Types Pauciarticular (4 or fewer joints involved) Age at presentation Involves large joints and is typically asymmetric Boys may develop Sacroiliitis Iridocyclitis may be present Polyarticular (>4 joints involved) Age at presentation 1-6 or 11-16 years [fpnotebook.com]

Acute polyarthritis has a very wide differential diagnosis, presenting significant diagnostic difficulties. [patient.info]

Oligoarticular or polyarticular arthritis of a duration of six weeks or longer must be present to diagnose JIA. Patients may present with an acute onset of symptoms or a more gradual onset. [radiopaedia.org]

Symptoms morning stiffness and joint pain visual changes fever Physical exam rash iridocyclitis (can lead to rapid loss of vision if untreated) Imaging Radiographs often negative at presentation juxta-articular, late osteopenia and joint destruction [orthobullets.com]

• Splenomegaly

  Individuals with systemic arthritis may also have a skin rash or enlargement of the lymph nodes (lymphadenopathy), liver (hepatomegaly), or spleen (splenomegaly).Oligoarticular juvenile idiopathic arthritis (also known as oligoarthritis) has no features [icdlist.com]

  […] similar presentation to Rheumatoid Arthritis in adults Systemic (Still's Disease) High daily Relapsing Fever >39 degrees persists over 2 weeks (most distinguishes this type from others) Polyarticular involvement with >4 joints involved Hepatomegaly and Splenomegaly [fpnotebook.com]

  Abdominal examination - may reveal evidence of hepatomegaly and/or splenomegaly. Examine other systems as indicated by the history and clinical hypotheses. [patient.info]

  Pericarditis, pleuritis, splenomegaly, and abdominal pain less commonly are observed. A low risk for uveitis exists. [emedicine.medscape.com]

• Fever

  A daily high spiking fever, often in the afternoon or evening, with swift return of temperature to normal. A rash appears with the fever - classically salmon pink. [patient.info]

  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. [rheumatology.org]

  It is characterized by the presence of arthritis, salmon-colored rash, spiking fevers, fatigue, and sore throats. [fpnotebook.com]

  […] are affected four times more often than boys in early-onset peak age 2-3 yrs late-onset seen more frequent in boys typical finding is a limp that improves during day best prognosis for long term remission (70%) Systemic (20%) systemic symptoms (rash, fever [orthobullets.com]

  The fever is usually low in the morning and must be present for a minimum of two weeks for diagnosis. [news-medical.net]

• Inflammation

  This tenderness, known as enthesitis, accompanies the joint inflammation of arthritis. [icdlist.com]

  Eye inflammation may persist independently of the arthritis. [rheumatology.org]

  This group of conditions, which includes spondyloarthropathy (inflammation of the joints of the spine), affects the soft tissues around the joints (ligaments, tendons, or joint capsules). [spineuniverse.com]

  Eye drops These will be used if you have eye inflammation. They’re used to reduce the inflammation, prevent the swollen iris from sticking to the lens and to reduce pressure inside the eye. [arthritisresearchuk.org]

  Arthritis causes inflammation (swelling, redness and pain) in your body’s joints. It’s common in people as they get older. [webmd.com]

• Lymphadenopathy

  Signs Similar to adult form of Rheumatoid Arthritis Joint Swelling with warmth (not red and hot) Differences from Adult Rheumatoid Arthritis Higher fever Rheumatoid Nodule s are rare Pericarditis and valvulitis are more common Lymphadenopathy and hepatosplenomegaly [fpnotebook.com]

  Individuals with systemic arthritis may also have a skin rash or enlargement of the lymph nodes (lymphadenopathy), liver (hepatomegaly), or spleen (splenomegaly).Oligoarticular juvenile idiopathic arthritis (also known as oligoarthritis) has no features [icdlist.com]

  Systemic-onset JRA is characterized by high intermittent fevers and other extra-articular manifestations: lymphadenopathy, rash, pleuritis, and pericarditis. [pedsinreview.aappublications.org]

