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Paediatrics > Rheumatology > Flashcards

Rheumatology Flashcards

1
Q

What is juvenile idiopathic arthritis?

A

Autoimmune inflammation in the joints of children (<16) that causes arthritis lasting >6 weeks, without any other cause

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2
Q

What are the key features of juvenile idiopathic arthritis?

A
  • joint pain
  • swelling
  • stiffness
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3
Q

What are the key five subtypes of juvenile idiopathic arthritis?

A
  • systemic JIA
  • polyarticular JIA
  • oligoarticular JIA
  • enthesitis related arthritis
  • juvenile psoriatic arthritis
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4
Q

What is another name for systemic juvenile idiopathic arthritis?

A

Still’s disease

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5
Q

What are the typical symptoms of Still’s disease?

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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6
Q

What blood tests are abnormal in Stills disease?

A
  • raised inflammatory markets - CRP and ESR
  • raised platelets and ferritin
    (antinuclear antibodies and Rheumatoid factor are typically negative)
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7
Q

What is a key complication of Stills disease?

A

Macrophage activation syndrome. Massive immune response. Causes DIC, anaemia, thrombocytopenia, bleeding and a non blanching rash. It is life threatening. There will be a low ESR.

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8
Q

What non infective causes can cause a fever >5 days?

A
  • Kawasaki disease
  • Still’s disease
  • Rheumatic fever
  • leukaemia
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9
Q

What is polyarticular juvenile idiopathic arthritis?

A

Idiopathic inflammatory arthrtisi in 5 or more joints. It tends to be symmetrical and can affect both small and large joints.

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10
Q

What is oligoarticular juvenile idiopathic arthritis?

A

Inflammatory arthritis in 4 or less joints - most commonly only one joint (and larger joints - e.g. knee or ankle). Most frequent in girls <6. It is also associated with anterior uveitis.

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11
Q

What blood test is often positive in oligoarticular juvenile idiopathic arthritis?

A

Antinuclear antibodies

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12
Q

What is enthesis-related arthritis?

A

Inflammation of where the tendon inserts into a bone - leading to arthritis.

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13
Q

What gene is associated with enthesis-related arthritis?

A

HLAB27 gene

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14
Q

How is juvenile idiopathic arthritis managed?

A
  • paediatric rheumatology and a MDT
  • NSAIDs
  • steroids
  • DMARDS - e.g. methotrxate, sulfasalazine
  • Biologic therapy - e.g. infliximab
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15
Q

What is Ehlers-Danlos syndrome?

A

A group of genetic conditions that cause defects in collagen causing hypermobility of joints and abnormalities in connective tissue (skin, bones, blood vessels and organs)

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16
Q

Name some of the different types of Ehlers Danlos syndromes?

A
  • hypermobile (most common and least severe)
  • classical
  • vascular (most dangerous)
  • kyphoscoliotic
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17
Q

What are the features of hypermobile Ehler-Danlos syndrome?

A
  • most common
  • joint hypermobility
  • soft and stretchy skin
  • no identified gene and no single mode of inheritance
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18
Q

What are the features of classical Ehlers-Danlos syndrome?

A
  • very stretchy skin that feels smooth and velvety to touch
  • severe joint hypermobility
  • joint pain
  • abnormal wound healing
  • prone to hernias, prolapsing, mitral regurgitation and aortic root dilation.
  • Inheritance is autosomal dominant
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19
Q

What are the features of vascular Ehlers-Danlos syndrome?

A
  • most dangerous
  • blood vessels are fragile
  • thin, translucent skin
  • skin, organs and arteries are prone to rupturing
  • monitoring is needed for vascular monitoring
  • autosomal dominant inheritance
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20
Q

What are the features of kyphoscoliotic Ehlers-Danlos syndrome?

