Randy Q. Cron, MD, PhD Univ. of Alabama at Birmingham

Temporomandibular Joint Arthritis in Pediatric

Inflammatory Arthropathies

What is the Temporomandibular Joint?

The temporomandibular joint (TMJ) is a typical sliding "ball and socket" which has a disc sandwiched between it. The TMJ is used many thousands of times a day in moving the jaw, biting and chewing, talking, yawning, etc. It is one of the most frequently used of all the joints in the body. http://www.medicinenet.com/temporomandibular_joint__disorder/page1.htm#1whatis

Diagnosis of TMJ Arthritis

• Clinical history • Physical exam findings • Imaging studies

Challenges in Assessing Pediatric TMJ disease

Asymptomatic TMJ Disease in JIA

• Twilt, et al. 2004 – 45% without pain

• Wallace, et al. 2000

– 70% asymptomatic UAB 2010

Percentage of Symptomatic Patients by Age Range

Ages 0-

6

Ages 7-

10

Ages 11

-1940

50

60

70

80

% o

f Pat

ient

s

50% 56% 74%

Tooth-to-tooth Gap/ Inter-incisor Distance

3 finger rule

Measure of Tooth-to-Tooth Gap

Mouth Opening by Age Twilt et al. 2004

Age (yrs):

0-6 6-11 11-16 16-21

Ingervall 1970

49 mm 51 mm

Sheppard 1965

42 mm 46 mm 51 mm 49 mm

- OPG 2004

43 mm 48 mm 53 mm 53 mm

+ OPG 2004

42 mm 43 mm 47 mm 57 mm

Normal range of mouth opening in children ages 5-17 years

97.5% 75% 25% 2.5%

N = 307 = 47 mm

Pediatr Rheumatol Online J. 2012 Jun 20;10(1):17. [Epub ahead of print]

Prevalence/Incidence of TMJ Arthritis in JIA

New Juvenile Idiopathic Arthritis (JIA) Criteria

Classification of JIA ACR 1977

JRA 1. Systemic onset 2. Polyarticular >4 joints

3. Pauciarticular <5 joints Spondyloarthropathies (HLA-B27) 1. Psoriatic 2. Ankylosing spondylitis 3. IBD associated 4. SEA syndrome

ILAR 1997 JIA 1. Systemic 2. Polyarticular RF- 3. Polyarticular RF+

4. Oligoarticular

a) Persistent (< 5 joints) b) Extended (>4 joints)

5. Psoriatic 6. Enthesitis related

7. Unspecified (none or

more than 1 category fulfilled) J Rheumatol. 2004 Feb;31(2):390-2.

Behrens EM, Beukelman T, Cron RQ.J Rheumatol. 2007 Jan;34(1):234

Behrens

JIA Subtype & Frequency of TMJ Arthritis (orthopantomogram)

0

10

20

30

40

50

60

70

So Oligo RF+ RF- SEA Psor

Subtype

% w

ith T

MJ

invo

lvem

ent

Twilt, et al. J. Rheumatol. 2004;31:1418.

N=97

Twilt

2010 UAB Data, n=183 JIA patients screened by MRI

Cannizzaro E, Schroeder S, Müller LM, Kellenberger CJ, Saurenmann RK. J Rheumatol. 2011;38:510-5. Stoll ML, Sharpe T, Beukelman T, Good J, Young D, Cron RQ. J Rheumatol., in press.

Saurenmann

Stoll

Morbidity with TMJ Arthritis in JIA

• TMJ Pain • Local morning stiffness • Impaired function (chewing, speaking) • Pain with chewing • Decreased mouth opening • Earache • Cosmetic appearance (micrognathia,

facial asymmetry)

Micrognathia

Pediatr Clin North Am. 2005 Apr;52(2):413-42, vi.

Destruction of the Growth Plate

• Growth plate is very superficial, located on the surface of the mandibular condyle head

• Arthritis leads to micrognathia • Costochondral graft surgery

AVOID THIS!

