Juvenile Idiopathic Arthritis (JIA) Juvenile Idiopathic Arthritis (JIA) is the most common chronic heterogenous rheumatological disorder that manifests in patients aged less than 16 years and, in some cases, can cause severe impairment and disability. It encompasses several distinct subtypes, each characterized by unique clinical presentations, genetic markers, and underlying causes (Skeie et al., 2019; Walton et al., 2000; Al-Mayouf et al., 2021).
Classification
The International League of Associations for Rheumatology (ILAR) classifies JIA into several subtypes: Systemic JIA, Oligoarthritis, Polyarticular JIA (Rheumatoid Factor Negative and Positive), Psoriatic JIA, Enthesitis-Related Arthritis, and Undifferentiated Arthritis. Each subtype has unique clinical presentations with varying impacts on the patient's overall health and specific dental considerations (Skeie et al., 2019; Walton et al., 2000; Al-Mayouf et al., 2021).
Impact of JIA on Oral Health
JIA's impact on oral health is multifaceted, involving periodontal disease, temporomandibular joint dysfunction, restricted mouth opening, and orofacial symptoms. The effects of JIA medications compound these issues and can result in a decreased oral health-related quality of life (Barut et al., 2017; Grevich et al., 2019; Rahimi et al., 2018; Skeie et al., 2019).
Plaque, gingivitis and periodontitis:
Periodontal indicators, including dental plaque, gingival bleeding, and probing depths, are commonly elevated in JIA patients, though some studies report no significant differences compared to healthy controls. Notably, Leksell et al. highlighted a higher prevalence of calculus in JIA patients, reflecting the broader trend of poorer periodontal health in this group (Walton et al., 2000; Skeie et al., 2019).
Dental Caries Risk in JIA Patients:
The risk of dental caries in children with JIA is influenced by multiple factors, though their specific impact is not fully understood. Reduced food intake during active disease, TMJ-related chewing difficulties, and medication-induced oral discomfort can alter dietary habits, often leading to increased frequency of small, sugary meals. This behaviour, compounded by well-intentioned relatives offering sweets for comfort, elevates the risk of caries (Walton et al., 2000; Skeie et al., 2019). Additionally, saliva, essential for maintaining oral health, can be diminished due to the xerostomic effects of JIA medications like NSAIDs and DMARDs. Studies show JIA patients often have lower salivary concentrations of key minerals and enzymes, which correlates with higher Decayed, Missing, and Filled Teeth (DMFT) scores and greater gingival inflammation compared to healthy peers (Siamopoulou et al., 1989).
Temporomandibular Joint (TMJ) Involvement
TMJ involvement is a significant concern in JIA, potentially leading to growth abnormalities, facial deformities, and limited mouth opening. TMJ disease in JIA often progresses without obvious symptoms, making early detection challenging. Radiographic changes in the TMJ, ranging from minor erosion to severe destruction, are prevalent and may contribute to a characteristic facial appearance in JIA patients, including a small mandible, Angle's class II malocclusion, and a 'bird face' deformity. These changes are due to the direct effects of JIA on the TMJ, restricted function, and altered mandibular growth patterns (Pedersen et al., 1995; Larheim and Haanaes, 1981).
Impact of Medications on Oral Health
JIA treatment typically involves NSAIDs, Disease-Modifying Antirheumatic Drugs (DMARDs), and corticosteroids, each with potential oral health implications. NSAIDs, commonly used in pediatric rheumatology, can cause soft tissue ulcers and tooth erosion, particularly when chewed rather than swallowed. Liquid formulations of these medications, often sugar-based, further increase the risk of caries, especially when administered before bedtime. DMARDs like methotrexate can cause ulcerative stomatitis and require careful management to mitigate oral side effects. Cyclosporin occasionally used for severe JIA, is associated with gingival hyperplasia and other systemic side effects that can complicate dental treatment (Walton et al., 2000; Skeie et al., 2019).
Dental Management in JIA Patients
Effective dental care for JIA patients requires an interdisciplinary approach involving pediatric rheumatologists, dentists, and other healthcare providers. Key considerations include:
Medication Assessment: Understanding the child's medication regimen is crucial, as certain JIA treatments can have significant oral health impacts, such as methotrexate-related stomatitis.
