Rheum-inationsFor the physical therapist

All you wanted to know about rheumatology and more!

Susan Shenoi MD, MS, RhMsUSAssistant Professor

Clinical DirectorSeattle Children’s Hospital

10-2017

Disclosures

No financial disclosuresWill reference off label use of drugs

Disclaimer

I am not kind enough,not patient enough,not skilled enough…..to ever do what you do!

Thank you for all that you do to help our kids!

Objectives• Case based learning• Recognize patterns of joint pain• Recognize signs of arthritis• Recognize Juvenile Idiopathic Arthritis

– Categories– Medications used– Complications

• Impact exercise on child • Brief overview of Juvenile dermatomyositis (JDM)• Identify resources for further help

Pediatric Rheumatology “pearls”

Recognize Patterns of Joint Pain

ANA leg pain 3 months

• activity rest• Exam normal• Labs - not needed

If done normalOr + ANA (1:80)

Mechanical• Usually no/ minimal am stiffness

• Worsening pain with increased activity

• Lack systemic symptoms like fatigue

• Swelling/ Warmth/ Decreased ROM +/-

• Examples: hypermobility, tendonitis, osteoarthritis*

* Seen more commonly in adults

ANA leg pain 3 months

• activity rest such as after sitting for long time

• Exam shows swollen warm joint(s) that are not moving well

• Labs – ESR and CRP are highOr + ANA (1:640)

Inflammatory• AM stiffness/ Gelling

• Pain better with activity

• Fatigue +/-

• Swelling/ Warmth/ Decreased ROM present

• Examples: JIA, Inflammatory bowel disease related arthritis

ANA body pain 3 months

• activity rest• Pain all day long• Pain with light touch• Exam normal except

for allodynia• Labs - normal

Or + ANA (1:80)

Central Pain Sensitization• Prolonged AM stiffness

• Pain all over, several days (months/ years)

• Increasing pain with activity

• Systemic symptoms, fatigue +, non-restorative sleep

• Tender points/ Allodynia/ Hyperalgesia

• Joint exam usually normal – no swelling, warmth, normal

ROM

Rheumatology “Pearl” #1Pattern recognition

3 patterns to joint pain:

– Inflammatory

– Mechanical

– Central Pain Sensitization

Recognize signs of Arthritis

Arthritis = Juvenile Arthritis

A) True

B) False

False – arthritis can be secondary to many causes infection, cancers etc ……

ANA leg pain 3 months • Waking up at night

with pain• Fevers on and off • Exam arthritis of

knee• Labs –ESR 30 CRP 2.5

CBC platelet 90KWBC 3 K/mm3

Rheumatology “Pearl” #2

Arthritis ≠ Rheumatology

Juvenile arthritis is a diagnosis of exclusionMust rule out

– infection, malignancies,– metabolic, mechanical etc

Arthritis ≠ RheumatologyMalignancy–may mimic JIA– 21-33% of ALL present with bone pain– 62% have musculoskeletal symptoms

Examples leukemia, lymphoma, neuroblastoma, localizedbone tumors (Ewing's)

AVOID STEROIDS TILL DIAGNOSIS CLEARJones OY, Pediatrics, 2006

When do you suspect it!– pain at rest, at night, or out of proportion – back pain, bone pain, bone tenderness– atypical fevers– night sweats– weight loss

Useful studies– low counts (>2 cell lines down)– lactate dehydrogenase (LDH), uric acid– Imaging (CT chest, abdomen – mass/ lymph

nodes)– BM/ LN biopsy

Recognize Juvenile Arthritis

Vignette• DR 7 yr boy limp for12 months• Saw orthopedic 8/15

- X-ray foot and ankle normal- Told limp was behavioral

• Second opinion - orthopedic 11/15• Decreased range of motion (ROM) hip• Inflammatory markers –

– CRP normal (<0.8) ESR 28 (0-10)

• Blood counts - CBC normal• Bone scan normal• MRI ankle and pelvis ordered

Right hip arthritis/ synovitisRheumatology referral• ROM, pain right hip• warmth swelling both

ankles • Enthesitis (inflammation . tendons, muscle

insertion to bone)• Plantar fasciitis

DiagnosisJIA - Enthesitis related arthritis (ERA)

Arthritis = inflammation of joints

A) True

B) False

True – red, warm, swollen, decreased

range of motion

Shenoi S. JIA Pediatr Rev. 2017 May;38(5):221-232.

