Rheumatology

Question 1

What is Kawasaki disease?

Answer 1

Systemic medium vessel vasculitis

Question 2

Who is Kawasaki disease seen in?

Answer 2

<5s

Males>females

Question 3

What is the presentation of Kawasaki disease?

Answer 3

Persistent high fever for >5 days
Strawberry tongue
Widespread erythematous rash
Lymphadenopathy
Unhappy, unwell child
Skin peeling on palms and soles
Cracked lips
Conjunctivitis

Question 4

What investigations are done for Kawasaki disease?

Answer 4

FBC- anaemia, leukocytosis, thrombocytosis
LFTs elevated
ESR elevated
Echo for coronary complications

Question 5

What is the management of Kawasaki disease?

Answer 5

High dose aspirin- reduce risk of thrombosis
IVIg- reduce risk of coronary artery aneurysm
Close follow up

Question 6

What is the main complication of Kawasaki disease?

Answer 6

Coronary artery aneurysm

Question 7

What is Henoch Schonlein purpura HSP?

Answer 7

IgA vasculitis that affects skin, kidneys and GI tract

Question 8

What are the triggers of HSP?

Answer 8

URTI
Gastroenteritis
Drugs

Question 9

What is the presentation of HSP?

Answer 9

Purpura
Joint pain
Abdo pain
IgA nephritis- nephritic= haematuria

Question 10

What investigations are done for HSP?

Answer 10

FBC and blood film
Renal profile
Urine dipstick and protein:creatinine ratio
BP

Question 11

What is the management of HSP?

Answer 11

Supportive

Kidney involvement- consider corticosteroids

Question 12

What is the prognosis for HSP?

Answer 12

No kidney involvement= 4-6 weeks

1/3 have recurrence at 6 months

Question 13

What is Ehlers Danlos?

Answer 13

Genetic defect in collagen

Question 14

What are 3 common types of Ehlers Danlos?

Answer 14

Hypermobile
Classic
Vascular

Question 15

What is the presentation of hyper mobile Ehlers Danlos?

Answer 15

Most common and least severe
Joint hypermobility
Soft and stretchy skin

Question 16

What is the presentation of classic Ehlers Danlos?

Answer 16

Stretchy skin that is smooth to touch
Severe joit hypermobility and pain
Abnormal wound healing

Question 17

What is vascular Ehlers Danlos?

Answer 17

Most dangerous form as blood vessels are fragile

Question 18

What is the presentation of vascular Ehlers Danlos?

Answer 18

Translucent skin

Skin, organs and blood vessels prone to rupture

Question 19

How is Ehlers Danlos assessed?

Answer 19

Beighton score- max score of 9

7= hypermobile

Question 20

How is Ehlers Danlos managed?

Answer 20

Physio

OT

Question 21

What is a complication of Ehlers Danlos?

Answer 21

Postural orthostatic tachycardia syndrome

Question 22

What is postural orthostatic tachycardia syndrome POTS?

Answer 22

Autonomic dysfunction causing inappropriate tachycardia upon standing/sitting up

Question 23

What is the presentation of postural orthostatic tachycardia syndrome?

Answer 23

Presyncope, syncope
Headaches
Disorientation, nausea
Tremor

Question 24

What is rheumatic fever?

Answer 24

Autoimmune condition triggered by group A strep

Question 25

What is the presentation of rheumatic fever?

Answer 25

2-4 weeks post strep infection, usually tonsillitis Fever, rash Hot, swollen painful joints, migratory pericarditis, myocarditis, endocarditis Chorea- irregular, uncontrolled rapid movements of limbs

Question 26

What are the cardiac symptoms in rheumatic fever?

Answer 26

Tachy/bradycardia Murmurs Pericardial rub

Question 27

How is rheumatic fever investigated?

Answer 27

Throat swab for culture ASO antibody Echo, ECG, CXR to assess cardiac involvement

Question 28

What is the management of rheumatic fever?

Answer 28

``` Prevention- vaccination, treat bacterial tonsillitis NSAIDs Aspirin Steroids Prophyactic antibiotics ```

Question 29

How is bacterial tonsillitis treatment?

Answer 29

10 days phenoxymethylpenicillin

Question 30

What are the complications of rheumatic fever?

Answer 30

Valvular heart disease- esp mitral stenosis Recurrence Heart failure