an inflammatory autoimmune disease that involves:

• Tired
• Muscle weakness manifested by difficulty climbing the stairs or getting out of bed
• Joint pain
• Stability together
• Periorbital edema (swelling of the eyelids)
• Heliotrope rash (purple-pink color of the eyelids)
• A breach (red cheeks).

In some cases, the disease may develop including the appearance of nodules with increased consistency (difficult to touch) made up of calcium deposits, these calcinosis lesions being difficult to treat because risk of ulcers that it connects.

Systemic lupus erythematosus it is an autoimmune condition with multiorgan exposure, in which both the joints and the skin, the renal system and the central nervous system are affected. The frequency of this pathology in the European pediatric population is about 1: 2,500 children, about 15% of the total number of cases diagnosed before the age of 18.

The skin lesions characteristic of SLE are typical and have the appearance of butterfly wings, 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 food)
• Unexpected weight loss
• Raynaud’s syndrome (blue color of the fingers caused by cold)
• Inflammation of the joints
• myalgia
• Anemia
• The tendency to brush easily with little difficulty
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• Edema in the lower limbs.

Juvenile scleroderma It is an autoimmune 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 who have been diagnosed with this disease have the general form.

Local or linear scleroderma includes pathological changes of the skin in the form of spots (morphea) or hard bands (linear scleroderma), of the tissues under the affected integumentary area, uveitis and arthritis. Systemic scleroderma includes Raynaud’s phenomenon, finger ulcers, joint inflammation and pain, calcinosis and clinical manifestations due to multiple organ 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 are represented by genetic predisposition and exposure to infectious environmental factors.

Juvenile idiopathic arthritis – symptoms

The clinical manifestations of JIA vary according to the type of juvenile arthritis and are mainly represented by:

• Chronic febrile syndrome
• Uveitis (inflammation of the membrane that covers the eyeball uvea)
• Photosensitivity (painful sensitivity of the eyes to light)
• Joint swelling and stiffness with restriction of movement
• Freckles especially associated with febrile pustules
• Shadow plates on the elbows and knees
• Myalgia (muscle pain)
• Inflammation of the insertion points of the tendons on the bones
• Hepatomegaly (increased liver size)
• Splenomegaly
• Lymphadenopathy (inflammation 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 specific studies that may include:

• Serum determination of rheumatoid factor
• Determination of AAN antinuclear antibodies
• HLA-B27 marker, a positive cell marker in approximately 80% of patients with JIA that correlates with psychosis
• Imaging studies such as joint ultrasound and nuclear magnetic resonance that allow to assess the extent of joint damage, but also long-term monitoring of patients undergoing treatment.

Liver and kidney function tests they will be done to assess the degree of damage to the internal organs and can be recommended by the attending doctor along with nonspecific inflammation tests:

• ESR
• C-reactive protein (CRP)
• Blood count – to assess the general health of the patient and possible toxicity of the applied treatment.

Treatment of juvenile idiopathic arthritis

The main therapeutic goals in the case of juvenile idiopathic arthritis are represented by control inflammatory processes under control, reduce pain, discomfort and joint stiffness, prevent joint pain, maintain and improve the level of mobility of the pediatric patient with confirmation of normal growth and development processes and long-term improvement of the patient’s quality of life.

The main classes of drugs used in the treatment of JIA are represented by non-steroidal anti-inflammatory drugs, analgesics, DMARDs (non-biological therapy), biological therapy and corticosteroids.

Physical therapy reduces the discomfort of the patient’s pain, improves at the same time the range of motion of the affected parts, strengthens the muscles and prevents injuries to the locomotor system. which may occur when they use physical activities.

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Unfavorable development of children with idiopathic arthritis can be associated with problems represented by:

• Delayed growth and development
• Eye damage
• Severe joint deformity
• Irreversible joint destruction requiring endoprosthesis.

Can juvenile idiopathic arthritis be prevented?

Unfortunately Juvenile idiopathic arthritis cannot be prevented or avoidedbut specific changes in the lifestyle of the little person that includes swimming, walking or cycling can improve the general condition of the child by reducing the discomfort of pain 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