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FACT SHEET: Juvenile Arthritis (also known as “JA”, “childhood arthritis”, and “juvenile rheumatoid arthritis” [“JRA”]1; includes “oligoarthritis” [also known as “pauciarticular juvenile idiopathic arthritis” and “pauciarticular juvenile rheumatoid arthritis”], “polyarthritis” [also known as “polyarticular juvenile idiopathic arthritis” and “polyarticular juvenile rheumatoid arthritis”], “systemic arthritis” [also known as “systemic juvenile idiopathic arthritis” or “SJIA”, as well as “Still’s disease” or “Still’s disease variant of juvenile rheumatoid arthritis”], “psoriatic arthritis”, and “enthesitis-related arthritis”)

Date of Publication: October 14, 2020
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Is the initiation of non-invasive dental hygiene procedures* contra-indicated?

  • No.

Is medical consult advised?  

  • No (assuming patient/client is already under medical care for juvenile arthritis and does not have cervical spine instability).

Is the initiation of invasive dental hygiene procedures contra-indicated?**

  • No.

Is medical consult advised? 

  • No, assuming patient/client is already under medical care for juvenile arthritis, is being appropriately monitored, and does not have cervical spine instability.  
  • Potentially, if the patient/client is likely to require pain medication post-procedures. 
  • Potentially, if the patient/client is on long-term corticosteroid therapy (to ensure adrenal insufficiency is not an issue).

Is medical clearance required? 

  • Yes, if the patient/client is being treated with medications associated with immunosuppression (e.g., corticosteroids, biologic response modifier drugs [including biosimilars], cyclosporine, methotrexate, etc.).
  • Yes, if the patient/client has recently undergone or is about to undergo joint replacement. (This is a rare occurrence in juveniles, and it would likely involve an older child/adolescent.) 

Is antibiotic prophylaxis required?  

  • No, in most circumstances (although extended use of corticosteroids and other immunosuppressive drugs may warrant consideration of antibiotic prophylaxis)2.

Is postponing treatment advised?

  • No, in most circumstances.  
  • Yes, if the patient/client lacks sufficient stamina.
  • Yes, if patient/client’s ability to open mouth is excessively compromised by temporomandibular joint (TMJ) involvement.
  • Yes, if there is cervical spine instability for which appropriate precautions are unable to be taken to ensure safe dental hygiene practice (e.g., neck brace to minimize C-spine movement).
  • Yes, following recent joint replacement or revision surgery unless clearance is given by the patient/client’s orthopaedic surgeon. Typically, all routine office-based dental hygiene, including invasive procedures, should be delayed for several weeks post-surgery. Practice patterns vary among orthopaedic surgeons, with some surgeons adopting 6 weeks post-joint replacement as reasonable for dental/dental hygiene procedures, and others adopting 3 to 6 months. 

Oral management implications

  • Given the high prevalence of arthritis of the jaw in children with JA, if the patient/client has continual difficulty eating or complains about pain during eating, referral to a physician is indicated for definitive diagnosis and management.  
  • When active, juvenile arthritis may adversely affect the child’s stamina for the dental hygiene visit. The joints of the neck may also be affected by arthritis, and TMJ involvement means the child may have difficulty keeping his/her mouth open for routine check-ups and procedures. Thus, dental hygiene appointments should be kept as short as possible, and particularly so when there is also multiple joint involvement with pain and immobility. The patient/client should be allowed to make frequent position changes as required for comfort, as long as safety is not compromised. The semi-supine position may be more comfortable than the supine position. Physical aids, such as a rolled towel or pillow, may be used to provide support for affected neck, joints, or limbs. 
  • Cervical spine instability may result from longstanding JA. In affected patients/clients, appropriate precautions should be taken to ensure safe dental hygiene practice (e.g., neck brace to minimize C-spine movement).
  • Juvenile arthritis that affects the temporomandibular joint can complicate dental work and create an overbite if the mandible does not develop properly. Orthodontic intervention may be indicated.
  • Limitation of jaw movement can make brushing and flossing difficult for the child, and jaw exercises may be indicated as therapy for pain and stiffness.
  • In addition to the “usual” rheumatology medications3, treatment may include local interventions such as mouth splints (typically involving an orthodontist).
  • Patients/clients with JA often take ASA or other non-steroidal anti-inflammatory drugs (NSAIDs), which can increase the risk of bleeding with scaling and root planing. However, the prolonged bleeding is usually not of clinical significance.
  • For patients/clients taking long-term corticosteroids, delayed wound healing and increased risk of infection are considerations. The oral health practitioner must also be alert to the possibility of adrenal insufficiency when any stressful treatment or surgery is planned.
  • JA that affects the wrist joints and the proximal finger joints may limit a patient/client’s ability to perform oral self-care measures. To reduce pain and swelling, the patient/client can use splints on wrists and hands to support the affected joints and let them rest. An occupational therapist can assist with splint and other assistive devices selection.
  • Brushing can be made easier for older affected children/adolescents by using an electric toothbrush (which has a larger handle and requires fewer motor skills), enlarging the handle of a standard toothbrush with a bike handlebar grip or tennis ball, or by using large-handled toothbrushes.
  • Flossing can be made easier for patients/clients by using U-shaped flossers or other floss holders as an alternative to regular dental floss.
  • Patient/client (and/or parental) education is indicated regarding the risk of temporomandibular joint involvement in certain types of juvenile arthritis (particularly polyarthritis and systemic arthritis). Panoramic radiographs may be periodically employed to assess mandibular condylar wear and the TMJs.
  • A potential long-term consequence of chronic juvenile arthritis (particularly of the types formerly referred to as “juvenile rheumatoid arthritis”) is the destruction of joint structures to the extent that joint replacement with synthetic materials may be indicated. Juvenile patients/clients with prosthetic joint replacements (such as hip and knee) very occasionally present in dental practice; in the absence of immunosuppressive therapy, for most such patients/clients, antibiotic prophylaxis is not recommended or required.
  • If arthritis severely affects the mandible, the chin may not grow normally, and surgery may be occasionally required.
  • Some medications (e.g., long-term corticosteroids) used to manage JA may affect the child’s teeth, gums, jaw, and general development.

