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Paediatric rotation > Paediatric rheumatology > Flashcards

Paediatric 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

Where there is arthritis without any other cause, lasting more than 6 weeks in a patient under the age of 16.

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3
Q

What are the key features of JIA?

A

joint pain, swelling and stiffness

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4
Q

What are the types of JIA?

A

Systemic
Polyarticular
Oligoarticular
Enthesistis related arthritis
Juvenile psoriatic arthritis

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5
Q

What is systemic JIA also known as?

A

Still’s disease

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6
Q

What are the typical features of systemic JIA?

A

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

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7
Q

What tests are typically negative on JIA?

A

Antonuclear antibodies and rheumatoid factors

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8
Q

What tests will be raised in systemic JIA?

A

Raised CRP, ESR, platelets and serum ferritin

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9
Q

What is a key complication of systemic JIA?

A

macrophage activation syndrome (MAS) where there is severe activation of the immune system with a massive inflammatory response.

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10
Q

What does macrophage activation syndrome present with?

A

Acutely unwell child with disseminated intravascular coagulation (DIC), anaemia, thrombocytopenia, bleeding and a non-blanching rash. It is life threatening

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11
Q

What is a key investigation finding in macrophage activation syndrome?

A

Low ESR

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12
Q

If a child has a fever for more than 5 days what are the key differentials?

A

Kawasaki disease, Still’s disease, rheumatic fever and leukaemia

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13
Q

What is polyarticular JIA?

A

Idiopathic inflammatory arthritis in 5 joints or more

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14
Q

What are the features of polyarticular JIA?

A

Symmetrical and can affect small or large joints. Minimal systemic features but there can be mild fever, anaemia and reduced growth

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15
Q

What is polyarticular JIA the equivalent of?

A

Rehumatoid arthritis in adults

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16
Q

What is the rheumatoid factor like in polyarticular JIA?

A

seronegative, may be seropositive in older adolescents where it is more similar to rheumatoid arthritis

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17
Q

What is oligoarthritis also known as?

A

pauciarticular JIA

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18
Q

What is oligoarticular JIA?

A

Involves 4 joints or less, usually only affects a single joint and tends to affect larger joints, either the knee or ankle

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19
Q

In whom does oligoarticular JIA occur in?

A

more frequent in girls under the age of 6 years

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20
Q

What is a classic feature of oligoarticular JIA?

A

anterior uveitis

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21
Q

What is the clinical picture of oligoarticular JIA?

A

Tend not to have systemic symptoms and inflammatory markers will be normal or mildly elevated. Antinuclear antibodies are often positive, however rheumatoid factor is usually negative

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22
Q

Who is enthesitis-related arthritis more common in?

A

male children over 6 years

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23
Q

What is enthesities-related arthritis the paediatric version of?

A

Seronegative spondyloarthropathy

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24
Q

What is an enthesis?

A

The point at which the tendon of a muscle inserts into a bone

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25
Q

What can enthesitis be caused by?

A

traumatic stress or autoimmune inflammatory process

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26
Q

What helps to diagnose enthesitis but what doesn’t it do?

A

MRI scan but cannot distinguhs between the causes

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27
Q

What gene is related to enthesitis?

A

HLA (Human Leukocyte gene) B27 gene

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28
Q

What should you check for when asking about enthesitis related arthritis?

A

Psoriasis and inflammatory bowel disease and anterior uveitis

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29
Q

Which areas should be palpated when checking for enthesitis in joints?

A

Interphrangeal joints of the hands
Wrist
Greater trochanter on lateral hip
Quadriceps insertion at the anterior superior iliac spine
Quadriceps and patella tendon insertion around the patella
Base of the achilles, at the calcaneous
Metatarsal heads on the base of the foot

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30
Q

What is psoriatic arthritis?

A

Seronegative inflammatory arthritis associoated with psoriasis

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31
Q

What is the pattern of psoriatic arthritis?

A

Symmetrical polyarthritis affectign the small joints, similar to rheumatoid or an asymmetrical athritis affecting the large joint in the lower limb

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32
Q

What signs are seen in juvenile psoriatic arthritis?

A

Plaques of psoriasis on the skin
Nail pitting
Onycholysis (separation of the nail from the nail bed)
Dactylitis
Enthesitis

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33
Q

What are the medical treatment options for juvenile idopathic arthritis?

A

NSAIDs
Steroids (intra-articular in oligoarthritis)
Disease modifying anti-rheumatic drugs (DMARDs) e.g. methotrexate, sulfasalazine and leflunomide
Biologic therapy e.g. tumour necrosis factor inhibitors; entanercept, infliximab and adalimumab

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34
Q

What is Ehlers-Danlos syndrome?

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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35
Q

What is a critical differential diagnosis for hypermobility than ehlers-danlos syndrome?