  It is classified into various types based on the pattern of joint involvement, the presence of extra-articular manifestations (e.g., uveitis, rashes, nail changes, lymphadenopathy, hepatosplenomegaly ), laboratory findings, and disease prognosis. [amboss.com]

  […] frequent in boys typical finding is a limp that improves during day best prognosis for long term remission (70%) Systemic (20%) systemic symptoms (rash, fever, multiple joint involvement) anemia, high WBC, elevated ESR, CRP, platelets hepatosplenomegaly, lymphadenopathy [orthobullets.com]

• Anemia

  […] than boys in early-onset peak age 2-3 yrs late-onset seen more frequent in boys typical finding is a limp that improves during day best prognosis for long term remission (70%) Systemic (20%) systemic symptoms (rash, fever, multiple joint involvement) anemia [orthobullets.com]

  Apart from joint involvement there are symptoms like irritability, fatigue, anemia, loss of appetite, growth retardation etc. This condition affects girls more often than boys. [news-medical.net]

  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. [rheumatology.org]

  Systemic symptoms, including anorexia, anemia, and growth retardation, are moderate. An intermediate risk for uveitis exists. Systemic onset JIA (10-20%) is equal frequency in boys and girls and can appear at any age. [emedicine.medscape.com]

• Fatigue

  It also can make you feel fatigued or lose your appetite. You might run a mild fever. In severe cases, it can make your spine stiffer. It also could damage the vertebrae or cause two or more of the bones to grow together. [webmd.com]

  Apart from joint involvement there are symptoms like irritability, fatigue, anemia, loss of appetite, growth retardation etc. This condition affects girls more often than boys. [news-medical.net]

  It is characterized by the presence of arthritis, salmon-colored rash, spiking fevers, fatigue, and sore throats. [fpnotebook.com]

  Physical Activity to Reduce Fatigue in Rheumatoid Arthritis: A Randomized, Controlled Trial. Arthritis Care Res (Hoboken). 2017 Apr 5. [Medline]. Lemmey AB, Marcora SM, Chester K, Wilson S, Casanova F, Maddison PJ. [emedicine.medscape.com]

  Identifying the specific biomarkers related to each type of JIA can help form more personalized treatment plans and decrease remission rates. [48] Children with JIA are more susceptible to cardiovascular disease, depression, sleep disturbance, anxiety and fatigue [en.wikipedia.org]

• Hepatosplenomegaly

  Migratory salmon colored light pink rash involving the trunk and/or extremities and hepatosplenomegaly are also frequently observed in patients with systemic onset. [radiopaedia.org]

  It is classified into various types based on the pattern of joint involvement, the presence of extra-articular manifestations (e.g., uveitis, rashes, nail changes, lymphadenopathy, hepatosplenomegaly ), laboratory findings, and disease prognosis. [amboss.com]

  […] late-onset seen more frequent in boys typical finding is a limp that improves during day best prognosis for long term remission (70%) Systemic (20%) systemic symptoms (rash, fever, multiple joint involvement) anemia, high WBC, elevated ESR, CRP, platelets hepatosplenomegaly [orthobullets.com]

  The children with the condition may have other systemic symptoms affecting other vital organs including hepatosplenomegaly (enlargement of the liver and spleen), lymphadenopathy (enlargement of the lymph nodes), and pericarditis (accumulation of fluid [news-medical.net]

  Signs Similar to adult form of Rheumatoid Arthritis Joint Swelling with warmth (not red and hot) Differences from Adult Rheumatoid Arthritis Higher fever Rheumatoid Nodule s are rare Pericarditis and valvulitis are more common Lymphadenopathy and hepatosplenomegaly [fpnotebook.com]

• Hepatomegaly

  Individuals with systemic arthritis may also have a skin rash or enlargement of the lymph nodes (lymphadenopathy), liver (hepatomegaly), or spleen (splenomegaly).Oligoarticular juvenile idiopathic arthritis (also known as oligoarthritis) has no features [icdlist.com]

  Factor confers similar presentation to Rheumatoid Arthritis in adults Systemic (Still's Disease) High daily Relapsing Fever >39 degrees persists over 2 weeks (most distinguishes this type from others) Polyarticular involvement with >4 joints involved Hepatomegaly [fpnotebook.com]

