Rheumatology Flashcards
What three criteria are required to diagnose JIA
Under 18 years old
More than one joint with unexplained arthritis
Lasting more than 6 weeks
What are the 4 subtypes of JIA using the new classification
1) systemic onset
2) rheumatoid positive
3) ANA positive
4) enthesitis
What is the best marker for uveitis in JIA
ANA positivity
What is macrophage activation syndrome otherwise called
Secondary HLH
What is the most telling sign on bloods with MAS
How does it present
High ferritin
Fevers, lymphadenopathy, encephalopathy and hepatosplenomegaly
What is telling about the fever in systemic onset JIA (3 things)
Twice daily
High
Rash will come with the fever
What joints are usually involved in oligoarticular JIA
Large joints- hips and knees
How many joints need to be involved to make JIA polyarticular
5 or more
Are X-ray findings present in JIA
No- not until very late
How does methotrexate work
What are it’s 3 main side effects
What is a more rare side effect
Anti folate
GI upset, mouth ulcers and mood changes
Hepatitis
What do the following DMARDs work on Infliximab/adalimumab/entanercept Rituximab Anakinara Tocilizumab Dupulimab Ecilizumab Omalizumab
What is the potential Adverse effect if used too early
TNF alpha Anti CD20 IL1 IL6 Il4/5 Terminal complement pathway IgE
Early onset interstitial lung disease
Dermatomyositis
How does it usually present
What are the 2 rashes seen
What three investigations indicate muscle involvement
Non specific weakness and fevers
Heliotropic and gottrons papules
Raised CK or LFTs, positive EMG, positive biopsy
Dermatomyositis
Which antibodies are specific
Which antibodies are associated
Anti jo
Anti sm and Ssa
Dermatomyositis
What are two possible complications
Calcifications
Interstitial lung disease
What mnemonic is used for the features of SLE
S-serositis
O- oral ulcers
A- arthritis
P- photosensitivity
B-blood R- renal A-Ana I- immunology N- nephritis
M- malar
D- discoid
What are early signs of lupus nephritis
How should it be investigated
How is it treated
Proteinuria and haematuria
Biopsy
Steroids and immunosuppression. Ace inhibitors and renal transplant
Lupus
What are the two most specific antibodies
Which antibody may be seen In Drug induced cases
Anti sm and dsDNA
Anti Histone
What are three ways antiphsopholipid syndrome can occur
Recurrent miscarriages
Clots
Valvular heart disease
What are the 2 main ways neonatal lupus presents
Does mum have to have symptoms? What antibodies is mum likely to have
What other disease could mum have
Rash from 6-8 weeks
Congenital heart block
Anti ro and la
Sjogrens
What are 4 markers of SLE disease activity
Anti DS DNA levels
Low complement levels
Raised ESR/CRP
Cytopenias
What is a serious side effect of hydroxychloroquinine
Retinal deposition causing blindness
What may be seen after blood tests in bechets
Pathergy (pustular reaction)
What is the most concerning complication of bechets
Pulmonary aneurysm
Scleroderma
What is the rash otherwise called
What is a lesion on the forehead called and why does it need more investigation
What may severe systemic scleroderma be associated with (2 things)
Morphoea
Coup de sabre- can be associated with seizures
Pulmonary hypertension and Raynauds
Familial Mediterranean fever syn What is associated with the fever What is the inheritance What protein is abnormal How is it treated What is the risk
Abdominal pain and joint pain AD Pyrin Colchicine Amyloidosis
Hyper igD syndrome What brings on the fever What other symptoms are associated What is seen in the urine Does it cause amyloidosis
Of there is a complete defect what metabolic syndrome is it?
Excitement eg holidays or birthdays
Lymphadenopathy, diarrhoea and vomiting
Mevalonic acid
Rarely
Mevalonic aciduria
TRAPs
What is associated with the fever
Can it cause amyloidosis
Severe abdominal pain and joint pain
Yes!
PFAPA
what is associated with the fever
What 2 treatments can help
Does it cause amyloidosis
What is another periodic fever syndrome with urticaria and no amyloidosis
Pharyngitis and lymphadenitis
Steroids and tonsillectomy
No!
NOMID
Which vessels are involved in
Small vessel vasculitis
Medium vessel
Large vessel
Give examples of each
Small- arteries to arterioles
Granulomatosis with polyangitis, HSP, anti GBM disease
Medium- main visceral arteries
Polyarteritis nodosa , Kawasaki
Large- aorta and main branches
Giant cell arteritis, takayasau‘s
Takayasau’s What is it otherwise called How does it present Will ANCA be positive How should it be investigated What type of inflammation is it
Pulseless disease
Non specific symptoms then pain, bruits and differential bps. Htn and seizures or strokes
No!
CT or MRI Angiography
Granulomatous
Polyarteritis nodosa
What type of inflammation is seen
Will ANCA be positive
Are the kidneys usually involved?
Necrotising
In 20%
No!
Small vessel vasculitis
What is the new name for wegeners. What finding may be seen
What type of ANCA will be positive. What else makes it likely
What does church Strauss have on bloods
Granulomatosis with polyangitis
Saddle nose deformity
CANCA
anti pr3
Eosinophilia
What can develop if a child has a bullous rash after starting naproxen
Pseudoporphyria
What antibody is highly specific for granulomatosis with polyangitis (wegners)
CANCA and PR3
Which antibody is highly specific for eosinophilic granulomatosis with polyangitis (churg Strauss)
PANCA
