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Y4 Paeds > Rheumatology > Flashcards

Rheumatology Flashcards

1
Q

What is juvenile idiopathic arthritis ?

A

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

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2
Q

What are the key features of inflammatory arthritis ?

A

Joint pain
Swelling
Stiffness

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3
Q

What is systemic juvenile idiopathic arthritis ?

A

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

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4
Q

What are some typical features of systemic juvenile idiopathic arthritis ?

A

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

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5
Q

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

A

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

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6
Q

What is the key complication of systemic juvenile idiopathic arthritis ?

A

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

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7
Q

How many joints are involved in polyarticular juvenile idiopathic arthritis ?

A

5 joints or more

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8
Q

What is a classic feature associated with oligoarticular JIA ?

A

Anterior uveitis

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9
Q

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

A

Plaques of psoriasis
Pitting of the nails
Oncholysis
Dactylitis
Enthesitis

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10
Q

What is the management of juvenile idiopathic arthritis ?

A

NSAIDs
Steroids
DMARDs - methotrexate
Biologic therapy

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11
Q

What is Ehler’s-Danlos syndrome ?

A

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.

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12
Q

What is a differential for Ehler’s danlos syndrome ?

A

Marfans syndrome

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13
Q

How does Ehler’s-Danlos syndrome present ?

A

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

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14
Q

What is the Beighton score ?

A

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

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15
Q

what is the management of Ehler’s danlos syndrome ?

A

No cure
Follow ups
Physiotherapy
Occupational therapy
Moderating activity

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16
Q

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

A

Premature osteoarthritis

17
Q

What is Henoch-Schonlein purpura ?

A

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.

18
Q

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

A

Purpura
Joint pain
Abdominal pain
Renal involvement

19
Q

How does a rash form in Henoch-schonlein purpura ?

A

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

20
Q

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

A

GI haemorrhage
Intussusception
Bowel infarction

21
Q

How is a diagnosis of henoch schonlein purpura ?

A

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

22
Q

What is the management of henoch-schonlein purpura ?

A

Supportive
Steroids is debatable

23
Q

How is henoch-schonlein purpura monitored ?

A

Urine dipstick
Blood pressure

24
Q

What is Kawasaki disease ?

A

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

25
Q

What is a key complication of Kawasaki disease ?

A

Coronary artery aneurysm

26
Q

What are some clinical features of Kawasaki disease ?

A

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

27
Q

What are some investigations when suspecting Kawasaki’s disease ?

A

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

28
Q

What is the disease course of Kawasaki disease ?

A

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 )

29
Q

What is the management of Kawasaki disease ?

A

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

30
Q

What is acute rheumatic fever ?

A

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

31
Q

What is the pathophysiology of rheumatic fever ?

A

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

32
Q

How does rheumatic fever present ?

A

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

33
Q

What are the key skin findings with rheumatic fever ?

A

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

34
Q

What investigations help support the diagnosis of rheumatic fever ?

A

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

35
Q

What are ASO antibodies ?

A

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

36
Q

What is the management of rheumatic fever ?

A

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

37
Q

What are some complications of rheumatic fever ?

A

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

Y4 Paeds (14 decks)

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