Constant pain is the major and most bothering symptom. The causes for the pain vary and depend on many factors [1]. Despite low disease activity, many children appear to have hand and/or wrist related symptoms and impairment, with resulting moderate to severe activity limitations and participation at school [5]. Patients present with painful, swollen joints with or without the presence of fever and rash.

• Disability

  Can Children Get Disability Benefits? Children who meet the Social Security Administration’s (SSA's) definition of disabled may be eligible for Supplemental Security Income (SSI), but not Social Security disability. [disabilitysecrets.com]

  Google Scholar | Crossref | Medline | ISI Missiuna, C, Pollock, N ( 1991 ) Play and deprivation in children with physical disabilities: The role of the occupational therapist in preventing secondary disabilities. [doi.org]

  Juvenile idiopathic arthritis (JIA), a term referring to a group of disorders characterized by chronic arthritis, is the most common chronic rheumatic illness in children and is a significant cause of short- and long-term disability. [ncbi.nlm.nih.gov]

• Fatigue

  OBJECTIVE: To compare polysomnography (PSG) and self-reported sleep, symptoms (pain and fatigue), and anxiety between children with active and inactive juvenile rheumatoid arthritis (JRA) and examine relations among sleep, symptoms, and anxiety. [ncbi.nlm.nih.gov]

  But if a child seems fatigued, lacks an appetite and is losing rather than gaining weight, it’s a sign that her problem could be juvenile arthritis. [kidsgetarthritistoo.org]

  Fatigue, anemia, suboptimal growth, and iritis (to a lesser degree than oligoarticular JRA) are complications. [medicinenet.com]

  In one or more joints (unilaterally or bilaterally) Pain Swelling Redness Increased warmth to palpation Tenderness Morning stiffness Rash Fever Uveitis (inflammation of eye) often asymptomatic Serositis (inflammation in lining heart, lungs, abdomen) Fatigue [accessphysiotherapy.mhmedical.com]

  The child with systemic disease may exhibit: signs of enlargement of the spleen and lymph nodes inflammation of the heart muscle and surrounding tissues a high white blood cell count anemia fatigue weight loss The prognosis of systemic disease is considered [arthritis.about.com]

• Weight Loss

  Low-grade fever, weight loss, and anemia may occur, as well as growth problems in severe cases. Most children with polyarticular disease are negative for rheumatoid factor and their prognosis is usually good. [arthritis.about.com]

  Finally, the condition can also cause weight loss. This can be a direct result of the disease, or it may be a side effect. Because of other symptoms, the child may simply not want to eat, which will naturally result in weight loss. [chronicbodypain.net]

  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]

  loss, or even that gluten can be harmful to every human being. [ncbi.nlm.nih.gov]

• Asymptomatic

  Asymptomatic erythematous scaly plaques developed on the trunk. A skin biopsy specimen revealed psoriasis. This is the first report of psoriasis developing in a patient with juvenile rheumatoid arthritis. [ncbi.nlm.nih.gov]

  The pericarditis may be asymptomatic. The usual physical findings consist of friction rub, tachycardia and tachypnea. X-ray and electrocardiographic changes are frequently useful in its detection. [pediatrics.aappublications.org]

  Extra-articular manifestations include nail changes, such as pitting and onycholysis, and asymptomatic uveitis. [web.archive.org]

  The onset of chronic uveitis is usually asymptomatic, which underlines the importance of the slit-lamp examination by an ophthalmologist at the time of diagnosis with arthritis and periodically thereafter. [aafp.org]

  4-year-olds and 9 to 11-year-olds; more common in females than males In one or more joints (unilaterally or bilaterally) Pain Swelling Redness Increased warmth to palpation Tenderness Morning stiffness Rash Fever Uveitis (inflammation of eye) often asymptomatic [accessphysiotherapy.mhmedical.com]

• Turkish

  In the healthy Turkish population, the frequencies of A and G alleles were 71 and 29%, respectively. [ncbi.nlm.nih.gov]

  Turkish Başlık:Jüvenil İdiopatik Artrit ve Ailesel Akdeniz Ateşi Birlikteliğinde Erken Tedavi ve Multidisipliner Yaklaşımın Önemi Anahtar Kelimeler:Jüvenil idiopatik artrit, ailesel Akdeniz ateşi, anti- tümör nekrozis faktörü Ailesel Akdeniz Ateşi (AAA [ftrdergisi.com]

  Turkish Journal of Rheumatology, vol. 26, no. 1, 2011, p. 71+. Accessed 21 Apr. 2020. Gale Document Number: GALE|A292713716 [go.gale.com]

