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Paediatrics > Paediatrics Rheumatology > Flashcards

Paediatrics Rheumatology Flashcards

1
Q

What is juvenile idiopathic arthritis (JIA)?

A

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

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2
Q

When is JIA diagnosed?

A

Arthritis without any other cause lasting more than 6 weeks in a patient under age of 16

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3
Q

What is juvenile idiopathic arthritis also known as?

A

Juvenile chronic arthritis

Juvenile rheumatoid arthritis

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4
Q

What are the features of JIA?

A

Inflammatory arthritis:

  • Joint pain
  • Swelling
  • Stiffness
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5
Q

What are five key subtypes of JIA (associated with different blood tests)?

A

Systemic JIA

Polyarticular JIA

Oligoarticular JIA

Enthesitis related arthritis

Juvenile psoriatic arthritis

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6
Q

What is systemic JIA also known as?

A

Still’s disease - systemic illness that can occur throughout childhood

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7
Q

What are the features of systemic JIA?

A

Subtle salmon pink rash

High swinging fever

Enlarged lymph nodes

Weight loss

Joint inflammation and pain

Splenomegaly

Muscle pain

Pleuritis and pericarditis

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8
Q

What are the blood test findings in systemic JIA?

A

Raised inflammatory markers (CRP, ESR)

Raised platelets and serum ferritin

ANA and rheumatoid factors are typically negative

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9
Q

What is a complication of systemic JIA?

A

Macrophage activation syndrome (MAS) = severe activation of the immune system, causing:

  • Disseminated intravascular coagulation (DIC)
  • Anaemia
  • Thrombocytopenia
  • Bleeding
  • Non-blanching rash
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10
Q

What is a key finding in macrophage activation syndrome?

A

Low ESR

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11
Q

What are the key non-infective differentials in children with fevers for more than 5 days?

A

Kawasaki disease

Still’s disease

Rheumatic fever

Leukaemia

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12
Q

What is polyarticular JIA?

A

Idiopathic inflammatory arthritis in 5 joints or more

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13
Q

Which joints are affected in polyarticular JIA?

A

Symmetrical - small joints of hands and feet and large joints e.g. hips and knees

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14
Q

What are the systemic symptoms of polyarticular JIA?

A

Minimal systemic symtoms (unlike systemic JIA), can be:

Mild fever

Anaemia

Reduced growth

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15
Q

What is the equivalent of rheumatoid arthritis in adults?

A

Polyarticular JIA

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16
Q

Are patients with polyarticular JIA seropositive?

A

Most children are negative

Older children can be positive - disease pattern = more similar to RA in adults

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17
Q

How many joints are affected in oligoarthritis? What is it also known as?

A

4 joints or less

Pauciarticular JIA

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18
Q

Which joints are typically affected by oligoarticular JIA?

A

Larger joints, knee or ankle

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19
Q

Who is typically affected by oligoarticular JIA?

A

Girls under the age of 6

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20
Q

What is classically associated with oligoarticular JIA?

A

Anterior uveitis (referred to opthalmologist for management and follow up)

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21
Q

What are the blood test findings on oligoarticular JIA?

A

ANA often positive

RF is negative

Inflammatory markers normal or mildly elevated

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22
Q

Who is enthesitis-related arthritis most common in?

A

Male children over 6

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23
Q

What is enthesitis-related arthritis the paediatric version of?

A

Seronegative spondyloarthropathy

  • Ankylosing spondylitis
  • Psoriatic arthritis
  • Reactive arthritis
  • IBD related arthritis
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24
Q

What is enthesitis?

A

Inflammation of the insertion point of the tendon into the bone

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25
Q

What can cause enthesitis?

A

Repetitive strain during sporting activities

Autoimmune inflammatory process

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26
Q

What imaging can be used for enthesitis?

A

MRI scan of affected joint (can’t distinguish cause)

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27
Q

Which gene is associated with enthesitis-related arthritis?

A

HLA B27 gene

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28
Q

What is look for on examination of enthesitis-related arthritis?

