juvenile idiopathic arthritis


arthritis

(ar-thri'tis ) plural.arthritides [ arthro- + -itis] Joint inflammation, often accompanied by pain, swelling, stiffness, and deformity. Arthritis is very common, affecting millions. The most prevalent type, osteoarthritis or degenerative arthritis, increases in incidence with age but is not considered a part of normal aging. Other forms of arthritis include rheumatoid arthritis, ankylosing spondylitis, and psoriatic arthritis. Arthritis differs from rheumatic disease in that arthritis is a disease of joints whereas rheumatic disease may also affect other tissues and organs. arthritic (-thrit'ik), adjective

Etiology

Arthritis may result from infections (e.g., rheumatic fever, staphylococcal infections, gonorrhea, tuberculosis), metabolic disturbances (e.g., gout, calcium pyrophosphate crystal disease), multisystem autoimmune diseases (e.g., psoriasis, rheumatoid arthritis, systemic lupus erythematosus), neuropathies (e.g., Charcot's joint), joint trauma, or endocrine diseases (e.g., acromegaly). See: bursitis; monoarthritis; osteoarthritis; polyarthritis; rheumatism

Treatment

Anti-inflammatory drugs, corticosteroids, monoclonal antibodies, antibiotics, joint aspiration, surgery, and occupational or physical therapies may play a role in the treating arthritis, depending on the cause and severity of the illness.

acne-associated arthritis

Abbreviation: AAA
Joint inflammation accompanying acne fulminans, typically in adolescent boys. It is a rare type of spondyloarthropathy. The joint disease in AAA commonly involves the acromioclavicular and sacroiliac joints. Painful hyperostosis of the sternum and clavicles are typical findings. The syndrome is also known as SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis, and osteomyelitis). Affected boys are HLA-B27 negative. Synonym: synovitis acne pustulosis hyperostosis and osteomyelitis syndrome

acute suppurative arthritis

Septic arthritis.

adjuvant arthritis

Abbreviation: AA
An experimental model of arthritis in rodents induced by injection of foreign substance, such as Freund's adjuvant, into the tail vein or paw. This model can be used to study new agents for human arthritis treatment. See: Rheumatoid arthritis

allergic arthritis

Arthritis occurring in serum sickness or, occasionally, as a result of food allergies. See: serum sickness

bacterial arthritis

Infection of joints associated with fever and other systemic symptoms. Joint destruction occurs if the infection is not treated expeditiously. Removal of pus from the joint is necessary. In older or immunosuppressed patients, the most common causative organism is Staphylococcus aureus. Staphylococci, anaerobes, or gram-negative bacteria are found in prosthetic joint infections. Gonococci and Borrelia burgdorferi, the spirochete that causes Lyme disease, differ from other forms of bacteria that cause joint infection in that they tend to affect younger and more active people. Synonym: acute suppurative arthritis; septic arthritis

cricoarytenoid arthritis

One of the causes of dysphonia and vocal fold immobility that does not involve laryngeal nerve damage. It is caused by degenerative changes of the cricoarytenoid joints.

degenerative arthritis

Osteoarthritis.

enteropathic arthritis

Joint disease associated with inflammatory bowel disease.

epidemic arthritis

Infectious arthritis, often accompanied by a rash, caused by the Ross River virus.

experimental arthritis

Any form of arthritis induced in laboratory animals, used to study pathophysiology, or to foster improvements in diagnosis or treatment of the disease.

gonococcal arthritis

Arthritis, often with tenosynovitis and/or rash, caused by gonococcal infection. The joints of the knees, wrists, and hands are most commonly affected. The disease may affect any sexually active person and may follow infection of a mucous membrane by gonorrhea. This presentation of gonorrhea is usually called “disseminated gonococcal infection” (DGI).