Courtesy of David D. Sherry, MD

*

Treatment of TMJ Arthritis

Do Biologics Treat TMJ Arthritis? Systemic Medication Use in TMJ Arthritic Patients Comparing Any Use vs. Use Only at Time of MRI

NSAIDMTX

TNF-a Inhibito

r (plus A

nakinra)

Steroid

0

20

40

60

80 Have Ever UsedUsed At Time of MRI

% o

f Pat

ient

sN=95

Stoll ML, Good J, Sharpe T, Beukelman T, Young D, Waite PD, Cron RQ.

J. Oral Maxillofac. Surg. 2012;70:1802-7.

Beukelman

Corticosteroid Injections of TMJs are Harmful?

• “A cortisone-wrecked and bony ankylosed temporomandibular joint.” – Plast Reconstr Surg. 1989;83:1084

• Temporomandibular joint osteoarthrosis. Histopathological study of the effects of intra-articular injection of triamcinolone acetonide. – Intra-articular injection of steroid into human

osteoarthritic temporomandibular joints acts as a lytic agent (n=44).

– Haddad. Saudi Med J. 2000 Jul;21(7):675-9.

Corticosteroids are NOT Evil! (for inflammatory TMJ disease)

• Vallon, et al. Long-term follow-up of intra-articular injections into the temporomandibular joint in patients with rheumatoid arthritis. Swed. Dent. J. 2002;26:149 – 12 year follow up of 21 adult RA patients following

corticosteroid injections (n=11) of TMJs – long-term progression of joint destruction was low for

both steroid and non-steroid agents

Intraarticular Corticosteroids are Used to Treat Other Joints in JIA

• Intraarticular corticosteroid injection in JIA are safe and effective – Review – Cleary, et al. Arch. Dis. Child.

2003;88:192 • Prevents leg length discrepancy

– Sherry, et al. Arthritis Rheum. 1999;42:2330 • 2nd most common therapy to treat

pauciarticular juvenile arthritis – Cron, et al. J. Rheumatol. 1999;26:2036

Intraarticular Corticosteroids for TMJ Arthritis in JIA

• Martini, et al. J. Rheumatol. 2001;28:1689 – Case report of arthroscopic synovectomy

followed by IA triamcinalone hexacetonide (10 mg) in 15 yo girl with JIA

– Decreased pain, increased function and mouth opening

Zulian

Retrospective Study of Intraarticular Steroid Injection of TMJ Arthritis in JIA

Demographics

Arabshahi, et al. Arthritis Rheum. 2005 Nov;52(11):3563-9.

Pre-Injection MRI Findings

• TMJ effusions in 13/23 • Bony erosions in 19/23 • Condylar flattening 17/23

Arabshahi, et al. Arthritis Rheum. 2005 Nov;52(11):3563-9.

Sedation for Treatment

• Deep intravenous sedation (in combination) – 1-3 µg/kg fentanyl citrate – 2-5 mg/kg pentobarbital sodium – 0.1-0.3 mg/kg midazolam hydrochloride

• Continuous cardio-respiratory monitoring – Cahill, et al. AJR Am. J. Roentgenol. 2007;188:182-186.

Therapeutic Approach

• Performed by experienced pediatric interventional radiologists

• Child placed supine in CT scanner with head rotated 45o away from TMJ to be injected

• Axial CT imaging in area of interest • Sterile preparation of access site anterior to tragus • Local anesthesia with bicarbonate buffered 1% lidocaine

(30 gauge needle) • CT confirmation of needle placement in mandibular fossa • Injection of triamcinalone acetonide (1cc = 40 mg) into TMJ

with 18 or 21 gauge needle – Cahill, et al. AJR Am. J. Roentgenol. 2007;188:182-186.

CT Guidance

Data Collection

• Tooth-to-tooth gap measurements • Pain assessment • MRI findings

– Effusions – Erosions – Condylar flattening

• Side effects

Bita Arabshahi, MD

TMJ Anatomy

Resolution of Effusion Following Intraarticular Steroid Injection

Arabshahi & Cron. Curr Opin Rheumatol. 2006;18:490-495.