A

Psoriasis (psoriatic plaques and nail pitting)

Inflammatory bowel disease (intermittent diarrhoea and rectal bleeding)

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29
Q

Why should patients with enthesitis-related arthritis see an opthalmologist?

A

As they are prone to anterior uveitis

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30
Q

What are the key areas to palpate in patients with enthesitis?

A

Interphalangeal joints in hand

Wrist

Greater trochanter on lateral aspect of hip

ASIS (quadriceps insertion)

Patella (quadriceps and patella tendon indertion)

Base of achilles at the calcaneus

Metatarsal heads on the base of the foot

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31
Q

What is juvenile psoriatic arthritis?

A

Seronegative inflammatory arthritis associated with psoriasis

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32
Q

Which joints are affected in juvenile psoriatic arthritis?

A

Varies: can be symmetrical polyarthritis affecting small joints similar to RA or can be symmetrical affecting large joints in the lower limb

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33
Q

What are the signs of juvenile psoriatic arthritis?

A

Plaques of psoriasis on the skin

Nail pitting

Onycholysis - separation of the nail from the nail bed

Dactylitis - inflammation of the full finger

Enthesitis

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34
Q

What is the medical management of juvenile idiopathic arthritis?

A

Coordinated by MDT:

  • NSAIDs e.g. ibuprofen
  • Steroids (oral / IM / intra-articular) in oligoarthritis
  • DMARDs e.g. methtrexate, sulfasalazine, leflunomide
  • Biologic therapy e.g. TNF inhibitors (etanercept, infliximab and adalimumab)
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35
Q

What is Ehlers-Danlos syndrome?

A

Umbrella term for group of genetic conditions causing defective collagen resulting in hypermobility of the patient’s joints and abnormal connective tissue e.g. skin, bones, blood vessels and organs

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36
Q

What are the four main types of Ehlers-Danlos?

A

Hypermobile Ehlers-Danlos syndrome

Classical Ehlers-Danlos syndrome

Vascular Ehlers-Danlos syndrome

Kyphoscoliotic Ehlers-Danlos syndrome

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37
Q

What are the features of hypermobild Ehlers-Danlos syndrome?

A

Least severe type of Ehlers-Danlos

Joint hypermobility

Soft and stetchy skin

Gene has not yet been found

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38
Q

What are the features of classical Ehlers-Danlos?

A

Stretchy skin

Smooth and velvety

Severe joint hypermobility, joint pain and abnormal wound healing

Lumps over pressure points e.g. elbows

Prone to hernias, prolapses, mitral regurgitation and aortic root dilatation

39
Q

What is the mode of inheritance for classical Ehlers-Danlos?

A

Autosomal dominant

40
Q

What are the features of vascular Ehlers-Danlos?

A

Thin, translucent skin

Most dangerous form of EDS

Blood vessels are fragile

Skin, internal organs and arteries are prone to rupturing

41
Q

What is the management of vascular Ehlers-Danlos?

A

Monitored for vascular abnormalities and told to seek urgent medical attention for sudden unexplained pain / bleeding

42
Q

What is the mode of inheritance of vascular Ehlers-Danlos?

A

Autosomal dominant

43
Q

What are the features of Kyphoscoliotic Ehlers-Danlos?

A

Hypotonia as a neonate / infant

Kyphoscoliosis as they grow

Joint hypermobility

Patients are tall and slim

Risk of rupture of medium sized arteries

44
Q

What is the mode of inheritance of kyphoscoliotic Ehlers-Danlos?

A

Autosomal dominant

45
Q

What are the two main symptoms in hypermobile EDS?

A

Joint pain

Hypermobility

46
Q

What are the other presenting features of hypermobile EDS?

A

Joint dislocation e.g. shoulders or hips

Soft stretchy skin

Bruising

Poor wound healing

Bleeding

Headaches

GORD

Abdo pain

IBS

Menorrhagia and dysmenorrhoea

PROM in pregnancy

Urinary incontinence

Pelvic organ prolapse

Temporomandibular joint dysfunction

Myopia

47
Q

What score is used for hypermobility? How is it calculated?

