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Rheumatology Flashcards

1
Q

Define JIA

A

Juvenile Idiopathic Arthritis: unknown etiology, begins before age 16, persists for >6 weeks

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2
Q

what are the types of JIA?

A

polyarthritis, RF negative, polyarthritis, RF positive, oligoarthritis - persistent/extended, psoriatic arthritis, undifferentiated.

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3
Q

what is the ILAR criteria for systemic arthritis?

A

Definition: Arthritis in one or more joints with or preceded
by fever of at least 2 weeks’ duration that is documented to be daily (“quotidian”) for at least 3 days, and accompanied by one or more of the following:
1.Evanescent (non fixed) erythematous rash
2. Generalized lymph node enlargement
3. Hepatomegaly and/or splenomegaly
4.Serositis

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4
Q

ddx for systemic onset JIA?

A

other autoinflammatory disease
- acute rheumatic fever, SLW, mixed connective tissue disorder, kawasaki, periodic fever syndromes, juvenile dermatomyositis, vasculitis, scleroderma, malignancy.
Hematologic disorders – Sickle CellDisease
– Haemophilia
– MAS
• Miscellaneous
– Inflammatoryboweldisease
– Chronicrecurrentmultifocalosteomyelitis/NBO – Sarcoidosis
Arthritis alone :
– ConnectiveTissueDisorders(eg,Ehlers-Danlossyndrome,Marfan syndrome)
– ConversionDisorder
– Hypermobilitysyndrome
– Villonodularsynovitis

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5
Q

what are the ILAR criteria for polyarthritis (RF negative)

A

Definition: Arthritis affecting 5 or more joints during the first 6
months of disease; a test for RF is negative.

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6
Q

what are the ILAR criteria for Polyarthritis (Rheumatoid Factor Positive)

A

• Definition: Arthritis affecting 5 or more joints during the
• first 6 months of disease; 2 or more tests for RF at least 3 months apart during the first 6 months of disease are positive.
more often have errosive, symmetric in small joints of hands/feet and rheumatoid nodules.

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7
Q

characteristics of RF neg polyarthritis?

A

Children with RF negative polyarthritis are frequently younger and have a better prognosis than those with RF positive disease.
ANA is positive in 25% of patients.
Affected joints are frequently symmetrical, affecting large and small joints alike.
Less than 50% of patients go into remission, and long- term sequelae are frequent, especially with hip and
shoulder involvement.
F>M

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8
Q

ddx for polyarthritis?

A 
• JIA
– Polyarth ritis (RF +ve/-ve), 
– Enthesitis Related Arthritis, 
– Psoriatic arthritis
• Reactivearthritis
-rubella,parvovirus, HepB 
• SLE/ Other autoinflammatory diseases
• Arthritis associated with IBD
• Malignancy –leukaemia, neuroblastoma
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9
Q

what are the charactersistics of rf positive polyarthritis?

A

Patients with RF positive polyarthritis share many characteristics with adults with rheumatoid arthritis.
Anti CCP more sensitive and specific
All patients, by definition, are RF positive. This affects mostly adolescent girls. The clinical symptoms are similar to the adult disease with symmetrical polyarthritis especially involving the PIP joints and MCP joints.
Children may develop rheumatoid nodules and similar complications to adult disease, including joint erosions and Felty syndrome
(neutropaenia and splenomegaly).

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10
Q

What is the ILAR criteria of oligoarthritis?

A

The most common subtype of JIA
Definition:
Arthritis affecting one to 4 joints during
the first
• 6 months of disease. Two subcategories are recognized:
– 1. Persistent oligoarthritis: Affecting not more than 4 joints throughout the disease course
– 2. Extended oligoarthritis: Affecting a total of more than 4 joints after the first 6 months of disease

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11
Q

Ddx of acute monoarthritis?

A

• Trauma
• Septic arthritis /osteomyelitis
• Malignancy – leukaemia, lymphoma, neuroblastoma, bone tumour
• Early JIA
• Acute rheumatic fever
• Reactive arthritis – post viral/ post enteric infection/ post strep
• Haemophilia
• Noninflammatory disorders
– Avascular necrosis syndromes/Osteochondroses
– SCFE /Transient Synovitis Hip
– Patellofemoral dysfunction

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12
Q

Ddx of chronic monarthritis?