  A Akcali, S Pehlivan, M Pehlivan, T Sever and M Neyal, Association of Macrophage Migration Inhibitory Factor Gene Promoter Polymorphisms with Multiple Sclerosis in Turkish Patients, Journal of International Medical Research, 38, 1, (69), (2010). [doi.org]

• Skin Rash

  All patients with systemic onset disease had fever (n = 10) followed by skin rash, hepatosplenomegaly and lymphadenopathy. Uveitis was found in 2 patients, and both belonged to the oligoarticular group. [ncbi.nlm.nih.gov]

  Systemic JIA causes inflammation in one or more joints and is often accompanied by a high spiking fever (103°F or higher) that lasts at least 2 weeks and a skin rash. [arthritis.org]

  A patient who develops pericarditis is also prone to develop a skin rash, lymphadenopathy, splenomegaly, pulmonary disease, and amyloid disease. [pediatrics.aappublications.org]

  Skin rashes that can’t be explained. Fever along with swelling of lymph nodes or inflammation in the body’s organs. To be sure that it is juvenile arthritis, doctors may: Perform a physical exam. Ask about family health history. [niams.nih.gov]

• Subcutaneous Nodule

  There is significant correlation between ANA seropositivity and HLA-B27, early erosion, response to treatment, uveitis and subcutaneous nodule. [ncbi.nlm.nih.gov]

  Results: One child had subcutaneous nodules; one (of 11) was rheumatoid factor positive; six were ANA positive. Median age at diagnosis was 10.7 years (2.5 to 15.9). [ard.bmj.com]

  Subcutaneous nodules, cervical spine fusion, chronic uveitis, and destructive hip disease occur RF+ PoJIA. [2] Rheumatoid factor negative (RF-) - patient tests negative on all tests for the RF. [4] Morning stiffness, fatigue and possibly a low grade fever [physio-pedia.com]

  The most characteristic lesions of rheumatoid arthritis are subcutaneous nodules, which may be present for weeks or months and are most commonly found over bony prominences, especially near the elbow. [medical-dictionary.thefreedictionary.com]

• Arthritis

  Juvenile rheumatoid arthritis, also termed as juvenile idiopathic arthritis sometimes, is not uncommon in children. Pain in the joint, stiff joints, and swelling are some of the common presenting complaints. [symptoma.com]

  Psoriatic arthritis (PsA): Establishing the diagnosis of psoriatic arthritis involves demonstration of both large and small joint arthritis and a characteristic rash (psoriasis). [medicinenet.com]

  Juvenile rheumatoid arthritis (JRA), also called juvenile idiopathic arthritis (JIA), is the most common type of arthritis that occurs in children, affecting 50,000 youth in the U.S. [labtestsonline.org]

• Joint Swelling

  He suffered from fever and generalized rash followed by multiple joints swelling for four months before admission. Physical examination revealed cervical lymphadenopathy and hepatosplenomegaly. [ncbi.nlm.nih.gov]

  Joint injections. In cases of extreme joint swelling, the doctor may administer local steroid injections directly into the swollen joint(s). Oral steroid medicines. [rileychildrens.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. [nlm.nih.gov]

  The signs and symptoms of JRA include: Joint swelling with pain and stiffness. This most often affects the knees and the joints in the hands and feet, and it may be more severe in the morning or after a nap. Fever and rash. [sobeyspharmacy.com]

  Juvenile rheumatoid arthritis (JRA) causes joint swelling, pain, stiffness and loss of motion. It can affect any joint, and in some cases it can affect internal organs as well. One early sign of JRA may be limping in the morning. [wexnermedical.osu.edu]

• Arthralgia

  We present a three-year-old boy with severe iron deficiency anemia (without any pulmonary symptoms) and arthralgia at the time of diagnosis. [ncbi.nlm.nih.gov]

  Related Cases Disease Juvenile Rheumatoid Arthritis Symptom/Presentation Arthritis and Arthralgia Extremity Problems Pain Specialty Immunology Pharmacology / Toxicology Rheumatology Age School Ager To Learn More To view pediatric review articles on this [pediatriceducation.org]

  Salmon rash of Adult JRA Arthritis Arthralgias, arthritis and myositis are universal features of adult JRA though often are preceded by fever by a few weeks. [clinicaladvisor.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]

  It is characterized by joint effusion (an increase in the amount of synovial fluid in the joint) and/or presence of a combination of symptoms including limitation in the range of motion, pain or tenderness associated with joint movement, and increased [rheumatoidarthritis.net]

  Ultrasonography is a noninvasive and inexpensive method to confirm a joint effusion. Bone scans help detect osteomyelitis, malignancy, and joints that have subclinical inflammation. [web.archive.org]

