Enthesitis related arthritis & Psoriatic arthritis - JIA subcategories of spondyloarthropathy? - SARAA Congress 2019
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Enthesitis related arthritis &
Psoriatic arthritis
JIA subcategories of spondyloarthropathy?
David Cabral
British 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 Dr Ross Petty
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
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
…
Me … …
Dr P …
……………
…
Idiopathic SEA syndrome! …
…
Do boys with a swollen …
knee and heel pain end up …
…
with ankylosing spondylitis?
…. 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
___________________________________________________
Spondyloarthropathy (SpA)
23 / 26 children ▪ Ankylosing Spondylitis
▪ Psoriatic Arthritis
with idiopathic SEA syndrome
______________________________________ ▪ Reiter’s Syndrome
▪ Arthritis of IBD
spondyloarthropathy 12
▪ Undifferentiated SpA
• 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
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 symptomsJuvenile Idiopathic Arthritis (JIA): subset %
ILAR Classification of JIA SA Can Ind W.Eur
• Systemic arthritis 8 6 8 16
• Oligoarthritis
- persistent 27 43 21 51
- extended
• Polyarthritis (RF +) 14 3 12
32
• Polyarthritis (RF - ) 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 2016Juvenile 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
UnclassifiedJuvenile 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
UnclassifiedJuvenile 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,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; dactylitis
characteristic
• Sacroiliac joint changes: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
JPsA a spondyloarthropathy?
Adult JPsA phenotypes
• Asymmetric oligoarthritis*
• Symmetric RA-like arthritis*
• Predominant DIP joint arthritis
Only 40% develop
• Arthritis mutilans sacroiliitis
• spondylitisQuestions about Psoriatic Arthritis
____________________________________________________
oligo/polyarthritis
or
spondyloarthropathy
Is presence of psoriasis simply
coincidental and a modifying
influence?
Similar questions can apply to
IBD-associated arthritisJuvenile Idiopathic Arthritis (JIA)
Other Classification of JIA
Systemic arthritis AInD
Idiopathic Peripheral Qualifiers
arthritis • Oligoarthritis
(no axial disease) • Polyarthritis • ANA
• RF
• HLA-B27
• Other
Idiopathic • ERA …….biomarkers
Spondyloarthropathy • JAS
(+/- peripheral arthropathy) • Psoriasis
• IBD
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
Enthesitis a critical defining feature of ERAEnthesitis difficult to evaluate: DD?
____________________________________________________
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
CRMOEvaluate enthesopathy (ERA) in context ____________________________________________________ • HLA-B27 +/or FH of SpA • arthritis • Transient • single site • Tender at non- entheseal sites • imaging
Enthesitis vs CRMO
7-yr-old girl:
proximal tibia pain
9-yr-old boy:
heel pain
5-yr-old boy:
knee painPeripheral Arthritis Characteristics
____________________________________________________
Asymmetric, lower limb oligoarthritis
common
• Hips
• Knees
• Ankles
Uncommon
• Mid foot
(characteristic)Examining for mid-foot disease
Evaluating axial disease
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 6Evaluating Back pain
Pre
Adolescents
Puberty
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.
15 yr-old mid-thoracic
sclerosis L3 vertebra
back painLow 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 ….Clinical manoevres
Reduced back range
Schober’s measurement
Flattening
FABER test
Mennel’s sign
Caution diagnosing sacroiliitis without arthritis imagingnormal
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 activityTreatment
… a cascade of drugs
• NSAIDS • peripheral arthritis
• enthesitis
– naproxen, ibuprofen, et al • Sacroiliitis
• corticosteroid May need to be
targeted differently
– Intra-articular
– Low dose oral
• DMARDs
– methotrexate, sulphasulazine,
• Biologic agents
– Anti-TNF…outcomes
Inactive disease within 2 years
Remission within 5 years
Flare one year after inactive
Oligoarthritis
Polyarthritis RF-Neg
Enthesitis-related
Psoriatic
Systemic
Polyarthritis RF-Pos
Undifferentiated
.
0 20 40 60 80 100
Guzman J, Oen K, et al Ann Rheum Dis 2016;75:1092-8.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
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