Arthritis is only for old people

A) True

B) False

False – kids get arthritis too,

CDC 1 in 250 kids have arthritis

Rheumatoid Arthritis Adult

Arthritis in Children

Juvenile Arthritis = JCA = JRA = JIA

A) True

B) False

True – one disease many names

Juvenile Idiopathic Arthritis

• < 16 years• > 6 weeks duration• No known cause

Petty R et al . ILAR Classification Edmonton J. Rheum. 2001

JIA is due to vitamin deficiency

A) True

B) False

False – we don’t know what causes it?

Complex Genetic Trait

Gene-Environmental Factors

Pathogenesis

JIA

Environment

GenesOther

Prakken B et al . Lancet 2011

Chronic inflammation: imbalance between mediators

TNF� IL-1�IL-8

IL-12IFN�

IL-4/IL-13IL-1Ra

TGF� IL-10

Chronic inflammation: Imbalance between mediators

All juvenile arthritis is the same

A) True

B) False

False – 7 categories of JIA

different presentations

ILAR ClassificationInternational League of Associations for Rheumatology

7 mutually exclusive categories:Systemic arthritisOligoarthritis (persistent, extended)Polyarthritis (RF +)*Polyarthritis (RF -)Psoriatic arthritisEnthesitis-related arthritisUndifferentiated arthritis

Petty R et al . ILAR Classification Edmonton J. Rheum. 2001

Categories of JIA

JIAOligoarticular��4 joints

Polyarticular> 4 joints

Extended oligo> 4 joints

Enthesitis-related

Psoriatic arthritis

Systemic-onset Undifferentiated!

RF(-) RF(+)

Image courtesy: Kristin Hayward

ANA 3 yr old girl Parents noticed her to be walking limp for 3 months

Preschool teachers -she limps too

ESR and CRP are normal

Oligoarticular JIA• 40-60% JIA• Females 3:1• 1-3 years• < 5 joints – knee, ankle, elbow• 70% ANA +• 30% asymptomatic uveitis• Labs often normal ESR CRP CBC

Oligoarticular JIA Criteria Exclusions

• 1-4 joints in first 6 months

• Persistent -same as above

• Extended - > 5 joints beyond 6 months

• Family history-psoriasis

• Family history-HLA B27 disease

• Positive RF test• HLA B27 male with

onset >6yrs• Systemic features

Polly2yr girl difficultyrunning, cranky inmorning, swollenfingers, knees, ankles

Labs - CRP 15.3 ESR 63 WBC 11.1 HCT 28 Platelet 472 ANA negativeRF negative

Polyarticular JIA• 30-40% JIA• Females • > 4 joints – large and small joints• C-Spine, TM joint• RF – (any age)• RF + (10%) usually adolescence• 30% ANA

Polyarticular JIA RF –Criteria Exclusions

• 5 or more joints in first 6 months

• RF -

• Family history-psoriasis

• Family history-HLA B27 disease

• Positive RF test• HLA B27 male

with onset >6yrs• Systemic features

Polyarticular JIA RF +Criteria Exclusions

• 5 or more joints in first 6 months

and• + RF test twice 3

months apart

• Family history-psoriasis

• Family history-HLA B27 disease

• HLA B27 male with onset >6yrs

• Systemic features

Rheumatoid Nodules

Micrognathia and Retrognathia

Spykar6 yr boy fevers high spiking103F X 2 wksRash on/ off,Recently shortnessof breathO/E big liver spleenarthritis

Systemic JIA

• Arthritis with or preceded by fever 2 weeks and

• any 1 of : -rash-adenopathy-serositis-liver-spleen

Systemic JIA– 10-20% JIA– fevers - daily spike (often pm) to >103F– 1/3 pericarditis, pleuritis– Adenopathy– Hepatosplenomegaly– Arthritis variable– Evanescent rash in about 80%– Anemia, Elevated WBC and platelets – Very high ESR, CRP, ferritin– ANA, RF negative

.Shenoi S, Wallace CA. Diagnosis & Rx of SJIA. J Pediatr. 2016 Oct;177:19-26.