Treatment

It is treated with intravenous ceftriaxone. A tetracycline antibiotic is usually given at the same time to treat possible co-infection with Chlamydia species.

gouty arthritis

Arthritis caused by gout.

hypertrophic arthritis

Osteoarthritis.

juvenile idiopathic arthritis

Abbreviation: JIA
The preferred name for juvenile rheumatoid arthritis.

juvenile rheumatoid arthritis

Abbreviation: JRA
Any of a group of chronic, inflammatory diseases involving the joints and other organs in children under 16. The age of onset is variable, as are the extra-articular manifestations. JRA affects about 1 in 1000 children (150,000 to 250,000 in the US alone) with overall incidence twice as high in females and is the most common form of arthritis in childhood. At least five subgroups are recognized. Synonym: Still's disease; juvenile idiopathic arthritis

Symptoms

Signs and symptoms depend on the type of JRA that is present.

Treatment

Anti-inflammatory agents are the mainstay of palliation but have little effect on the outcome of the disease. Corticosteroids may have adverse effects on bone growth; therefore most rheumatologists try to minimize their use. Disease-modifying drugs, such as methotrexate or leflunomide are current mainstays of treatment. Hematopoietic stem cell transplantation may be used in specialized treatment centers. Surgery is used to release ankylosed joints once the child reaches physical maturity and is able to carry out vigorous rehabilitation. Physical and occupational therapy are needed to maintain muscle strength and joint range of motion to prevent contractures, deformities, and disability. Gait training and joint protection also are helpful. Splinting joints in correct alignment reduces pain and prevents contractures. Regularly scheduled slit-lamp examinations help in the early diagnosis of iridocyclitis, which should be managed by an ophthalmologist, usually with corticosteroids and mydriatics. Other extra-articular manifestations should be referred to medical and surgical specialists.

Patient care

The child and family are instructed about the disease, treatment, and coping strategies, and are encouraged to express concerns. A well-balanced diet, regular exercise and rest periods, and avoidance of overexertion are encouraged. The child should be encouraged to be independent and involved in education and have an active social life. Moist heat helps relieve pain and stiffness. Placing the child in a warm bath, immersing painful hands and feet in pans of warm water for 10 min two to three times daily, or using daily whirlpool baths, a paraffin bath, or hot packs provide temporary relief of acute swelling and pain. Swimming and aerobic exercise in warm water are recommended to strengthen muscles and maintain mobility. Good posture and body mechanics are important; sleeping on a firm mattress without a pillow or with only a thin pillow is recommended to maintain proper body alignment. The patient should lie prone to straighten the hips and knees when resting or watching television. When braces or splints are required, their use is explained and demonstrated. Activities of daily living and playing provide opportunities to maintain mobility and incorporate therapeutic exercises using assistive and safety devices. The child with photophobia due to iridocyclitis should wear sunglasses. The child and family are referred to local and national support and information groups like the Arthritis Foundation (404-872-7100) (www.arthritis.org). Desired outcomes include the child's ability to achieve and maintain optimal health with joints that are movable, flexible, and free of deformity; to move with minimal or no discomfort; to engage in activities suitable to his or her interests, capabilities, and developmental level; and to perform self-care activities to maximum capabilities.

Lyme arthritis

The large-joint arthritis that develops in approx. 35% to 80% of patients with Lyme disease, caused by the spirochete Borrelia burgdorferi. It appears 2 weeks to 2 years after infection and is marked by periodic episodes of pain that move among different joints; the shoulders, knees, elbows, and ankles are involved most commonly. Approx. 10% of patients develop permanent deformities. The likelihood of chronic arthritic complaints is markedly diminished if patients are treated with amoxicillin or other appropriate antibiotics. See: Lyme disease

arthritis mutilans

Severe joint destruction, a characteristic of several inflammatory joint diseases, including some instances of psoriatic arthritis.

neuropathic arthritis

Arthritis associated with diseases of the nervous system. It occurs most commonly as a result of diabetes but can occur in tabes dorsalis, syphilis, and syringomyelia.

oligoarticular type I juvenile idiopathic arthritis

A form of JIA that accounts for about 33% of all cases; 80% of cases occur in girls, usually presenting in early childhood. Only a few joints are involved, typically the large joints of the knee, ankle, or elbow. One third of cases develop chronic iridocyclitis. Results of rheumatoid factor evaluation are usually negative. Ultimately, 10% of these children develop ocular damage, and 20% go on to develop polyarthritis.