A

Marfan syndrome

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36
Q

What is the most common and least severe type of Ehlers-Danlos syndrome?

A

Hypermobile Ehlers-Danlos syndrome

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37
Q

What are the key features of hypermobile Ehlers-Danlos sydnrome?

A

joint hypermobility and soft, stretchy skin

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38
Q

What pattern of inheritance is hypermobile Ehlers-Danlos syndrome?

A

Autosomal dominant

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39
Q

What are the classical Ehlers-Danlos syndrome features?

A

stretchy skin that feels smooth and velvety
Severe joint hypermobility, joint pain and abnormal wound healing

40
Q

What are patients prone to with Ehlers-Danlos syndrome?

A

hernias, prolapses, mitral regurgitation and aortic root dilatation

41
Q

What is the most dangerous and severe form of Ehlers-Danlos?

A

Vascular Ehlers-Danlos syndrome

42
Q

What are the features of vascular Ehlers-Danlos syndrome?

A

Thin, translucent skin
Gastrointestinal perforation
Spontaneous pneumothorax

43
Q

What is kyphoscoliotic Ehlers-Danlos syndrome?

A

Initially poor muscle tone (hypotonia) as a neonate and infant followed by kyphoscoliosis as they grow.
significant joint hypermobility

44
Q

What is the inheritance of kyphoscoliotic Ehlers-Danlos syndrome?

A

Autosomal recessive

45
Q

What is the inheritance of vascular Ehlers-Danlos syndrome?

A

Autosomal dominant

46
Q

What symptoms can occur with Ehlers-Danlos syndrome/

A

Joint pain
Hypermobility
Joint dislocations
Soft and stretchy skin
Stretch marks
Easy bruising
Poor wound healing
Bleeding
Chronic pain
Chronic fatigue
Headaches
Autonomic dysfunction
Gastro-oesophageal reflux
Abdominal pain
IBS
Menorrhagia and dysmenorrhea
Premature rupture of membranes in pregnancy
Urinary incontinence
Pelvic organ prolapse
Temporomandibular joint dysfunction

47
Q

What can occur in hypermobile Ehlers-Danlos syndrome due to autonomic dysfunction?

A

Postural orthostatic tachycardia syndrome

48
Q

What score is used to assess for hypermobility and support a diagnosis of Ehlers-Danlos?

A

Beighton score

49
Q

What are the tests for the Beighton score (1 point for each side of the body for 4 of the points)?

A

Place their palms flat on the floor with their straight legs
Hyperextend their elbows
Hyperextend their knees
Bend their thumb to touch their forearm
Hyperextend their little finger past 90 degrees

50
Q

What is the management for Hypermobile Ehlers-Danlos syndrome?

A

Physio
Occupational therapy
Moderating activity to minimise flares

51
Q

What can hypermobility in joints leasd to?

A

Premature osteoarthritis

52
Q

What is henoch-Schonlein Purpura?

A

IgA Vasculitis that presents with a purpuric rash affecting lower limbs and buttocks in children

53
Q

Why does inflammation occur in Henoch-Schonlein purpura?

A

IgA deposits in blood vessels

54
Q

What is HSP often triggered by?

A

Upper airway infection or gastroenteritis

55
Q

Where does HSP normally affect?

A

Skin, Kidneys and gastro-intestinal tract

56
Q

What are the four classic features of HSP?

A

Purpura
Joint pain
Abdominal pain
Renal involvement

57
Q

What are pupura?

A

Red-purple lumps under the skin containing blood

58
Q

What can severe HSP lead to in the gastrointestinal tract?

A

Gastrointestinal haemorrhage, intussusception and bowel infarction

59
Q

If IgA affects the kidneys what does it cause?

A

IgA nephritis leading to microscopic or macroscopic haematuria and proteinuria

60
Q

What is indicative of nephrotic syndrome in HSP?

A

2+ protein on urine dipstick

61
Q

What tests might you do in HSP to exclude other causes and help support HSP diagnosis?

A

FBC an dblood film for thrombocytopenia, sepsis and leukaemia
Renal profile for kidneys
Serum albumin for nephrotic syndrome
CRP for sepsis
Blood cultures for sepsis
Urine dipstick for proteinuria
Urine protein:creatinine ratio to quantify proteinuria
BP for HTN

62
Q

What is the most recent criteria for HSP diagnosis?

A

EULAR/PRINTO/PRES criteria

63
Q

What does the EULAR/PRINTO/PRES criteria diagnose and what does it include?

A

Palpable purpura (not petechiae) + at leats one of:
Diffuse abdominal pain
Arthritis or arthralgia
IgA deposits on biopsy
Protienuria or haematuria

64
Q

What is the management for HSP?

A

Supportive with analgesia, rest and hydration

65
Q

What must be monitored in HSP?

A

Urine dipstick for renal involvement
BP for HTN

66
Q

What is the prognosis of HSP?