Oral manifestations

  • JA is associated with xerostomia, which increases the risk of dental caries.
  • As with other autoimmune diseases, compromise of the immune system (be it from the disease itself or its treatment) elevates the risk of periodontal disease.
  • Arthritis of the jaw occurs in many children with juvenile arthritis, and in all types and at any time during the disease. The child may have no symptoms, or the symptoms (such as preauricular pain) may be attributed to recurrent ear problems rather than arthritis of the jaw.
  • Pain, swelling, stiffness, restricted mouth opening (e.g., during talking and mastication), growth disturbance, and facial deformity may occur when arthritis affects the temporomandibular joint (TMJ). Clicking and snapping of the TMJ may result from alterations in articular cartilage and meniscus.
  • One side of the jaw may grow faster than the other. 
  • Systemic juvenile idiopathic arthritis (i.e., Still’s disease), when it involves the temporomandibular joint, may cause malocclusion with protrusion of maxillary incisors and an anterior open bite. There may also be deformation of the mandible, with shortening of the body and reduction in the height of the ramus. The combination of small mandible, mandibular retrognathia, anterior open bite, lower incisor crowding, and incisal protrusion is sometimes referred to as “bird face deformity”.  
  • The use of corticosteroids (such as prednisone) in the treatment of JA can lead to oral candidiasis (yeast infection) and other oral infections as a result of immunosuppression. Bone mineral density of the jaw may also be reduced.
  • Some disease-modifying anti-rheumatic drugs (DMARDs), as well as cyclosporine, have side-effects which include gingival bleeding, oral ulcerations, stomatitis, and tender or swollen gums. Methotrexate can cause mouth sores, in addition to sore throat.
  • Some biologic response modifier drugs (i.e., tumour necrosis factor [TNF] inhibitors [e.g., etanercept, infliximab, adalimumab, and golimumab] and non-TNF targeted biologic agents (e.g., tocilizumab and abatacept) can cause sore throat and/or nasal congestion.
  • The side-effect profile of anakinra (interleukin-1 inhibitor) includes runny nose, while sore throat and cough are associated with the use of abatacept (selective co-stimulation modulator).

