Rheumatology

Question 1

What is juvenile rheumatoid arthritis?

How is it managed?

Answer 1

Autoimmune inflammation of the joints occurring in <16yrs, lasting >6wks.

Features include joint pain, swelling and stiffness.

Management includes NSAIDs, steroids, DMARDs and biologics.

Question 2

What is Henoch-Schonlein Purpura?
How does it present?
How is it investigated?

Answer 2

IgA vasculitis.
Deposition of IgA in the small blood vessels (100%)- get inflammation and leaky vessels therefore leading to skin manifestation as purpura. Purpuric rash of lower limbs and buttocks only.
Arthritis of knee and elbow (75%)
Deposition of IgA in the GI tract- abdominal pain (50%)- can complication to infarction, intussusception and haemorrhage.
Deposition in kidneys- causing IgA nephritis (50%)

Need to rule out leukaemia, meningococcal septicaemia, HUS etc
FBC and film to see reduced platelets, see if septicaemia, leukaemia
CRP- sepsis
Blood cultures- sepsis
U+Es- kidney function
Albumin- nephrotic syndrome
Urine dip- proteinuria
BP- HTN

Question 3

What is the diagnostic criteria of Henoch-Schonlein purpura?

How is it managed?

Answer 3

Diagnostic criteria=
Palpable purpura + one of; IgA deposits on histology, abdominal pain, Arthritis/arthralgia OR haematuria/proteinuria.

Management:
Supportive with hydration and analgesia.
Steroid usage is debatable- benefits vs risks

Need to monitor BP and urine dip (protein/blood) closely.

Most w/o renal involvement will recover within 6-8wks. Some can have relapse. Some can develop end stage renal disease.

Question 4

What is Kawasaki disease?

How does it present?

Answer 4

Medium-sized vessel arteritis, affecting <5yrs old, usually male and Asian.
THINK IF FEVER>39 degrees FOR >5 DAYS!

Will also present with maculopapular rash, desquamation, strawberry tongue, cracked lips, bilateral conjunctivitis, cervical lymphadenopathy.

Acute phase- Fever, arthralgia and lymphadenopathy. (1-2wks)
Subacute phase- Acute symptoms will resolve, develop arthralgia and desquamation. Can also develop coronary aneurysms. (2-4wks)
Convalescent (recovering) phase- Symptoms beginning to resolve, bloods returning to normal and coronary aneurysms subside (2-4wks)