Enthesitis related arthritis & Psoriatic...
Transcript of Enthesitis related arthritis & Psoriatic...
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
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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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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…….. 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
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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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 +
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
When to do MRI?
• To diagnose sacroiliitis (radiographs normal)
• To exclude sacroiliitis
• To track disease activity
… 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
Flare one year after inactive
Guzman J, Oen K, et al Ann Rheum Dis 2016;75:1092-8.
0 20 40 60 80 100
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Undifferentiated
Polyarthritis RF-Pos
Systemic
Psoriatic
Enthesitis-related
Polyarthritis RF-Neg
Oligoarthritis
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