Enthesitis related arthritis &Psoriatic arthritis JIA subcategories of spondyloarthropathy?

David CabralBritish Columbia Children’s Hospital

Vancouver, CANADA

Learning objectives

• ERA /JPsA are they spondyloarthopathies?

• How do we classify / diagnose

• How are they the same / different

• How do we evaluate

• How do we treat what is the outcome

A Long time ago in a city far,

far away ……..

Winnipeg 1970s

PATIENT: 12 y Difficulty walking (John)

painful swollen knee & painful heel

Diagnosis: Juvenile rheumatoid arthritis

ANA - , RF - , HLA-B27+, Enthesitis!

Treatment: naproxen, predisone, Sulphasalazine

joint injection, PT, orthotics,

By 17 referred to adult rheumatologist

– no arthritis, naproxen only

– Occasional Knee + back pain

Dr Ross Petty

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Seronegative Enthesitis & Arthritis (SEA) Syndrome

• 39 patients (35 m, 4 f)

• Onset 9.8 y (2-16 y)

• Enthesitis + arthritis

• Negative ANA, RF

• Diagnoses

– JAS 8

– IBD 2

– Reactive arthritis 2

– Reiter syndrome 1

– Idiopathic* 26

Rosenberg AM, Petty RE. Arthritis Rheum 25:1041, 1982

….1990, 10 years later: Vancouver

Idiopathic SEA syndrome!

Do boys with a swollen knee and heel pain end up with ankylosing spondylitis?

…

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…

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…Me

Dr P

…. 1990, Winnipeg story continues

Back to John (25y)

• First rheumatology contact for 5y

• Intermittent NSAID use

• Increasing back pain attributed to warehouse work & sport

• poor fitness, quit football, missing work

• Hunched, poor posture, joint contractures, low self esteem

→ Ankylosing spondylitis first diagnosed!

SEA Syndrome: 10 year outcome

23 / 26 children with idiopathic SEA syndrome

spondyloarthropathy 12

• Definite JAS 6

• Possible JAS 5

• Possible PsA 1

• Non-inflammatory disease 5

• JRA (ACR criteria) 4

• Idiopathic SEA syndrome 2

Cabral DA, Oen K, Petty RE. J Rheumatol 19: 1992

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Spondyloarthropathy (SpA)

▪ Ankylosing Spondylitis

▪ Psoriatic Arthritis

▪ Reiter’s Syndrome

▪ Arthritis of IBD

▪ Undifferentiated SpA

Winnipeg was a landmark experience, learn from it you will!

• SEA syndrome patients likely develop AS.

• Window of improvement before relapse.

• Prognostication and close follow-up especially through transition period is important

• Relapse / onset of back disease may be insidious

• SEA syndrome became ERA

ERA = enthesitis related arthritis

Enthesitis related arthritis

Arthritis and enthesitis

or

Arthritis or Enthesitis, and 2 of

• SI joint tenderness +/or back pain

• HLA-B27

• FH (10) HLA-B27 disease

• Acute anterior uveitis

• Onset in boy over 6

and do not have:

Psoriasis; FH of psoriasis;

RF; systemic symptoms

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Juvenile Idiopathic Arthritis (JIA): subset %

ILAR Classification of JIA SA Can Ind W.Eur

• Systemic arthritis 8 6 8 16

• Oligoarthritis- persistent- extended

27 43 21 51

• Polyarthritis (RF +)

• Polyarthritis (RF - )

14 3 1232

27 18 17

• Psoriatic arthritis 1 7 1 ?

• Enthesitis related arthritis 23 14 36 ?

• Unclassified ? 10 5 ?

• Weakly, Esser, Scott, Ped Rheumatol 2012

• Guzman, Oen et al Annals Rheum.Dis 2016

Juvenile Idiopathic Arthritis (JIA): subsets

ILAR Classification of JIA Adult equivalents

• Systemic arthritis AInD

• Oligoarthritis- persistent- extended

RA-like• Polyarthritis (RF +)

• Polyarthritis (RF - )

• Psoriatic arthritis ??

• Enthesitis related arthritis SpA-like

Unclassified

Juvenile Idiopathic Arthritis (JIA): subsets

ILAR Classification of JIA Adult equivalents

• Systemic arthritis AInD

• Oligoarthritis- persistent- extended

RA-like• Polyarthritis (RF +)

• Polyarthritis (RF - )

• Psoriatic arthritis ??

• Enthesitis related arthritis SpA-like

Juvenile ankylosing spondylitis

Unclassified

Juvenile Psoriatic arthritis (JPsA)

Arthritis and Psoriasis

or

Arthritis and >2 of

• dactylitis

• nail pitting or onycholysis

• FH (10) of psoriasis

and do not have:

HLA B-27 boy>6; systemic symptoms

RF; FH of HLA-B27 disease,

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JPsA Characteristics

• Age at onset: Bimodal peaks

• Sex ratio: 1:1.6 (M:F)

• Joint involvement: typically asymmetric small and large joints pattern: oligoarticular -> polyarticular; dactylitischaracteristic

• Sacroiliac joint changes: <10 %

• Enthesitis: <10 %

• Chronic anterior uveitis: 15 %

• RF: absent; ANA: 30 – 60 %

Huemer C, Malleson PN, Cabral DA, et al: J Rheumatol 29:1531, 2002

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JPsA: Diagnosis obvious!