Periodontal Care: Rigorous oral hygiene practices are essential to prevent and manage periodontal disease in JIA patients.
TMJ Evaluation: Regular assessment of TMJ function, including checking for pain, clicking, or limited jaw movement, is necessary.
Orthodontic Considerations: Orthodontic evaluation may be needed to address malocclusions resulting from TMJ involvement.
Preventive Measures: Fluoride treatments and dental sealants are recommended to reduce caries risk.
Pain Management: Collaboration with rheumatologists is essential to manage jaw pain and discomfort during dental procedures.
General Anesthesia (GA): GA may be required for some procedures, necessitating consultation with the child's rheumatologist and anesthesiologist.
Regular Follow-Up: Continuous monitoring by both the pediatric dentist and rheumatologist is critical for maintaining overall and oral health in JIA patients.
Name: SJ
DOB: 09/06/2018
Age on presentation: 4 years
Gender: Male
Accompanied by his mother
Consent signed by his mother
History
History of chief complaint:
SJ's mother said his teeth are rotten, but he doesn't have pain. Dr. Manoto from Tygerberg Hospital's Pediatric Rheumatology Department referred him.
"Dear Dr,
Patient NB, a male, requires multiple restorations and extraction under GA. The patient has idiopathic juvenile arthritis (JIA) and received intra-articular steroid therapy on 30/8/2023.
No known allergies.
Current medication: Phenoxymethyl penicillin 125mg/5ml, Multivitamin syrup, Aspelone 15 mg per 5 ml, Abitrexate, Chloroquine sulphate, Folic acid.
Please advise if this child could be booked for GA."
Social history:
SJ is an only child to a single mother. He lives with his mom, his 80-year-old grandmother, and his stay-at-home aunt, who lives next door and assists his grandmother in caring for him.
Prenatal History:
Prenatal History:
SJ's mother reported a healthy pregnancy. She did not take any medications aside from the standard prenatal supplements, including multivitamins, iron, and folic acid. Her diet was normal, with no special dietary restrictions or modifications followed.
Natal History:
SJ was born at full term with no reported history of neonatal jaundice, birth complications, or injuries. Additionally, no congenital abnormalities were identified.
Postnatal History:
SJ was breastfed for the first three months. Due to the mother returning to work, she expressed milk and fed him using a bottle. SJ continued bottle feeding until 2.5 years of age, initially receiving breast milk for the first three months, followed by formula, tea with added sugar, sugary juice, and water. During this period, his mother did not wipe his mouth after feeding, and he often slept with the bottle. After weaning, SJ transitioned to drinking from a regular cup, consuming full-cream milk with added sugar, sugary juice, and water.
Medical history:
SJ has been diagnosed with Juvenile Idiopathic Arthritis. He has no known allergies. His current medications include Phenoxymethyl penicillin 125mg/5ml, multivitamin syrup, Aspelone 15 mg per 5 ml (Prednisolone), Abitrexate (Methotrexate), Chloroquine sulphate, and folic acid.
Dental history and oral hygiene practice:
This is SJ's first dental visit.
SJ's mother brushes his teeth once a day in the morning, but not consistently. She mentioned that it is often challenging to wake him up in the morning, and due to her early work schedule, she sometimes has to leave without brushing his teeth when he doesn't wake up on time.
Extra-oral examination
SJ's facial appearance was normal, with no notable asymmetries or abnormalities. No palpable lymph nodes were detected. The temporomandibular joint was functioning normally without any signs of dysfunction or pain. His muscle tone was relaxed, and no abnormalities were observed.
ORTHODONTIC EVALUATION
Morphological examination of the face:
The facial profile is slightly convex, with a slightly prognathic maxilla and a slightly retrognathic mandible. The chin fold appears normal, and the lips are competent.
Occlusal analysis:
The molar classification shows a distal step on both sides. Overjet and overbite are not applicable. There is no crossbite, and the buccal canine bulges are not palpable. No habit.
Intra-oral examination
No abnormalities were found in the tongue or frenum. However, there is alveolar bone fenestration related to the roots of 51 and 62.
Tooth 55op: Confirmed by radiography to extend halfway through the dentin (D2). SJ is not experiencing any pain, and there are no signs of pathology such as abscess, mobility, or fistula. The tooth can be restored, and more than half its root is intact.