A 
• Chronic (>6 weeks) :
– JIA – oligoarthritis, ERA, psoriatic arthritis 
– Chronic infections 
– TB, fungal, brucellosis 
– Malignacy
– Villonodular synovitis
– Sarcoidosis
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13
Q

What are the 5 systemic diseases associated with uveitis?

A
  1. JIA - anterior > posterior
  2. Ensethetis Related Arthritis - anterior
  3. Infantile sarcoidosis - posterior and anterior
  4. Bechet disease - posterior
  5. Kawasaki disease - anterior
  6. tubulointerstitial athrtitis and uveitis - anterior
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14
Q

what is the definition of extended oligoarthritis ?

A

Extended oligoarthritis: Affecting a total of more than 4 joints after the first 6 months of disease

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15
Q

What are the features of enthesitis related arthritis?

A

Children with ERA have inflammation predominantly affecting joints and entheses of the lower extremities, which may also eventually affect the sacroiliac (SI) joints and spine. It is characterized by a strong association with human leukocyte antigen (HLA)-B27 and absence of rheumatoid factor (RF). In many instances, the disease evolves to closely resemble ankylosing spondylitis (AS), (cousin of) although SI joint and spine involvement at disease onset is uncommon in childhood or adolescence.
sacroilitis; rare to have spinal involvement.
Male>6 years of age
tendon insertion into the calcaneus

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16
Q

What is the ILAR classification of enthesitis related arthritis?

A

Arthritis and enthesitis
or
Arthritis or enthesitis with at >2 of the following:
• Sacroiliac joint tenderness and/or inflammatory lumbosacral pain
• Presence of HLA-B27
• Onset of arthritis in a boy after 6 years of age
• Family history of HLA-B27–associated disease (AS, ERA, sacroiliitis with inflammatory bowel disease, reactive arthritis, acute anterior uveitis) in a first-degree relative
• Acute symptomatic anterior uveitis
EXCLUSION CRITERIA
Psoriasis in patient or first-degree relative
• Presence of IgM RF on at least two occasions at least 3 months apart
• Systemic JIA in the patient
• Arthritis fulfilling two JIA categories

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17
Q

What is ANA positive oligoathritis most associated with?

A

ANA positive 60-80%
• Uveitis more common (20-30% if ANA positive)
• Uveitis can be asymptomatic

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18
Q

What other joints can be affected?

A

TMJ

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19
Q

What are the findings in hands?

A

swan neck deformities, on xray narrowing of joint space, advanced maturation of carpal bones.

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20
Q

psoriatic arthritis features?

A

Arthritis and psoriasis; or arthritis plus: – Dactylitis
– Nail pitting / onycholysis
– Psoriasis in 1st degree relative

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21
Q

Systemic JIA features?

A 
Fever for 2 weeks
rash
lymphadenopathy
arthritis
hepatosplenomegaly
serositis
quotidian fever (one or 2 spikes per day)
salmon pink confluent rash
Koebner phenomenon ( can write on skin)
Athritis can develop later, usually oligoarticular
pericardial effusions in ~10%, myocarditis less common
ANA positive 5-10% ; RF usually negative
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22
Q

DDx for Systemic JIA

A
  1. malignancy
  2. infection
  3. IBD
  4. other autoimmune (SLE KAWASAKI, periodic fever syndromes)
    MAS/Secondary HLH emergency
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23
Q

What is the risk of uveitis accross the majority of JIA?

A

uveitis

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24
Q

What are the characteristics of JIA associated Uveitis?