…….. but not always!

• Diagnosis may not be considered

• Psoriasis not present, or not looked for..

• Psoriasis may be difficult to diagnose

• Nail pitting is frequently transitory

• The characteristic pattern of joint involvement may not be noted.

• Reliable family histories are difficult

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JPsA a spondyloarthropathy?

Adult JPsA phenotypes• Asymmetric oligoarthritis*• Symmetric RA-like arthritis*• Predominant DIP joint arthritis• Arthritis mutilans• spondylitis

Only 40% develop sacroiliitis

Questions about Psoriatic Arthritis

oligo/polyarthritis

or

spondyloarthropathy

Is presence of psoriasis simply coincidental and a modifying influence?

Similar questions can apply to IBD-associated arthritis

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Juvenile Idiopathic Arthritis (JIA)

Other Classification of JIA

Systemic arthritis AInD

Idiopathic Peripheral arthritis (no axial disease)

• Oligoarthritis• Polyarthritis

Qualifiers

• ANA• RF• HLA-B27• Other …….biomarkers

• Psoriasis• IBD

Idiopathic Spondyloarthropathy

(+/- peripheral arthropathy)

• ERA • JAS

Unclassified arthritis ?

Evaluating JSpA

.. & some pearls and myths

Evaluating JSpA

Usually no inflammatory back pain or sacroiliitis but presents as undifferentiated disease: i.e. ERA

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Enthesitis a critical defining feature of ERA

Mechanical / structural / Muscle imbalance• Flat feet

• poor fitting shoes?

• repetitive stress

• patello-femoral / iliotibial band

Osteochondroses• Severs, Osgood-Schlatter’s , iselins, Sinding-Larssen-

Johansen’s etc

Pain syndromes

CRMO

Enthesitis difficult to evaluate: DD?____________________________________________________

Evaluate enthesopathy (ERA) in context

• HLA-B27 +/or FH of SpA

• arthritis

• Transient

• single site

• Tender at non-entheseal sites

• imaging

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7-yr-old girl: proximal tibia pain

9-yr-old boy: heel pain

5-yr-old boy: knee pain

Enthesitis vs CRMO

Peripheral Arthritis Characteristics

Asymmetric, lower limb oligoarthritis

common

• Hips

• Knees

• Ankles

Uncommon

• Mid foot

(characteristic)

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Examining for mid-foot disease

Evaluating axial disease

Arthritis and enthesitisor

Arthritis or Enthesitis, and 2 of

•SI joint tenderness +/or back pain

•HLA-B27

•FH (10) HLA-B27 disease

•Acute anterior uveitis

•Onset in boy over 6

Evaluating Back painPre

PubertyAdolescents

Localised

+ Discitis

+ Osteomyelitis +

+ Tumor +

Spondylo –lysis /-listhesis +

Scheuermann’s disease +

+ CRMO +

Diffuse

malignancy +

structural / ergonomic +

pain amplification +

+/- inflammatory back pain +

4-yr-old with back pain. sclerosis L3 vertebra

15 yr-old mid-thoracic back pain

Low Back pain 15 yr-old

Axial Arthritis in kids: Clinical Characteristics

• Often asymptomatic

• Buttock pain

(more likely ischial tuberosity enthesitis)

• Localized morning Stiffness

• Tenderness over SI Joints, LS-Spine, (? C-Spine)

• Clinical manoevres ….

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FABER test

Mennel’s sign

Clinical manoevres

Reduced back range

Schober’s measurement

Flattening

Caution diagnosing sacroiliitis without arthritis imaging

normal

sclerosis

Bilateral erosions & joint space widening

7-year-old boy with back pain.

16 year old with low back /SI pain

When to do MRI?

• To diagnose sacroiliitis (radiographs normal)

• To exclude sacroiliitis

• To track disease activity

Treatment

… a cascade of drugs

• NSAIDS

– naproxen, ibuprofen, et al

• corticosteroid

– Intra-articular

– Low dose oral

• DMARDs

– methotrexate, sulphasulazine,

• Biologic agents

– Anti-TNF

• peripheral arthritis• enthesitis• Sacroiliitis

May need to be targeted differently

…outcomes

Inactive disease within 2 years

Remission within 5 years

Flare one year after inactive

Guzman J, Oen K, et al Ann Rheum Dis 2016;75:1092-8.

0 20 40 60 80 100

.

Undifferentiated

Polyarthritis RF-Pos

Systemic

Psoriatic

Enthesitis-related

Polyarthritis RF-Neg

Oligoarthritis

Flare one year after treatment stop

JIA outcomes with & without enthesitis

Overview

• ERA (SpA) the most difficult category of JIA to diagnose

• Distinguishing inflammatory vs non- inflammatory disease is an imperative

• The morbidity & poor outcome associated with enthesitis is under appreciated

• Earlier more aggressive treatment may help