• 9 yr girl swelling of DIP/ PIP hands X 3 mths

• Silvery scaly rash elbows and scalp

• BTW mom says always had funny nails

• Labs at PMD –CBC, ESR/ CRP normal, ANA 1:40

Psoriatic JIA

• Asymmetric oligoarthritis, DIP joint involvement, dactylitis

• Uveitis• ANA + 30-50%

Psoriatic JIA Criteria Exclusions

• Arthritis and psoriasis

or• Arthritis and two of

- Dactylitis- Nail signs- FH

• Family history-HLA B27 disease

• Positive RF test• HLA B27 male with

onset >6yrs• Systemic features

• Arthritis and Enthesitis or

• Arthritis or Enthesitis two of -

- Sacroiliac pain- HLA B 27 +- FH- Anterior uveitis- Male > 6 yrs

• Family history of psoriasis

• Systemic features• RF +

Enthesitis Related ArthritisCriteria Exclusions

Enthesitis Related Arthritis• Adolescent boys, familial 10-20% • Lower extremity oligoarthritis; knees and ankles • Spine - Sacroiliitis decreased mobility of the

lumbar spine • Enthesitis • Labs - mildly elevated WBC and ESR• RF, ANA - negative. • HLA-B27 + > 90%

Enthesitis

Enthesitis

Undifferentiated JIACriteria Exclusions

• Not fulfilling criteria for any category

• Fulfills criteria for more than one category

• Meeting criteria for other category

Recognize common medications used to treat JIA

There is no cure for arthritis

A) True

B) False

True – current therapy controls disease

Pharmacologic managementPT / OT OphthalmologyLab/ Radiology

Rheumatology

Psychosocial supportNutrition and growth

. monitoringPediatrician Nursing supportSchool supportFamily/ Social support

Rheumatology “Pearl” #3JIA requires multidisciplinary management

Arthritis can lead to blindness

A) True

B) False

True – some children have uveitis

if untreated can cause blindness

ANA positive children more risk

regular ophthalmologic screening

Rheumatology “Pearl” #4Look into the Eyes

Besides the PT the ophthalmologist is the rheumatologists best friend!

JIA Anterior Uveitis - Asymptomatic

Increasedrisk if

ANA+

Chronic Anterior UveitisSlit Lamp Exam

Late Uveitis -Posterior Synechiae

Weblike attachment pupillary margin to anterior lens capsule

AAP Guidelines Pediatrics 2006

We use chemotherapy to Rx JIAA) True

B) False

True – many medications are known

chemotherapeutic meds (methotrexate,

cyclosporine, cyclophosphamide)

Powerful medicines

Knowing how and when to use them

Medications Used - JIA

A) Nonsteroidal anti-inflammatory (do not combine)– Ibuprofen, Naproxen, Meloxicam, Piroxicam, Diclofenac

B) Steroids– Prednisolone, IV methylprednisolone, Intraarticular

injections triamcinolone acetonide, topical (eyes)

Medications Used - JIA

C) Disease Modifying Antirheumatic drugs (DMARDs)– Methotrexate (po/ iv/ subcutaneous)– Others

• Sulfasalazine• Azathioprine• Cyclosporine• Hydroxychloroquine• Leflunomide

Medications Used - JIAD) Biologics • Anti-TNF

– Etanercept (Enbrel®), Adalimumab (Humira®)– Infliximab (Remicade®), Golimumab (Simponi®), Certolizumab (Cimzia®)

• Anti-IL1– Anakinra (Kineret®), Canakinumab (Ilaris®), Rilonacept (Arcalyst®)

• AntiCD20 - Rituximab (Rituxan®)• Anti-IL6 - Tocilizumab (Actemra®)• CTLA4 - Abatacept (Orencia®)

E) Small molecule: JAK kinase inhibitor - Tofacitinib (Xeljanz®)Infusion Infusion or sc Oral pillSubcutaneous (SC)

Children outgrow their arthritis

A) True

B) False

False – it may undergo remission

but flares are frequent

• 80% of children continue to have active disease

• Joint damage frequently occurs, and is greatest in the first 2 years of disease

• 44% achieve remission

• But by 2 yrs off of meds, most have flared

• <10% are successfully off meds >5 years

Ringold & Wallace. Rheumatology 2009.

Children DO NOT outgrow their arthritis

Early treatment

TREAT – Inactive disease (ID) and clinical remission on medications are

achievable – Best chance of achieving

• Rapid and early initiation of therapy • For each month earlier a subject was treated

odds ID increased by 1.32; p = 0.011

Wallace CA et al. TREAT trial A & R 2012

Early treatmentACUTE - JIA

– Compelling evidence for early aggressive therapy – 3 groups: open label study

• Infliximab and methotrexate• Combination DMARD therapy (methotrexate, hydroxychloroquine,

sulfasalazine)• Methotrexate

– Response seen in • 100% infliximab and methotrexate• 65% combination DMARD• 50% methotrexate (p<0.0001) Tynjälä P et al. Ann. Rheum. Dis. 2011

Rheumatology “Pearl” #5

EARLY RECOGNITION IS KEY (you can help!)