  Abdominal examination - may reveal evidence of hepatomegaly and/or splenomegaly. Examine other systems as indicated by the history and clinical hypotheses. [patient.info]

  Systemic onset is associated with fever (high in evening and normal in morning), macular rash, leukocytosis, lymphadenopathy, and hepatomegaly. Pericarditis, pleuritis, splenomegaly, and abdominal pain less commonly are observed. [emedicine.medscape.com]

• Anterior Uveitis

  uveitis that occurs with JIA may be asymptomatic (especially in the case of chronic anterior uveitis ). [amboss.com]

  Acute anterior uveitis with hypopyon in a child. Courtesy of Manolette Roque, MD, Roque Eye Clinic. [emedicine.medscape.com]

  Other exclusion criteria include: the presence of ankylosing spondylarthritis, enthesitis and arthritis, sacroiliitis with an inflammatory enteropathy or acute anterior uveitis in the patient or a family history of one of these conditions in a parent [orpha.net]

  Acute anterior uveitis. Onset in a male over the age of 6. Features of enthesitis-related JIA include: It affects males nine times more commonly than females with age of onset usually over 10. It often affects a lower limb. [patient.info]

  Early childhood onset patients are at risk for developing a chronic iridocyclitis or anterior uveitis, which is inflammation of the eye. [en.wikipedia.org]

• Arthritis

  Juvenile Arthritis Also called: Childhood arthritis, JRA, Juvenile idiopathic arthritis, Still's disease Juvenile arthritis (JA) is arthritis that happens in children. It causes joint swelling, pain, stiffness, and loss of motion. [icdlist.com]

  The ICD code M08 is used to code Childhood arthritis Childhood arthritis, also known as juvenile arthritis (JA), is any form of arthritis or arthritis-related conditions which affects individuals under the age of 16. [icd.codes]

  Whipple’s Disease, and various types of arthritis caused by bacteria (Lyme arthritis, Gonococcal arthritis, Rheumatic fever, tuberculous arthritis). [rheumatoid-arthritis-decisions.com]

  Juvenile Idiopathic Arthritis Is Not a Child-Sized Type of Adult Rheumatoid Arthritis The most common type of arthritis in children and adolescents is juvenile idiopathic arthritis (JIA) (idiopathic means that no cause has been determined). [spineuniverse.com]

  National Institute of Arthritis and Musculoskeletal and Skin Disease. 2015. Juvenile Arthritis. Available online at https://www.niams.nih.gov/Health_Info/Juv_Arthritis/default.asp. Accessed 6/20/2017. Rheumatoid Arthritis Support Network. 2016. [labtestsonline.it]

• Joint Swelling

  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. [rheumatology.org]

  It causes joint swelling, pain, stiffness, and loss of motion. It can affect any joint, but is more common in the knees, hands, and feet. In some cases it can affect internal organs as well. [icdlist.com]

  Arthritis causes inflammation (swelling, redness and pain) in your body’s joints. It’s common in people as they get older. [webmd.com]

  These agents have a varied mechanism of action and they act by reducing joint swelling and pain, decreasing markers of acute inflammation in blood and halting the progressive joint damage. [news-medical.net]

  Signs Similar to adult form of Rheumatoid Arthritis Joint Swelling with warmth (not red and hot) Differences from Adult Rheumatoid Arthritis Higher fever Rheumatoid Nodule s are rare Pericarditis and valvulitis are more common Lymphadenopathy and hepatosplenomegaly [fpnotebook.com]

• Osteoporosis

  Osteoporosis. Growth restriction - related to the degree of inflammation. Complications from medications. [patient.info]

  The long-term use of steroids is associated with side effects such as weight gain, poor growth, osteoporosis, cataracts, avascular necrosis, hypertension, and risk of infection. [rheumatology.org]

  Other muskuloskeletal issues may include joint contractures, muscle weakness or muscle loss, and osteoporosis. [9] Children who delay treatment or do not participate in physical therapy, or children with severe disease, can often develop joint deformities [en.wikipedia.org]

