Rheumatology Flashcards
What are the features of systemic juvenile idiopathic arthritis
Still’s disease
Salmon pink rash
Swinging high fevers
Enlarged lymph nodes
Weight loss
Joint inflam and pain
Splenomegaly
Muscle pain
Pleuritis/ pericarditis
Systemic- affects everything
Markers - CRP and ESR, platelets and serum ferritin will be up
No antibodies positive
When fever lasts over 5 days think stills disease or Kawasaki’s
What is a key complication of Systemic JIA
Macrophage activation syndrome - massive inflam response
Acute unwell child with DIC
Anaemia, thrombocytopenia
Bleeding and non blanching rash
Life threatening
Key finding: LOW ESR
What are the features of polyarticular JIA
Idiopathic inflam arthritis in 5 joints or more
Symmetrical arthritis with minimal systemic symptoms
Mild fever, anaemia and reduced growth but mild compared to systemic
The equivalent of RA in adults
Negative RF as it is seronegative
Can be seropositive (RF positive)- in older/ adolescent kids
What are the features of oligoarticular JIA
Involves 4 joint or less
Usually only a single joint- larger joints usually knee or ankle
Girls under 6 usually
Associated with ANTERIOR UVEITIS
Refer to opthalmology
ANA antibodies are positive but inflam markers neg or mildly elevated
What are the features of enthesitis related arthritis
Male children over 6
Inflam arthritis and enthesitis
HLA B 27 =gene
Look for signs and symptoms of psoriasis and IBD
Prone to anterior uveitis
Patient will be tender at the entheses
What are the features of juvenile psoriatic arthritis
Symmetrical affecting small joints or asymmetrical affecting large joints
Will have
Pllawues of psoriasis
Nail pitting
Onycholysis
Dactlylitis
Enthesitis
What is the management of JIA
MDT and paeds rheumatology
NSAIDs
Steroids
DMARDS- methotrexate, sulfasalaizne, leflunomide
Biologics
What are the features of hyper mobile Ehlers- Danlos syndrome?
Autosomal dominant
Hypermobile Ehlers-Danlos syndrome - most common and least severe- joint hyper mobility and soft stretchy skin
Joint dislocations
Stretch marks
Easy bruising
Poor wound healing
Bleeding
Chronic pain/ fatigue
Headaches
GORD
IBD
Menorrhagia/dysmenorrhea
Urinary incontinence
Pelvic organ prolapse
Temporomandibular joint dysfunction
What are the features of Classic Ehlers danlos
Classical Ehlers-Danlos syndrome - very stretchy skin, smooth and velvety, severe joint hyper mobility, joint pain, abnormal wound healing, lumps over pressure points, hernia, prolapse, mitral regurgitation, aortic root dilation- autosomal dominant
What are the features of vascular ehlers danlos syndrome
Vascular Ehlers Danlos syndrome- Most severe- blood vessels prone to rupture, thin translucent skin.
GI perforation, spontaneous pneumothorax. Autosomal dominant
What are the features of kyphoscoliotic ehlers-Danlos syndrome
Poor muscle tone as a neonate and infant
Kyphoscoliosis
Joint hypermobility
Joint dislocation is common
Autosomal recessive
What are the features of postural orthostatic tachycardia syndrome (POTS)
Can occur with hyper mobile Ehlers-Danlos syndrome
Autonomic dysfunction
Tachycardia, presyncope, syncope, headaches, disorientation, nausea and tremors
What scoring system is used in Ehlers Danlos
Beighton score- assess hyper mobility
One point for each side of the body- max score of 9
-Put arms flat on floor with straight legs
-Hyperextend elbows
-Hyperextend knees
-Bend thumb to forearm
-Hyperextend little finger past 90 degrees
What is the management of Hypermobile Ehlers Danlos
No cure
Follow up
Physio, OT, psychology
What are the classic features of Henoch Schonlein purpura
IgA vasculitis- IgA deposits in blood vessels
Children under 10
Purpuric rash affecting lower limb and bum
Usually triggered by an upper airway infection/ gastroenteritis
4 features
Purpura
Joint pain
Abdo pain (GI haemorrhage, intussception, bowel infarction)
Renal involvement (IgA nephritis- haematuria and proteinuria with some oedema)
How is Henoch Schonlein purpura diagnosed
Rule out meningococcal septicaemia and leukaemia
Patient needs to have palpable purpura (not petechiae- bigger)
And at least one of
-Abdo pain
-Arthritis/ arthralgia
-IgA deposits on histology
-Proteinuria/ haematuria
What is the management of Henoch Schonlein purpura
Supportive
Monitor urine dipstick and BP
Without kidney involvement full recovery in 4-6 weeks
1/3 patients will have reoccurrence in 6 months
Small portion will have end stage renal failure
What are the features of Kawasaki disease
Systemic medium sized vasculitis
Typically under 5s
More common in boys and asians
Key complication- Coronary artery aneurysm
Features
*Persistent high fever >39 for more than 5 days
*Erythematous maculopapular rash with desquamation on palms and soles (peeling)
*Strawberry tongue
-Cracked lips
-Cervical lymphadenopathy
-Bilateral conjunctivitis
Bloods will show
Low plts and low WBC
LFTS- hypoalbuminaemia and high ALT/AST
ESR raised
What is the disease course in Kawasaki disease
Acute phase- 1-2 weeks- child most unweel- fever, rash, lymphadenopathy
Subacute phase- 2-4 weeks desquamation and arthralgia- risk of coronary artery aneurysms forming
Convalescent stage- 2-4 weeks- Symptoms settle and aneurysms may regress
What is the management of Kawasaki disease
High dose aspirin (reduce thrombosis risk)
IV immunoglobulins to reduce coronary artery aneurysm risk
Close follow up with echos
What are the features of rheumatic fever
Autoimmune condition triggered by strep
Usually group A strep -Strep pyogenes causing tonsillitis
Type 2 hypersensitivity reaction - 2 -4 weeks after infection - immune system attacks own cells
Presentation
Previous tonsillitis
Fever
Joint pain - migratory- different joints at different times
SOB
Rash- erythema marginatum- pink rings or torso and upper limbs
Chorea
Subcutaneous firm painless nodules on extensor surfaces
Carditis- murmurs, pericardial rub, HF, tachycardia/brady
Complications
Recurrence
Mitral stenosis
Chronic HF
What investigations are carried out in rheumatic fever
Throat swab
Echo, ECG, CXR
ASO antibodies
What criteria is used for diagnosing rheumatic fever
Jones criteria- Scott Jones has rheumatic fever and gives me the fear
Two major criteria or One major and two minor
Major criteria JONES
-Joint arthritis
-Organ inflam eg. carditis
-Nodules
-Erythema marginatum rash
-Sydenham chorea
Minor criteria - FEAR
-Fever
-ECG changes (prolonged PR) without carditis
-Arthralgia without arthritis
-Raised inflam markers
What is the management of rheumatic fever
Refer immediately for specialist
-NSAIDs for joint pain
-Aspirin and steroids for carditis
-Propylactic abx (oral/ IM penicillin)
Monitor for complications