Related signs and symptoms

  • Juvenile arthritis is chronic arthritis in childhood — with onset usually less than 16 years of age — that results in joint pain and swelling. It is characterized by inflammation of one or more joints (particularly the synovium lining the joint) that lasts at least six weeks. While most types of JA are considered to be autoimmune diseases of unknown cause4, they often differ from adult rheumatoid arthritis, which is why the term “rheumatoid” has been used less in recent years to describe the various forms of juvenile arthritis.
  • Juvenile arthritis affects as many as 1 in 250 Canadian children, typically manifesting between the ages of 6 months and 16 years.5 Affecting both boys and girls, the main types6 are: 
    • a. oligoarthritis (pauciarticular), the most common, which
      • affects about 50 percent of children with juvenile arthritis
      • involves 4 or fewer joints, most likely knees, elbows, wrists, or ankles
      • may cause eye inflammation
      • will be outgrown by many children by the time they become adults 
    • b. polyarthritis (polyarticular), which
      • affects about 30 percent of children with juvenile arthritis
      • involves 5 or more joints, including
        • jaw and neck
        • smaller joints, such as those in the hands and feet
        • large joints 
      • often closely resembles the adult form of rheumatoid arthritis  or evolves into it
    • c. systemic arthritis (body-wide), also called Still’s disease, which
      • is the least common main type
      • affects about 20 percent of children with juvenile arthritis
      • involves many body parts, including joints, and internal organs, such as
        1. heart
        2. liver
        3. lymph nodes
        4. spleen
      • often begins with
        • bouts of fever and chills
        • light pink rash on the thighs and chest. 
  • Signs/symptoms of juvenile arthritis may begin with joint stiffness, pain, warmth, swelling, erythema (redness), or range of motion limitation. Other manifestations, depending on the type of JA, include: limping; avoidance of use of affected limb; fever (often high every day); rash on trunk and limbs that varies with the fever; pale skin; ill appearance; swelling of lymph nodes, liver, and/or spleen; and uveitis7 (which may be accompanied by red or cloudy eyes, photophobia8, and vision changes).
  • Signs/symptoms tend to be unpredictable, and they last for a matter of months and then disappear, or, more commonly, follow an up-and-down course for many years.
  • Anemia of chronic disease may be present, particularly in children affected by systemic arthritis. Pericarditis (inflammation of the pericardial sac that surrounds the heart) may also occur.
  • In addition to persistent pain, continuing inflammatory activity can cause joint destruction. Slow rate of growth and uneven growth of limbs may also occur.

References and sources of more detailed information


Date: August 20, 2020
Revised:


FOOTNOTES

1 Juvenile rheumatoid arthritis is an older term gradually falling out of favour.
2 Note: Most patients/clients with prosthetic joints do not require antibiotic prophylaxis for invasive dental hygiene procedures, as per the 2016 Canadian Orthopaedic Association/Canadian Dental Association/Association of Medical Microbiology and Infectious Disease Canada Consensus Statement. Children with juvenile arthritis, however, may be on immunosuppressive medications that warrant antibiotic consideration.
3 Medications used to manage juvenile arthritis include acetylsalicylic acid (ASA); other non-steroidal anti-inflammatory medications (NSAIDs, including ibuprofen, naproxen, indomethacin, and tolmetin); corticosteroids (e.g., prednisone, dexamethasone, hydrocortisone, and methylprednisolone); disease modifying anti-rheumatic drugs (DMARDs, including hydroxychloroquine, methotrexate, and sulfasalazine); immunosuppressants (e.g., cyclosporine); biologic response modifier drugs (which cause immunosuppression, including tumour necrosis factor blockers [e.g., adalimumab, etanercept, golimumab, and infliximab], non-TNF targeted biologic agents [e.g., abatacept and tocilizumab], and interleukin-1 inhibitors [e.g., anakinra]). At the time of writing this fact sheet (August 2020), newer DMARDs — such as JAK inhibitors baricitinib and tofacitinib (used in treatment of adult rheumatoid arthritis) — are being investigated for treatment of juvenile arthritis.
4 Systemic juvenile idiopathic arthritis (Still’s disease) is classified by some authorities as an auto-inflammatory rather than autoimmune disease.
5 Medical investigation of juvenile arthritis includes: physical examination (including joints and skin, liver, spleen, and lymph nodes); blood tests (such as antinuclear antibody [ANA], rheumatoid factor [RF], human leukocyte antigens [HLA], erythrocyte sedimentation rate [ESR], and complete blood count [CBC]); and other tests (such as joint x-rays, chest x-ray, bone scan, aspiration and examination of synovial fluid from swollen joints, and eye examination).
6 Two less common forms of juvenile arthritis are psoriatic arthritis (in which children have both arthritis and the skin disorder psoriasis, with either preceding the other; signs include pitted fingernails) and enthesitis-related arthritis (that tends to affect the spine, hips, eyes, and entheses [the places where tendons attach to bones], and which typically occurs in boys older than 8 years of age who have a family history of ankylosing spondylitis [a form of back arthritis] in male relatives).
7 Uveitis is swelling and irritation of the uvea, the middle layer of the eye that supplies blood to the retina.
8 Photophobia is eye discomfort or pain caused by sensitivity to light.


* Includes oral hygiene instruction, fitting a mouth guard, taking an impression, etc.
** Ontario Regulation 501/07 made under the Dental Hygiene Act, 1991. Invasive dental hygiene procedures are scaling teeth and root planing, including curetting surrounding tissue.