Once diagnosed early and aggressive therapy improves outcomes!

Treatment JIA - CARRA • Consensus Treatment Plans• Childhood Arthritis and Rheumatology Research Alliance • North American organization • > 400 pediatric rheumatologists, researchers and research

coordinators

www.carragroup.org

CARRA - CTP

Consensus treatment plans (CTP) – SJIA– Polyarticular JIA

Dewitt E et all. AC & R 2012Ringold S, Shenoi S et al . AC & R 2014

Kimura Y et al . AC & R 2014

Ringold S, Shenoi S et al. AC & R 2014

Polyarticular JIA CTP

Recognize common complications JIA

Complications Ana who had OligoJIA is doing well and in remission without active arthritis – she still has a gait that is limping and off – why?

Affected leg grows longer Leg length discrepancy Shoe lift

Other complications of JIA

• Joint space narrowing• Destruction of cartilage• Erosions• Chronic pain • Joint damage

Impact Exercise in rheumatic diseases

PT = great resource for children with joint pain

A) True

B) False

100% TRUE

How can JIA impact ChildPhysicalpain, weakness, stiffness after sitting, swelling joints, difficulty writing, unable to participate PE, medication side effects (steroids – chubby, methotrexate - nausea)

Emotionalneed for chronic medications, long term injections/ infusions, sense of being different from peers, drain on family resources (doctors appts, bills, copay, hospital stays)

Educationmissing school (doctor visits, OT/PT, infusions, flares), difficulty with vision (rarely), completing assignments/ exams (stiff hands)

Exercise can damage the joints A) True

B) False

False - exercise helps maintain

good range of motion

- modifications in case of flares

Rheumatology “Pearl” #6Exercise is the ideal drug

• Safe • Inexpensive• Widely available• Dose it (pace, self-limit, modify)

Houghton K. The Physician and Sports medicine. 2012 Sep 1;40(3):77 82.Hebestreit, H et al. Journal of Rheumatology, 1998. 25(8): p. 1626 33.Lelieveld, O.T. et al. Arthritis & Rheumatism, 2008. 59(10): p. 1379Henderson, C.J. et al. Arthritis Care & Research, 1995. 8(2): p. 11Houghton K et al. Journal of Rheumatology, 2013:40(6):979.

Exercise in JIA

• Exercise may improve ROM, active joint count, function, QOL & fitness

• Exercise DOES NOT worsen disease activity or cause flares• Intervention trials small to no effect

Takken T et al. Eur J Phys Rehab Med 2008;44(3):287 97.Cavallo S et al. 2016. Arch Phys Med Rehabil. 2016 Dec 6.

Switching gears!

What is this?4 year old girl Dermatologist

rash on her handsfirst noticed after she had been swimmingdid not respond to topical steroids

preschool teacher - wasn’t keeping up with her classmatesparents were complaining she was clumsy

Visual diagnosis

Juvenile dermatomyositis

– Rash • face (includes eyelids)• extensor surfaces hands, knees, elbows

– Muscle weakness • proximal more than distal• palate, pharynx, proximal esophagus

– Vasculitis • GI tract, skin

JDM evaluationLabs:

General – blood counts, inflammatory markersCPK, aldolase, LDHAntibodies

Imagingswallowing study, muscle MRI

ElectromyogramMuscle biopsy

Treatment of JDMSupportive – airway, nutritionPhysical Therapy – splints, ranging, active strengtheningMedications

sunscreensteroids (pulse if severe, daily oral) - need prolonged treatmenthydroxychloroquinemethotrexate, IVIGCytoxanMMF, cyclosporineRituximab

Identify resources for further help

Resources

• Pediatric Rheumatology Staff – Seattle Children’s– 206-987-2193 (parents permission)– Doctor on call 206-987-7777– susan.shenoi@seattlechildrens.org

• Other resourceswww.arthritis.orgwww.rheumatology.org

Arthritis Foundation

This is a picture of Arthritis Camp – 1970’s

A) True

B) False

True

This is a picture ofArthritis Camp in 2010

A) True

B) False

True

THANK YOU

For the invitation

And your attention

AND MY HOPE…...

“Too late is the medicine prepared, when the diseasehas gained strength by long delay”

Questions