oligoarticular type II juvenile idiopathic arthritis

A form of JIA that 90% of the time occurs in boys. As with type I, few joints are involved in this form of JIA; the hip girdle is usually involved. Sacroiliitis and acute iridocyclitis are the important extra-articular manifestations; an unknown percentage of children develop chronic spondyloarthropathy.

palindromic arthritis

Transient recurrent arthritis, of unknown cause, usually affecting large joints, such as the knees and elbows.

polyarticular juvenile idiopathic arthritis, rheumatoid factor–negative

A form of JIA that accounts for about 25% of all cases; 90% of cases occur in girls. It may involve multiple joints. Iridocyclitis, its most severe extra-articular manifestation, is rare. Severe arthritis develops in 10% to 15% of these children.

polyarticular juvenile idiopathic arthritis, rheumatoid factor–positive

A form of JIA that accounts for 5% to 10% of all cases; 80% of cases occur in girls. Typically presenting later in childhood, this arthritis may affect multiple joints. There are few extra-articular manifestations but 50% or more of these children develop severe arthritis.

psoriatic arthritis

Arthritis associated with psoriasis. The exacerbations and remissions of arthritic symptoms do not always parallel those of psoriasis. “Sausage-shaped” deformities of the fingers and toes are often present.

reactive arthritis

Arthritis that occurs shortly after an infection of the urinary or gastrointestinal tract. It often affects large joints in the lower extremities, usually in people under 50. Reiter's syndrome may be a form of reactive arthritis. RHEUMATOID ARTHRITISRHEUMATOID ARTHRITIS

rheumatoid arthritis

A chronic systemic disease marked by inflammation of multiple synovial joints. The disease usually affects similar groups of joints on both sides of the body and can create bony erosions that can be seen radiographically. Subcutaneous nodule formation and elevated serum rheumatoid factor levels are common. Patients typically complain of joint stiffness in the morning rather than after activities. Women are affected three times more often than men. Members of some ethnic groups, such as certain Native Americans, have higher rates of this disease than the general population. The illness usually begins in mid-life, but any age group can be affected. See: illustration See: Gripping with Rheumatoid Arthritis

Etiology

Factors implicated in the development and the severity of this disease include genetics (e.g., HLA haplotypes), autoimmune phenomena, and environmental influences.

Symptoms

Joint pains, morning stiffness, gelling, malaise, and fatigue are often present. Systemic disease marked by pleural effusions, pericarditis, pulmonary fibrosis, neuropathies, and ocular disorders may occu. Symptoms usually develop gradually over the course of several months but may begin abruptly in some patients.

Treatment

Most rheumatologists recommend aggressive therapy with disease-modifying antirheumatic drugs (DMARDs) early in the course of the illness to prevent bony erosions and loss of joint function. Drugs in this class include agents like methotrexate. Nonsteroidal anti-inflammatory drugs, e.g., ibuprofen or corticosteroids are often prescribed for palliation. Many patients may continue to take low-dose corticosteroids for years, but the benefits of long-term steroid use must be weighed against the risks, such as diabetes, osteoporosis, and adrenal suppression. Gold compounds can be used, but they are weaker than DMARDs and newer agents. Newer agents include antibodies to tumor necrosis factor and other immunomodulatory drugs. Powerful immunosuppressive agents like cyclosporine, azathioprine, and mycophenolate may also be used. Combination therapies involving several agents from different classes can be used. Joint replacement surgery can be helpful for some patients. Homeopathic substances such as black currant (gamma linolenic acid) and fish oil have demonstrated efficacy in rheumatoid arthritis

