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Juvenile Seronegative Polyarthritis

Juvenile Polyarthritis (seronegative)


Presentation

  • Musculoskeletal System, Sixth Edition , delivers comprehensive, abundantly illustrated coverage of all aspects of MR musculoskeletal imaging--beginning with basic principles of interpretation, physics, and terminology--before moving through a systematic presentation[books.google.com]
  • 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]
  • It usually presents in those under 6 years old and is more common in females. It typically presents with one or two swollen joints causing stiffness and reduced movement but often not much pain. The child usually feels well.[patient.info]
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]
  • Hepatomegaly and/or splenomegaly. Serositis (pericarditis, pleuritis, peritonitis). Features of systemic JIA include: It affects males and females equally, with average age of onset before the age of 5 years.[patient.info]
  • Pericarditis, pleuritis, splenomegaly, and abdominal pain less commonly are observed. A low risk for uveitis exists.[emedicine.medscape.com]
Fever
  • A high daily fever that lasts at least 2 weeks either precedes or accompanies the arthritis.[icdlist.com]
  • 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]
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]
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]
  • Other laboratory markers of a poor prognosis include early radiologic evidence of bony injury, persistent anemia of chronic disease, elevated levels of the C1q component of complement, and the presence of ACPA (see Workup ).[emedicine.medscape.com]
Weight Loss
  • May be associated with weight loss or family history. Malignancy: Night pain, weight loss, easy bruising can all be features. Leukaemia , lymphoma , neuroblastoma , Ewing's sarcoma, bony tumours .[patient.info]
  • Systemic symptoms should also be sought - eg, fever and weight loss [ 5 ] . Note whether there any other associated presenting symptoms - eg, abdominal or respiratory disease. Examination Check temperature.[patient.info]
Malaise
  • 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]
Hepatosplenomegaly
  • Migratory salmon coloured light pink rash involving the trunk and/or extremities and hepatosplenomegaly are also frequently observed in patients with systemic onset.[radiopaedia.org]
  • ., 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]
  • Hepatomegaly and/or splenomegaly. Serositis (pericarditis, pleuritis, peritonitis). Features of systemic JIA include: It affects males and females equally, with average age of onset before the age of 5 years.[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]
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]
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: An Overview. . Accessed October 5, 2015. Types of Juvenile Arthritis. Arthritis Foundation. Kids Get Arthritis Too. . Accessed October 5, 2015. Understanding JIA. Kids Get Arthritis Too. Arthritis Foundation. .[spineuniverse.com]
  • : arthropathy in Whipple's disease ( M14.8* ) M09.0* Juvenile arthritis in psoriasis ( L40.5 ) M09.1* Juvenile arthritis in Crohn's disease [regional enteritis] ( K50.- ) M09.2* Juvenile arthritis in ulcerative colitis ( K51.- ) M09.8* Juvenile arthritis[apps.who.int]
Joint Swelling
  • 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]
  • 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 can include redness, joint swelling and pain, often in knees , ankles , and feet. Psoriatic arthritis affects major joints of the body, as well as the fingers and toes, along with the back and pelvis.[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]
Joint Effusion
  • MRI MRI shows synovial hypertrophy, joint effusions as well as osseous and cartilaginous erosions. Active synovitis is characterised by enhancement on T1-weighted gadolinium contrast studies.[radiopaedia.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]

Workup

Complete Blood Count Abnormal
HLA-DR4
  • Other HLA-DR4 molecules (eg, HLA-DR beta *0402) lack this epitope and do not confer this risk. Genes other than those of the major histocompatibility complex (MHC) are also involved.[emedicine.medscape.com]
  • DR4 and HLA DW4 .[en.wikipedia.org]

Treatment

  • Still osteopathic methods, treatment of acutely ill hospital patients, somatic dysfunction, clinical research and trials, outcomes research, and biobehavioral interactions with disease and health.[books.google.com]
  • 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]
  • […] the Treatment of Arthritis and Systemic Features.[autoinflammation.de]

Prognosis

  • ., uveitis , rashes, nail changes, lymphadenopathy , hepatosplenomegaly ), laboratory findings, and disease prognosis.[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

  • 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 ) invasion[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]
  • Etiology The cause of RA is unknown. Genetic, environmental, hormonal, immunologic, and infectious factors may play significant roles.[emedicine.medscape.com]
  • The etiology is unknown and it leads to multisystem inflammation. It can progress to aphthous ulcers, genital ulcerations as well as blindness form uvitis.[en.wikipedia.org]

Epidemiology

  • 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]
Sex distribution
Age distribution

Pathophysiology

  • 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 ) invasion[amboss.com]
  • Arthritis Care & Research 2012; 64(7):1001–1010. [9] Frosch M, Roth J: New insights in systemic juvenile idiopathic arthritis-from pathophysiology to treatment.[autoinflammation.de]
  • 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]
  • Pathophysiology The pathogenesis of RA is not completely understood.[emedicine.medscape.com]

Prevention

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

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