Growing up with arthritis can be challenging. However, with care from a team of rheumatology professionals, most children with arthritis live full and active lives and are able to do everything their peers do. There are various types of chronic childhood arthritis, which can last from several months to many years.
In every instance, early diagnosis and treatment can help avoid joint damage. There are many terms used to describe a child with chronic arthritis. These include juvenile rheumatoid arthritis, juvenile chronic arthritis, and juvenile idiopathic arthritis. Juvenile arthritis may involve one or many joints and can also cause silent eye inflammation. It can also cause other symptoms such as fevers or rash.
Several types of arthritis, all involving chronic long-term joint inflammation, fall under the JIA heading. This inflammation begins before patients reach the age of 16, and symptoms must last more than 6 weeks to be called chronic. JIA may involve one or many joints, and may also affect the eyes. It can cause other symptoms such as fevers or rash.
Systemic onset JIA affects about ten percent of children with arthritis. Systemic onset JIA may cause inflammation of the internal organs as well as the joints, though joint swelling may not appear until months or even years after the fevers began.
Anemia a low red blood cell count and elevated white blood cell counts are also typical findings in blood tests ordered to evaluate the fevers and ongoing symptoms. Arthritis may persist even after the fevers and other symptoms have disappeared.
Oligoarticular JIA , which involves fewer than five joints in its first stages, affects about half of all children with arthritis. Girls are more at risk than boys. Children who develop the oligoarticular form of JIA when they are younger than seven years old have the best chance of having their joint disease subside with time.
They are, however, at increased risk of developing an inflammatory eye problem iritis or uveitis. Basic research in pediatric rheumatology has blossomed, with at least 10 National Institutes of Health—funded investigators in this field at present, and several National Institutes of Health Center and Training Grants focused on pediatric rheumatology.
Advances in understanding human immunology have contributed to better understanding of the pathophysiology of rheumatic diseases, although this knowledge remains imperfect. The identification of immune complex disease as a basic mechanism in rheumatic diseases, particularly in systemic lupus, has furthered our understanding of tissue damage and has provided a useful mechanism for monitoring disease activity.
The sequencing of the human genome represents an enormous step forward in science and offers promise for better future understanding of many crucial factors influencing rheumatic diseases: mechanisms of inflammation and immune response, host-predisposition to disease, and pharmacogenomics and host responsiveness to drugs. Histocompatibility typing has established that a genetic predisposition exists in a number of rheumatic diseases.
The association is strongest for ankylosing spondylitis and the spondyloarthropathies, all of which are associated with HLA-B Associations have also been shown for adult-type rheumatoid factor—positive rheumatoid arthritis, pauciarticular JRA, SLE, and childhood dermatomyositis — However, in none of these conditions is HLA testing diagnostic, nor is it certain whether the HLA antigens or some linked factor explain the disease associations.
Concerning other rare rheumatic syndromes of childhood, extensive studies have led to better understanding of the various febrile syndromes, a number of which are now recognized as single-gene diseases [ e. Advances in imaging techniques including computer tomography, magnetic resonance imaging, and ultrasound have revolutionized the localization of organ involvement and tissue damage. Geographic and ethic differences in prevalence of various rheumatic disease syndromes have also become apparent; in most instances it is not known whether these differences arise from a genetic or environmental basis, or combination of both.
The spondyloarthropathies have a strong association with a genetic factor, HLA B27; these conditions are rare in populations with a low prevalence of B27 e. Japan and relatively common in populations of a high prevalence of this gene e. Bechet syndrome is relatively rare in North America but relatively common in the countries such as Turkey and Iran Kawasaki disease has been described most frequently in children of Japanese origin, even where there are mixed racial populations in areas such as Hawaii The explanations for most of these geographic or ethnic diversities in rheumatic disease distribution remain to be found, but offer possible clues to basic disease mechanisms.
Pediatric rheumatologists have become increasingly aware of the importance of accurate differential diagnosis in the evaluation and management of children with rheumatic complaints. Several observations have been made concerning infectious agents. Lyme arthritis, originally described in children thought to have pauciarticular juvenile arthritis, is now known to be caused by infection with a spirochete Parvovirus, the virus causing Fifth disease, can be accompanied by arthritis in one or a number of joints Childhood leukemia, and indeed any of the childhood malignances, can be the basis for frank arthritis, sometimes with other systemic complaints also reminiscent of rheumatic disease Pediatric rheumatologists everywhere are seeing an increasing number of children with either diffuse or localized musculoskeletal pain without apparent underlying organic disease.