Pre Post

Retrospective Study Results

• 13/23 with pain prior to injections (only 3 with pain following injections)

• Tooth to tooth gap increased from 3.59+/-0.725 to 4.07+/-0.606 (P=0.0017) – 43% of patients had a T-T gap increase >0.5 cm.

• In 23 TMJs followed up by MRI: – 11/23 absent or decreased effusions – 2/23 increased effusions (both re-injected) – Bony resorption remained stable in the majority of pts

Arabshahi, et al. Arthritis Rheum. 2005 Nov;52(11):3563-9.

Increase in Tooth-to-Tooth Gap (< 6 yrs old)

Tooth-tooth gap, ages 0-6 (n=5)

pre-in

jectio

n

post-

injec

tion

norm

als2

3

4

5

Increase in Tooth-to-Tooth Gap (7-10 yrs old)

Tooth-tooth gap, age 7-10 (n=10)

pre-in

jectio

n

post-

injec

tion

norm

als2

3

4

5

6

P=

cm

Increase in Tooth-to-Tooth Gap (11-16 yrs old)

Tooth-tooth gap, age 11-16(n=5)

pre-in

jectio

n

post-

injec

tion

norm

als

2.5

5.0

7.5

P=

P=

cm

Complications/Side Effects

• Accidental injection of 1cc of ethanol prior to injection of corticosteroids

• Increase in TMJ pain following injection (n=2) • No infections, subcutaneous atrophy, or

hypopigmentation at injection sites

• Cushingoid features in one child injected by oromaxillofacial surgery (prior to this study)

Arabshahi, et al. Arthritis Rheum. 2005 Nov;52(11):3563-9.

Summary of Retrospective Study

• CT-guided corticosteroid injection of the TMJ in children with JIA appears safe

• Corticosteroid injection of TMJ arthritis in children with JIA is associated with decreased TMJ pain, increased mouth opening, and decreased TMJ effusions as detected by MRI

• +ANA and polyarticular disease may be risk factors for TMJ arthritis

Arabshahi, et al. Arthritis Rheum. 2005 Nov;52(11):3563-9.

Intraarticular corticosteroids for TMJ arthritis in JIA

Ringold S, Cron RQ. Pediatr Rheumatol Online J. 2009 May 29;7(1):11.

Zurich Seattle Germany Philly

Pediatr Radiol. Pediatr Radiol. 2010;40:1498-504.

Toronto

Connolly

• Determine the point prevalence of TMJ arthritis at disease onset in children with JIA using MRI and ultrasound • Subaim: comparative study of MRI versus ultrasound

for diagnosing TMJ arthritis • Development of a screening protocol to predict those

children with JIA at greatest risk for developing TMJ arthritis • Using demographics, serologies, physical

examination, CHAQ, and questionnaire on TMJ functionality/pain

Prospective Study of TMJ Arthritis in JIA

• Meet the diagnostic criteria for JIA • Able to complete study within 8 weeks of

diagnosis

Exclusion Criteria: • Inability to undergo MRI due to metal

implants, braces, pacemakers

Inclusion Criteria:

New-onset JIA Cohort

Jaw Symptoms & PE Findings

MRI: Condylar Flattening & Erosion

MRI: Joint Effusion & Condylar Erosion

MRI Findings

N MRI pattern Unilateral Bilateral Oligo:Poly

8/20 (40%)

Minimal to mild effusion

62% 38% 1:1

17/20 (85%)

Enhancement 31% 69% 0.9:1

9/20 (45%)

Condylar Flattening

50% 50% 1:3

MRI Findings

• All the patients with effusion AND enhancement AND condylar flattening had polyarticular disease.

• All the patients with effusion AND enhancement but NO condylar flattening had oligoarticular disease.

• No other correlations with MRI pattern and age/ duration of disease/ JIA subtype/

CHAQ score/ serologies.