A

Beighton Score

Palms flat on floor with straight legs (score 1)

Elbows hyperextend

Knees hyperextend

Thumb can bend to touch the forearm

Little finger hyperextends past 90 degrees

48
Q

What is it important to exclude in EDS?

A

Marfan syndrome (examine for high arch palate, arachnodactyly and arm span)

49
Q

What is the management of EDS?

A

No cure - only maintain healthy joints, monitor for complications

  • ​Physio - to strengthen joints
  • OT to maximise function
  • Maintain good posture
  • Psychology to manage chronic condition and pain
50
Q

What can EDS often lead to?

A

Generalised osteoarthritis

51
Q

What can be the result of the autonomic dysfunction in hypermobile EDS?

A

Postural orthostatic tachycardia syndrome:

  • Inappropriate tachycardia on sitting or standing up
  • Presyncope, syncope, headaches, nausea and tremor
52
Q

What is Henoch-Schonlein Purpura (HSP)?

A

IgA vasculitis presenting with purpuric rash affecting the lower limbs and buttocks in children

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

53
Q

Where does Henoch-Schonlein Purpura affect?

A

Skin

Kidneys

GI tract

54
Q

What is HSP triggered by?

A

Upper airway infection

Gastroenteritis

55
Q

What are the four classic features of HSP?

A

Purpura

Joint pain

Abdo pain

Renal involvement

56
Q

What is the purpura caused by?

A

Inflammation and leaking of blood from small blood vessels causing purpura - red-purple lumps under skin containing blood

57
Q

How can purpura develop in severe cases?

A

Skin ulceration and necrosis develops

58
Q

What is the difference between arthritis and arthralgia?

A

Arthritis is pain with swelling - arthralgia is just pain

59
Q

What can severe cases of GI involvement with HSP lead to?

A

Gastrointestinal haemorrhage

Intussusception

Bowel infarction

60
Q

What does HSP cause in the kidneys? What are the features?

A

IgA nephritis in the kidneys

Microscopic/macroscopic haematuria

Proteinuria (if 2+ on dip = nephrotic syndrome - oedema)

61
Q

What are differentials for a non-blanching rash?

A

Meningococcal septicaemia

Leukaemia

Idiopathic thrombocytopenic purpura

Haemolytic uraemic syndrome

62
Q

What investigations are there for HSP?

A

FBC and blood film for thrombocytopenia, sepsis and leukaemia

Renal profile for kidney damahe

Serum albumin for nephrotic syndrome

CRP for sepsis

Urine dip for proteinuria

Urine protein:creatinine ratio to quantify proteinuria

Blood pressure for hypertension

63
Q

What set of criteria is used to diagnose HSP?

A

EULAR/PRINTO/PRES criteria

64
Q

What is the EULAR/PRINTO/PRES criteria for diagnosing HSP?

A

Have palpable purpura (not petichiae) + at least one:

  • Diffuse abdo pain
  • Arthritis or arthralgia
  • IgA deposits on histology (biopsy)
  • Proteinuria or haematuria
65
Q

What is the management of HSP?

A

Analgesia

Rest

Hydration

Steroid use is debatable - may shorten duration of illness but won’t affect long term outcomes (may be used in severe GI pain / renal involvement)

66
Q

How are patients with HSP monitored?

A

Urine dip for renal involvement

Blood pressure monitoring for HTN

67
Q

What is the prognosis of HSP?

A

Abdo pain settles in a few days

Full recovery in 4 to 6 weeks (if no kidney involvement)

1/3 have recurrence in 6 months

Small proportion develop end stage renal failure

68
Q

What is Kawasaki disease? What is it also known as?

A

Systemic, medium-sized vessel vasculitis affecting young children (under 5)

Also known as mucocutaneous lymph node syndrome

69
Q

Who does Kawasaki disease typically affect?

A

Asian children, japanese and korean (boys)

No clear cause or trigger

70
Q

What are the key features of Kawasaki disease?

A

Persistent high fever (above 39) for more than 5 days

Widespread erythmatous maculopapular rash

Desquamation (skin peeling) on palms and soles

71
Q

What are the other features of Kawasaki disease?

