Rheumatology Flashcards

1
Q

What are the causes of a non-blanching purpuric rash in children?

A
  • henoch-schonlein purpura
  • ITP
  • meningitis
  • following viral infection
  • haemolytic uraemic syndrome
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2
Q

What is henoch-schonlein purpura (HSP)?

A

IgA vasculitis

Inflammation occurs in affected organs due to IgA deposits.

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3
Q

Which organs does henoch-schonlein purpura affect?

A

Skin
Kidneys
GI tract

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4
Q

What can precipitate HSP?

A

URTI

Gastroenteritis

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5
Q

What ages is HSP most common?

A

Children <10 years

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6
Q

What are the 4 classic features of HSP?

A
  • purpuric rash (100%)
  • arthralgia/arthritis (75%)
  • abdominal pain (50%)
  • renal involvement, IgA nephritis (50%)
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7
Q

What causes the purpuric rash?

A

Inflammation and leaking of blood from small blood vessels under the skin, forming purpura.

Purpura are red-purple lumps under the skin containing blood.

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8
Q

Where is the purpura mostly found in HSP?

A

Typically start on the legs; spread to the buttocks

Can also affect the trunk and arms.

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9
Q

In severe cases of HSP what skin changes can occur?

A

Skin ulceration and necrosis

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10
Q

What joints are most commonly affected in HSP?

A

Knees
Ankles

Become swollen, painful, reduced ROM

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11
Q

In severe cases of HSP, what GI involvment can occur?

A
  • gastrointestinal haemorrhage
  • intussusception
  • bowel infarction
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12
Q

What is present in IgA nephritis?

A
  • haematuria

- proteinuria

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13
Q

What is recorded on the urine dipstick that would indicate the child has developed nephrotic syndrome in HSP?

A
  • 2+ protein

Will also have a degree of oedema

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14
Q

What serious pathology should be ruled out in investigations for HSP?

A
  • meningococcal septicaemia
  • leukaemia
  • ITP
  • haemolytic uraemic syndrome
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15
Q

What investigations should be done for HSP?

A
  • FBC, blood film (thrombocytopenia, sepsis, leukaemia)
  • renal profile (kidney involvement)
  • serum albumin (nephrotic syndrome)
  • CRP (sepsis)
  • blood cultures (sepsis)
  • urine dipstick (proteinuria)
  • urine protein:creatinine ratio (quantify proteinuria)
  • BP (HTN)
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16
Q

What set of criteria is used to diagnose HSP?

A

EULAR/PRINTO/PRES criteria

Requires patient to have palpable purpura AND at least one of:

  • diffuse abdo pain
  • arthritis/arthralgia
  • IgA deposits on histology
  • proteinuria/haematuria
17
Q

What is the management for HSP?

A

Supportive

  • simple analgesia
  • rest
  • hydration

Consider steroids in severe GI pain, renal involvement

Monitor:

  • renal involvement (proteinuria/haematuria) with urine dipstick
  • HTN with BP
18
Q

What is the prognosis for HSP?

A
  • abdo pain should settle within a few days
  • patients without kidney involvement should recover within 4-6 weeks
  • third have recurrence within 6 months
  • small & develop end stage renal failure
19
Q

What is Kawasaki disease?

A

A systemic, medium sized vessel vasculitis

20
Q

Who does Kawasaki affect?

A

Young children <5 years

More common in Asian children (Japanese, Korean)

More common in boys

21
Q

What are the causes of Kawasaki disease?

A

No clear cause of trigger

22
Q

How does Kawasaki disease present?

A

“warm cream”

  • persistent fever >39C for >5 days
  • Conjunctivitis
  • Rash (widespread erythematous, maculopapular)
  • Erythema/Oedema to palms/soles (and desquamation, skin peeling)
  • Adenopathy, cervical
  • Mucositis, mucous membranes dry, red, strawberry tongue (red tongue with large papillae), cracked lips
23
Q

What investigations should be done in Kawasaki disease?

A
  • FBC (anaemia, leukocytosis, thrombocytosis)
  • LFTs (hypoalbuminaemia, elevated liver enzymes)
  • ESR, CRP (raised)
  • urinalysis (raised WBC without infection)
  • ECHO (coronary artery pathology)
24
Q

What are the 3 phases to Kawasaki disease?

A

1) Acute phase
2) Subacute phase
3) Convalescent stage

25
Q

What happens in acute phase of Kawasaki disease?

A
  • most unwell with fever, rash, lymphadenopathy

- lasts 1-2 weeks

26
Q

What happens in subacute phase of Kawasaki disease?

A
  • acute symptoms setlle
  • desquamation, arthralgia occur
  • risk of coronary artery aneurysms forming
  • lasts 2-4 weeks
27
Q

What happens in the convalescent stage of Kawasaki disease?

A
  • remaining symptoms settle
  • blood tests slowly return to normal
  • coronary artery aneurysms may regress
  • lasts 2-4 weeks
28
Q

What is the management for Kawasaki disease?

A
  • High dose aspirin (reduce thrombosis risk)
  • IV immunoglobulins (reduce risk of coronary artery aneurysms)
  • monitor with echocardiograms for evidence of coronary artery aneurysms
29
Q

Why is aspirin usually avoided in children?

A

The risk of Reye’s syndrome (very rare; encephalopathy and liver damage)