• Arthralgia

  Signs and symptoms In most patients with RA, onset is insidious, often beginning with fever, malaise, arthralgias, and weakness before progressing to joint inflammation and swelling. [emedicine.medscape.com]

• Joint Effusion

  MRI MRI shows synovial hypertrophy, joint effusions as well as osseous and cartilaginous erosions. Active synovitis is characterized by enhancement on T1-weighted gadolinium contrast studies. [radiopaedia.org]

• Skin Disease

  This type of arthritis usually occurs along with the skin disease, psoriasis (skin condition; dry, scaly skin), but it also may occur in the presence of a few other factors. Enthesitis-related JIA. [spineuniverse.com]

  NIH: National Institute of Arthritis and Musculoskeletal and Skin Diseases Juvenile rheumatoid arthritis (Medical Encyclopedia) [ Read More ] Juvenile idiopathic arthritis Juvenile idiopathic arthritis refers to a group of conditions involving joint inflammation [icdlist.com]

  National Institute of Arthritis and Musculoskeletal and Skin Disease. 2015. Juvenile Arthritis. Available online at https://www.niams.nih.gov/Health_Info/Juv_Arthritis/default.asp. Accessed 6/20/2017. Rheumatoid Arthritis Support Network. 2016. [labtestsonline.it]

  With psoriatic arthritis, children have both arthritis and a skin disease called psoriasis or a family history of psoriasis in a parent or sibling. [rheumatology.org]

  National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) Information Clearinghouse National Institutes of Health. October 2012. Retrieved 19 December 2012. [en.wikipedia.org]

• Skin Rash

  Individuals with systemic arthritis may also have a skin rash or enlargement of the lymph nodes (lymphadenopathy), liver (hepatomegaly), or spleen (splenomegaly).Oligoarticular juvenile idiopathic arthritis (also known as oligoarthritis) has no features [icdlist.com]

  Pisetsky gives these examples: A person diagnosed with seronegative RA may eventually develop a skin rash that would cause the doctor to change the diagnosis to psoriatic arthritis. [arthritis.org]

  Other possible signs are a skin rash associated with psoriasis, or an inflammatory intestinal illness. People with ankylosing spondylitis sometimes develop arthritis in their hips and shoulders early on. [webmd.com]

• Subcutaneous Nodule

  It is associated with subcutaneous nodules, erosions, and a poor prognosis. Approximately 40% of these patients test positive for ANA. Systemic symptoms, including anorexia, anemia, and growth retardation, are moderate. [emedicine.medscape.com]

  Patients with Farber disease typically have subcutaneous nodules and a hoarse or weak voice due to growth of nodules on the larynx. [en.wikipedia.org]

• Complete Blood Count Abnormal

  Blood Count Abnormal in systemic presentation IX. [fpnotebook.com]

• HLA-DR4

  HLA-DR1 (HLA-DR beta *0101) also carries this shared epitope and confers risk, particularly in certain southern European areas. Other HLA-DR4 molecules (eg, HLA-DR beta *0402) lack this epitope and do not confer this risk. [emedicine.medscape.com]

  DR4 and HLA DW4. [en.wikipedia.org]

Case-specific questions were asked regarding (1) initial treatment of oligoarthritis, (2) treatment of refractory oligoarthritis, (3) treatment of complications (uveitis refractory to topical steroids). III. [ped-rheum.biomedcentral.com]

Management and treatment Treatment is best managed by a paediatric rheumatologist in collaboration with physiotherapists and ophthalmologists. Initial treatment revolves around administration of NSAIDS. [orpha.net]

Just as treatment has changed through the years, so has the thinking about RA in some cases. [arthritis.org]

Read more: Antibiotics may play a role in juvenile arthritis » Could research lead to treatments? But it is possible that these findings may eventually provide better, more targeted novel treatment therapies for JA and RA patients alike. [healthline.com]

Many of these treatments are approved for use in children as well as adults. In addition, researchers are developing new treatments. [rheumatology.org]

Prognosis Early disease onset is associated with a greater degree of growth impairment and deformity! References: [17] [1] [amboss.com]

