Rheumatology Flashcards
What is juvenile idiopathic arthritis ?
A condition affecting children and adolescents where autoimmune inflammation occurs in the joints.
Arthritis lasting more than 6 weeks in patients under 16.
What are the key features of inflammatory arthritis ?
Joint pain
Swelling
Stiffness
What is systemic juvenile idiopathic arthritis ?
Also known as Still’s disease
Systemic illness that occurs in boys and girls
What are some typical features of systemic juvenile idiopathic arthritis ?
Subtle salmon pink rash
High swinging fevers
Enlarged lymph nodes
Weight loss
Joint inflammation and pain
Splenomegaly
Muscle pain
Pleuritic and pericarditis
What are some abnormalities seen in blood tests in systemic juvenile idiopathic arthritis ?
Antinuclear antibodies and rheumatoid factors are negative
Raised inflammatory markers with raised CRP, ESR, platelets and serum ferritin.
What is the key complication of systemic juvenile idiopathic arthritis ?
Macrophage activation syndrome - severe activation of the immune system with a massive inflammatory response.
How many joints are involved in polyarticular juvenile idiopathic arthritis ?
5 joints or more
What is a classic feature associated with oligoarticular JIA ?
Anterior uveitis
What are some signs that can be seen in juvenile psoriatic arthritis ?
Plaques of psoriasis
Pitting of the nails
Oncholysis
Dactylitis
Enthesitis
What is the management of juvenile idiopathic arthritis ?
NSAIDs
Steroids
DMARDs - methotrexate
Biologic therapy
What is Ehler’s-Danlos syndrome ?
A group of genetic conditions involving defects in collagen causing hypermobility in the joints and abnormalities in the connective tissue of the skin, bones, blood vessels and organs.
What is a differential for Ehler’s danlos syndrome ?
Marfans syndrome
How does Ehler’s-Danlos syndrome present ?
Joint pain and hypermobility
Joint dislocations
Soft and stretchy skin
Stretch marks
Easy bruising
Poor wound healing
Bleeding
Chronic pain and fatigue
Headaches
What is the Beighton score ?
It is used to assess for hypermobility and support the diagnosis of Ehler’s danlos syndrome.
what is the management of Ehler’s danlos syndrome ?
No cure
Follow ups
Physiotherapy
Occupational therapy
Moderating activity
What can be the complication of Ehler’s danlos syndrome ?
Premature osteoarthritis
What is Henoch-Schonlein purpura ?
An IgA vasculitis that presents with a purpuric rash affecting the lower limbs and buttocks in children. Inflammation occurs in the affected organs due to IgA deposits in the blood vessels.
What are the 4 classic features of Henoch-Schonlein purpura ?
Purpura
Joint pain
Abdominal pain
Renal involvement
How does a rash form in Henoch-schonlein purpura ?
The rash is caused by inflammation and leaking of blood from small blood vessels under the skin forming purpura.
In severe cases how can Henoch-schonlein purpura cause GI conditions ?
GI haemorrhage
Intussusception
Bowel infarction
How is a diagnosis of henoch schonlein purpura ?
FBC and blood film
Renal profile
Serum albumin
CRP
Urine dipstick
Blood cultures
Blood pressure
What is the management of henoch-schonlein purpura ?
Supportive
Steroids is debatable
How is henoch-schonlein purpura monitored ?
Urine dipstick
Blood pressure
What is Kawasaki disease ?
Mucocutaneous lymph node syndrome.
It is a systemic, medium-sized vessel vasculitis.
What is a key complication of Kawasaki disease ?
Coronary artery aneurysm
What are some clinical features of Kawasaki disease ?
Persistent high fever
Children look unhappy and unwell
Widespread erythematous maculopapular rash
Desquamation - skin peeling
Strawberry tongue
Cracked lips
Cervical lymphadenopathy
Bilateral conjunctivitis
What are some investigations when suspecting Kawasaki’s disease ?
FBC - anaemia, Leucocytosis and thrombocytosis
LFT
Inflammatory markers - ESR
Urinalysis -WBC
Echocardiogram
What is the disease course of Kawasaki disease ?
Acute phase - most unwell with fever, rash and lymphadenopathy ( 1-2 weeks )
Subacute phase - acute symptoms settle, desquamation and Arthralgia occurs ( lasts 2-4 weeks )
Convalescent stage - remaining symptoms settle, blood tests slowly return to normal ( last 2-4 weeks )
What is the management of Kawasaki disease ?
High dose aspirin to reduce the risk of thrombosis
IV immunoglobulins to reduce risk of coronary artery aneurysm
What is acute rheumatic fever ?
An autoimmune condition triggered by streptococcus bacteria. It is caused by antibodies created against the strep bacteria that also target tissues in the body.
What is the pathophysiology of rheumatic fever ?
Caused by group A beta-haemolytic streptococcal ( typically strep pyogenes ) causing tonsillitis. Antibodies are produced which target the bacteria but also antigens on the cells of the persons body.
This results in a type 2 hypersensitivity reaction
How does rheumatic fever present ?
Usually follows a strep infection like tonsillitis
Fever
Joint pain
Rash
SOB
Chorea
Nodules
What are the key skin findings with rheumatic fever ?
Subcutaneous nodules - firm over the extensor surfaces
Erythema marginatum rash - pink rings affecting the torso and proximal limbs
What investigations help support the diagnosis of rheumatic fever ?
Throat swab for bacterial culture
ASO antibody titres
Echocardiogram, ECG and chest X-ray
What are ASO antibodies ?
Antibodies against streptococcus and indicate a recent strep infection and can be helpful in supporting a diagnosis of rheumatic fever
What is the management of rheumatic fever ?
Tonsillitis should be treated with penicillin V
NSAIDs
Aspirin
Prophylactic abx
Monitoring and management of complications
What are some complications of rheumatic fever ?
Recurrence of rheumatic fever
Valvular heart disease - mitral stenosis
Chronic heart failure