These pain syndromes are often labeled as reflex sympathetic dystrophy pain generally localized to one limb or body part , the fibromyalgia syndrome diffuse pain syndrome , or the chronic fatigue syndrome Such conditions can be extremely disabling and usually have a strong psychological overlay.
There is no ready explanation for their apparent increased prevalence. Management with restoration of function, attention to psychosocial issues, and a minimum of drug therapy is often difficult and time consuming. A number of basic laboratory studies are frequently used in the rheumatic diseases.
Both rheumatoid factors, antibodies that react with immunoglobulin , , and antinuclear antibodies, a family of antibodies that react with various antigens found in cell nuclei 40 , 41 , were described in the s. Both are useful in the classification of rheumatic diseases, but neither has proved to be a diagnostic test for any one disease.
The classic rheumatoid factors, IgM antibodies to IgG, are associated with adult-type rheumatoid arthritis. Some specific ANA are useful in disease classification: antibodies reacting with RNA for mixed connective tissue disease, antinuclear cytoplasmic antibodies for Wegener granulomatosis, antibodies to DNA in monitoring the activity of the immune complex disease of SLE.
Positive tests for ANA are strongly associated with the iridocyclitis of JRA , but are also found in children with pauciarticular or polyarticular arthritis without eye disease, and children with dermatomyositis and scleroderma Serum complement studies are useful in following the activity of the immune complex of SLE, but are not diagnostic of any disease , The rare deficiency states of components of the complement system may be associated with rheumatic disease syndromes, particularly SLE , The antiphospholipid antibodies are directed against negatively charged phospholipids, and have been associated with various rheumatic diseases, notably SLE lupus anticoagulants and Sjogren syndrome.
These antibodies may also occur with infectious diseases and malignancies, or in the absence of disease. They are associated with an increased risk of hypercoaguability, stroke, and fetal loss, and their presence raises the question of anticlotting or coagulation therapy Rheumatic fever remains a major disease of children in the developing world, and to a lesser extent of poor children in the industrialized world 12 , Preventing or treating the antecedent streptococcal infections of rheumatic fever involves addressing socioeconomic factors such as poverty, crowding, poor nutrition, and lack of access to adequate health care.
Efforts to find a suitable vaccine against streptococcal infections have not yet been successful, and adequate therapy of streptococcal pharyngitis remains crucial to control rheumatic fever In the s, outbreaks of rheumatic fever occurred in several parts of the United States, perhaps related to relatively asymptomatic streptococcal pharyngitis that was not recognized or treated , No convincing studies exist to show that any form of antiinflammatory therapy during active rheumatic disease alters the long-term cardiac damage.
When cardiac failure from valvular dysfunction has occurred, definitive therapy of valve replacement surgery is expensive and not available for most children in the developing world. Although the role of antecedent streptococcal infection in causing acute rheumatic fever has been known for decades, extensive research has not yet fully elucidated the pathophysiologic mechanisms involved, or the matter of host predisposition to disease.
Juvenile rheumatoid arthritis is now accepted as a syndrome of chronic arthritis with several different subgroups that probably represent different diseases.
This concept was suggested by Schaller and Wedgwood in the late s and early s , The occurrence of chronic iridocyclitis is virtually limited to a group of patients with early childhood onset of pauciarticular arthritis and frequent occurrence of antinuclear antibodies The spondyloarthropathies, all associated with HLA-B27 , , are now recognized within the spectrum of juvenile arthritis, and indeed account for most instances of pauciarticular arthritis beginning in later childhood, especially in males Classic IgM rheumatoid factors, the markers for seropositive adult rheumatoid arthritis, are restricted to a relatively small group of older children and adolescents with a severe form of polyarthritis that appears identical to classic adult rheumatoid arthritis and is associated with HLA-DR4 , The other two generally accepted subgroups of juvenile arthritis are systemic onset disease, clearly delineated by its characteristic systemic complaints of high intermittent fevers and other system involvement, and rheumatoid factor—negative polyarthritis, a much less well characterized group , , Such detailed classifications may permit more accurate subgrouping, and thus provide a basis for better study of basic disease mechanisms , Better recognition of children with systemic lupus and more aggressive therapy have improved the outlook.
However, aggressive therapy is difficult and fraught with potential side effects. Corticosteroid therapy remains a mainstay, and intravenous cyclophosphamide is now the most commonly used regime to control severe disease Ideal management requires the input of a pediatric rheumatologist or other specialist who is experienced in the control of this difficult disease in children and adolescents.
