Juvenile idiopathic arthritis Flashcards

1
Q

What is JIA?

A

Group of inflammatory disorders affecting children below 16yrs

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2
Q

What is the most commonly diagnosed rheumatic disease in children?

A

JIA

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3
Q

What type of disease is JIA?

A

Autoimmune

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4
Q

What is the etiology and pathogenesis of JIA?

A

Genetic
Environmental
Immunologic
= multi-factorial

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5
Q

What is the criteria for diagnosis for JIA?

A

Age of onset = <16yrs
Duration of disease = >6wks
Presence of arthritis = joint swelling or 2 of the following: painful of limited joint motion, tenderness, warmth

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6
Q

After 6mo, what are the 3 major subtypes of JIA?

A

Pauciarticular
Polyarticular
Systemic onset

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7
Q

Why do the clinical subtypes of JIA matter?

A

Help suspect natural history
Complications
Prognosis
Strategy of treatment

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8
Q

What is the most common subtype of JIA?

A

Pauciarticular

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9
Q

What is the mean onset age for pauciarticular JIA?

A

2-4yrs

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10
Q

Which sex is more affected in type I pauciarticular JIA?

A

Female

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11
Q

How many joints does pauciarticular JIA affect?

A

4 or less joints

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12
Q

What is the presentation of type I pauciarticular JIA?

A

Limp rather than pain

No constitutional manifestations

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13
Q

What types of joint does type I pauciarticular JIA affect?

A

Knee, ankle, hand/elbow

Hip = rare

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14
Q

What joint is rarely affected in pauciarticular JIA?

A

Hip

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15
Q

Is type I pauciarticular JIA usually ANA-positive or negative?

A

+ve ANA

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16
Q

What is a common complication in 20% cases of type I pauciarticular JIA?

A

Chronic uveitis

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17
Q

How many types of pauciarticular JIA are there?

A

3

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18
Q

Which sex is more affected in type II pauciarticular JIA?

A

Males

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19
Q

What is the mean age for onset of type II pauciarticular JIA?

A

After 8/9yrs

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20
Q

What is the presentation for type II pauciarticular JIA?

A

Constitutional rare
Limp
Knee, ankle

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21
Q

What is a complication in 10-20% cases of type II pauciarticular JIA?

A

Acute iridocyclitis - vision blurred, eyes red

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22
Q

Which sex is more affected in type III pauciarticular JIA?

A

Females

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23
Q

What is the mean age for onset of type III pauciarticular JIA?

A

Any age

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24
Q

What is the presentation for type III pauciarticular JIA?

A

Asymmetric UL and LL arthritis

Dactyltitis

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25
What psoraisis like in type III pauciarticular JIA?
FHx psoriasis 40% +/- nail pitting May develop psoriasis later in life
26
How many joints does polyarticular JIA affect?
5 or more joints
27
Is polyarticular JIA RF-ve or +ve?
Either
28
What is the age of onset for RH-ve polyarticular JIA?
Any age, often early
29
Which sex is more affected in RH-ve polyarticular JIA?
Female
30
What is the presentation of RH-ve polyarticular JIA?
Constitutional manifestations: low grade fever, malaise Hepato-splenomegaly Mild anaemia Growth abnormalities
31
What joints does RH-ve polyartciular JIA affect?
Symmetric large and small joints: | knees, wrists, ankles, MCPs, PIPs, neck
32
What is the age of onset for RH+ve polyarticular JIA?
Late childhood, teens, 12-16yrs
33
Which sex is more affected in RH+ve polyarticular JIA?
Female
34
What is the presentation of RH+ve polyarticular JIA?
Constitutional manifestions: low grade fever, malaise, weight loss Anaemia Nodules
35
What can complicate RH+ve polyarticular JIA?
``` Sjogren's, Felty or vasculitis AR (aortic regurgitation) Pulmonary fibrosis AAS (acute aortic syndrome) CTS (carpal tunnel) ```
36
Which type of RH JIA is similar to RA but in a child?
RH+ve
37
What is the least common type of JIA?
Systemic onset
38
What is systemic onset JIA also known as?
Still's disease
39
What defines systemic onset JIA?
Extra-articular features
40
What is the age of onset for systemic onset JIA?
Throughout childhood (4-6yrs)
41
What are the extra-articular features of systemic onset JIA?
``` Fever Rash Lymph nodes Hepatosplenomegaly Abdominal pain Serositis Rare: pleural effusion/pulmonary fibrosis Arthritis ```
42
What are the features of fever in systemic onset JIA?
Rise to 39.5 daily for at least 2wks Late in afternoon evening/normal morning Child appear bad with fever/chills, then normal when fever gone
43
What are the features of rash in systemic onset JIA?
Evanescent salmon red eruption On trunk and thighs Accompanies fever Can be brought by scratching
44
What are the features of lymph nodes in systemic onset JIA?
Generalised lymphadenopathy | Non tender
45
What are rare pulmonary manifestations of systemic onset JIA?
Pleural effusion | Pulmonary fibrosis
46
What are the features of arthritis in systemic onset JIA?
Within 3-12mo of onset of fever | Wrists, knees, ankles, cervical spine, hips, TMJ
47
What type of JIA sees higher levels of uveitis?
Pauciarticular/oligoarticular JIA
48
Is uveitis related to ANA-ve or +ve?
ANA +ve
49
What is the most common non-infective cause of uveitis?
JIA
50
What are complications of uveitis in JIA?
Posterior synechiae Cataract Band keratopathy
51
What is important to do in children with JIA?
Screening for uveitis
52
What is the treatment for uveitis?
``` Steroids Mydriatic and cycloplegia agents Methotrexate MMF Ciclosporin Anti-TNF ```
53
What is the 1st line therapy for JIA?
Pain killers | NSAIDs
54
What is the 2nd line therapy for JIA if no response to NSAIDs/joint injections?
Methotrexate Anti-TNF IL therapy
55
What are systemic steroids used in in JIA?
Systemic JIA Serious disease complications Bridge between DMARDs Undergoing surgery
56
What are the risks of systemic steroids?
Osteoporosis Infections Growth abnormalities
57
What are other treatments for JIA apart from pharmacological?
Physio | OT
58
What type of JIA uses local steroids?
``` Oligo/pauci-articular JIA Eye disease (ANA+ve oligoarticular disease) ```
59
What are surgical options for JIA?
Synovectomy | Reconstructive/joint replacement surgery
60
What are growth failures in JIA?
``` Leg length discrepencies Shortening of fingers, hands, forearms, toes, feeet Micrognathia Short stature Delayed puberty ```