A

Full recovery in 4 to 6 weeks- a third of patients will have recurrence in 6 months

67
Q

What is kawasaki also known as?

A

Mucocutaneous lymph node syndrome

68
Q

What is kawasaki disease?

A

Medium-sized vessel vasculitis

69
Q

Who is kawasaki more common in?

A

children under 5 years, asian children and boys

70
Q

What is a key complication of kawasaki disease?

A

Coronary artery disease

71
Q

What are key features of kawasaki disease?

A

Persistent high fever for more than 5 days
Widespread erythematous maculopapular rash and desquamation

72
Q

What are features of kawasaki apart from fever and rash?

A

Strawberry tongue
Cracked lips
Cervical lymphadenopathy
Bilateral conjunctivitis

73
Q

What are the investigations for kawasaki disease?

A

FBC can show anaemia, leukocytosis and thrombocytosis
LFTs can show hypoalbuminaemia and elevated liver enzymes
Inflammatory markers (particularly ESR) are raised
Urinalysis can show raised WBC without infection
ECHO can show coronary artery pathology

74
Q

How many phases of kawasaki disease are there and what are they?

A

three; acute subacute, convalescent

75
Q

What is the acute phase of kawasaki disease?

A

child is most unwell with fever, rash and lymphadenopathy- lasts 1-2 weeks

76
Q

What is the subacute phase of kawasaki disease?

A

Acute symptoms settle, desquamation and arthralgia occur and there is a risk of coronary artery aneuysms forming- 2-4 weeks

77
Q

What is the convalescent stage of kawasaki disease?

A

Remaining symptoms settle, blood tests slowly return to normal and coronary aneurysms may regress- this last 2-4 weeks

78
Q

What are the two first line medical treatments given to patients with kawasaki disease?

A

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

79
Q

Why is aspirin usually avoided in children?

A

Reye’s syndrome

80
Q

What is acute rheumatic fever and what is it triggered by?

A

Autoimmune condition triggered by streptococcus bacteria. It is a multi-system disorder that affects joints, heart, skin and nervous system

81
Q

What is rheumatic fever caused by?

A

Antibodies created against group A beta-haemolytic streptococcal bacteria, usually streptococcus pyogenes causing tonsilitis that also target tissues in the body

82
Q

What type of hypersensitivity reaction is rheumatic fever?

A

Type 2, where the immune system begins attacking cells throughout the body. This is usually dleayed 2-4 weeks after the initial infection

83
Q

What is the presentation of rheumatic fever?

A

Occurs 2-4 weeks after streptococcal infection e.g. tonsilitis
Fever
Joint pain
Rash
SOB
Chorea
Nodules

84
Q

What does rheumatic fever due to joints?

A

Causes migratory arthritis affecting the large joints, with hot, swollen painful joints.

85
Q

What does rheumatic fever do to the joints?

A

Carditis with pericasrditis, myocarditis and endocarditis leads to:
Tachycardia or bradycardia
murmurs from valvular heart disease
Pericardial rub on ascultation
Heart failure

86
Q

What does rheumatic fever do to the skin?

A

Subcutaneous nodules
Erythema marginatum rash or pink rings of varying sizes affecting torso and proximal limbs
Firm, painless nodules over extensor surfaces e.g. elbows

87
Q

What does rheumatic fever do to the skin?

A

Chorea is the key symptom of the nervous system

88
Q

What investigations are used for rheumatic fever?

A

throat swab for bacterial culture
ASO antibody titres
ECHO, ECG and chest xray

89
Q

What criteria is used to diagnose rheumatic fever?

A

Jones criteria

90
Q

What happens to the levels of ASO antibodies after an acute infection?

A

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

ASO levels repeated after 2 weeks to confirm a negative test and assess whether levels are rising or falling

91
Q

What is the diagnosis with jones criteria for rheumatic fever?

A

Two major criteria or one major and 2 minor

92
Q

What is the major criteria for rheumatic fever?

A

J- joint arthritis
O- organ inflammation such as carditis
N- nodules
E- erythema marginatum rash
S-sydenham chorea

93
Q

What is the minor criteria for rheumatic fever using Jones criteria?

A

Fever
ECG changes (prolonged PR interval) without carditis
Arthralgia without arthritis
Raised inflammatory markers (CRP and ESR)

94
Q

What is the prevention of rheumatic fever?

A

Tretament of tponsilitis caused by streptococcus should be treated with phenoxymethylpenicillin (penicillin V) for 10 days

95
Q

What is the manageemnt for possible rheumatic fever?

A

NSAIDs
Aspririn and steroids
Prophylactic antibiotics

96
Q

What are the complications of rheumatic fever?

A

Recurrence of rheumatic fever
Valvular heart disease, most notably mitral stenosis
Chronic heart failure

Paediatric rotation (14 decks)

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