Juvenile Rheumatoid Arthritis. Sakharova Inna. Ye., MD, Univ. assistant

Juvenile Rheumatoid Arthritis (JRA) is classified as a connective tissue disease. It is a chronic systemic inflammatory disorder that damages the joints of the body.

Clinical features of JRA: Joint pain, stiffness, and swelling: These are the most common symptoms of JRA, but many children do not recognize, or do not report, pain. Stiffness and swelling are likely to be more severe in the morning. Loss of joint function: Pain, swelling, and stiffness may impair joint function and reduce range of motion. Some children are able to compensate in other ways and display little, if any, disability. Severe limitations in motion lead to weakness and decreased physical function and sometimes to invalidization.

Clinical features of JRA: Limp: A limp may indicate a particularly severe case of JRA, although it also may be due to other problems that have nothing to do with arthritis, such as an injury. In JRA, a limp often signals knee involvement.

Clinical features of JRA: Eye irritation, pain, and redness: These symptoms are signs of eye inflammation. The eyes may be sensitive to light. In many cases, however, eye inflammation has no symptoms. If the inflammation is very severe and not reversed, it can cause loss of vision. The most common types of eye inflammation in JRA are uveitis and iritis. The names refer to the part of the eye that is inflamed.

Recurrent fevers: Fever is high and comes and goes with no apparent cause. Fever may “spike” (go high) as often as several times in one day. Rash: A light rash may come and go without explanation.

Myalgia (muscle aches): This is similar to that achy feeling that comes with the flu. It usually affects muscles throughout the whole body, not just one part.

Clinical features of JRA: Lymph node swelling. Swollen lymph nodes are noticed most often in the neck and under the jaw, above the clavicle, in the armpits, or in the inguinal region. Weight loss. This is common in children with JRA. It may be due to the child’s simply not feeling like eating.

Growth problems: Children with JRA often grow more slowly than average Growth problems: Children with JRA often grow more slowly than average. Growth may be unusually fast or slow in an affected joint, causing one arm or leg to be longer than the other. General growth abnormalities may be related to having a chronic inflammatory condition such as JRA or to the treatment, especially glucocorticoids

Diagnostic criteria of JRA: Clinical signs: Arthritis more than 3 months (in Europe), more than 6 weeks (in USA). Next joint arthritis in 3 months and more after the first joint affection Symmetrical affection of small joints Effusion in joint cavity (under the capsule) Joint contracture.

Diagnostic criteria of JRA: Tendosynovitis or bursitis. Muscular atrophy. Morning stiffness of joints. X-ray signs: Osteoporosis. Articular cavities narrowing Bone growth disturbances

Symmetrical affection of small joints

Joint contracture

Joint contracture

Bursitis

Muscular atrophy

Osteoporosis

Osteoporosis

Articular cavities narrowing

Bone growth disturbances

The main JRA subsets: Subset # of joints Gender Age ANA RF Out-come Pauciarticular 1-4 F 2 yrs ++ - Good 50 % Polyarticular RF-  5 3-9 yrs + Poor 25 % Polyarticular RF+ Teens Very poor Systemic Any Either 0-16

Laboratory tests in JRA ANA (antinuclear antibody) RF (Rheumatoid factor ) CRP (C-reactive protein) ESR (erythrocyte sedimentation rate) CCP (Cyclic Citrullinated Peptide Antibody) test

Nonsteroidal anti-inflammatory drugs (NSAIDs) Medications Doses (mg/kg) Side effects Aspirin 50-120 Stomack pain, vomiting, gastrointestinal bleedings, headache, blood in the urine, fluid retention, thinning and scarring of the skin (especially with naproxen), stomach ulcer (aspirin). Ibuprofen 10-30 Tolmentin 10-15 Naproxen 5-20

Slow-acting anti-rheumatic drugs (SAARDs) Medications Doses (mg/kg) Side effects Hydroxychlo-roquine (Plaquenil) 5-7 Upset stomach, skin rash and a eye damage. A child who takes this drug should have his/her eyes examined at least every six months by an ophthalmologist Sulfasalazine (Azulfadine)

Doses(weekly, depending from body weight ) Gold compaunds Medications Doses(weekly, depending from body weight ) Side effects Auranofin, Ridaura, Myochrysine Solganol 20 kg – 10 mg 30 kg – 20 mg 40 kg – 30 mg 50 kg – 40 mg > 50 kg – 50 mg Skin rash, mouth sores, kidney problems, a low blood count or anemia

Slow-acting anti-rheumatic drugs (SAARDs) Medications Week of treatment Doses (mg/ kg) Side effects Penicillamine (DePen, Cuprimine) 0-2 2-4 4-6 6-8 10-14 25-50 50-100 100 100-150 150-200 Diarrhea, skin rash, low blood counts, nausea or vomiting, stomach pain, loss of appetite, swollen glands, unusual bleeding or bruising

Immune System Medications (Cytostatics) Doses Side effects Methotrexate (Rbeumatrex) Azathioprine (Imuran) Cyclophosphamide (Cytoxan) Typically 7.5 to 25 mg a week Loss of appetite, nausea or vomiting, skin rash, unusual bleeding or bruising, tiredness or weakness, sterility.

Other medications Biologic Agents, which blocks the protein TNF Etanercept (Enbrel) Infliximab (Remicade) Glucocorticoid Drugs (Dexamethasone, Methylprednisolone, Cortef, Prednisolone and Prednisone) Analgesics (acetaminophen [Tylenol, Panadol], tramadol [Ultram])

Very important parts of treatment for juvenile arthritis: Therapeutic exercises Sports and Recreational Activities Splints

Very important parts of treatment for juvenile arthritis: Morning Stiffness Relief Diet Eye Care Dental Care Surgery