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FRACP > Rheumatology > Flashcards

Rheumatology Flashcards

1
Q

What three criteria are required to diagnose JIA

A

Under 18 years old
More than one joint with unexplained arthritis
Lasting more than 6 weeks

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2
Q

What are the 4 subtypes of JIA using the new classification

A

1) systemic onset
2) rheumatoid positive
3) ANA positive
4) enthesitis

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3
Q

What is the best marker for uveitis in JIA

A

ANA positivity

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4
Q

What is macrophage activation syndrome otherwise called

A

Secondary HLH

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5
Q

What is the most telling sign on bloods with MAS

How does it present

A

High ferritin

Fevers, lymphadenopathy, encephalopathy and hepatosplenomegaly

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6
Q

What is telling about the fever in systemic onset JIA (3 things)

A

Twice daily
High
Rash will come with the fever

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7
Q

What joints are usually involved in oligoarticular JIA

A

Large joints- hips and knees

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8
Q

How many joints need to be involved to make JIA polyarticular

A

5 or more

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9
Q

Are X-ray findings present in JIA

A

No- not until very late

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10
Q

How does methotrexate work
What are it’s 3 main side effects
What is a more rare side effect

A

Anti folate
GI upset, mouth ulcers and mood changes
Hepatitis

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11
Q 
What do the following DMARDs work on
Infliximab/adalimumab/entanercept 
Rituximab 
Anakinara
Tocilizumab
Dupulimab
Ecilizumab
Omalizumab

What is the potential Adverse effect if used too early

A 
TNF alpha
Anti CD20
IL1
IL6
Il4/5
Terminal complement pathway 
IgE

Early onset interstitial lung disease

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12
Q

Dermatomyositis
How does it usually present
What are the 2 rashes seen
What three investigations indicate muscle involvement

A

Non specific weakness and fevers
Heliotropic and gottrons papules
Raised CK or LFTs, positive EMG, positive biopsy

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13
Q

Dermatomyositis
Which antibodies are specific
Which antibodies are associated

A

Anti jo

Anti sm and Ssa

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14
Q

Dermatomyositis

What are two possible complications

A

Calcifications

Interstitial lung disease

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15
Q

What mnemonic is used for the features of SLE

A

S-serositis
O- oral ulcers
A- arthritis
P- photosensitivity

B-blood 
R- renal 
A-Ana 
I- immunology 
N- nephritis

M- malar
D- discoid

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16
Q

What are early signs of lupus nephritis
How should it be investigated
How is it treated

A

Proteinuria and haematuria
Biopsy
Steroids and immunosuppression. Ace inhibitors and renal transplant

17
Q

Lupus
What are the two most specific antibodies
Which antibody may be seen In Drug induced cases

A

Anti sm and dsDNA

Anti Histone

18
Q

What are three ways antiphsopholipid syndrome can occur

A

Recurrent miscarriages
Clots
Valvular heart disease

19
Q

What are the 2 main ways neonatal lupus presents
Does mum have to have symptoms? What antibodies is mum likely to have
What other disease could mum have

A

Rash from 6-8 weeks
Congenital heart block
Anti ro and la
Sjogrens

20
Q

What are 4 markers of SLE disease activity

A

Anti DS DNA levels
Low complement levels
Raised ESR/CRP
Cytopenias

21
Q

What is a serious side effect of hydroxychloroquinine

A

Retinal deposition causing blindness

22
Q

What may be seen after blood tests in bechets

A

Pathergy (pustular reaction)

23
Q

What is the most concerning complication of bechets

A

Pulmonary aneurysm

24
Q

Scleroderma
What is the rash otherwise called
What is a lesion on the forehead called and why does it need more investigation
What may severe systemic scleroderma be associated with (2 things)

A

Morphoea
Coup de sabre- can be associated with seizures

Pulmonary hypertension and Raynauds

25
Q 
Familial Mediterranean fever syn 
What is associated with the fever 
What is the inheritance 
What protein is abnormal 
How is it treated 
What is the risk
A 
Abdominal pain and joint pain 
AD
Pyrin 
Colchicine
Amyloidosis
26
Q 
Hyper igD syndrome 
What brings on the fever 
What other symptoms are associated 
What is seen in the urine 
Does it cause amyloidosis

Of there is a complete defect what metabolic syndrome is it?

A

Excitement eg holidays or birthdays
Lymphadenopathy, diarrhoea and vomiting
Mevalonic acid
Rarely

Mevalonic aciduria

27
Q

TRAPs
What is associated with the fever
Can it cause amyloidosis

A

Severe abdominal pain and joint pain

Yes!

28
Q

PFAPA
what is associated with the fever
What 2 treatments can help
Does it cause amyloidosis

What is another periodic fever syndrome with urticaria and no amyloidosis

A

Pharyngitis and lymphadenitis
Steroids and tonsillectomy
No!
NOMID

29
Q

Which vessels are involved in
Small vessel vasculitis
Medium vessel
Large vessel

Give examples of each

A

Small- arteries to arterioles
Granulomatosis with polyangitis, HSP, anti GBM disease

Medium- main visceral arteries
Polyarteritis nodosa , Kawasaki

Large- aorta and main branches
Giant cell arteritis, takayasau‘s

30
Q 
Takayasau’s 
What is it otherwise called
How does it present 
Will ANCA be positive 
How should it be investigated 
What type of inflammation is it
A

Pulseless disease

Non specific symptoms then pain, bruits and differential bps. Htn and seizures or strokes

No!

CT or MRI Angiography

Granulomatous

31
Q

Polyarteritis nodosa
What type of inflammation is seen
Will ANCA be positive
Are the kidneys usually involved?

A

Necrotising

In 20%

No!

32
Q

Small vessel vasculitis
What is the new name for wegeners. What finding may be seen
What type of ANCA will be positive. What else makes it likely

What does church Strauss have on bloods

A

Granulomatosis with polyangitis
Saddle nose deformity
CANCA
anti pr3

Eosinophilia

33
Q

What can develop if a child has a bullous rash after starting naproxen

A

Pseudoporphyria

34
Q

What antibody is highly specific for granulomatosis with polyangitis (wegners)

A

CANCA and PR3

35
Q

Which antibody is highly specific for eosinophilic granulomatosis with polyangitis (churg Strauss)

A

PANCA

FRACP (23 decks)

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