Patient care

All joints are assessed for inflammation, deformities, and contractures. The patient's ability to perform activities of daily living (ADLs) is evaluated. The patient is assessed for fatigue. Vital signs are monitored, and weight changes, pain (location, quality, severity, inciting and relieving factors), and morning stiffness (esp. duration) are documented. Use of moist heat is encouraged to relieve stiffness and pain. Prescribed anti-inflammatory and analgesic drugs are administered and evaluated; the patient is taught about the use of these medications. Patient response to all medications is evaluated, esp. after a change in drug regimen, and the patient and family are taught to recognize the purpose, schedule, and side effects of each. Over-the-counter drugs and herbal remedies may interact with prescribed drugs and should not be taken unless approved by physicians or pharmacists. Inflamed joints are occasionally splinted in extension to prevent contractures. Pressure areas are noted, and range of motion is maintained with gentle, passive exercise if the patient cannot comfortably perform active movement. Once inflammation has subsided, the patient is instructed in active range-of-motion exercise for specific joints. Warm baths or soaks are encouraged before or during exercise. Cleansing lotions or oils should be used for dry skin. The patient is encouraged to perform ADLs, if possible, allowing extra time as needed. Assistive and safety devices may be recommended for some patients. The patient should pace activities, alternate sitting and standing, and take short rest periods. Referral to an occupational or physical therapist helps keep joints in optimal condition as well as teaching the patient methods for simplifying activities and protecting joints. The importance of keeping PT/OT appointments and following home-care instructions should be stressed to both the patient and the family. A well-balanced diet that controls weight is recommended (obesity further stresses joints). Both patient and family should be referred to local and national support and information groups. Desired outcomes include cooperation with prescribed medication and exercise regimens, ability to perform ADLs, slowed progression of debilitating effects, pain control, and proper use of assistive devices. For more information and support, patient and family should contact the Arthritis Foundation (404-872-7100) (www.arthritis.org).

septic arthritis

Inflammation of the synovial tissues in a joint as the result of a pyogenic bacterial infection. Once infection occurs, cartilage is destroyed and the joint space narrows. Patients at greatest risk are those with pre-existing arthritis, joint trauma, or immune deficiencies and those who use intravenous drugs. Synonym: bacterial arthritis; acute suppurative arthritis

Etiology

The primary site of infection is usually elsewhere, with joint infection occurring as the result of bacteremia or spread from osteomyelitis in an adjacent bone. The most common pathogen for those 16 to 40 years old is Neisseria gonorrhoeae; other common bacteria include Staphylococcus aureus, group B streptococci, and gram-negative bacilli such as Escherichia coli and Salmonella spp.

Symptoms

Suppurative arthritis is marked by an acutely painful, warm, swollen joint with limited range of motion and fever; the white blood cell count and erythrocyte sedimentation rates are increased. Except in gonococcal arthritis, only one joint is affected, most commonly the knee, hip, or shoulder.

Treatment

Prompt treatment is necessary, including drainage of the joint and antimicrobial drug therapy (intravenous penicillinase-resistant penicillins and third-generation cephalosporins). The affected joint is supported with a sling or pillows, and the patient's pain is treated with mild opioids and nonsteroidal anti-inflammatory agents. Without vigorous treatment, significant joint destruction can occur.

syphilitic arthritis

Arthritis occurring in the secondary and tertiary stages of syphilis and marked by tenderness, swelling, and limitation of motion.

systemic juvenile idiopathic arthritis

A form of JIA that accounts for 20% of all cases; boys are affected 60% of the time. Fever and rash may be the presenting symptoms, either with or without joint involvement. Ultimately, 25% of these children develop severe arthritis.

tuberculous arthritis

Chronic, slowly progressive arthritis of hips, knees, ankles, or intervertebral disks caused by Mycobacterium tuberculosis. The organism usually spreads via the blood or from osteomyelitis in an adjacent bone. The macrophage and lymphocyte response to the mycobacterium destroys the bone along the joint margins, resulting in progressive pain, fibrosis, and restricted movement. See: granuloma

arthritis, juvenile idiopathic 

An inflammation of a joint or joints beginning in childhood. The entity is commonly divided into three types: pauciarticular (oligoarthritis) in which four joints or less are involved. It affects girls much more frequently than boys, especially in early onset, and it is associated with anterior uveitis in about 20% of cases. Complications from the uveitis include posterior synechia, band keratopathy, glaucoma and cataract; polyarticular (polyarthritis) in which more than four joints are involved with only a few percent of cases developing uveitis; and the most severe form systemic (Still's disease) which affects boys and girls equally and in which uveitis rarely develops. Syn. juvenile chronic arthritis; juvenile rheumatoid arthritis. Note: both synonyms were used formerly.