A
  • Anterior chamber – inflammatory cells / protein
  • Posterior synechiae
  • Band keratopathy
  • Cataracts
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25
What guides the screening for opthalmology?
depending, on oligoarticular, Polyarticular or Enthesitis Related Arthritis ANA positive or negative, and age of onset, as well as duration of disease: 3,6 or 12 monthly.
26
How do we treat JIA?
Oligo/Poly/ERA within 3 months we start a DMARD 1. (methotrexate). 2.Etanercept/Adalimumab/Tocilizumab 3. Abatacept 5. infliximab, rituximab, tofacitinib and finally Golimumab
27
How do we treat systemic JIA?
Systemic features + arthritis (failing NSAID/Steroid/MTX) Tocilizumab/anakinra Arthritis without systemic features (failing NSAID/Steroid/MTX) Anti TNF/anakinra, failing that : infliximab or rituximab
28
What is the routine JIA treatment?
``` Naproxen (liquid format) Piroxicam comes in dispersible tablets Corticosteroids - PO, IV methylpred, Intra-articular corticosteroid injection (Triamcinolone Hexacetonide (Aristospan) Triamcinolone Acetonide (Kenacort)) ```
29
What is SLE?
Autoimmune multisystem chronic relapsing / remitting disease - mimic of other diseases - SLE is a ddx in almost all patients, and manifests differently in every patients.
30
Childhood SLE?
20% presents in childhood, usually more severe (sicker at disease onset) - active disease at presentation and over time - active renal disease intensive treatment - higher long term risk of organ damage
31
What are the ACR diagnostic criteria of SLE?
``` 4 or more of: • Malar rash • Discoid rash • Serositis (pleuritis, pericarditis) • Oral / nasal mucocutaneous ulcers • Arthritis (non-erosive) • Photosensitivity • Blood disorder (cytopaenia, Coombs + haemolytic anaemia) • Renal nephritis • ANA positivity • Immunoserology positive (anti-DNA, Sm, antiphospholipid) • Neurologic encephalopathy (headaches, seizures, psychosis) “MD SOAP BRAIN” ```
32
What are the SLICC criteria for diagnosis of SLE?
``` 4 or more of (at least 1 clinical AND 1 immunologic; or Lupus nephritis as sole clinical + ANA/anti-dsDNA): Clinical: • Acute/chronic cutaneous lupus • Oral ulcers • Alopecia (non-scarring) • Synovitis/arthritis • Serositis • Renal • Neurologic • Haemolytic anaemia • Leukopaenia • Thrombocytopaenia ``` ``` Immunologic: • ANA positive • Anti-dsDNA • Anti-Sm • Antiphospholipid antibody + • Complement low • Direct Coombs + (in absence of haemolytic anaemia) ```
33
What are some of the SLE manifestations?
``` • Constitutional symptoms – Fever, weight loss, lethargy, anorexia • Rashes – maculopapular rash, vasculitic rash, livedo reticularis • Polyarthralgia • Raynaud phenomenon • Alopecia • Hepato / splenomegaly • GI vasculopathy (abdominal pain) ```
34
What are the most serious complications of SLE?
Lupus Neprhitis, Neuropsychiatric SLE, serositis and MAS
35
What are the classification and clinical fx of lupus nephritis?
Approx 50% of cSLE will have renal involvement – 80-90% within 1st year of diagnosis low threshold for renal biopsy - unexplained or worsening proteinuria (nephritic or nephrotic flares)
36
What are the lab features of SLE and what are they indicative of?
1. Raised ESR (crp normal) 2. Low C3/C4 (esp. with lupus nephritis) 3. Anti dsDNA and Anti-Smith (most specific antibodies) 4. Anti-RO, Anti La, RNP may be positive
37
Anti-dsDNA increase and C3/4 decrease is MOST accurate in reflecting what?
disease activity
38
What are the most disease specific antibodies for SLE?
Anti dsDNA and Anti-Smith
39
Initial Treatments for SLE?
aim: use minimal therapy for clinical and lab quiesence Initial: NSAIDs – Arthritis • Corticosteroids – For moderate/severe disease – Tapering dependent on organ involvement – Pulse therapy for severe LN, haematologic crises, CNS disease • Methotrexate (medium or mod) – Steroid sparing, arthritis, skin
40
Major treatment for SLE
