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Paeds > Rheumatology > Flashcards

Rheumatology Flashcards

1
Q

What is JIA?

A

Juvenile idiopathic arthritis

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2
Q

What is juvenile idiopathic arthritis?

A

Condition affecting children and adolescents where autoimmune inflammation occurs in the joints

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3
Q

When is JIA diagnosed?

A

Where there is arthritis with no other known cause, lasting > 6 weeks in patients <16

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4
Q

What are the key features of JIA?

A

Joint pain
Swelling
Stiffness

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5
Q

What are the 5 key subtypes of JIA?

A 
Systematic JIA
Polyarticular JIA
Oligoarticular JIA
Enthesitis related arthritis
Juvnile psoriatic arthritis
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6
Q

What is the difference between each of the 5 JIA subtypes?

A

The serology (blood tests)

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7
Q

What is systemic JIA?

A

Systemic idiopathic inflammatory condition that occurs in childhood

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8
Q

What is systemic JIA also known as?

A

Still’s disease

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9
Q

What are the typical features of systemic JIA?

A 
Subtle salmon-pink rash
High fevers
Enlarged lymph nodes
Weight loss
Joint inflammation/ pain
Splenomegaly
Muscle pain
Pleuritis and pericarditis
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10
Q

What will be raised in systemic JIA and what will stay negative?

A

Raised inflammatory markers (CRP/ ESR0, platelets, serum ferritin
Antinuclear antibodies and rheumatoid factors stay negative

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11
Q

What is a key complication of systemic JIA?

A

Macrophage activation syndrome

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12
Q

What is macrophage activation syndrome?

A

Severe activation of the immune system with massive immune response

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13
Q

How does MAS present?

A 
Acutely unwell child
DIC
Anaemia
Thrombocytopenia
Bleeding
Non-blanching rash 
Low ESR
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14
Q

In a child with a fever for > 5 days, what are the key non-infective differentials?

A

Kawasaki disease
Still’s disease
Rheumatic fever
Leukaemia

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15
Q

What is polyarticular JIA?

A

Idiopathic inflammatory arthritis in 5 joints or more (equivalent of RA in adults)

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16
Q

Where is usually affected in polyarticular JIA?

A

Symmetrical in small joints of hands and feet, as well as large joints like hips and knees

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17
Q

Does polyarticular JIA present with systemic symptoms?

A

May be mild (e.g. mild fever, anaemia, reduced growth)

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18
Q

What is the serology of polyartiular JIA?

A

seronegative (negative for rheumatoid factor)

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19
Q

What is oligoarticular JIA?

A

JIA involving 4 joints or less (usually only a single joint)

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20
Q

Where does oligoarticular JIA usually affect?

A

A larger joint (e.g. ankle or knee)

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21
Q

In which patients is oligoarticular JIA more common?

A

Girls < 6

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22
Q

What is a common associated feature with oligoarticular JIA?

A

Anterior uveitis

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23
Q

What is the serology of oligoarticular JIA?

A

Normal inflammatory markers
Seronegative (no rheumatoid factor)
Positive antinuclear antibodies

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24
Q

What is enthesitis-related arthritis?

A

Inflammatory arthritis in the joints as well as enthesitis

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25
Q

What is enthesitis?

A

Inflammation of the entheses

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26
Q

What is an enthesis?

A

Point where a tendon of a muscle inserts into a bone

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27
Q

In which patients is enthesitis-related arthritis more common?

A

Male children >6

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28
Q

What is enthesitis-related arthritis comparable to in adults?

A

Seronegative spondylarthopathies

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29
Q

What are the causes of enthesitis?

A 
Traumatic stress (e.g. repeptitive strain during sport) 
Autoimmune inflammatory process
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30
Q

How is enthesitis diagnosed?

A

MRI scan

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31
Q

What is enthesitis-related arthritis associated with?

A

HLA B27 gene

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32
Q

What other signs and symptoms should be looked for in patients with enthesitis-related arthritis?

A

Psoriasis
IBD symptoms
Anterior uveitis
Tenderness to entheses

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33
Q

What is juvenile psoriatic arthritis?

A

Seronegative inflammatory arthritis associated with psoriases

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34
Q

What are the examination signs of juvenile psoriatic arthritis?

A 
Plaques of psoriases
Pitting of nails
Onycholysis
Dactylitis
Enthesitis
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35
Q

How is JIA managed?

A 
MDT
NSAIDs 
Steroids (Oral, IM or intra-articular) 
DMARDs (e.g. Methotrexate) 
Biologic therapy (e.g. anti-TNF's )
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36
Q

What is EDS?

A

Ehlers- Danlos syndrome

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37
Q

What is Ehlers- Danlos syndrome?

A

Group of genetic conditions that cause defects in collagen

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38
Q

What does collagen defect cause?

A

Hypermobility of joints and abnormalities in connective tissues

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39
Q

What are the different types of Ehlers-Danlos syndrome?

A

Hypermobile
Classical
Vascular
Kyphoscoliotic

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40
Q

What is the most common EDS?

