Rheumatology Flashcards
JUVENILE IDIOPATHIC ARTHRITIS
i) what occurs in the joints? how long does it last? under what age? name three key features
ii) what is systemic JIA aka? what type of rash is seen? is there fever? will ANA and RF be pos or neg? which blood markers will be raised? what is a key complication?
iii) how many joints does polyarticular JIA involve? is it symm or asymm? name three symptoms? what is it similar to in adults?
iv) what is pauciarticular JUA aka? how many joints does it involve? what type of joints does it affect? what is it especially associated with?
v) name four drugs that can be given
i) autoimmune inflammation in the joints under the age of 16 and lasting more than 6 weeks
joint pain, swelling, stiffness
ii) systemic aka stills disease
see subtle salmon pink rash with high swinging fevers
negative ANA, RF but raised inflam markers eg CRP, ESR, plats and ferritin
key complication is macrophage activation syndrome - massive imm sys activation > DIC > low ESR
iii) polyarticular is 5 joints or more > symmetrical
mild fever, anaemia, reduced growth
similar to RA in adults but most children will be RF negative
iv) pauciarticular aka oligoarticular - 4 joints or less
affects larger joints eg knee or ankle
classic assoc with anterior uveitis - ANA positive
v) NSAIDS eg ibuprofen, steroids (oligo), DMARDs eg mtx, sulfasalazine, biologics eg TNF inhibitors eg etanercept, inflix, adalimumab
EHLERS DANLOS SYNDROME
i) what is it an umbrella term for? what is the key feature seen in patients? how does the skin appear?
ii) how is wound healing impacted? name two things they are prone to? what is the inheritance?
iii) which type has fragile blood vessels, thin translucent skin and arteries prone to rupture
iv) name five symptoms?
v) what score is used to assess hypermobility? what does it comprise of (5)
i) umbrella term for genetic conditions that cause defects in collagen
results in hypermobility and abnormalities in connective tissue
skin appears soft and stretchy
ii) abnormal wound healing, prone to hernias, prolapse, mitral regurg and aortic root dilatation
inheritance is auto dominant
iii) vascular EDS
iv) hypermobile joints, joint pain post exercise/inactivity, joint disloc, soft skin, easy bruise, bleeding, headaches
v) beighton score for hypermobility used to support dx
palms flat on floor, elbows hyperext, knees hyperext, thumb bends to touch forearm, little finger extends past 90 degrees
EDS MANAGEMENT
i) what is used to make a diagnosis? what is it important to exclude?
ii) what may patients develop at an earlier age?
iii) what other syndrome can occur with EDS? what is it a result of?
i) use beighton score to diagnose
exclude marfans - will have high arch palate, archnodactyly and high arm span
ii) may dev OA at an earlier age
iii) POTS can also occur with EDS due to autonomic dysfunction
HENOCH SCHONLEIN PURPURA
i) what is it? what does it present with? why does inflammation occur? which three things does it affect? what can it be triggered by?
ii) what are the four classic features? which one is seen in all patients? where does it start? what other problem do 75% of patients develop?
iii) name three gastro complications that can occur?
iv) what is seen in the kidneys? what may be seen in the urine? what has developed if there is more than 2+ protein on dipstick?
i) IgA vasculitis that presents with purpuric rash on lower limbs and buttocks
inflam occ due to IgA deposits in the blood vessels > leaky > purpura
aff skin, kidneys and GI tract > often triggers by UTRI or gastroenteritis
ii) classic features are purpura, joint pain, abdo pain, renal involvement
all patients have purpura - start on legs > buttocks
75% of patients have joint pain in knees and ankles
iii) gastro involvement > can lead to GI haemmorhage, intussception and bowel infarction
iv) kidneys - IgA nephritis
can lead to macro/micro haematuria and proteinuria
if more than 2+ urine dip = child has nephrotic syndrome and will have a degree of oedema
DX AND MX OF HSP
i) name two things that need to be excluded? name two other DDs for a non blanching rash
ii) what blood marker will show whether there is nephrotic syndrome? which criteria may be used to diagnose
iii) name four other things patient must have one of to use EULAR criteria
iv) how is it managed? are steroids routinely used? name two things that need to be monitored during course of disease
v) how long does abdo pain take to settle? how long does it take to recover if kidney involvement?
