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Juvenile Rheumatoid Arthritis

Juvenile Idiopathic 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.


Presentation

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.

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 Myalgia (pain in the muscles) No one knows for sure the exact cause of juvenile rheumatoid arthritis.[casapalmera.com]
Short Stature in Children
  • Short stature in children with coeliac disease. Pediatr. Endocrinol. Rev. 6, 457–463 (2009). 104. Bona, G., Marinello, D. & Oderda, G. Mechanisms of abnormal puberty in coeliac disease. Horm. Res. 57, 63–65 (2002). 105. Volta, U. et al.[dx.doi.org]
Cerebral Palsy
  • A child with spina bifida, cerebral palsy and juvenile rheumatoid arthritis is presented, and strategies to approach a patient with multiple paediatric onset disabling conditions with possible overlaps are discussed.[ncbi.nlm.nih.gov]
Cyanosis
  • An 8.5-year-old girl evaluated for central cyanosis, hypoxia and normocarbia was found to have aorticopulmonary window and pulmonary hypertension. The diagnosis of Eisenmenger syndrome (ES) was made and treatment with bosentan was started.[ncbi.nlm.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]
  • 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]
  • 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
  • arthritis juvenile chronic polyarthritis monarticular juvenile rheumatoid arthritis pauciarticular juvenile arthritis systemic juvenile rheumatoid arthritis juvenile idiopathic arthritis chronic childhood arthritis[wikidata.org]
  • Childhood arthritis is called juvenile arthritis or juvenile idiopathic arthritis. It is also referred to as juvenile rheumatoid arthritis (JRA), though that is an older term.[arthritis.about.com]
  • MAIN OUTCOME MEASURES: First consultation with an arthritis specialist subsequent to diagnosis by a non-arthritis specialist and time to first consultation with an arthritis specialist.[ncbi.nlm.nih.gov]
  • Previously called juvenile rheumatoid arthritis, the name has been changed to reflect the difference between the juvenile (childhood) forms of arthritis and adult forms of arthritis.[dx.doi.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]
  • This inflammation leads to chronic joint swelling and damage. JIA is the most common type of arthritis in children under the age of 17.[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]
  • If your child has joint swelling, stiffness, or pain for no obvious reason, consult your family doctor. If you have any questions about JRA, your Sobeys Pharmacist will be happy to answer them.[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]
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]
Knee Effusion
  • She is found to have a right knee effusion and associated soft-tissue swelling with no redness or warmth. Lab work reveals negative Rheumatoid factor, a positive low titer ANA and a normal WBC. Radiographs are normal for her age.[orthobullets.com]
Platyspondyly
  • From the age of 3 years, he developed JRA, which resulted in severe restrictive joint disease, osteopenia, and platyspondyly. Patient 2, born in 1976, had tetralogy of Fallot and peripheral pulmonary artery stenosis.[ncbi.nlm.nih.gov]

Workup

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.

Elevated Sedimentation Rate
  • Many patients with JIA, especially the polyarticular and systemic types, have elevated sedimentation rates and anemia. Antinuclear antibody is a useful test in classifying patients with JIA and determining the risk of uveitis.[unboundmedicine.com]
Synovial Cyst
  • […] radiographic changes in JIA include the following: Soft tissue swelling Osteopenia or osteoporosis Joint-space narrowing Bony erosions Intra-articular bony ankylosis Periosteitis Growth disturbances Epiphyseal compression fracture Joint subluxation Synovial[emedicine.medscape.com]

Treatment

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

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.

Etiology

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.

Epidemiology

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.

Sex distribution
Age distribution

Pathophysiology

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.

Prevention

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].

Summary

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).

Patient Information

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.

References

Article

  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. 

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Last updated: 2018-06-21 19:33