Rheumatic Diseases In Children

Question 1

Describe the diagnostic criteria of Kawasaki disease

6

Answer 1

Fever persisting for at least 5days
AND
4 of the following 5 principal features
Conjunctivitis
Polymorphous skin rash ( anything BUT Vesicular)
Oromucosal changes ( NOT mouth ulcers)
Erythematous changes / peripheral changes ( red hands and feet) later can see peeling of the hands and feet
Cervical lymphadenopathy ( often painful and unilateral)

Question 2

Describe other clinical features

Answer 2

Diarrhoea 
Aseptic meningitis 
Arthritis / arthalgia 
Sterile Pyrrha 
Hydrops of the gallbladder 
Cardiac failure due to myocarditis 
30% of cases can be with an associated infection eg URTI/UTI
But the pt has the persistent FEVER

Question 3

Kawasaki disease

Answer 3

Self limited vasculitis of unknown aetiology
2nd commonest vasculitis in childhood (HSP)
Complications Coronary A Aneurysm reduced if diagnosed and treated within 10days
Commonest cause of acquired heart disease in the developed world
Early Diagnosis and Treatment reduces the complication rate
From 25% to 5% (may be even lower 2-3%)

Question 4

Age group and sex predominance in Kawasaki disease

Answer 4

80% of cases occur at <5years of age
Males predominances
Does occur in INFANTS and older children ( over 7yrs)
Higher risk of complications because diagnosis missed in infants and older children watch out for persisting fever

Question 5

Investigations NOTHING diagnostic ( clinical diagnosis)

No specific test or pathonomonic sign

Answer 5

Raised WCC
Low HB
High Platelets ( during the 2nd week)
Raised ESR and CRP
Elevated LFTs
Low Albumin
Sterile pyuria
ECHO

Question 6

DD lots of overlap

Answer 6

Viral infections eg measles adenovirus ( fevers in these generally not as persistent
Scarlet fever (toxin mediated illnesses) usually no ocular signs
Drug reactions eg SJS
Systemic Juvenile idiopathic arthritis
Mercury poisoning

Question 7

Pitfalls in the diagnosis of Kawasaki disease

Answer 7

1 the features of Kawasaki disease can appear sequentially rather than simultaneously ( ask about the features on the history)
2 common childhood infections eg UTI can precede them
Also viral illness and scarlet fever look a lot like that)

3 incomplete Kawasaki disease (30% of pt have this and are still at risk of Coronary A Aneurysm). More common in infants and older children
High risk of delayed diagnosis and increased risk of complications
Pt can have the fever but NOT 4/5 features
It’s possible that the features were missed /subtle
High index of suspicion

Question 8

Treatment of Kawasaki disease

Answer 8

1referral to peadiatrician
2 admit
3 IVIG 2gm/kg  (10-20% of kids with K fail to response rpt the IVIG) DONT give LIVE Vaccinations for 6-12/12
4 aspirin 5mg/kg
5 ECHO in the acute phase

Question 9

Follow up of Kawasaki disease

Answer 9

1 minimum of 12 m follow up with a cardiologist
2 prompt review if redevelops
3 delay LIVE immunizations for 6-12/12

Question 10

Coronary Aneurysm in Kawasaki disease

Incidence and complications

Answer 10

Caused by the coronary a vasculitis

Prevalence
25% of Kawasaki WITHOUT treatment
5% with prompt treatment IVIG and aspirin within 10days of fever onset
Complications of Coronary Aneurysm
Depends on the size
Small ones often resolve 90% regress
Large ones can develop a thrombosis and an acute MI
Development of stenotic lesions increasing risk of MI in the future

Question 11

Henoch-Schonlein purpura HSP
Commonly present with rash on lower limbs abdomen pain
Joint pain esp the ankles

Answer 11

Commonest vasculitis in childhood. Small vessels venues and capillaries
Commonly effects skin joints ( esp the ankles) GIT/ Kidneys
Skin scalp/scrotum ( scalp not common but pathonomonic ) rash on the lower legs non blanching and blanching rash0
Acute morbidity assoc with GIT (bleeding intusseption)
Chronic morbidity associ with Kidney involvement not common but serious 5%
Ix PT/PTT urine
Bx shows non specific changes leukocytoclasitic vasculitis
Scrotal swelling look for a rash because dd is an acute scrotum but DDx is HSP look at the lower legs and the buttocks

Question 12

Classification criteria of HSP ( must have at least 2 of the4)

Answer 12

1 palpable purpura
2Age <20years at disease onset
3 bowel angina ( abdominal pain after meals or bowel ischemia usually with bloody Diarrhoea )
4 Graulocytes in the walls of the arterioloes /venues on bx
Sn and Sp =87.7%

Question 13

DD of HSP

Answer 13

Acute abdomen
Acute scrotum
Meningococcal disease ( kids are sicker with Meningitis than HSP )
Other vasculitis PAN

Question 14

Treatment of HSP

Answer 14

1 generally mild only requires NSAI for joint pain
2 sever abdo pain may need STERIODS
3 urine BP needs follow up freq initially and then annually
4 30% recurrence

Question 15

Juvenile SLE

Answer 15

Multisystem inflammatory disease characterized by presence of autoantibodies
Characteristised by exacerbation and remissions
Pathology immunoglobulins deposits and vasculitis
20% of all SLE begins <18years
Females >M 4-5:1
Average age 11-14yrs

Question 16

SLE effects most organs

Answer 16

Renal disease Big one very important for mortality
Poly articular arthritis treat with steroids often
Vasculitis in the fingers
Mouth Vascular ulcer oral ulcer Not painful but if you see it is pathognomonic of SLE

Question 17

Lupus Nephritis

Answer 17

Worrying part of SLE
Clinical presentation can vary from a symptomatic mild haematuria or proteinuria
To nephritic/ nephrotic syndrome with severe Hypertension and renal failure
Need a Renal Biopsy to Know the histology classification that will direct the treatment

Question 18

Juvenile dermatomyositis features and complications

Answer 18

History is of struggling to keep up with his peers / struggling on stairs
Dermatomyocisits inflamation of the skin and muscles
Grottons papules on the extensor surface of the hands red and atrophic lesion on the knuckles and then around the nail folds

Complications 
Muscle weakness complications 
Difficulty swallowing 
Aspiration pneumonia 
Respiratory difficulty 
Weakness getting around ( need a wheelchair)
Calcinosis Ca++ deposits modular lumps

Question 19

Linear scleroderma

Answer 19

Scleroderma in children can be limited to the skin
But if it crosses joints in can cause lots of problems and limitation
Need steroids