54om: Confirmed by radiography to be limited to the dentin (D1). SJ is not experiencing any pain, and there are no signs of pathology such as abscess, mobility, or fistula. The tooth can be restored, and more than half its root is intact.
64om: Radiography confirmed to extend close to the pulp (D3). SJ is not experiencing any pain, and there are no signs of pathology such as abscess, mobility, or fistula. The tooth can be restored, and more than half of its root length is intact.
65o: Confirmed by radiography to be limited to the dentin (D2). SJ is not experiencing pain, and there are no signs of pathology, such as abscess, mobility, or fistula. However, the distobuccal root is completely resorbed due to the abnormal mesioangular eruption path of the 26.
74o: D3, as confirmed by radiography. SJ is not experiencing any pain, and there are no other indications of pathology, such as abscess, mobility, or fistula. The tooth is restorable, with more than half of its roots intact.
75o: Occlusal caries, confirmed by radiography D3. SJ is not experiencing any pain, and there are no other indications of pathology, such as abscess, mobility, or fistula. The tooth is restorable and has more than half of its root intact.
36: Partially erupted.
84o: Confirmed by radiography to be confined to the enamel.
85: Sound.
46: Partially erupted.
53 and 63: Present with labio-incisal caries and incisal attrition. More than half of the root lengths are intact, and they are restorable.
52, 51, 61, 62: Remaining roots, with the roots of 51 and 62 fenestrated through the mucosa.
83, 82, 72, and 73: Show labial caries confined to the enamel.
Radiographic examination
The bony and soft tissue anatomy appears normal, and all teeth are accounted for except the wisdom teeth. The canines are in a favourable position, and the sequence of eruption is also favourable. Leeway spaces are compromised in the first and second quadrants, interproximal caries 54&64. The dental age is approximately six years.
Teeth 16 and 26: These teeth are in an ectopic position due to an abnormal mesioangular eruption path. This results in impaction at the distal prominence of the primary second molar crowns.
Teeth 55 and 65: The distal roots are prematurely resorbed due to the abnormal mesioangular eruption path of the permanent molars.
Cries Risk Assessment
SJ is classified as a high caries risk child due to several factors. He is a child with special needs. His mother brushes his teeth once daily but inconsistently, and flossing is not part of the routine. Multiple cavitated and non-cavitated lesions are present, including anterior teeth. SJ has not received professionally applied topical fluoride treatments nor attended regular dental check-ups. Additionally, his mother has limited dental health literacy.
Treatment sequence and behaviour management
According to Frankl's behaviour rating scale, SJ's attitude was positive (Shao et al., 2016).
The behaviour management techniques included tell-show-do, distraction, and positive reinforcement. Additionally, since SJ did not speak English but understood some words, his mother acted as an interpreter to facilitate communication and ensure SJ's comfort and understanding throughout the procedures.
Treatment sequence:
Since this was SJ's first dental visit, we decided to begin with the least invasive procedures, gradually progressing to more invasive ones as we earned his trust and cooperation. However, SJ's mother informed us that she cannot commit to a lengthy treatment plan involving multiple visits. As a single mother, she cannot frequently take time off work and has already taken significant time off to manage his Arthritis treatment. Additionally, SJ will soon start a series of injections into his ankles, which will also require her to take time off from work.
As such, we decided to have as much as possible done during this visit and take him to GA to finish the rest in one session. SJ's mom was very pleased with the plan.
Treatment plan
Preventive Phase:
Oral hygiene:
With the use of a disclosing agent, active brushing and flossing
Scaling and polishing.
Fissure sealant:
36,46&85
Restorative treatment:
55op, 54mod, 53&63 strip crowns, 64mo&b; possible pulp therapy, 65o; possible pulp therapy, 75&74o; possible pulp therapy. 73l, 72l, 82l&83l, 84o.
Oral surgery:
51, 52, 61, 62 XLA
Orthodontics:
Consultation regarding the ectopic position of 16&26.
Pediatric Rheumatology: Consultation regarding the safety of GA.
Treatment done
First visit:
1/ Diagnosis and treatment plan.
2/ Oral Hygiene Instruction and Demonstration:
Active brushing and flossing techniques were demonstrated using a disclosing agent. SJ's mom was advised to brush his teeth twice a day and floss as much as possible. It was suggested that SJ's stay-at-home aunt, who lives next door, assist with brushing his teeth in the morning when his mom has to leave early for work and again before bed.
