Rheumatology

Question 1

What is juvenile idiopathic arthritis ?

Answer 1

A condition affecting children and adolescents where autoimmune inflammation occurs in the joints.
Arthritis lasting more than 6 weeks in patients under 16.

Question 2

What are the key features of inflammatory arthritis ?

Answer 2

Joint pain
Swelling
Stiffness

Question 3

What is systemic juvenile idiopathic arthritis ?

Answer 3

Also known as Still’s disease
Systemic illness that occurs in boys and girls

Question 4

What are some typical features of systemic juvenile idiopathic arthritis ?

Answer 4

Subtle salmon pink rash
High swinging fevers
Enlarged lymph nodes
Weight loss
Joint inflammation and pain
Splenomegaly
Muscle pain
Pleuritic and pericarditis

Question 5

What are some abnormalities seen in blood tests in systemic juvenile idiopathic arthritis ?

Answer 5

Antinuclear antibodies and rheumatoid factors are negative
Raised inflammatory markers with raised CRP, ESR, platelets and serum ferritin.

Question 6

What is the key complication of systemic juvenile idiopathic arthritis ?

Answer 6

Macrophage activation syndrome - severe activation of the immune system with a massive inflammatory response.

Question 7

How many joints are involved in polyarticular juvenile idiopathic arthritis ?

Answer 7

5 joints or more

Question 8

What is a classic feature associated with oligoarticular JIA ?

Answer 8

Anterior uveitis

Question 9

What are some signs that can be seen in juvenile psoriatic arthritis ?

Answer 9

Plaques of psoriasis
Pitting of the nails
Oncholysis
Dactylitis
Enthesitis

Question 10

What is the management of juvenile idiopathic arthritis ?

Answer 10

NSAIDs
Steroids
DMARDs - methotrexate
Biologic therapy

Question 11

What is Ehler’s-Danlos syndrome ?

Answer 11

A group of genetic conditions involving defects in collagen causing hypermobility in the joints and abnormalities in the connective tissue of the skin, bones, blood vessels and organs.

Question 12

What is a differential for Ehler’s danlos syndrome ?

Answer 12

Marfans syndrome

Question 13

How does Ehler’s-Danlos syndrome present ?

Answer 13

Joint pain and hypermobility
Joint dislocations
Soft and stretchy skin
Stretch marks
Easy bruising
Poor wound healing
Bleeding
Chronic pain and fatigue
Headaches

Question 14

What is the Beighton score ?

Answer 14

It is used to assess for hypermobility and support the diagnosis of Ehler’s danlos syndrome.

Question 15

what is the management of Ehler’s danlos syndrome ?

Answer 15

No cure
Follow ups
Physiotherapy
Occupational therapy
Moderating activity

Question 16

What can be the complication of Ehler’s danlos syndrome ?

Answer 16

Premature osteoarthritis

Question 17

What is Henoch-Schonlein purpura ?

Answer 17

An IgA vasculitis that presents with a purpuric rash affecting the lower limbs and buttocks in children. Inflammation occurs in the affected organs due to IgA deposits in the blood vessels.

Question 18

What are the 4 classic features of Henoch-Schonlein purpura ?

Answer 18

Purpura
Joint pain
Abdominal pain
Renal involvement

Question 19

How does a rash form in Henoch-schonlein purpura ?

Answer 19

The rash is caused by inflammation and leaking of blood from small blood vessels under the skin forming purpura.

Question 20

In severe cases how can Henoch-schonlein purpura cause GI conditions ?

Answer 20

GI haemorrhage
Intussusception
Bowel infarction

Question 21

How is a diagnosis of henoch schonlein purpura ?

Answer 21

FBC and blood film
Renal profile
Serum albumin
CRP
Urine dipstick
Blood cultures
Blood pressure

Question 22

What is the management of henoch-schonlein purpura ?

Answer 22

Supportive
Steroids is debatable

Question 23

How is henoch-schonlein purpura monitored ?

Answer 23

Urine dipstick
Blood pressure

Question 24

What is Kawasaki disease ?

Answer 24

Mucocutaneous lymph node syndrome.
It is a systemic, medium-sized vessel vasculitis.

Question 25

What is a key complication of Kawasaki disease ?

Answer 25

Coronary artery aneurysm

Question 26

What are some clinical features of Kawasaki disease ?

Answer 26

Persistent high fever Children look unhappy and unwell Widespread erythematous maculopapular rash Desquamation - skin peeling Strawberry tongue Cracked lips Cervical lymphadenopathy Bilateral conjunctivitis

Question 27

What are some investigations when suspecting Kawasaki’s disease ?

Answer 27

FBC - anaemia, Leucocytosis and thrombocytosis LFT Inflammatory markers - ESR Urinalysis -WBC Echocardiogram

Question 28

What is the disease course of Kawasaki disease ?

Answer 28

Acute phase - most unwell with fever, rash and lymphadenopathy ( 1-2 weeks ) Subacute phase - acute symptoms settle, desquamation and Arthralgia occurs ( lasts 2-4 weeks ) Convalescent stage - remaining symptoms settle, blood tests slowly return to normal ( last 2-4 weeks )

Question 29

What is the management of Kawasaki disease ?

Answer 29

High dose aspirin to reduce the risk of thrombosis IV immunoglobulins to reduce risk of coronary artery aneurysm

Question 30

What is acute rheumatic fever ?

Answer 30

An autoimmune condition triggered by streptococcus bacteria. It is caused by antibodies created against the strep bacteria that also target tissues in the body.

Question 31

What is the pathophysiology of rheumatic fever ?

Answer 31

Caused by group A beta-haemolytic streptococcal ( typically strep pyogenes ) causing tonsillitis. Antibodies are produced which target the bacteria but also antigens on the cells of the persons body. This results in a type 2 hypersensitivity reaction

Question 32

How does rheumatic fever present ?

Answer 32

Usually follows a strep infection like tonsillitis Fever Joint pain Rash SOB Chorea Nodules

Question 33

What are the key skin findings with rheumatic fever ?

Answer 33

Subcutaneous nodules - firm over the extensor surfaces Erythema marginatum rash - pink rings affecting the torso and proximal limbs

Question 34

What investigations help support the diagnosis of rheumatic fever ?

Answer 34

Throat swab for bacterial culture ASO antibody titres Echocardiogram, ECG and chest X-ray

Question 35

What are ASO antibodies ?

Answer 35

Antibodies against streptococcus and indicate a recent strep infection and can be helpful in supporting a diagnosis of rheumatic fever

Question 36

What is the management of rheumatic fever ?

Answer 36

Tonsillitis should be treated with penicillin V NSAIDs Aspirin Prophylactic abx Monitoring and management of complications

Question 37

What are some complications of rheumatic fever ?

Answer 37

Recurrence of rheumatic fever Valvular heart disease - mitral stenosis Chronic heart failure