Paediatric rheumatology and immunology Flashcards Preview

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Flashcards in Paediatric rheumatology and immunology Deck (17):
1

HSP - what does it stand for, and what is another name for it

Henoch-Schonlein purpura

AKA IgA vasculitis

2

Classic tetrad of HSP

1. Palpable purpura in absence of thrombocytopaenia or coagulopathy
2. Arthritis/arthralgia
3. Abdominal pain
(4. Renal disease)

3

Epidemiology of HSP

Most common form of systemic vasculitis in children
Peak incidence between 4-6y
Slight male predominance
Rarely occurs in summer months
5% higher in patients with familial mediterranean fever

4

Presentation of HSP

50% preceded by URTI
Palpable purpura - mainly on extensor surfaces of the lower limb and the buttocks)
Arthritis/arthralgia (usually lower limb large joints)
Abdominal pain +/- intussuception (colicky pain in 50%)
Renal disease
May be associated with subcutaneous oedema (scrotum, face, hands)

Manifestations may develop over the course of days-weeks and may vary in their order of presentation

Purpura and joint pain are usually the presenting symptoms

5

Investigations in suspected HSP

Urine dipstick - MCS if haematuria
CBE
EUC
Blood culture

6

Management of HSP

Symptomatic (NSAIDs and analgesia)
Prednisolone 1mg/kg if severe abdo or joint pain
Admit if (abdominal or renal complications, or for severe symptoms needing supportive treatment)
Discharge with information for family, ensure follow-up

7

Follow-up required for HSP

Follow-up early on to ensure remission of symptoms (rash usually last to go)
Urine checked regularly first 6 months (renal involvement can present up to 6 months after initial presentation)
BP checked 3-monthly over first 6 months

8

Rationale for prednisolone in HSP

Symptomatic relief only
Shortens duration of abdominal and joint pain
Does not improve rate of long-term renal complications
May reduce risk of abdominal complications

9

Diagnostic criteria for Kawasaki disease

Fever for 5 or more days
PLUS 4 out of 5:
- polymorphous rash
- bilateral (non purupent) conjunctival injection
- Mucous membrane changes (reddened/dry cracked lips, strawberry tongue, diffuse redness of oral or pharyngeal mucosa)
- Peripheral changes (erythema of palms or soles, oedema of hands/feet, in convaescence desquamation)
- Cervical lymphadenopathy (usually unilateral, single and painful

+ exclusion of diseases with similar presentation (TSS, scalded skin syndrome, scarlet fever, measles, Steven Johnson's, drug reaction)

10

Definition of atopy

the ability of an individual to form specific IgE antibodies to one or more common inhaled aeroallergens

11

Definition of an allergen

An antigen (usually a protein) that is recognised by the immune system, is usually harmless, and induces an allergic inflammatory response

12

Atopic diseases (types)

Eczema
Asthma
Allergic rhinoconjunctivitis

13

Epidemiology of Kawasaki disease (age, gender, ancestry)

Males more than females
90% below the age of 5
Highest incidence in children of Asian (esp Japanese) descent

14

Pathophysiology in Kawasaki disease

Systemic inflammatory illness particularly affecting medium-sized arteries (e.g. coronary), inflammatory cell infiltration into vascular tissue causes blood vessel damage
Strawberry tongue occurs due to sloughing of filiform papillae and denuding of inflamed glossal tissue

15

Investigations in Kawasaki disease

CRP/ESR/Ferritin all increased
Normocytic normochromic anaemia is common
Pyuria
EUC - hypoNa+ associated with higher risk of CA aneurysms
Echo at time of diagnosis and repeat 6-8 weeks later if normal

16

Complications of Kawasaki disease

Shock
Coronary artery disease (aneurysm, ectasia, stenosis)
Mitral regurg
Pericardial effusion
Acute MI (most common cause of death)
Peripheral vascular disease (ischaemia, gangrene)
Acute interstital nephritis/acute kidney injury (uncommon)

17

Management of Kawasaki disease

Low dose aspirin - not just for acute episode
IVIG over 8-12 hours (repeat if fever persists more than 36h)
IV methylprednisolone if fever persists after 2nd dose of IVIG