A
  • initial poor tone as a neonate and infant (hypotonia)
  • kyphoscholiosis
  • joint hypermobility
  • tall and slim
  • risk of rupture of medium sized arteries
  • autosomal dominant
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21
Q

How can hypermobile Ehlers-Danlos present?

A
  • hypermobility
  • joint pain after exercise or inactivity
  • joint dislocations - e.g. shoulders or hips
  • soft stretchy skin
  • easy bruising
  • poor healing of wounds
  • bleeding
  • headaches
  • autonomic dysfunction - dizziness and syncope
  • GORD
  • abdo pain
  • IBS
  • Menorrhagia and dysmenorrhea
  • premature rupture of membranes in pregnancy
  • urinary incontinence
  • pelvic organ prolapse
  • TMJ dysfunction
  • Myopia and other
22
Q

What scoring system is used to assess hypermobility?

A

Beighton score

23
Q

Explain the Beighton scoring for hypermobility

A

One point for each side of the body (max 9)
- palms flat on floor with straight legs
- elbows hyperextend
- knees hyperextend
- thumb can bend to touch forearm
- little finger hyperextends past 90 degrees

24
Q

How is Ehlers-danlos managed?

A
  • physiotherapy - to strengthen and stabilise joints
  • occupational therapy - to maximise function
  • moderating intensity of activity to minimise flares
  • follow up with specialities for any complications - e.g. vascular / cardio
  • psychology can also be useful
25
Q

What is POTS?

A

Postural orthostatic tachycardia syndrome. Autonomic dysfunction causes inappropriate tachycardia on sitting or standing up. This leads to presyncope, syncope, headaches, disorientation, nausea and tremor.

26
Q

What is Kawasaki disease?

A

A systemic, medium sized vessel vasculitis. Also known as mucocutaneous lymph node syndrome.

27
Q

Who is most commonly affected by Kawasaki disease?

A
  • children under 5
  • more common in asian children - particularly japanese or korean
  • more common in boys
28
Q

What is a key complication of Kawasaki disease?

A

Coronary artery aneurysm

29
Q

How does Kawasaki disease present?

A
  • persistent high fever (>39) for more than 5 days
  • widespread erythematous maculopapular rash and desquamation (skin peeling) on the palms and soles
  • strawberry tongue
  • cracked lips
  • cervical lymphadenopathy
  • bilateral conjunctivitis
30
Q

What investigations are done for Kawasaki disease?

A
  • FBC - can show anaemia, leukocystosis and thrombocytosis
  • LFTs - can show hypoalbuminaemia and elevated liver enzymes
  • Inflammatory markers - raised CRP and ESR
  • urinalysis - raised WBC without infection
  • Echocardiogram - to look for coronary artery pathology
31
Q

How is Kawasaki disease managed?

A
  • high dose aspirin to reduce risk of thrombosis
  • IV immunoglobulins - to reduce risk of coronary artery aneurysms

Follow up with echocardiograms.

32
Q

Why is aspirin normally avoided in children?

A

Due to the risk of Reye’s syndrome (causing serious liver and brain damage)

33
Q

What is rheumatic fever?

A

Autoimmune condition triggered by streptococcus bacteria. Antibodies are created against this bacteria however they also target tissues in the body.

34
Q

What organism causes rheumatic fever?

A

Group A beta-haemolytic streptococcal - typically streptococcus pyogenes causing tonsilitis

35
Q

When does rheumatic fever symptoms present?

A

2-4 weeks after the initial infection (e.g. tonsilitis)

36
Q

What type of sensitivity reaction is rheumatic fever?

A

Type 2 - antibody mediated

37
Q

How does rheumatic fever present?

A

Systemic symptoms 2-4 weeks after acute infection.
- fever
- joint pain
- rash
- shortness of breath
- chorea - uncontrolled and rapid movements of the limbs
- migratory arthritis - hot swollen painful large joints
- cardiac involvement - pericarditis, myocarditis etc. - tachy or bradycardia, murmurs, pericardial rub and heart failure
- skin involvement - subcut. nodules, erythema marginatum rash (pink rings varying in sizes on the torso and proximal limbs)

38
Q

What investigations are done for rheumatic fever?

