Rheumatology Flashcards
(32 cards)
What is JIA?
A broad spectrum of childhood rheumatic diseases that occur before the age of 16 and are characterised by joint inflammation that lasts more than 6 weeks.
What type of JIA is most common?
Oligoarticular JIA îs the most common with asymmetrical involvement of upto 4 joints
What is still’s disease?
Systemic Juvenile idiopathic arthritis. A systemic illness
Features of still’s disease
Subtle salmon pink rash
Intermittent fevers
Enlarged lymph nodes
Weight loss
Joint inflammation and pain
Splenomegaly
Muscle pain
What type of JIA is most common?
Oligoarticular JIA îs the most common with asymmetrical involvement of upto 4 joints
How to diagnose Systemic juvenile idiopathic arthritis?
ANA and RF is typically negative. There will be raised inflammatory markers - CRP, ESR, platelets and serum ferritin
Key complication of systemic JIA
macrophage activation syndrome where there is severe activation of the immune system with a massive inflammatory response. Key investigation finding is low ESR.
Classic features of oligoarticular JIA
Anterior uveitis
It involves 4 joints or less.
ANA usually positive but RF usually negative
What is Ehler-Danlos?
An umbrella term that encompasses a group of genetic conditions caused by defects in collagen causing hyper mobility.
Presentation of Ehler-Danlos
Hypermobility
Joint pain after exercise or inactivity
Joint dislocations
Soft stretchy skin
Easy bruising
Poor wound healing
Bleeding
How would you access hyper mobility?
Beighton score - one point is scored for each side of the body. Max 9 points
Management of Ehler-Danlos
Physio to strengthen and stabilise the joints
Occupational therapy to maximise function
Maintaining good posture
Psychology may be required to help manage the chronic condition and pain
What is Henoch-Schonlein purpura?
IgA vasculitis that presents with a purpuric rash that affects the lower limbs and buttocks in children. IgA deposits in blood vessels
Classical features of HSP?
Purpura
Joint pain
Abdominal pain
Renal involvement
Diagnosis of HSP
FBC
U&Es - for kidney involvement
Serum albumin - nephrotic syndrome
CRP - for sepsis
Blood cultures for sepsis
Blood pressure for HTN
What is the management of HSP?
Management is supportive with simple analgesia, rest and proper hydration
Monitoring of HSP
Monitoring of renal involvement using urine dipstick
Monitoring of hypertension via blood pressure
What is Kawasakis?
It is a systemic medium sized vessel vasculitis that affects children under the age of 5. Common in Japanese and Korean children. Key complication coronary artery aneurysm
Clinical features Kawasaki’s
Persistent high fever above 39 degrees Celsius more than 5 days
Strawberry tongue with large papillae
Cracked lips
Cervical lymphadenopathy
Bilateral conjunctivitis
Investigations of Kawasaki’s
FBC - can show anaemia, leukocytosis and thrombocytosis
LFTs - hypoalbuminaemia and elevated LFTs
Inflammatory markers especially ESR raised
ECHO - to demonstrate coronary artery pathology
Kawasaki disease course
3 phases
Acute - when the child is most unwell with fever, rash and lymphadenopathy. Lasts 1-2 weeks
Subacute phase - acute symptoms settle and there is a risk of coronary artery aneurysms
Convalescent stage - blood tests and symptoms return back to normal and coronary aneurysm may regress
Management of Kawasaki’s
high dose aspirin
IV immunoglobulins
Why is aspirin rarely used in children?
Due to the risk of Reyes disease - rare and serious condition that causes swelling of the brain and liver. Can cause confusion, seizures and loss of consciousness.
What is rheumatic fever?
It is an autoimmune condition caused by group A beta-haemolytic strep - typically strep progenies. The antibodies that the body produces to fight the infection also targets the host cells. Causing type 2 hypersensitivity reaction
