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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
What can enthesitis be caused by?
traumatic stress or autoimmune inflammatory process
26
What helps to diagnose enthesitis but what doesn't it do?
MRI scan but cannot distinguhs between the causes
27
What gene is related to enthesitis?
HLA (Human Leukocyte gene) B27 gene
28
What should you check for when asking about enthesitis related arthritis?
Psoriasis and inflammatory bowel disease and anterior uveitis
29
Which areas should be palpated when checking for enthesitis in joints?
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
30
What is psoriatic arthritis?
Seronegative inflammatory arthritis associoated with psoriasis
31
What is the pattern of psoriatic arthritis?
Symmetrical polyarthritis affectign the small joints, similar to rheumatoid or an asymmetrical athritis affecting the large joint in the lower limb
32
What signs are seen in juvenile psoriatic arthritis?
Plaques of psoriasis on the skin Nail pitting Onycholysis (separation of the nail from the nail bed) Dactylitis Enthesitis
33
What are the medical treatment options for juvenile idopathic arthritis?
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
34
What is Ehlers-Danlos syndrome?
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
35
What is a critical differential diagnosis for hypermobility than ehlers-danlos syndrome?
Marfan syndrome
36
What is the most common and least severe type of Ehlers-Danlos syndrome?
Hypermobile Ehlers-Danlos syndrome
37
What are the key features of hypermobile Ehlers-Danlos sydnrome?
joint hypermobility and soft, stretchy skin
38
What pattern of inheritance is hypermobile Ehlers-Danlos syndrome?
Autosomal dominant
39
What are the classical Ehlers-Danlos syndrome features?
stretchy skin that feels smooth and velvety Severe joint hypermobility, joint pain and abnormal wound healing
40
What are patients prone to with Ehlers-Danlos syndrome?
hernias, prolapses, mitral regurgitation and aortic root dilatation
41
What is the most dangerous and severe form of Ehlers-Danlos?
Vascular Ehlers-Danlos syndrome
42
What are the features of vascular Ehlers-Danlos syndrome?
Thin, translucent skin Gastrointestinal perforation Spontaneous pneumothorax
43
What is kyphoscoliotic Ehlers-Danlos syndrome?
Initially poor muscle tone (hypotonia) as a neonate and infant followed by kyphoscoliosis as they grow. significant joint hypermobility
44
What is the inheritance of kyphoscoliotic Ehlers-Danlos syndrome?
Autosomal recessive
45
What is the inheritance of vascular Ehlers-Danlos syndrome?
Autosomal dominant
46
What symptoms can occur with Ehlers-Danlos syndrome/
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
What can occur in hypermobile Ehlers-Danlos syndrome due to autonomic dysfunction?
Postural orthostatic tachycardia syndrome
48
What score is used to assess for hypermobility and support a diagnosis of Ehlers-Danlos?
Beighton score
49
What are the tests for the Beighton score (1 point for each side of the body for 4 of the points)?
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
What is the management for Hypermobile Ehlers-Danlos syndrome?
Physio Occupational therapy Moderating activity to minimise flares
51
What can hypermobility in joints leasd to?
Premature osteoarthritis
52
What is henoch-Schonlein Purpura?
IgA Vasculitis that presents with a purpuric rash affecting lower limbs and buttocks in children
53
Why does inflammation occur in Henoch-Schonlein purpura?
IgA deposits in blood vessels
54
What is HSP often triggered by?
Upper airway infection or gastroenteritis
55
Where does HSP normally affect?
Skin, Kidneys and gastro-intestinal tract
56
What are the four classic features of HSP?
Purpura Joint pain Abdominal pain Renal involvement
57
What are pupura?
Red-purple lumps under the skin containing blood
58
What can severe HSP lead to in the gastrointestinal tract?
Gastrointestinal haemorrhage, intussusception and bowel infarction
59
If IgA affects the kidneys what does it cause?
IgA nephritis leading to microscopic or macroscopic haematuria and proteinuria
60
What is indicative of nephrotic syndrome in HSP?
2+ protein on urine dipstick
61
What tests might you do in HSP to exclude other causes and help support HSP diagnosis?
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
What is the most recent criteria for HSP diagnosis?
EULAR/PRINTO/PRES criteria
63
What does the EULAR/PRINTO/PRES criteria diagnose and what does it include?
