Paediatric Rheumatology

Question 1

Juvenile idiopathic arthritis pathophysiology

Answer 1

Autoimmune inflammation of the joints lasting more than 6 weeks with no identifiable cause

Question 2

Presentation of JIA

Answer 2

• Joint pain
• Swelling
• Stiffness

Question 3

Still’s disease

Answer 3

Systemic JIA:

• salmon pink rash
• high swinging fevers
• enlarged lymph nodes
• weight loss
• joint inflammation and pain
• splenomaegaly
• muscle pain
• pleuritis and pericarditis

Question 4

Investigations for JIA

Answer 4

Raised inflammatory markers - CRP, ESR, platelets and serum ferritin
Antinuclear antibodies and rheumatoid factor is negative

Question 5

Complications of still’s disease

Answer 5

Macrophage activation syndrome:

• Disseminated intravascular coagulation (DIC)
• Anaemia
• Thrombocytopenia
• Bleeding
• Non blanching rash

Question 6

Polyarticular JIA

Answer 6

Idiopathic arthritis in > 5 joints

• symmetrical
• minimal or mild systemic features
• can be RF postive (seropositive)

Question 7

Oligoarticular JIA features

Answer 7

Involves < 4 joints

Normally 1 joint (monoarthritis)
Normally larger joints

Associated with uveitis - refer to opthalmologist

Antinuclear antibodies often positive

Question 8

Who is more likely to get oligoarticular arthritis

Answer 8

Girls under 6 yo

Question 9

Enthesitis related arthritis

Answer 9

Includes:

• Ankylosing spondylitis
• psoriatic arthritis
• reactive arthritis
• IBD related arthritis

Inflammatory arthritis + enthesitis

Question 10

Who commonly gets enthesitis related arthritis

Answer 10

Boys over the age of 6 yo

Question 11

What HLA is associated with enthesitis related arthritis

Answer 11

HLA - B27

Question 12

What is an enthesis

Answer 12

Point at which a muscle tendon inserts into bone

Question 13

Presentation of juvenile psoriatic arthritis

Answer 13

Either symmetrical polyarthritis affecting small joints or asymmetrical arthritis affecting large joints in the lower limb

Question 14

Henoch Schonlein purpura pathophysiology

Answer 14

IgA vasculitis that presents with a purpuric rash affecting the lower limbs and buttocks in children due to IgA deposits in the blood vessels. It affects the skin, kidneys and GI tract

Question 15

Features of Henoch - Schonlein purpura

Answer 15

Purpura - non blanching, can cause skin necrosis
Joint pain - often knees and ankles
Abdominal pain
Renal involvement - IgA nephritis - haematuria

Question 16

What is Henoch - Schonlein purpura triggered by?

Answer 16

Often triggered by a URTI or gastroenteritis

Question 17

Complications of henoch - scholein purpura

Answer 17

GI haemorrhage
Intussuseption
Bowl infarction

Question 18

How is Henoch - Schonlein purpura diagnosed

Answer 18

Exclude other pathology:

• FBC and blood film - thrombocytopenia, leukemia, sepsis
• U+Es
• Serum albumin - nephrotic syndrome
• CRP - sepsis
• Blood culture - sepsis
• Urine dipstick - proteinuria
• Urine protein: creatinine ratio
• Blood pressure

Use diagnostic criteria - EULAR/PRINTO/PRES

Question 19

EULAR criteria

Answer 19

Palpable purpura + at least 1 of:

• diffuse abdo pain
• arthritis or arthralgia
• IgA deposits on histology
• proteinuria or haematuria

Question 20

Management of Henoch - Schonlein purpura

Answer 20

Supportive - analgesia, rest and hydration

Monitor with repeated urine dipstick and BP

Question 21

Who commonly gets Henoch - Schonlein purpura

Answer 21

Children under 10 yo

Question 22

Pathophysiology of Kawasaki disease

Answer 22

Systemic medium sized vessel vasculitis affecting young children, typically under 5 years old.

No clear trigger

Question 23

Whi is most likely to get Kawaski disease

Answer 23

Asian boys under 5 yo

Question 24

Presentation of Kawasaki disease

Answer 24

Persistent high fever for more than 5 days without a clear cause

• widespread erythematous maculopapular rash
• skin peeling (desquamation) on the palms and soles
• strawberry tongue
• cracked lips
• cervical lymphadenopathy
• bilateral conjunctivitis

Question 25

Investigations for Kawasaki disease

Answer 25

Bloods: - FBC - anaemia, thrombocytopenia, leucocytosis - LFTs - hypoalbuminaemia and elevated liver enzymes - CRP and ESR - Urinalysis - Echocardiogram - coronary artery anuerysms

Question 26

Kawasaki disease course

Answer 26

Acute phase - 1 - 2 weeks - most unwell with fever, rash and lymphadenopathy Subacute phase - 2 - 4 weeks acute synotoms settle, can get desquamation and arthralgia Convalescent stage - further 2 - 4 weeks - remaining symptoms settle, tests normalise and coronary artery aneurysms may regress

Question 27

Management of Kawasaki disease

Answer 27

High dose aspirin IV immunoglobulin Monitor and follow up with echocardiogram

Question 28

Complications of Kawasaki disease

Answer 28

Coronary artery aneurysm

Question 29

Rheumatic fever pathophysiology

Answer 29

Autoimmune condition triggered by antibodies specific to Group A beta haemolytic - streptococcus (typically Streptococcus pyogenes) bacteria affecting the joints, heart, skin and nervous system Type 2 hypersensitivity reaction

Question 30

Presentation of rheumatic fever

Answer 30

Symptoms occure 2 - 4 weeks after a streptococcal infection like tonsillitis Present with: - Fever - Joint pain - hot swollen painful joints - Rash - Shortness of breath - pericarditis, myocarditis and endocarditis - may get murmur - Chorea - involuntary muscle movements - Nodules

Question 31

Cardiac signs due to rheumatic fever

Answer 31

Tachycardia or bradycardia Murmurs Pericardial rub Heart failure - crackles in chest

Question 32

What skin signs are seen in rheumatic fever

Answer 32

Subcutaneous nodules - painless, firm nodules on extensor surfaces Erythema marginatum rash - pink rings of varies size on torso and proximal limbs

Question 33

Chorea

Answer 33

Irregular, rapid and uncontrolled movements of limbs - sydenham chorea also known as St Vitus dance

Question 34

Assessment of rheumatic fever

Answer 34

``` Throat swab - bacterial culture ASO - antistreptoccal antibody titres Echocardiogram ECG Chest Xray ```

Question 35

Which criteria is used to daignose rheumatic fever

Answer 35

Jones criteria

Question 36

Jones criteria

Answer 36

2 major or 1 major + 2 minor Major: - Joint involvement - Organ inflammation - carditis - Nodules - Erythema marginatum - Sydenham chorea Minor: - Fever - ECG changes - prolonged PR interval with carditis - Arthralgia - Raised inflammatory markers

Question 37

Management of rheumatic fever

Answer 37

Treat streptococcal infection with phenoxymethylpencillin for 10 days Refer for specialist management - NSAIDS - joint pain - Aspirin if > 16 yo and steroids for carditis

Question 38

Complications of rheumatic fever

Answer 38

Chronic heart failure Mitral stenosis Recurrence