Childhood dermatomyositis has been the focus of intensive study in several settings — A genetic predisposition to disease has been identified but is imperfectly understood. Extensive studies of pathophysiology have increased understanding of basic disease mechanisms of this condition, but cause and effect remain elusive. Magnetic resonance imaging of muscle has been accepted as a valuable tool for determining the extent of muscle involvement and to a certain extent the activity of disease.
Therapy with a number of agents has been found to be effective, but no effective therapies have been found for the discouraging long-term complication of calcinosis. Kawasaki disease remains essentially a childhood condition, and has now been recognized all over the world — In the United States, it is the most common cause of acquired heart disease in children. Despite much research effort, an understanding of basic disease mechanisms remains incomplete.
The promising concept that Kawasaki disease is the result of a toxin produced by bacteria such as staphylococci or streptococci seems difficult to prove Intravenous gamma-globulin has been found to be an effective tool in preventing coronary vasculitis, but we still do not understand its mechanism of action. Other forms of vasculitis, aside from Schonlein-Henoch, remain rarities in childhood Scleroderma remains a rarity during childhood years. It is now accepted that the cutaneous forms are rarely if ever associated with involvement of internal organs.
Systemic sclerosis is quite rare in childhood; Raynaud's phenomenon is often the harbinger of this disease. There have been no major research or therapeutic breakthroughs Despite many recent advances in science, we are still left with an incomplete understanding of the pathophysiology of most of the rheumatic diseases, and hampered by our inability to prevent them or to predict accurately those patients at risk for severe disease.
We have been successful at better defining diseases, and have made significant advances in therapy. Continuing emphasis on clinical, epidemiologic, and basic research is needed; a recent burgeoning of basic research in pediatric rheumatology holds promise for enhanced understanding and care of patients. The scope of pediatric rheumatology has broadened to include pain syndromes, sports-related injury, musculoskeletal dysfunction, and overall fitness and musculoskeletal health.
Cutbacks in health care, particularly in ancillary services such as physical and occupational therapy, pose problems for children with all kinds of chronic conditions including rheumatic diseases.
Pediatric rheumatology has become a well-organized specialty area, and one that deserves preservation as a valid subspecialty that enhances both the recognition and the care of children with these difficult conditions. Bywaters EG The history of pediatric rheumatology. Arthritis Rheum 20 : — Harvard University Press, Cambridge, p Book Google Scholar.
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Colver T The prognosis in rheumatoid arthritis in childhood. Arch Dis Child pp — J Pediatr 29 : — Schlesinger B Rheumatoid arthritis in the young. The child with joint pain in primary care. Best Pract Res Clin Rheumatol. Download references. LA: data acquisition and interpretation, writing and analysis, LRS: revising the article and discussion, data interpretation, IKP: revising the article and discussion, data interpretation, PD: conception, design, writing, analysis and interpretation of data.
All authors read and approved the final manuscript. You can also search for this author in PubMed Google Scholar. Correspondence to Perrine Dusser. Reprints and Permissions. Aoust, L. Time to diagnosis in juvenile idiopathic arthritis: a french perspective. Orphanet J Rare Dis 12, 43 Download citation. Received : 13 August Accepted : 04 February Published : 28 February Anyone you share the following link with will be able to read this content:. Sorry, a shareable link is not currently available for this article.
Provided by the Springer Nature SharedIt content-sharing initiative. Skip to main content. Search all BMC articles Search. Download PDF. Abstract Background Juvenile idiopathic arthritis JIA is a rare disease that is not widely known by paediatricians and general practitioner GP leading to diagnostic error and delayed care provision.
Results Sixty-seven patients were finally analysed 42 girls. Background Juvenile idiopathic arthritis, JIA, is the most frequent cause of inflammatory arthritis in children before 16 years. Table 1 Population characteristics Full size table.
Graph representing the different physicians seen at first, second and third referral. Full size image. References 1. PubMed Google Scholar 2. Article PubMed Google Scholar 3. PubMed Google Scholar 4. Article PubMed Google Scholar 6. PubMed Google Scholar JIA affects large joints such as knees, wrists, and ankles as well as small joints.
Another subgroup is Systemic JIA, which affects the whole body, and usually causes fever and skin rashes. In the past, the first line of treatment for children with juvenile arthritis has been to relieve pain and inflammation with non-steroidal anti-inflammatory drugs NSAIDs such as aspirin and ibuprofen.
But polyarticular and systemic JIA are now also treated with newer medicines called biologics, which are manufactured in or extracted from biological sources. Biologics used in the treatment of juvenile arthritis are generally given intravenously or subcutaneously under the skin , and usually are taken for years.
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