Goldsmith

Ultrasound Appearance of Condyle Flattening (L>R)

Right

Left

Comparison of MRI and US Findings

Comparison of MRI and US indetection of effusions and

condylar erosions(n=40 TMJs)

effusions erosions0

10

20MRIUSConcordance

TMJ appearance

num

ber

of T

MJs

TMJ Arthritis Detection (Dis)agreement by MRI & US

Summary of Acute vs Chronic Findings

• Acute: presence of effusion or enhancement – Seen in all but two patients (83% bilateral)

• Chronic: presence of condylar flattening – Seen in 69% by MRI, most with Poly JIA, 26% by US

• Concordance of MRI and US: – 0% agreement in detection of effusions – 22% agreement in detection of condylar flattening

• Length of disease, CHAQ score, and erythrocyte sedimentation rate (ESR) did

NOT correlate significantly with either chronicity or acuity on MRI.

Predictors of TMJ Arthritis in New-onset JIA

Change in MIO after Corticosteroid Injection

TMJ Arthritis: Prevalence, Diagnosis, and Predictors of Active Disease

• What we’ve learned: – Prevalence of TMJ arthritis is quite high – Unable to establish predictors of active

disease at this time given the high prevalence

– MRI appears much more sensitive than US in detecting early inflammatory changes in the TMJ, especially given operator dependence of US

Weiss, et al. Arthritis Rheum. 2008;58:1189-96.

Pam Weiss, MD

Funding

Nickolett Family Awards

Program for JRA Research

Ethel Brown Foerderer

Fund for Excellence

Credit Where Credit is Due

CHOP Rheumatology CHOP Radiology Bita Arabshahi Anne Marie Cahill

Esi DeWitt Robin Kaye

Pam Fitch Marissa Bilaniuk

Sandy Burnham Ann Johnson

David Sherry Kevin Baskin

Carol Wallace (Seattle)

Questions that Arise:

• Since bilateral enhancement is so common, could it be a normal post-contrast finding?

• Could condylar flattening by itself, or with enhancement, be a normal finding?

• If the above is true: 50% of the kids currently found to have abnormal TMJs by MRI could be normal.

• Therefore: Important to have controls, especially to help make treatment decisions.

Synovial Enhancement in a Normal Control

T1-weighted parasagittal MRI image with fat saturation of the TMJ joint of a normal 7 year old child, showing synovial enhancement (arrow) superior to the condyle (C).

C

96 Children without autoimmune disease screened

94% entirely normal TMJ MRI

Acta Radiol. 2009 Dec;50(10):1182-6.

Tzaribachev

Treatment of TMJ Arthritis in JIA without radiographic guidance

Peter D. Waite, M.P.H., D.D.S., M.D.

University of Alabama at Birmingham

1.2 mm Arthroscope

P = .001

J. Oral Maxillofac. Surg. 2012;70:1802-7.

Post-lnjection MIO Changes

65%

27%

7%

ImprovementWorsening

Unchanged

Stoll ML, Good J, Sharpe T, Beukelman T, Young D, Waite PD, Cron RQ.

J. Oral Maxillofac. Surg. 2012;70:1802-7.

Mouth Opening Improved Following IA-Steroids to TMJs

MIO Change by Subtype

System

icOlig

o

Poly (R

F-Neg

)ERA

Psoria

tic

Undifferen

tiated

-2

-1

0

1

2

3

4

5

6

mm

4.56

2.82

2.20

1.54

-0.67

1.50

Stoll ML, Good J, Sharpe T, Beukelman T, Young D, Waite PD, Cron RQ.

J. Oral Maxillofac. Surg. 2012;70:1802-7.

All JIA Subtypes Respond to IA-Steroids

Post-Injection MRI Results

34%

17%

49%

Some ImprovementComplete ResolutionUnchanged or Worse

Stoll ML, Good J, Sharpe T, Beukelman T, Young D, Waite PD, Cron RQ.

J. Oral Maxillofac. Surg. 2012;70:1802-7.

MRI Findings Improved Following IA-Steroids to TMJs

Young

What do we do for TMJ arthritis not responsive to IA-steroids? • Many have already failed repeated (2 or

more) IA-steroid injections. • The vast majority are already on high

dose, aggressive systemic arthritis therapy (e.g. methotrexate and anti-TNF agents at high doses).