Palpable purpura (not petechiae) + at leats one of: Diffuse abdominal pain Arthritis or arthralgia IgA deposits on biopsy Protienuria or haematuria
64
What is the management for HSP?
Supportive with analgesia, rest and hydration
65
What must be monitored in HSP?
Urine dipstick for renal involvement BP for HTN
66
What is the prognosis of HSP?
Full recovery in 4 to 6 weeks- a third of patients will have recurrence in 6 months
67
What is kawasaki also known as?
Mucocutaneous lymph node syndrome
68
What is kawasaki disease?
Medium-sized vessel vasculitis
69
Who is kawasaki more common in?
children under 5 years, asian children and boys
70
What is a key complication of kawasaki disease?
Coronary artery disease
71
What are key features of kawasaki disease?
Persistent high fever for more than 5 days Widespread erythematous maculopapular rash and desquamation
72
What are features of kawasaki apart from fever and rash?
Strawberry tongue Cracked lips Cervical lymphadenopathy Bilateral conjunctivitis
73
What are the investigations for kawasaki disease?
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
How many phases of kawasaki disease are there and what are they?
three; acute subacute, convalescent
75
What is the acute phase of kawasaki disease?
child is most unwell with fever, rash and lymphadenopathy- lasts 1-2 weeks
76
What is the subacute phase of kawasaki disease?
Acute symptoms settle, desquamation and arthralgia occur and there is a risk of coronary artery aneuysms forming- 2-4 weeks
77
What is the convalescent stage of kawasaki disease?
Remaining symptoms settle, blood tests slowly return to normal and coronary aneurysms may regress- this last 2-4 weeks
78
What are the two first line medical treatments given to patients with kawasaki disease?
High dose aspirin to reduce risk of thrombosis IV immunoglobulins to reduce coronary artery aneurysms
79
Why is aspirin usually avoided in children?
Reye's syndrome
80
What is acute rheumatic fever and what is it triggered by?
Autoimmune condition triggered by streptococcus bacteria. It is a multi-system disorder that affects joints, heart, skin and nervous system
81
What is rheumatic fever caused by?
Antibodies created against group A beta-haemolytic streptococcal bacteria, usually streptococcus pyogenes causing tonsilitis that also target tissues in the body
82
What type of hypersensitivity reaction is rheumatic fever?
Type 2, where the immune system begins attacking cells throughout the body. This is usually dleayed 2-4 weeks after the initial infection
83
What is the presentation of rheumatic fever?
Occurs 2-4 weeks after streptococcal infection e.g. tonsilitis Fever Joint pain Rash SOB Chorea Nodules
84
What does rheumatic fever due to joints?
Causes migratory arthritis affecting the large joints, with hot, swollen painful joints.
85
What does rheumatic fever do to the joints?
Carditis with pericasrditis, myocarditis and endocarditis leads to: Tachycardia or bradycardia murmurs from valvular heart disease Pericardial rub on ascultation Heart failure
86
What does rheumatic fever do to the skin?
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
What does rheumatic fever do to the skin?
Chorea is the key symptom of the nervous system
88
What investigations are used for rheumatic fever?
throat swab for bacterial culture ASO antibody titres ECHO, ECG and chest xray
89
What criteria is used to diagnose rheumatic fever?
Jones criteria
90
What happens to the levels of ASO antibodies after an acute infection?
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
What is the diagnosis with jones criteria for rheumatic fever?
Two major criteria or one major and 2 minor
92
What is the major criteria for rheumatic fever?
J- joint arthritis O- organ inflammation such as carditis N- nodules E- erythema marginatum rash S-sydenham chorea
93
What is the minor criteria for rheumatic fever using Jones criteria?
Fever ECG changes (prolonged PR interval) without carditis Arthralgia without arthritis Raised inflammatory markers (CRP and ESR)
94
What is the prevention of rheumatic fever?
Tretament of tponsilitis caused by streptococcus should be treated with phenoxymethylpenicillin (penicillin V) for 10 days
95
What is the manageemnt for possible rheumatic fever?
NSAIDs Aspririn and steroids Prophylactic antibiotics
96
What are the complications of rheumatic fever?
Recurrence of rheumatic fever Valvular heart disease, most notably mitral stenosis Chronic heart failure

Paediatric rotation (14 decks)

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