A

Strawberry tongue (red tongue with large papillae)

Cracked lips

Cervical lymphadenopathy

Bilateral conjunctivitis

72
Q

What are the investigations for Kawasaki disease?

A

FBC for anaemia, leukocytosis, thrombocytosis

LFTs for hypoalbuminaemia, and elevated liver enzymes

Inflammatory markers (particularly raised ESR)

Urinalysis raised WBCs without infection

Echo for coronary artery pathology

73
Q

What are the three phases for Kawasaki?

A

Acute phase: 1-2 weeks - most unwell with fever, rash and lymphadenopathy

Subacute phase: 2-4 weeks - acute symptoms settle - desquamation and arthralgia (risk of coronary artery aneurysm)

Convalesent stage: 2-4 weeks - remaining symptoms settle - blood tests normalise and coronary aneurysms may regress

74
Q

What is the management of Kawasaki disease?

A

High dose aspirin - reduce risk of thrombosis

IV immunoglobulins - reduce risk of coronary artery aneurysm

Close following with echo for coronary artery aneurysm

75
Q

Why is aspirin usually avoided in children?

A

Risk of Reye’s syndrome

76
Q

What is acute rheumatic fever?

A

Autoimmune condition triggered by streptococcus bacteria

77
Q

What is acute rheumatic fever caused by?

A

Antibodies created against streptococcus bacteria which also targets tissues in the body

78
Q

What is rheumatic fever usually caused by?

A

Group A beta-haemolytic streptococcal typically strep pyogenes causing tonsillitis

79
Q

What type of hypersensitivity reaction is rheumatic fever?

A

Type 2 hypersensitivity reaction where immune system begins attacking cells throughout body

80
Q

How does rheumatic fever present?

A

2-4 weeks following a streptococcal infection e.g. tonsillitis

  • Fever
  • Joint pain
  • Rash
  • RoB
  • Chorea
  • Nodules
81
Q

Why does rheumatic fever cause migratory arthritis?

A

Affecting large joints which become hot, swollen and painful - become inlammed and improve at different times

82
Q

What does the carditis in rheumatic fever cause?

A

Pericarditis

Myocarditis

Endocarditis

83
Q

How does the carditis in rheumatic fever manifest?

A

Tachycardia or bradycardia

Murmurs from valvular heart disease, typically mitral valve disease

Pericardial rub on auscultation

Heart failure

84
Q

What are the two key skin findings in rheumatic fever?

A

Subcutaneous nodules (firm, painless, extensor surfaces)

Erythema marginatum rash (pink rings, torso, proximal limbs)

85
Q

What is the nervous system involvement in rheumatic fever?

A

Chorea - irregular, uncontrolled and rapid movements of the limbs

86
Q

What is chorea also known as?

A

Sydenham chorea

St Vitus’ Dance

87
Q

What investigations are there for rheumatic fever?

A

Throat swab for bacterial culture

ASO antibody titres

Echo, ECG and CXR

88
Q

How do the anti-streptococcal antibodies (ASO) vary with rheumatic fever?

A
89
Q

When are ASO levels repeated?

A

Confirm negative test

Assess if levels rising / falling

90
Q

What diagnostic criteria is used for rheumatic fever?

A

Jones Criteria

91
Q

What does the Jones criteria for diagnosis involve?

A

Evidence of recent streptococcal infection

Plus: two major criteria or one major plus two minor

Major:

J - Joint arthritis

O - Organ inflammation

N - Nodules

E - Erythema marginatum rash

S - Sydenham chorea

Minor:

Fever

ECG changes (prolonged PR interval) without carditis

Arthralgia without arthritis

Raised inflammatory markers (CRP and ESR)

92
Q

What is the management of rheumatic fever?

A

Treat strep infections to prevent

NSAIDs for joint pain

Aspirin and steroids for carditis

Prophylactic abx (oral / IM penicillin to prevent further strep infections)

Monitor for complications

93
Q

What are the complications of rheumatic fever?

A

Recurrence

Valvular heart disease e.g. mitral stenosis

Chronic heart failure

Paediatrics (18 decks)

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