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Year 4 Paediatrics > Paediatric Rheumatology > Flashcards

Paediatric Rheumatology Flashcards

1
Q

Juvenile idiopathic arthritis pathophysiology

A

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

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2
Q

Presentation of JIA

A
  • Joint pain
  • Swelling
  • Stiffness
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3
Q

Still’s disease

A

Systemic JIA:

  • salmon pink rash
  • high swinging fevers
  • enlarged lymph nodes
  • weight loss
  • joint inflammation and pain
  • splenomaegaly
  • muscle pain
  • pleuritis and pericarditis
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4
Q

Investigations for JIA

A

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

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5
Q

Complications of still’s disease

A

Macrophage activation syndrome:

  • Disseminated intravascular coagulation (DIC)
  • Anaemia
  • Thrombocytopenia
  • Bleeding
  • Non blanching rash
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6
Q

Polyarticular JIA

A

Idiopathic arthritis in > 5 joints

  • symmetrical
  • minimal or mild systemic features
  • can be RF postive (seropositive)
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7
Q

Oligoarticular JIA features

A

Involves < 4 joints

Normally 1 joint (monoarthritis)
Normally larger joints

Associated with uveitis - refer to opthalmologist

Antinuclear antibodies often positive

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8
Q

Who is more likely to get oligoarticular arthritis

A

Girls under 6 yo

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9
Q

Enthesitis related arthritis

A

Includes:

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

Inflammatory arthritis + enthesitis

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10
Q

Who commonly gets enthesitis related arthritis

A

Boys over the age of 6 yo

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11
Q

What HLA is associated with enthesitis related arthritis

A

HLA - B27

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12
Q

What is an enthesis

A

Point at which a muscle tendon inserts into bone

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13
Q

Presentation of juvenile psoriatic arthritis

A

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

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14
Q

Henoch Schonlein purpura pathophysiology

A

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

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15
Q

Features of Henoch - Schonlein purpura

A

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

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16
Q

What is Henoch - Schonlein purpura triggered by?

A

Often triggered by a URTI or gastroenteritis

17
Q

Complications of henoch - scholein purpura

A

GI haemorrhage
Intussuseption
Bowl infarction

18
Q

How is Henoch - Schonlein purpura diagnosed

A

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

19
Q

EULAR criteria

A

Palpable purpura + at least 1 of:

  • diffuse abdo pain
  • arthritis or arthralgia
  • IgA deposits on histology
  • proteinuria or haematuria
20
Q

Management of Henoch - Schonlein purpura

A

Supportive - analgesia, rest and hydration

Monitor with repeated urine dipstick and BP

21
Q

Who commonly gets Henoch - Schonlein purpura

A

Children under 10 yo

22
Q

Pathophysiology of Kawasaki disease

A

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

No clear trigger

23
Q

Whi is most likely to get Kawaski disease

A

Asian boys under 5 yo

24
Q

Presentation of Kawasaki disease

A

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
25
Q

Investigations for Kawasaki disease

A

Bloods:

  • FBC - anaemia, thrombocytopenia, leucocytosis
  • LFTs - hypoalbuminaemia and elevated liver enzymes
  • CRP and ESR
  • Urinalysis
  • Echocardiogram - coronary artery anuerysms
26
Q

Kawasaki disease course

A

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

27
Q

Management of Kawasaki disease

A

High dose aspirin
IV immunoglobulin

Monitor and follow up with echocardiogram

28
Q

Complications of Kawasaki disease

A

Coronary artery aneurysm

29
Q

Rheumatic fever pathophysiology

A

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

30
Q

Presentation of rheumatic fever

A

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
31
Q

Cardiac signs due to rheumatic fever

A

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

32
Q

What skin signs are seen in rheumatic fever

A

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

33
Q

Chorea

A

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

34
Q

Assessment of rheumatic fever

A 
Throat swab - bacterial culture 
ASO - antistreptoccal antibody titres 
Echocardiogram 
ECG 
Chest Xray
35
Q

Which criteria is used to daignose rheumatic fever

A

Jones criteria

36
Q

Jones criteria

A

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
37
Q

Management of rheumatic fever

A

Treat streptococcal infection with phenoxymethylpencillin for 10 days

Refer for specialist management

  • NSAIDS - joint pain
  • Aspirin if > 16 yo and steroids for carditis
38
Q

Complications of rheumatic fever

A

Chronic heart failure
Mitral stenosis
Recurrence

Year 4 Paediatrics (19 decks)

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