3/ Fissure Sealant: 85. As SJ is a high-caries-risk child and good moisture control was achieved, the resin-based fissure sealant 3M™ Clinpro™ Sealant was selected for its fluoride-releasing, caries-preventive benefits (Simonsen, 2002).
36&46 As they partially erupted, satisfactory moisture control could not be achieved; GIC, GC Fuji TRIAGE® was chosen as it is less sensitive to moisture (Welbury et al., 2004).
4/ 73, 72, and 82, Dyract Flow was used as the restorative material. This choice was made because its ease of handling, good moisture control was achieved, SJ was cooperative during the procedure, and the material's fluoride-releasing properties were beneficial.
5/84o: Filling material Dyract XP.
6/55, 54, 65, 75, 74: SDF was applied to keep the caries progression at bay till we can schedule a GA appointment. Silver diamine fluoride (SDF) has been used successfully to stop the progression of carious lesions in people who cannot tolerate invasive treatment, including children, the elderly, people with medical conditions, and people who require additional care and support (Bridge et al., 2021).
7/ Scaling and polishing were performed, followed by the application of 5% sodium fluoride varnish (2.26% fluoride, 22,600 ppm F).
5% sodium fluoride varnish (2.26% fluoride, 22,600 ppm F) is the only high-concentration fluoride formulation that can be used in children under six. It effectively arrests and reduces the prevalence of dental caries (AAPD, 2023).
Considering the high likelihood of TMJ involvement in JIA and its potential progression without obvious symptoms, SJ was given a 10-minute break between each procedure. He was advised to let us know if he feels any discomfort. The session took a long time, but it was worthwhile.
SJ's mom was pleased with the progress made in a single visit and was motivated to follow through with the treatment plan and oral hygiene instructions.
Referral letter to SJ's physician
SJ's physician was consulted and informed of the treatment plan through a letter, and she has given her approval.
SJ's physician reply
Referral letter to Tygerberg’s Anesthesiologists
Referral letter to Tygerberg’s Anesthesiologists
Anesthesiologists replay
Treatment done under GA
55op, 54om,64om, 74o, 75o,84o
Selective caries removal strategy was used. Selective caries removal arrests carious lesion activity while reducing the risk of pulpal exposure and preserving the odontoblastic barrier. This barrier is crucial for inducing the more ordered deposition of reactionary rather than reparative tertiary dentinogenesis. Additionally, it reduces the risk of bacterial ingress into the pulp, thereby maintaining pulp vitality and maximizing the tooth's prognosis (Lim et al., 2023). Given that the cavities were in high-stress-bearing areas and MG is a child with a high caries risk, the fluoride-releasing properties of compomer Dyract XP made it the material of choice. Other options included Vitremer, both with similar failure rates (Pummeret, 2019).
52, 51, 61, 62, 65: XLA.
Orthodontic consultation was sought to address the premature resorption of the distal roots 55 and 65, caused by the abnormal mesioangular eruption path of teeth 16 and 26, and to decide whether to retain or extract 55 and 65. The ectopic eruption of 26 was deemed irreversible, and the 16 had the chance to be reversible, so the decision was made to extract the 65 and retain the 55.
Third visit
SJ missed his scheduled follow-up appointment two weeks after the general anaesthesia session, returning after three months.
Treatment provided:
Reinforcement of oral hygiene instructions.
Application of topical fluoride.
Fourth visit
Three-month follow-up
At this appointment, it was noted that tooth 16 did not disimpact as expected, and a dentoalveolar abscess was identified associated with tooth 55. Additionally, tooth 26 had drifted mesially, occupying the space of tooth 25.
Treatment Provided:
Reiteration of oral hygiene instructions.
Application of fluoride varnish (5% sodium fluoride).
Extraction of tooth 55.
A space regainer will be planned once both tooth 16 and tooth 26 have fully erupted.
Planned Upper Space Regainer Options:
Pendulum Appliance
Distal Jet Appliance
Gerber Space Regainer
Wilson Rapid Molar Distalizer
Reflection
In retrospect, extracting tooth 55 during the initial GA session would have been the best course of action. Although the orthodontic department initially advised against extraction, considering the impaction of the 16 to be reversible, the circumstances changed after I sought their consultation. General anaesthesia had not been part of the original plan; however, the decision was made later due to SJ's mother expressing difficulty in committing to a multi-visit treatment plan because of her work constraints as a single mother. Extracting the tooth during the GA session could have spared SJ and his mother the additional stress of undergoing the procedure in the dental chair.