A
  • throat swabs for bacterial culture
  • ASO (anti-streptococcal antibodies) titres
  • echocardiogram, ECG and chest XRAY to assess cardio involvement

Diagnoses is then made using the Jones criteria.

39
Q

Describe the Jones Criteria for diagnosis

A

Evidence of a recent streptococcal infection +
2 major criteria OR
1 major + 2 minor criteria

MAJOR
- J - joint arthritis
- O - organ inflammation - e.g. carditis
- N - nodules (skin)
- E - erythema marginatum rash
- S - sydenham chorea

MINOR
- F - fever
- E - ECG changes
- A - arthralgia (without arthritis)
- R - raised inflammatory markers - CRP and ESR

40
Q

How is rheumatic fever managed?

A

Treatment of streptococcal infections with antibiotics is preventative (e.g. penicillin V)

  • NSAIDs - for joint pain
  • Aspirin and steroid for carditis
  • Prophylactic antibiotics - to prevent recurrence
  • monitor and manage any complications
41
Q

What are some complications of rheumatic fever?

A
  • recurrence
  • valvular heart disease - mitral stenosis
  • chronic heart failure
42
Q

What is Henoch-Schonlein Purpura?

A

IgA vasculitis - where IgA deposits in the blood vessels causing inflammation. Leads to symptoms such as a purpuric rash affecting lower limbs and buttocks in children.

43
Q

What can trigger Henoch-Schonlein purpura?

A
  • URTI
  • gastroenteritis
44
Q

What are the four classic features of Henoch-Schonlein purpura?

A
  • purpuric rash
  • joint pain
  • abdominal pain
  • renal involvement
45
Q

Describe the rash seen in Henoch-Schonlein purpura?

A

Inflammation and leaking of blood from small blood vessels under the skin, forming purpura (red/ purple lumps palpable under the skin).
This typically starts on the legs and spreads to the buttocks. It can also affect the trunk and arms.

46
Q

How does Henoch-Schonlein purpura affects joints?

A

Causes arthralgia or arthritis in 75% of patients. Mainly knees and ankles. Become swollen and painful with a reduced range of movement.

47
Q

How does Henoch-Schonlein purpura affects the GI system?

A

Causes abdominal pain in about 50% of patients. In severe cases it can lead to GI haemorrhage, intussusception and bowel infarction.

48
Q

How does Henoch-Schonlein purpura affect the kidneys?

A

In 50% of patients it causes and IgA nephritis. This leads to microscopic or macroscopic haematuria and proteinuria. It can also lead to nephrotic syndrome and oedema.

49
Q

How is Henoch-Schonlein purpura investigated?

A

Exclude other serious pathologies.
- FBC and blood film
- renal profile for kidney involvement
- serum albumin for nephrotic syndrome
- CRP for sepsis
- blood cultures for sepsis
- urine dipstick - for proteinuria
- urine protein- creatinine ratio - to quantify the proteinuria
- blood pressure for hypertension

50
Q

How is Henoch-Schonlein purpura managed?

A

Supportive - simple analgesia, rest and proper hydration.
Steroids may be considered.
Close monitoring
- urine dipstick for renal involvement
- blood pressure for hypertension

51
Q

What is the prognosis for Henoch-Schonlein purpura?

A

Abdominal pain usually settles within a few days.
If no kidney involvement - full recovery within 4-6wks.
A third of patients have recurrent disease within 6 months.
A very small proportion of patients will develop end stage renal failure

52
Q

What are important differentials for Henoch-Schonlein purpura?

A
  • meningococcal septicaemia
  • leukaemia
  • idiopathic thrombocytopenic purpura
  • haemolytic uraemic syndrome

Paediatrics (14 decks)

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