A

Hypermobile Ehlers-Danlos syndrome

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41
Q

What are the key features of hypermobile EDS?

A

Joint pain and hypermobility

Soft, stretchy skin

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42
Q

What are the key features of classical EDS?

A

Remarkably stretchy skin (smooth and velvety)
Severe joint hypermobility, joint pain and abnormal wound healing
Lumps over pressure points

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43
Q

What are patients with classical EDS more at risk of?

A

Hernias
Prolapses
Mitral regurg
Aortic root dilation

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44
Q

What is the inheritance pattern of classical EDS?

A

Autosomal dominant

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45
Q

What is the most dangerous form of EDS?

A

Vascular EDS

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46
Q

What happens in vascular EDS?

A

The blood vessels are partiularly fragile as a result of defective collagen

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47
Q

What are the characteristic features of vascular EDS?

A

Thin, transluscent skin

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48
Q

What is the inheritance pattern of vascular and kyphoscoliotic EDS?

A

Autosomal dominant

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49
Q

What are the key features of kyphoscoliotic EDS?

A

Hypotonia as neonate and infant
Kyphoscoliosis as they grow
Joint hypermobility
Tall and slim

50
Q

What is the key complication of kyphoscoliotic EDS?

A

Rupture of medium sized arteries

51
Q

What other symptoms may you get in hypermobile EDS?

A 
Hypermobility 
Joint pain and dislocations
Soft stretchy skin
Easy bruising/ poor healing
Bleeding
Headaches
Syncope
GORD
Abdo pain/ IBS
Menorrhagia/ dysmenorrhea
Urinary prolapse 
ect
52
Q

What score is used to assess hypermobility?

A

Beighton score

53
Q

What things are assessed in Beighton score?

A

Palms flat on floor with straight legs
Elbows/ knees hyperextension
Thumb bend to touch forearm
Little finger hyperextends past 90 degrees

54
Q

How is hypermobile EDS diagnosed?

A

Using beighton score

55
Q

What should be excluded when diagnosing IDS and how?

A

Marfan syndrome by examining for high arch palate, arachnodactyly and arm span

56
Q

How is EDS managed?

A

Maintaining healthy joints (OT, PT)
Monitor for complications
Minimise symptoms

57
Q

What do patients with hypermobility often develop?

A

Osteoarthritis at a younger age

58
Q

What is a key complication of hypermobile EDS?

A

Postural orthostatic tachycardia syndrome (POTS)

59
Q

What is POTS and why does it occur?

A

Autonomic dysfunction causes inappropriate tachycardia on sitting or standing, resulting in syncope, headaches, nausea ect

60
Q

What is HSP?

A

Henoch-Schonlein Purpura

61
Q

What kind of condition is HSP?

A

IgA vasculitis

62
Q

What happens in HSP?

A

Inflammation occurs in affected organs due to IgA deposits in blood vessels

63
Q

What organs are particularly affected by HSP?

A

Skin
Kidneys
GI tract

64
Q

What usually triggers HSP?

A

URTI

Gastroenteritis

65
Q

In which age range is HSP most common?

A

Children under 10

66
Q

What are the 4 classic features of HSP?

A 
Purpura (100%)
Joint pain (75%)
Abdominal pain (50%)
IgA nephritis (50%)
67
Q

What causes the rash in HSP?

A

Inflammation and leaking of blood from small blood vessels under the skin

68
Q

What are purpura?

A

Red-purple lumps under the skin containing blood

69
Q

Where are the common sites for purpura in HSP?

A

Start on legs and spread to buttocks

70
Q

What joints are most affected by arthralgia/ arthritis as a result of HSP?

A

Knees and ankles

71
Q

What can associated abdominal features with HSP lead to in severe cases?

A

GI haemorrhage
Intussusception
Bowel infarction

72
Q

What does IgA nephritis lead to?

A

Haematuria and proteinuria

73
Q

What differentials should be excluded when diagnosing HSP?

A

Meningococcal septicaemia
Leukaemia
ITP
Haemolytic uraemic syndrome

74
Q

What investigations are done into HSP?

A 
FBC and Blood film
U&E's
Serum albumin (nephrotic syndrome)
CRP (Sepsis)
Blood cultures (sepsis) 
Urine dipstick + Protein:creatinine ration 
Blood pressure
75
Q

What is the criteria for diagnosing HSP?

A

Palpable purpura + at least on of:

  • Diffuse abdominal pain
  • Arthritis/ arthralgia
  • IgA deposits on histology
  • Protein/ haematuria
76
Q

What is the management of HSP?

A

Supportive

Close monitoring with repeat urine dipstick and blood pressure for renal involvement

77
Q

What is the prognosis for HSP?

A

Abdominal pain settles in a few days
If no kidney involvement, fully recover within 4-6 weeks
1/3 patients have recurrence within 6 months

78
Q

What kind of condition is Kawasaki disease?

A

Systemic medium-sized vessel vasculitis

79
Q

What is kawasaki disease also known as?

A

Mucocutaneous lymph node syndrome

80
Q

What age range is typically affected by kawasaki?

A

Young children (<5)

81
Q

In which children is Kawasaki disease more common?