References
BARRIGA, B., LEWIS, T. M. & LAW, D. B. 1974. An investigation of the dental occlusion in children with juvenile rheumatoid arthritis. The Angle Orthodontist, 44, 329-335.
BARUT, K., ADROVIC, A., ŞAHIN, S. & KASAPÇOPUR, Ö. 2017. Juvenile idiopathic arthritis. Balkan medical journal, 34, 90-101.
BRIDGE, G., MARTEL, A.-S. & LOMAZZI, M. 2021. Silver diamine fluoride: transforming community dental caries program. international dental journal, 71, 458-461.
GABRIEL, S. E. & MICHAUD, K. 2009. Epidemiological studies in incidence, prevalence, mortality, and comorbidity of the rheumatic diseases. Arthritis research & therapy, 11, 1-16.
GREVICH, S., LEE, P., LEROUX, B., RINGOLD, S., DARVEAU, R., HENSTORF, G., BERG, J., KIM, A., VELAN, E. & KELLY, J. 2019. Oral health and plaque microbial profile in juvenile idiopathic arthritis. Pediatric Rheumatology, 17, 1-9.
HAYNES JR, R. 1975. Adrenocorticotropic hormone: Adrenocortical steroids and their synthetic analogs; inhibitors of adrenocortical steroid biosynthesis. The pharmacological basis of therapeutics, 1486-1488.
HAZELRIGG, C. O., DEAN, J. A. & FONTANA, M. 2003. Fluoride varnish concentration gradient and its effect on enamel demineralization. Pediatr Dent, 25, 119-126.
LARHEIM, T. & HAANAES, H. 1981. Micrognathia, temporomandibular joint changes and dental occlusion in juvenile rheumatoid arthritis of adolescents and adults. European Journal of Oral Sciences, 89, 329-338.
PEDERSEN, T. K., GRØNHØJ, J., MELSEN, B. & HERLIN, T. 1995. Condylar condition and mandibular growth during early functional treatment of children with juvenile chronic arthritis. The European Journal of Orthodontics, 17, 385-394.
RAHIMI, H., TWILT, M., HERLIN, T., SPIEGEL, L., PEDERSEN, T. K., KÜSELER, A. & STOUSTRUP, P. 2018. Orofacial symptoms and oral health-related quality of life in juvenile idiopathic arthritis: a two-year prospective observational study. Pediatric Rheumatology, 16, 1-10.
SHAO, A., KAHABUKA, F. & MBAWALLA, H. 2016. Children’s behaviour in the dental setting according to Frankl behaviour rating and their influencing factors. J Dent Sci, 1, 1-12.
SIAMOPOULOU, A., MAVRIDIS, A., VASAKOS, S., BENECOS, P., TZIOUFAS, A. & ANDONOPOULOS, A. 1989. Sialochemistry in juvenile chronic arthritis. Rheumatology, 28, 383-385.
SIMONSEN, R. J. 2002. Pit and fissure sealant: review of the literature. Pediatric dentistry, 24, 393-414.
SKEIE, M. S., GIL, E. G., CETRELLI, L., ROSÉN, A., FISCHER, J., ÅSTRØM, A. N., LUUKKO, K., SHI, X., FEUERHERM, A. J. & SEN, A. 2019. Oral health in children and adolescents with juvenile idiopathic arthritis–a systematic review and meta-analysis. BMC Oral Health, 19, 1-16.
THIERRY, S., FAUTREL, B., LEMELLE, I. & GUILLEMIN, F. 2014. Prevalence and incidence of juvenile idiopathic arthritis: a systematic review. Joint Bone Spine, 81, 112-117.
WALTON, A., WELBURY, R., THOMASON, J. & FOSTER, H. 2000. Oral health and juvenile idiopathic arthritis: a review. Rheumatology, 39, 550-555.
WELBURY, R., RAADAL, M. & LYGIDAKIS, N. 2004. EAPD guidelines for the use of pit and fissure sealants. European journal of paediatric dentistry, 5, 179-184.