As with pauciarticular-onset JIA, it is now recognized to contain many distinct subgroups with varying prognosis. [eldersarthritis.com]

[…] for long term remission (70%) Systemic (20%) systemic symptoms (rash, fever, multiple joint involvement) anemia, high WBC, elevated ESR, CRP, platelets hepatosplenomegaly, lymphadenopathy, pericarditis M=F, age 5-10y includes Stills disease poorest prognosis [orthobullets.com]

Prognosis In total, 50% of cases are non-progressive by adulthood. Joint sequelae are minor (possible asymmetry in the length of the lower limbs). [orpha.net]

This theory is supported by the much worse prognosis of RA among patients with positive RF results. [emedicine.medscape.com]

Etiology Pathophysiology Autoimmune and/or autoinflammatory disease → chronic synovial inflammation with infiltration of plasma cells, B lymphocytes, and T lymphocytes → joint capsule hyperplasia → growth of fibrovascular connective tissue ( pannus ) [amboss.com]

Etiology Oligoarticular juvenile arthritis is an autoimmune disorder, but the exact disease mechanism and triggering factors are unknown. However, the disease has been associated with HLA class II antigens, HLA DR3 and DRB1*08. [orpha.net]

To more accurately assess the incidence of childhood arthritis and to better understand its etiology, it is necessary to both more rigorously and more inclusively define the diseases which cause arthritis in children. [eldersarthritis.com]

Unraveling the Phenotypic Variability of Juvenile Idiopathic Arthritis across Races or Geographic Areas--Key to Understanding Etiology and Genetic Factors?. J Rheumatol. 2016;43(4):683-5. doi: 10.3899/jrheum.160173. [ Links ] 15. [scielo.cl]

Etiology The cause of RA is unknown. Genetic, environmental, hormonal, immunologic, and infectious factors may play significant roles. [emedicine.medscape.com]

Epidemiology References: [1] Epidemiological data refers to the US, unless otherwise specified. [amboss.com]

Summary Epidemiology Its prevalence is estimated at 1-10 in 6,500 children, with an annual incidence of 1-20 in 1,000,000 children. [orpha.net]

"Polyarticular juvenile idiopathic arthritis - epidemiology and management approaches". Clinical Epidemiology. 6 : 379–93. doi : 10.2147/CLEP.S53168. PMC 4216020. PMID 25368531. ^ Weber J, Weber D, Tzaribachev N (2011). [en.wikipedia.org]

Epidemiology The overall prevalence is estimated to be 1-2 per 1,000 children, with an incidence of 1 per 10,000. [ 3 ] It is more common in females, although there are differences depending on the subset. [patient.info]

Epidemiology United States JIA has an estimated prevalence of about 113 cases per 100,000 children. It is estimated that JIA afflicts 60,000-70,000 children, but only a minority develop eye disease. [emedicine.medscape.com]

Etiology Pathophysiology Autoimmune and/or autoinflammatory disease → chronic synovial inflammation with infiltration of plasma cells, B lymphocytes, and T lymphocytes → joint capsule hyperplasia → growth of fibrovascular connective tissue ( pannus ) [amboss.com]

Pathophysiology Rheumatoid disease in children III. [fpnotebook.com]

Pathophysiology The cause of uveitis and arthritis in JIA remains unknown. [9] Akin to many other autoimmune diseases, the target antigen is unidentified. [emedicine.medscape.com]

Preventing the progression of joint damage To prevent progression of joint damage Disease-modifying anti-rheumatic drugs or DMARDs are widely used. [news-medical.net]

Therapists may construct splints to prevent permanent joint tightening or deformities, and work with school-based therapists to address issues at school. [rheumatology.org]

Using condoms is important to prevent sexually transmitted infections (STIs) even when using hormonal contraception. [arthritisresearchuk.org]

They may also help prevent and treat complications. [icdlist.com]

Their hope is that a better understanding of the differences between the diseases we call RA may provide insights that could eventually lead to new ways to treat and, perhaps in some cases, even prevent it. [arthritis.org]