A

Asian boys

82
Q

What is the key complication of kawasaki?

A

Coronary artery aneurysm

83
Q

What is the key feautre that would make you consider kawasaki disease?

A

Persisent high fever for > 5 days

84
Q

What are the key skin findings in kawasaki?

A

Widespread erythematous maculopapular rash

Desquamation (skin peeling) on palms and soles

85
Q

What are the key features of kawasaki?

A 
Prolonged fever
Rash
Skin peeling
Strawberry tongue
Cracked lips
Cervical lymphadenopathy
Bilateral conjunctivitis
86
Q

What investigations can help diagnose kawasaki disease?

A 
FBC
LFTs
Inflammatory markers
Urinalysis
Echocardiogram
87
Q

What will FBC show in kawasaki?

A

Anaemia
Leukocytosis
Thrombocytosis

88
Q

What might urinalysis show in kawasaki disease?

A

Raised WCC without infection

89
Q

What are the 3 phases of kawasaki disease?

A

Acute
Subacute
Convalescent

90
Q

How long is the acute phase of kawasaki and what happens in it?

A

Child is unwell with fever, rash and lymphadenopathy

Lasts 1-2 weeks

91
Q

What happens in the subacute phase and how long does it last?

A

Acute symptoms settle, desquamation and arthralgia occur.

Lasts 2-4 weeks

92
Q

What is the convalescent stage and how long does it last?

A

Remaining symptoms settle blood tests slowly return to normal
Lasts 2-4 weeks

93
Q

What are the two first line medications used to treat Kawasaki disease?

A

High dose aspirin

IV immunoglobulins

94
Q

Why are patients with kawasaki given high dose aspirin?

A

To reduce the risk of thrombosis

95
Q

Why are patients with Kawasaki given Iv immunoglobulins?

A

To reduce the risk of coronary artery aneurysms

96
Q

Is aspirin usually given to children?

A

No-Kawasaki is one of the rare cases

97
Q

What kind of condition is acute rheumatic fever?

A

Autoimmune condition

98
Q

What triggers rheumatic fever?

A

Streptococcus bacteria

99
Q

What is the most common cause of rheumatic fever?

A

Strep pygones causing tonsillits

100
Q

What happens in rheumatic fever?

A

Antibodies are created against strep bacteria which also target tissues in the body.

101
Q

What kind of reaction does rheumatic fever result in?

A

Type 2 hypersensitivity reaction

102
Q

How long after initial infection does they hypersensitivity reaction usually occur?

A

2-4 weeks later

103
Q

How does rheumatic fever present?

A 
Following strep infection
Fever
Joint pain
Rash
SOB
Chorea
Nodules
104
Q

How does rheumatic fever affect the joints?

A

Causes migratory arthritis, making large joints hot, swollen and painful

105
Q

How does rheumatic fever affect the heart?

A 
Causes carditis throughout (pericarditis, myocarditis & endocarditis), leading to: 
Tachy/bradycardia
Murmurs
Pericardial rub
Heart failure
106
Q

How does rheumaic fever affect the skin?

A

Subcutaeneous nodules &

Erythema marginatum rash

107
Q

Where do nodules form in rheumatic fever?

A

Over extensor surfaces of joints

108
Q

What is the key nervous system symptom in rheumatic fever?

A

Chorea

109
Q

What is chorea?

A

Irregular, uncontrolled and rapid movements of the limbs

110
Q

What investigations help support diagnosis of rheumatic fever?

A

Throat swab for bacterial culture
ASO antibody titres
Echo, ECG and CXR for heart involvement

111
Q

What are ASO antibodies?

A

Anti-streptococcal antibodies

112
Q

What do ASO antibodies indicate?

A

A recent streptococcus infection

113
Q

What happens to ASO antibody levels after an acute strep infection?

A

Rise over 2-4 weeks
Peak around 3-6 weeks
Gradually fall over 3-12 months

114
Q

What is the criteria for diagnosis of rheumatic fever?

A

Jones criteria

115
Q

What is the Jones criteria for diagnosing rheumatic fever?

A

Evidence of recent strep infection + Two major citeria OR one major and two minor criteria

116
Q

What is the mnemonic for the Jones criteria?

A

JONES-FEAR

117
Q

What are the major criteria used for diagnosing rheumatic fever?

A 
Joint arthritis
Organ inflammation
Nodules
Erythema marginatum rash
Sydenham chorea
118
Q

What is the minor criteria used for diagnosing rheumatic fever?

A

Fever
ECG changes
Arthralgia (without arthritis)
Raised inflammatory markers

119
Q

What can prevent the development of rheumatic fever?

A

Treatment of strep infections with antibiotics (e.g. penicillin V)

120
Q

How are patients with rheumatic fever managed?

A

Refer to specialist
NSAIDS
Aspirin and steroids
Prophylactic antibiotics

121
Q

What are the key complications of rheumatic fever?

A

Recurrene
Valcular heart disease
Chronic heart failure

122
Q

What is the most common valvular heart disease associated with rheumatic fever?

A

Mitral stenosis

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