Rheumatology

Question 1

What three criteria are required to diagnose JIA

Answer 1

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

Question 2

What are the 4 subtypes of JIA using the new classification

Answer 2

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

Question 3

What is the best marker for uveitis in JIA

Answer 3

ANA positivity

Question 4

What is macrophage activation syndrome otherwise called

Answer 4

Secondary HLH

Question 5

What is the most telling sign on bloods with MAS

How does it present

Answer 5

High ferritin

Fevers, lymphadenopathy, encephalopathy and hepatosplenomegaly

Question 6

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

Answer 6

Twice daily
High
Rash will come with the fever

Question 7

What joints are usually involved in oligoarticular JIA

Answer 7

Large joints- hips and knees

Question 8

How many joints need to be involved to make JIA polyarticular

Answer 8

5 or more

Question 9

Are X-ray findings present in JIA

Answer 9

No- not until very late

Question 10

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

Answer 10

Anti folate
GI upset, mouth ulcers and mood changes
Hepatitis

Question 11

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

Answer 11

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

Early onset interstitial lung disease

Question 12

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

Answer 12

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

Question 13

Dermatomyositis
Which antibodies are specific
Which antibodies are associated

Answer 13

Anti jo

Anti sm and Ssa

Question 14

Dermatomyositis

What are two possible complications

Answer 14

Calcifications

Interstitial lung disease

Question 15

What mnemonic is used for the features of SLE

Answer 15

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

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

M- malar
D- discoid

Question 16

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

Answer 16

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

Question 17

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

Answer 17

Anti sm and dsDNA

Anti Histone

Question 18

What are three ways antiphsopholipid syndrome can occur

Answer 18

Recurrent miscarriages
Clots
Valvular heart disease

Question 19

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

Answer 19

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

Question 20

What are 4 markers of SLE disease activity

Answer 20

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

Question 21

What is a serious side effect of hydroxychloroquinine

Answer 21

Retinal deposition causing blindness

Question 22

What may be seen after blood tests in bechets

Answer 22

Pathergy (pustular reaction)

Question 23

What is the most concerning complication of bechets

Answer 23

Pulmonary aneurysm

Question 24

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)

Answer 24

Morphoea
Coup de sabre- can be associated with seizures

Pulmonary hypertension and Raynauds

Question 25

``` 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 ```

Answer 25

``` Abdominal pain and joint pain AD Pyrin Colchicine Amyloidosis ```

Question 26

``` 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?

Answer 26

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

Question 27

TRAPs What is associated with the fever Can it cause amyloidosis

Answer 27

Severe abdominal pain and joint pain | Yes!

Question 28

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

Answer 28

Pharyngitis and lymphadenitis Steroids and tonsillectomy No! NOMID

Question 29

Which vessels are involved in Small vessel vasculitis Medium vessel Large vessel Give examples of each

Answer 29

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

Question 30

``` 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 ```

Answer 30

Pulseless disease Non specific symptoms then pain, bruits and differential bps. Htn and seizures or strokes No! CT or MRI Angiography Granulomatous

Question 31

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

Answer 31

Necrotising In 20% No!

Question 32

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

Answer 32

Granulomatosis with polyangitis Saddle nose deformity CANCA anti pr3 Eosinophilia

Question 33

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

Answer 33

Pseudoporphyria

Question 34

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

Answer 34

CANCA and PR3

Question 35

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

Answer 35

PANCA