i) need to exclude meningococcal septicaemia and leukaemia
other DDs are ITP and haemolytic uraemic syndrome
ii) raised serum albumin > nephrotic syndrome
EULAR/PRINTO/PRES criteria - patient needs to have palpable purpura
iii) diffuse abdo pain, arthritis or arthralgia, IgA deposits on histol, proteinuria/haematuria
iv) managed supportively - analgesia, rest, hydration
steroid use is debateable - may not affect long term outcomes but may be used when severe GI pain or renal inolve
monitor BP and urine dip for prot/haematuria
v) abdo pain settles in a few days, kidney involve 4-6 weeks
KAWASAKI
i) what is it? who does it usually affect? what is a key complication
ii) what type of fever is seen? for how long? what type of rash is seen? what happens to soles and palms?
iii) name three other features seen?
iv) what is seen in the acute phase? (3) how long does it last? what is seen in subacute phase? (2) how long? hwat is seen in convalescent phase? how long?
v) what are the two medical treatments given and what is each for? what monitoring till a patient need?
i) systemic medium sized vessel vasculitis > affects children under 5yrs
key complication is coronary artery aneurysm
ii) persistent high fever above 39 for more than 5 days
widespread erythematous maculopap rash and desquamation of soles and palms
iii) strawberry tongue, cracked lips, cervival LNs, bilateral conjunctivitis
iv) acute lasts 1-2 weeks - fever, rash, lymphadeno
subacute lasts 2-4 weeks - acute symp settle > desquam and arthalgia, risk of CA aneurysm
convalescent stage - 2-4 weeks - remaining symptoms settle, blood tests return to normal and CA aneurysms may regress
v) high dose aspirin to reduce risk of thrombosis
IV Ig to reduce risk of CA aneurysm
monitor with echo
aspirin is usually avoided due to risk of reyes syndrome
RHEUMATIC FEVER
i) what is it? what is it triggered by? what specific bacteria is it caused by? what type of hypersensitivity reaction is it?
ii) when does RF presentation occur after bacterial infection? what infection may be seen? what type of arthritis can it cause?
iii) how does it affect the heart? name two skin findings?
iv) what is a key nervous system symptom? name three investigations that should be done
?
i) autoimmune condition triggered by streptococcus bacteria (group A strep - strep pyogenes that has caused tonisilitis)
type II hypersens reaction
ii) occurs 2-4 post strep infection eg tonisilits
may see migratory arthritis - different joints become inflamed and improve at different times
iii) causes carditis (heart inflam) eg pericarditis, myocarditis, endocarditis
skin - subcut nodules (firm painless nodules on ext surfs of joints eg elbows) and erythema marginatum rash (pink rings on torso and limbs)
iv) chorea is key NS complication
investigations: bacterial throat swab, ASO antibody titres, echo/ECG/CXR
RHEUMATIC FEVER IX AND MX
i) what criteria is used to make a diagnosis? what are ASO antibodies?
ii) when do ASO level peak? when are they repeated?
iii) what criteria is used (JONES FEAR) name each
iv) what abx is usually given to treat the strep? name three other medications that may be given
v) name three complications
i) jones criteria
ASO antibodies - anti strep antibodies > indicate recent strep infection and can help support a dx
ii) peak around 3-6 weeks > repeat after 2 weeks
iii) Joint arthritis Organ inflam eg carditis Nodules Erythema marginatum rash Sydenham chorea
Fever Ecg cganges (prolonged PR) Arthalgia Raised inflam markers
iv) pen V for 10 days
also give NSAIDs for joint pain, aspirin/steroids for carditis, prophylactic abx to prevent further strep infections and recurrence
v) recurrence, valvular heart disease eg mitral stenosis, chronic hear failure