Intra-articular anti-TNF to treat TMJ arthritis

• Scand J Rheumatol. 2008 Mar-Apr;37(2):155-7. • Successful treatment with multiple intra-articular injections of

infliximab in a patient with psoriatic arthritis. • Alstergren P, Larsson PT, Kopp S. • Department of Clinical Oral Physiology, Institute of Odontology, Karolinska

Institutet, Huddinge, Sweden. per.alstergren@ki.se • Abstract • This case report presents the clinical and radiographic course of

temporomandibular joint (TMJ) involvement in a patient with severe TMJ symptoms from psoriatic arthritis (PsA) resistant to both systemic infliximab and intra-articular glucocorticoid and who therefore received multiple intra-articular infliximab injections for 36 weeks. TMJ symptoms improved after the first bilateral intra-articular infliximab injections but even more so after the second injections. The considerable improvement remained for the 36 weeks studied. Bilateral computerized tomography showed no progression in radiographic changes during the treatment. No adverse reaction was observed from the intra-articular injections.

Alstergren

Intra-articular Infliximab Treatment of Refractory TMJ Arthritis in Children with JIA

Unchanged or improved Pre-post IACI Pre-post IAII p-value

Acute changes 9 / 34 (26%) 23 / 34 (68%) 0.001 Chronic changes 9 / 34 (26%) 21 / 34 (62%) 0.008

Intra-articular: steroids anti-TNF

Stoll ML, Morlandt A, Terrawattanapong S, Young D, Waite PD, Cron RQ. Manuscript submitted.

Morlandt

Do non-JIA children with other rheumatic diseases develop TMJ

arthritis? • Many other pediatric rheumatic disorders

are associated with arthritis (SLE, myositis, sarcoidosis, Sjogren, MCTD, etc.).

• Some children with the above disorders have PE findings or complaints suggestive of TMJ arthritis.

Parotitis seen on TMJ MRI

C

Fain ET, Atkinson GP, Weiser P, Beukelman T, Cron RQ. J Rheumatol. 2011 Oct;38(10):2272-3

Screening for TMJ Arthritis in Other Pediatric Arthritides

Fain

TMJ Arthritis in Pediatric Sjogren and Sarcoidosis

Fain ET, Atkinson GP, Weiser P, Beukelman T, Cron RQ. J Rheumatol. 2011 Oct;38(10):2272-3

Atkinson

Contrast weighted MRI sagittal section through the TMJ of a child with juvenile dematomyositis.

C: condyle; Arrow indicates synovial enhancement after administration of contrast.

TMJ Arthritis in Pediatric JDMS and MCTD

Patient number

Age at dx Gender Dx

MIO with positive TMJ

MRI Deviation Peripheral

arthritis

Post injection

MIO Repeat

TMJ

1 15y female MCTD 3.2 yes yes

2 16y female MCTD 3.6 yes yes

3 12y female MCTD 4.8 no yes

4 4y female JDMS 3 no no 3.4 Negative

5 20m female JDMS 3.1 no no 4.20 Negative

6 10y female JDMS 4.6 no yes Active

7 5y male JDMS 1.85 yes yes

Peter Weiser, Stephen Johnson, Robert M. Lowe, Randy Q. Cron. Submitted for publication.

Weiser

Things to Consider • 50-75% of children with JIA develop TMJ arthritis. • All subtypes of JIA develop TMJ arthritis. • TMJ arthritis is frequently asymptomatic. • Inflammation of the TMJ leads to growth plate arrest

(micrognathia). • MRI is the most sensitive modality for detecting TMJ arthritis. • Intraarticular corticosteroid injection is effective treatment for

TMJ arthritis in JIA. • TMJ arthritis can develop while being treated with methotrexate

plus a TNF inhibitor. • TMJ arthritis may be active while other joints are in remission. • Intraarticular infliximab injection treats refractory TMJ arthritis. • Children with sarcoidosis, Sjogren, JDMS, and MCTD can

develop destructive TMJ arthritis.

In Memory of Dr. Frida Gudmundsdottir

Questions??