Hydroxychloroquine: generalised immunosuppresent and anti inflam effect Reduces overall morbidity and mortality • Delays onset of new organ disease • Decreases frequency/severity of flares • May improve lipid profile and thrombosis risk Adverse effects • Common: nausea, GI symptoms, headache, rash • Retinotoxicity rare in children • Retinotoxicity risk factors: – Antimalarial use > 5-6 years – Dosage higher than recommended (HCQ >6.5mg/kg) – Obesity – Previous eye disease – Age >65 years
41
Autoimmune ITP?
Can sometimes be the first manifestations of SLE | - if ANA positive, ~10% risk of developing SLE prospectively
42
What is Antiphospholipid syndrome?
Autoimmune hypercoagulable disorder - vascular thrombosis or previous recurrent miscarriages, fetal loss antiphospholipid antibodies positive - anti cardiolipin - anti- B2 glycoprotein I - Lupus Anticoagulant - paradoxical aPTT prolongation
43
What are the categories of antiphospholipid syndrome?>
Primary (idiopathic) | Secondary (SLE, other autoimmune, infections, drugs, malignancy)
44
Neonatal Lupus Erythematosis (NLE)
fetal and neonatal disease involving the transplacental passage of maternal anti-Ro and or Anti-La antibodies - mothers hx of SLE, Gjogren, other autoimmune but can also be healthy - most childen born healthy and unaffected Most important complication is cardiac
45
What are the cardiac manifestations of SLE?
1. congenital atrioventricular block - 1-5% - increases to 6-25% for those with previously affected child - myocardial dysfunction - endocardial fibroelastosis
46
What are the dermatologic manifestations of NLE?
``` 15-25% of children with NLE - photosensitive maculopapular with a scale frequently around eyes but also other areas of body - anti-ro antibodies found in >95% ```
47
treatment for NLE?
1. cardiology - pacemaker for complete heart block + regular screening 2. often NO treatment as problems usually transient - reassurance, careful observation for 1st year - corticosteroid cream for severe NLE rash
48
What is Juvenile scleroderma?
autoimmune disease affecting skin/subcutaneous tissue (and deeper) issues rare: incidence jLS - 0.3-2.7/100 000 very rare. subtypes: circumscribed morphea, linear scleroderma, generalised morphea, pansclerotic morphea can lead to systemic sclerosis
49
what are the skin manifestations of juvelnile scleroderma?
hypo or hyperpigmented, skin is usually hard or indurated (thickened), deeper and cause muscle involvement. this can be seen in MRI as well, subcutaneous is thinner, skin is thicker.
50
Treatment of Scleroderma?
corticosteroids, methotrexate - sometimes cyclosporin Topical agents: corticosteroid, tacrolimus, imiquimod, Vit D analogues phototherapy (UVA) - ?long term cancer risk, physiotherapy, OT..
51
What is Anti-CCP? What is it useful for?
sometimes positive in Polyarthritis JIA RF _ (mimics RA) | We don't really do it
52
what is RF?
IgM autoantibody, reacts to Fc of IgG antibodies less commonly positive in JIA (5-10%) not useful in a diagnosis of JIA NOT useful in classification/prognosis of polyarticular JIA
53
HLA-B27?What is it useful for?
``` MCH HLA class 1 General population are ~8% positive; only 5% of the 8% actually have associated disease -ERA and PsA - IBD - Anterior uveitis - Reactive arthrtitis *not necessarily a diagnostic marker ```
54
ANA? What is it useful for?
Immunoglobulins/antibodies binding to antigens of nucelus of human cella - testing methods: indirect IF; immunoassays (EIA or ELISA) - IF: two fold dilution; 1:160 weak positive, 1:1280 strong positive - EIA/ELISA: intensity of flurescence in IU/mL (>7IU/mL = positive) only consider testing if pre-test probablility of disease is high
55
ANA - is it sensitive or specific
``` Sensitive, but not specific SLE: 93% scleroderma: 85% MCTD (93%) poly/dermatomyositis (61%) Sjogrens drug induced lupus (100%) ```
56
What is ANA useful for?
• JIA = increased risk of uveitis • If negative, autoimmune diagnosis unlikely – High negative predictive value • The greater the positivity, the more likely they may have an autoimmune diagnosis - (but not specific) • ENA can help to subclassify: – anti-dsDNA, anti-Sm = SLE – anti-histone = Drug induced lupus
