Rheumatology Flashcards
7 types of JIA
SUP POPE
Systemic arthritis
Undifferentiated arthritis
Polyarthritis (RF negative) 5+ joints in 6 months need RF tested two occasions at least 3m apart
Polyarthritis (RF positive)
Oligoarthritis (1-4 involved, often ANA positive, most common)
Psoriatic arthritis
Enthesis-related arthritis
ALL have eye exam (uveitis in 20-30%)
Felty syndrome (3)
RA, Neutropenia and splenomegaly
Enthesitis related arthritis
HLA B27 80% Male over 6y Anterior uveitis Ank spend IBD Dactylitis (also with sickle cell and psoriatic arthritis)
therapy for SLE
Hydroxychloroquine (standard) Steroids Azathioprine Mycophenolate mofetil Cyclophosphamide Rituximab
SLE most deaths related to..
Infection
Drugs that cause drug induced lupus
Minocycline
Anticonvulsants
Hydralazime
TNF agents
Granulomatosis with polyangitis triad
Small vessel
Upper and lower respiratory tract inflammation and renal disease
ANCA positive
HSP other name
IgA vasculitis
HSP recurrence rat
and end stage renal disease rate
Recurrence in 1/3rd
ESRF in 1-3%
Behcet criteria and mx
Recurrent mouth ulcers (exhibit pathergy) Recurrent genital ulcers Eye lesions Skin lesions Pathergy
Mx steroid, colchicine etc
Juvenile dermatomyositis criteria
Symmetric proximal muscle weakness
Skin changes - gottron papules knuckles and eyelid rash (50% have photosensitivity and can have malar rash)
Elevated CK, AST, LDH, Aldolase
Abnormal EMG and muscle biopsy
Positive anti jo and Ro indicate high risk early death
Limited systemic sclerosis 5 features
CREST previous name Calcinosis Raynaud Eosophageal dysmotiliy Sclerodactyly Telangiectasia
Raynauds Tx
Nifedipine
Nitroglycerin 2% ointment for digital ulcers
Tx for chorea
Carbamazepine
Phenobarbital
Haloperidol
Chlorpromazine
Macrophage activation syndrome
Cytokines storm with 10-30% mortality Pancytopenia Fever Splenomegaly High TG, High ferritin, high ddimer Decreased fibrinogen (DIC) Deranged LFTs
Haemophagocytosis of bone marrow
Persistent high CRP but decreasing ESR (consumption of fibrinogen - DIC)
Anti-glomerular basement membrane antibodies seen in
Goodpasture syndrome (IgG deposits BM with crescenteric changes))
B cell depletion agent
Rituximab
IL-1 inhibitors
Anakinra
Canakinumab (very impressive SJIA)
Rilonacept
How much normal population HLA B27 positive
10%
Criteria for SLE
Rash on maids
Renal Arthritis Serotitis Haematological Oral uk era Neurological Malar rash ANA (most sensitive) Immunological Discoid rash Sunlight sensitivity
Periodic fevers (PFAPA vs FMF)
PFAPA well in between and self resolves, 0% risk amyloidosis and FmF 40-75% risks amyloidosis
Livedo reticularis indicates..
Mottled purple skin can be from: Anti phospholipid Systemic lupus erythematosus Dermatomyositis Rheumatoid arthritis Polyarteritis nodosa Granulomatosis with polyangiitis Sjogren syndrome
C-ANCA in
Wegeners
Reacts against proteinase 3
(P-ANCA in microscopic polyangitis against myeloperoxidase)
Churg-Strauss
Atopic/asthma and multi system vasculitis
P ANCA positive
Large vasculitis
Medium vasculitis
Small vasculitis
Large vasculitis - GCA, takayasu
Medium vasculitis - PAN, Kawasaki
Small vasculitis -Chung-Strauss, HSP, wegners, microscopic polyangitis
JIA arthritis for how long at least
6w
Intra articulate steroid lasts for at least
4 months
Triamcinolone hexacetonide
Joints most effected by psoriatic arthritis
Polyarticular arthritis involving DIP joints
Radiologic changes diagnostic of ank spond
Sclerosis of sacroiliac joint margins on X-ray
Most specific marker SLE
Anti smith
DsDNA
Pemphigus biopsy
Is a rare autoimmune skin disease causing blisters skin and mucous membranes
Biopsy shows intraepidermal blister with IgG deposits
SJS biopsy
Full thickness epidermal necrosis
Can eight loss improve cutaneous psoriasis
Yes
Psoriasis treatment
Calcipotriol (vitamin d analogue) Steroids topically Dithranol Coal tar UVB MTX Biological agents
SLE percent ANA positive
ANA- 97% positive
What does aspirin do in Kawasaki
Aspirin protects from coronary events but not development of aneurysms
Biological big risk
Infection (deactivation TB)
Anti CCP antibody positive in most with…
JIA
Nikolskys sign is and reflects what
Elicit by applying pressure to affected skin and there is extension of blister or removal of epidermis where touched
Positive in pemphigus Vulgaris and negative in bullies pemphigoid
Reflects autoimmune disease
Heliotrope in what? Blue violet discolouration of eyelids
Dermatomyositis
RF positive in
Sjogren (also anti ro and la) and JIA
Raynauds, tight thick skin, ulcers
Salt and pepper skin
Positive anticentromere
Systemic sclerosis (scleroderma) Rare in children
AntiJo antibodies in dermstomyositis relates to
Risk of interstitial lung disease
Rash of systemic JIA
Salmon colour evanescent discrete borders best den with fever migratory
Hunchback child called
Sheuermanns disease
Back pain and fever in preschool child with narrowing of vertebral disc on X-ray consistent with
Discitis
Percent with arthritis in IBD
15%
Reiters/reactive arthritis occurs 1-4 weeks following what infections
Salmonella, shigella, campylobacter
TB
Yersinia
Chlamydia
Calcinosis in
Inherited CT diseases
Hypercalcaemia
Connective tissue disease (dermatomyositis, systemic sclerosis, cutaneous lupus erythematosus)
10 year old with 1-3 day fever episodes wth serositis, knee arthritis with rash over joint
Familial Mediterranean fever
Colchine
Painful rash migrates distally with exercise in 3 year old 2-6 times per year
TRAPS
AD
Gene TNFRSF1A
25% get amyloidosis
Fever every 21-28 days and ulcers adenitis syndrome
PFAPA
Periodic fever, aphthous ulcer, stomatitis, pharyngitis, adenitis
Prednisone
Adenotonsillectomy
(cutaneous hypersensitivity to superficial
trauma).
Koebner phenomenom
Dermatomyositis facial rash
Facial erythema crossing nasolabial folds (malar rash in SLE does not
involve nasolabial folds).
FMF and PFAPA treatment
FMF colchicine and amyloid
PFAPA p for prednisone
Keppra/ leveteracitam SE
Tiredness
Aggression/behaviour change
DIP joints and Nails may show pitting, yellowing, transverse ridges or destruction (onycholysis)
Psoriatic arthritis
Three causes of photosensitivity
SLE
Dermatomyositis
Psoriasis
