Juvenile Idiopathic Arthritis Flashcards

1
Q

Whats makes an arthritis JIA? [2]

A

Systemic inflammatory disorders

Has to be <16yrs old

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2
Q

Diagnostic criteria for JIA [3 components]

A
Onset <16yrs
Duration >6wks
Symptoms:
Joint Swelling OR 2 of:
- Tenderness
- Painful/limited ROM
- Warmth
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3
Q

There are 3 major subtypes of JIA which are only determinable after 6 months)?

A
Pauciarticular (55%) - <5 joints
Polyarticular (25%) - >4 joints
Systemic Onset (20%) - Still's Disease
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4
Q

There are 3 subtypes of Pauciarticular JIA (Type 1, 2, 3). How does Type 1 present?
Gender, age, sites
2 features

A

Female dominant
Peaks at 1-3yrs.
Limp due to LL arthritis
20% also get chronic uveitis

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5
Q

How can we test for Type 1 Pauciarticular JIA? [1]

A

40-75% are ANA +ve

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6
Q

How does Type 2 Pauciarticular JIA present?
Gender and demographic
2 features
NB Watch out for hip or back involvement, while rare it can progress rapidly to AS (worth testing for HLA-B27)

A

Pre-school boys >8 yo

Limp due to LL arthritis

10-20% get Acute Iridocyclitis

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7
Q

How Does Type 3 Pauciarticular JIA present?
Gender, age
4 features

A

Female Predominant
Any age during childhood

Asymmetric UL and LL arthritis
Dactylitis
\+/- Nail pitting
Psoriasis
40% have FH/o Psoriasis, develops later in life
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8
Q

What are the subtypes of Polyarticular JIA? [2]

NB in both iridocyclitis is rare

A

RF -ve (15%)

RF +ve (10%

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9
Q
How does RF -ve Polyarticular JIA present?
Gender 
Main presenting complaint
Constitutional manifestations [2]
Signs [3]
A

Female predominant, any age

•Symmetric large and small joint effusions
•Constitutional manifestations
	§ Low grade fever
	§ Malaise
• Hepato-splenomegaly
• Mild anemia
• Growth abnormalities
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10
Q
How does RF +ve Polyarticular JIA present?
Gender, age
Main presenting complaint +
Systemic sx [4]
Complications [5]
X-ray signs
A

Female mostly, 12-16yrs

5 or more joints + systemic features:

  • Anaemia
  • Low grade fever
  • Malaise
  • Rheumatoid Nodules

Can be complicated by:

  • Secondary Sjogren’s
  • Atlanto-Axial subluxation
  • Pulm. Fibrosis
  • Felty’s disease
  • Cubital tunnel syndrome

• X-ray erosion seen early

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11
Q

Whats special about how Still’s Disease (Systemic onset JIA) presents?

A

Systemic features start first with arthritis not occuring till 3-12 months in.

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12
Q

Who gets Still’s Disease?

A

1.5 F:M, mostly 4-6yrs old

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13
Q

Still’s disease fever [2]

A

It rises daily in the afternoon/evening for at least 2 weeks.
Look for a child toxic with fever that goes away in the morning

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14
Q

50-75% of Still’s Patients get Abdominal features such as… [3]
2 symptoms and 1 sign

A

Hepatosplenomegaly
Abdo Pain
~Raised Transaminases

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15
Q

Lymphadenopathy problems in 50-75% of still’s patients:

2 features

A

Non-tender and generalized

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16
Q

Describe Still’s rash (90%) [1]

Name the phenomenon associated with this rash

A

Evanescent Salmon Red Eruption on their trunk/thighs along with the fever.
It can be brought on by scratching (aka Koebner’s Phenomenon)

17
Q

Pulmonary sx in Still’s patients are rare [2]

A

Pleural effusion

Pulm. Fibrosis

18
Q

Inflammation of Serous membranes can come with Still’s Disease, what type is most common?
Give 2 rare complications assoc with inflammation of serous membranes

A

36% of patient’s get Polyserositis including pericarditis

Rarely they can get tamponade and myocarditis

19
Q

How do the actual arthritis symptoms appear in Still’s Disease? (Name 6 sites)

A

Wrist, ankle, knee, C-spine, hips, TMJ

20
Q

Many types of JIA can present with Eye symptoms such as uveitis, these are often asymptomatic but can lead to blindness, how do we respond?

A

Every JIA patient gets regular Ophthalmology Screenings (PArticularly those with Pauciarticular JIA)

21
Q

1st Line treatment of JIA? [3]
Treatment for pauciartuclar
Treatment for polyarticular

A

Simple analgesia
NSAIDs
Intra-articular steroids for single joint involvement
Systemic steroids for polyarticular

22
Q

What if NSAIDs and local steroids fail? [4] options

A

Methotrexate
Anti-TNF - infliximab
IL-1R antagonist
IL-6 antagonist tocilizumab

23
Q

How do we treat Refractory Systemic Arthritis?

A

IL-1 Receptor Antagonist - Anakinra

IL-6 Antagonist (Tocilizumab)

24
Q

When would we risk using systemic steroids? [3]

- Serious disease complications [5]

A

Systemic JIA to control pain and fever

Bridge between DMARDs

Surgery

Serious disease complications in JIA e.g. pericardial effusion, tamponade, vasculitis, severe anaemia or severe eye disease

25
What are the risks of systemic steroids?
Osteoporosis Infection Growth abnormality
26
Major difference between pauciarticular and polyarticular JIA in presentation
Rare constitutional manifestations in pauci vs | poly
27
Surgical management of JIA involves [2]
• Surgical mx ○ Synovectomy Reconstructive/joint replacement surgery
28
``` Examinations [2] Investigations No specific tests for JIA But what lab investigations can you consider [4] What imaging techniques can you do [2] ```
``` pGALS as screening measure and pREMS ANA HLA-B27 Inflammatory markers RF X-ray, MRI ```
29
Management approach Do's [2] Dont's [3]
``` Do - intensify treatment, remain active Don't: - Advise rest - Splints, crutches, wheelchairs - Accept less than normal function ```