[57] Henoch-Schonlein Purpura Flashcards

1
Q

What is Henoch-Schonlein Puprura (HSP)?

A

A small vessel vasculitis with particular characteristics

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2
Q

What are the characteristics of HSP?

A
  • Skin rash on extensor surface
  • Arthralgia
  • Periarticular oedema
  • Abdominal pain
  • Glomerulonephritis
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3
Q

What age range is HSP most common in?

A

3-10 years

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4
Q

When does the incidence of HSP peak?

A

In winter months

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5
Q

How does HSP begin?

A

Genetic predisposition and antigen exposure increases circulating IgA levels and disrupts IgG synthesis

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6
Q

What happens to IgA and IgG in HSP?

A

They interact to form complexes that activate complement and are deposited in affected organs precipitating an inflammatory response with vasculitis

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7
Q

What are the risk factors for HSP?

A
  • Male
  • White
  • Asian
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8
Q

What are the symptoms of HSP?

A
  • Fever
  • Rash
  • Joint pain
  • Colicky abdominal pain
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9
Q

If there is GI involvement in HSP how can this present?

A

Haematemesis and melena

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10
Q

Describe the rash in HSP

A

Initially urticarial then maculopapular, purpuric and palpable

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11
Q

Where does the rash in HSP typically affect?

A

Buttocks and extensor surfaces

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12
Q

What is normally spared by the HSP rash?

A

The trunk

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13
Q

How many patients with HSP are affected by arthralgia?

A

2/3

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14
Q

What joints are particularly affected by arthralgia in HSP?

A

Knees and ankles

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15
Q

What is diagnosis based on?

A

Combination of symptoms with supportive tests

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16
Q

Why can diagnosis be made on symptoms alone?

A

Very few conditions present in the same way

17
Q

What supportive tests can be used in diagnosing HSP?

A
  • Bloods
  • Urine dipstick
  • Renal biopsy
  • Skin biopsy
  • Abdominal investigations
18
Q

What may be elevated in bloods in HSP?

A
  • Creatinine
  • Urea
  • CRP
19
Q

What are the urine dipstick findings in HSP?

A

Microscopic or macroscopic haematuria or mild proteinuria in 80% of patients

20
Q

When may renal biopsy be indicated?

A

To establish diagnosis or assess severity of suspected kidney disease

21
Q

What are the main findings on renal biopsy?

A

Increased cells and Ig deposition in the mesangium

22
Q

Is skin biopsy necessary?

A

Only rarely

23
Q

What might skin biopsy show?

A

Leucocytoclastic vasculitis

24
Q

What may atypical HSP be confused for?

A
  • Papular urticaria
  • SLE
  • Meningococcemia
25
Q

Does HSP usually require treatment?

A

No - most have a benign course that resolve in 6 weeks

26
Q

What medication may be needed whilst HSP resolves?

A

NSAIDs to treat arthritis

27
Q

What can be used to manage severe abdominal pain in HSP?

A

Steroids

28
Q

What should occur after HSP has resolved in children with persistent renal symptoms and that required treatment?

A

Long-term follow-up

29
Q

What is long-term follow-up to look for?

A

CKD and hypertension

30
Q

When may hypertension and CKD develop after HSP?

A

After several years

31
Q

What are the potential complications of HSP?

A
  • Intussusception
  • Ileua
  • Protein-losing enteropathy
  • CNS invovlement
  • Progressive CKD
32
Q

What are the risk factors for developing CKD after HSP?

A
  • Heavy proteinuria
  • Oedema
  • Hypertension
  • Deteriorating renal function