  English arthritis (joint swelling, polyarthritis and joint effusion) Maltese artrite (nefħa fil-ġogi, poliartrite u effużjoni mill-ġogi) Last Update: 2017-04-26 Usage Frequency: 1 Quality: English Treatment of septicaemia, polyarthritis or polyserositis [mymemory.translated.net]

  Results —In 30 (75%) patients we found joint effusion (bursa suprapatellar and prepatellar), 24 (60%) with synovial proliferation, 15 (38%) with cartilage thickness, 4 (10%) quadriceps tendonitis, and 3 (7.5%) Baker's cysts. [ard.bmj.com]

• Enthesopathy

  1/32 (3.1) Psoriatic 1/15 (6.7) JIA subset No. of patients with amyloid (%) Systemic onset 10/52 (19.2) Oligoarticular 0/15 (0.0) Extended oligoarticular 2/55 (3.6) Polyarticular, RF − 4/41 (9.8) Polyarticular, RF + 1/37 (2.7) Enthesopathy‐related 1/ [academic.oup.com]

Workup consists of a detailed history and physical examination.

Laboratory tests

Laboratory tests are not diagnostic but are performed to both confirm the presence of infection and to eliminate the possibility of other diseases.

They include:

• Complete blood count
• Erythrocyte Sedimentation Rate
• Serum ANA
• Serum C Reactive Protein assay
• Liver function tests
• Kidney function tests
• Urinalysis
• Angiotensin Converting Enzyme levels (to rule out sarcoidosis)

Imaging 

Synovial thickness, and volume of effusion monitored by ultrasound is used to examine the progression, and prognosis of the disease [6]. Imaging studies include X-Ray of involved joints, CT scan and MRI (highly sensitive). MRI can give details about the status of soft tissue, cartilage of the joint, synovial membrane, and joint integrity as a whole [7].

Test results

On the basis of physical examination and history and by eliminating all other diseases with the help of laboratory tests, a diagnosis of juvenile rheumatoid arthritis can be made.

• Synovial Cyst

  […] radiographic absorptiometry (DRA) Arthrocentesis and synovial biopsy Pericardiocentesis See Workup for more detail. [emedicine.medscape.com]

• HLA-B27

  There is significant correlation between ANA seropositivity and HLA-B27, early erosion, response to treatment, uveitis and subcutaneous nodule. [ncbi.nlm.nih.gov]

  It appears to have association with HLA alleles other than B27 (e.g. HLA-DR5). [pathophys.org]

  HLA B27-positive. Family history of HLA B27-related disease. Acute anterior uveitis. Onset in a male over the age of 6. [patient.info]

  HLA-B27 is present more commonly in enthesitis-related arthritis. [msdmanuals.com]

• HLA-A2

  […] association HLA DRB1*0401, 0404, 0101 in Caucasians EOPA: HLA-A2, -DR5, -DR8, - DPB1*0201. [doi.org]

  Among children who carry HLA-A2 and any two HLA-DR alleles (HLA-DR3, -DR5, -DR6, -DR8), the median age at onset of pauciarticular disease was only 2.7 years. [arthritis-research.biomedcentral.com]

Pain management

It includes non-steroidal antiinflammatory drugs (NSAIDs) and opioids. Counselling plays an important role in the pain management. Physical therapy and psychological interventions are of great benefit for the patient [1].

Other medications

Many new drugs have been prepared to help manage rheumatic diseases. They include methotrexate, a DMARD, etanercept [8] and Tumor Necrosis Factor alpha blockers. Celecoxib has been found effective in one study [9].

Physiotherapy

Regular physiotherapy sessions may help improve movement and functioning of the affected joints.

Prognosis depends upon the subtype. Generally, JIA has a good prognosis, provided it is appropriately treated. Polyarticular JIA has the worst prognosis of all subtypes and needs to be treated aggressively to improve patient's health.

Complications

Complications of JIA are rarely serious. They mainly involve a restricted life style due to physical impairment of motion. In polyarticular JIA, involvement of internal organs may complicate the disease. Overall, children suffering from JIA have weak bones and muscles and slower growth.

High adiposity: According to a study, patients with severe JIA have high adiposity accompanied by increased bone resorption [4]. Such children become obese and remain short in height.

As the name indicates, JIA has no known etiology. It is an autoimmune reaction which may or may not be triggered by genetic as well as external factors. Whatever the precipitating cause may be, JIA affects young children and usually persists for life.

Incidence

JIA affects between 8-150 out of every 100,000 children, depending upon the analysis [2].

Age

JIA may occur anywhere between the 1st year of life to age 16. It is however, more common between 1-3 years of age.

Sex

Like other rheumatic diseases, JIA is more common in females.