57
What diseases can cause raised ANA other than
Other autoimmune disease – e.g. autoimmune hepatitis, PBC, Graves’, Hashimoto’s • Acute infectious diseases • Chronic infectious diseases – TB, EBV, Hep C, HIV • Lymphoproliferative disease • Normal population – (~40% low; 5% moderate positive)
58
what is Anti-Sm, Anti-dsDNA associated with?
SLE
59
Anti-histone associated with?
Drug-induced lupus
60
Anti-U1RNP associated with?
MCTD
61
Anti-Ro/SSa, La/SSb
SLE (40%), NLE, Sjogren’s
62
Anti-Scl-70
Scleroderma
63
Anti-Jo/SRP/Mi-2
JDM
64
Anti-Mitochondrial, Smooth muscle, LKM
Autoimmune hepatitis
65
Anti-DFS70
Non-specific, can be protective of SLE
66
what are the characteristics of children with JIIM?
1. proximal muscle weakness 2. characteristic muscle biopsy changes 3. increased serum muscle enzyme levels 4. electromyography abnormalities 1) polyphasic, short, small motor-unit poten- tials; (2) fibrillation, positive sharp waves, insertional irritability; and (3) bizarre, high-frequency repetitive discharges 5.characteristic skin rash
67
what is the characteristic skin rash of dermatomyositis? and what does it look like?
1. Gottron papules (Fig. 1) are erythematous, raised, and scaly lesions, classically located over the metacarpophalangeal joints, the proximal and distal inter- phalangeal joints of the fingers, and less frequently the toes. They may also be seen over the elbows, knees, and medial malleoli. 2. heliptrope rash:It is a red or purple discoloration of the upper eyelid, often with diffuse swelling.
68
skin lesions seen in dermatomyositis in active disease?
``` Considered to indicate active diseasea Gottron papules/sign Heliotrope rash Malar/facial erythema Linear extensor erythema V-sign rash Shawl-sign rash Non–sun-exposed erythema Erythroderma Livedo reticularis Skin ulcers Mucous membrane lesions (including oral ulcers) Periungual capillary loop changes Mechanic’s hands Cuticular overgrowth Subcutaneous edema Panniculitis Alopecia ```
69
What are 6 core outcome variables in JIA?
1. active joints 2. Restricted joints 3. ESR 4. PGA (global assessment) 5. CHAQ (childhood health assessment questionaire) 6. Parental Visual Analogue Scale
70
Bloods for suspected JIA?
FBC/UEC/LFt/ESR/CRP ANA, RF, HLAb27 Strep titres, mycoplasma, EBV, CMV, adenovirus VZV (prior to MTX)
71
bloods for Suspected CT disease (SLE)
FBC/UEC/LFt/ESR/CRP/TFT ANA/AntidsDNA, ENA, C3/c4 ck, ast, ldh urinalysis, urine P:Cr ratio strep titres, mycoplasma, EBV, CMV, adenovirus lupus anticoagulant, anticardiolipin ab, coags, coags DCT Consider ACE, ANCA if vasculitic process suspected
72
bloods for suspected JDM
``` FBC/UEC/LFt/ESR/CRP/TFT CK, LDH, AST +/- myositus specific antibodies strep titres, mycoplasma, EBV, CMV, adenovirus ANA/AntidsDNA, ENA VZV (prior to MTX) ```
73
bloods for monitoring on MTX
FBC, UEC, LFT, ESR, CRP
74
bloods prior to starting biologic
VZV, IgG, Quant gold
75
bloods in suspected MAS
FBC/UEC/LFT/ESR/CRP | Ferretin/LDH/coags+fibrinogen/D-dimer, TGLs
76
what is the CMAS scale?
Childhood Myositis Assessment Score
77
what is Schober's test and how is it performed?
Assessment of Lumbar function. 1. Patient is standing, examiner marks the L5 spinous process by drawing a horizontal line across the patients back. 2. A second line is marked 10 cm above the first line. 3. Patient is then instructed to flex forward as if attempting to touch his/her toes, examiner remeasures distance between two lines with patient fully flexed. 4. The difference between the measurements in erect and flexion positions indicates the outcome of the lumbar flexion
78
What antibodies are associated with ILD in dermatomyositis?
Anti-ARS and anti-MDA5 antibodies are associated with ILD, while anti-Mi-2, SRP, and TIF1-γ/α antibodies are less so. Anti-MDA5 antibodies are associated with rapidly progressive ILD, while anti-ARS antibodies are not associated with rapidly progressive ILD but with a recurrent

Paeds FRACP (23 decks)

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