Juvenile arthritis consists of various conditions that start before the age of 16 years and last for longer than 6 weeks [3]. It may be classified into 3 subtypes. They include:

• Oligoarticular JIA

This subtype is also known as pauci-articular JIA. In this type, there is only involvement of 4 or less than 4 joints. The affected joints are asymmetrical, more often than not affecting just one side of the body. Larger joints are more frequently involved, however, smaller joints such a those of fingers and toes may sometimes become involved as well. High serum ANA levels are a marker for oligo articular JIA.

• Polyarticular JIA

This subtype occurs when there is involvement of 5 or more joints in the first 6 months of development of disease. Large as well as small joints are symmetrically involved and there is even involvement of the jaw and neck. Due to the aggressiveness of this subtype, prognosis is worse.

• Systemic JIA

In this subtype, as the name indicates, there are systemic signs of infection and inflammation such as fever, maculopapular rash on the trunk and extremities as well as both large and small joint involvement. Internal organs may become involved, commonly hepatosplenomegaly occurs. The fever and rash come and go making diagnosis difficult. Serum ANA levels are also low.

Since JIA is an autoimmune disease of unknown etiology, it cannot be easily prevented. An increased consumption of omega 3 fatty acids has proved to be beneficial in 2 small studies [10].

Juvenile rheumatoid arthritis (JRA) is the most common cause of chronic rheumatic disease in childhood [1]. It is an autoimmune, noninfective condition in which inflammation of one or more joints occurs at an early age. This name, JRA, has been replaced by the more appropriate term: Juvenile idiopathic arthritis (JIA).

Definition

Juvenile rheumatoid arthritis (JRA), also known as JIA, is inflammation of at least one joint that persists for at least 6 weeks, even after other causes are excluded, in an individual younger than 16 years.

Cause

JRA is a disease of unknown etiology. It is an autoimmune disorder in which our body's immune system, that normally protects our body, turns against our own cells.

Symptoms

JRA presents with pain and swelling of a joint, most commonly the knee, elbow, wrist and ankle joint. Pain and swelling are accompanied with stiffness and restricted movement, and sometimes there may be presence of rash on the trunk and extremities, and fever.

Treatment

Immunosuppressive medications and painkillers taken along with physiotherapy can help relieve symptoms.

1. Weiss JE, Luca NJ, Boneparth A, Stinson J. Assessment and management of pain in JIA. Peadiatr Drugs. 2014 Oct 21. PMID 25331986.
2. Hoffart C and Sherry D. Early identification of juvenile idiopathic arthritis. J Musculoskel Med. 2010. 247:2.
3. McKeever K, Kelly MM. Growing up with juvenile idiopathic arthritis. MCN Am J Matern Child Nurse. 2014 Oct 18. PMID 25333886.
4. Markula-Patjas KP, Ivaska KK, Pekkinen M, Andersson S, Moilanen E, Viljakainen HT, Makitie O. High adiposity and serum leptin accompanied by altered bone turnover markers in severe juvenile idiopathic arthritis. J Rheumatol. 2014 Oct 15. pii:jrheum.131107. PMID 25320222.
5. Hoeksma AF, Va Rossum MA, Zinger WG, Dolman KM, Dekkar J, Roorda LD. High prevalence of hand and wrist symptoms, impairments, activity limitations, participation restrictions in children with juvenile rheumatoid arthritis. J Rehabil Med. 2014 Sept 3 doi 10.2340/16501977-1879. PMID 25188280.
6. Shanmugavel C, Sodhi KS, Sandhu MS, Sidhu R, Singh S, Katariya S, et al. Role of power Doppler sonography in evaluation of the therapeutic response of the knee in juvenile rheumatoid arthritis. Rheumatol Int. Apr2008;28(6):573-8 [Medline].
7. McHugh K, Gupta R, Murray K. Imaging in Juvenile Chronic Arthritis. Imaging. 1999;11:91-7.
8. Kaminiarczyc-Pyzalka D, Adamczak K, Mikos H, Klimecka I, Mockzo J, Niedziela M. Proinflammatory cytokines in monitoring the course of disease and effectiveness of treatment with Etanercept with children with oligo and polyarticular juvenile idiopathic arthritis. Clin Lab. 2014;60(9):1481-90. PMID 25291994.
9. Foeldvari I, Szer IS, Zemel S, et al. A prospective study comparing Celecoxib with Naproxen in children with Juvenile Rheumatoid Arthritis. J Rheumatol. Nov 2008;36(1):175-82 doi 10.3899/jrheum.080073. PMID 19012356.
10. Alpigiani MG, Ravera G, Buzzanca C, Devescovi R, Fiore P, Lester A. The use of omega 3 fatty acids in juvenile idiopathic arthritis. Peadiatr Med Chir. 1996 Jul-Aug;18(4);387-90.