Rheumatology / orthopaedics Flashcards
Name a large vessle / medium / small primary vasculitis
Large - giant cell / Takayasu
[Giant samurai]
medium
PAN, kawasaki
[Cows are cooked in medium PANS]
small
Henoch-schonlein purpura, Wegner’s granulomatosis, churg strauss, microscopic polyangitis
[German sounding + microscopic obvs]
Secondary causes of vasculitis
Aortitis in RA
Autoimmune, malignancy, durgs, infection
Eg of infective / drug / autoimmune causes of secondary vasculitis
BBV, strep
penicillin, steroids
RA, SLE, sjorgrens
What are the ANCA associated vasculities?
Wegners, microscopic polyangitis, Churg strauss, drug induced
What is ANCA? How are they detected? 2 types and associated antibody?
Anti-neutrophil cystoplasmic antibodies
Detected with indirect immunofluorescence microscopy
Cytoplasmic ANCA
Peri-nuclear ANCA
C-ANCA = Proteinase 3 (PR3)
P-ANCA = Myeloperoxidase (MPO)
69 M
Sinus problems, blocked nose with bloody discharge
No improvement with antibiotics and steroid spray
Constitutional symptoms
Hearing problems
Arthralgia
Rash on legs
Nasal tone of voice
Vasculitic rash on legs
Small joint and ankle swelling
Ix
Low Hb
Raised Ur & Cr
CRP 109
Wegners
For exams
c-ANCA / PR3 ?
P-ANCA / MPO?
c anca - wegners
p anca - churg strauss
Triad of affected areas in wegners
lung, kidney, ENT
Usual vasculitis with raised eosinophils
Churg Strauss
Often get new / worsening asthma
New name for wegners?
what and Who usually affected?
Granulomatosis with polyangitis
Necrotising vasculitis of arterioles, capillaries and post-capillary venules
Typically affects men aged 25-60
Causes of cavitation on lung XR?
wegners, malignancy, septic emboli, TB, Klebsiella, Staph aureus, pulmonary infarcts
main 3 affected organs features of Granulomatosis with polyangitis?
Upper resp - Sinusitis
Otitis
Nasal crusting & bleeding
Saddle nose deformity
Lungs
Cavitation
haemorrhage
Renal
Glomerularnephritis (haematuria/proteinura)
Also
Eyes
nervous system
heart
Gi
52 F
Increasing SOB on minimal exertion
Poor appetite
5 stone weight loss in previous 6 months
Rash on legs
PMH
Asthma
Nasal polyps
Psoriasis
Syndrome X
Β thalassaemia trait
DH
Diltiazem
Amitriptyline
Serotide
Salbutamol
Betnovate cream
O/E
Scattered wheeze
Vasculitic rash on legs
No joint swelling
Ix
Low Hb
Raised EO 5.8
CRP 291, ESR 35
CK 654
RF +
cANCA +
MPO +
What is it?
Usual organs and features?
Eosinophilic granulomatosis with polyangitis
(Churg Strauss)
Systemic necrotising vasculitis of arterioles, capillaries and post-capillary venules
Lungs
New/worsening asthma
Pulmonary infiltrates
Alveolar haemorrhage
CNS
Mono/polyneuropathy
ENT
Polyps
conductive hearing loss
allergic rhinitis
Skin
Pupura
nodules
Renal disease RARE
Common Ix in wegners/churg strauss
Hb, RF, CRP, ESR, CK
Eosinophils (raised in churg)
ANCA
Biopsy if needed
Mx of Churg / Wegners ?
Maintanence of remission?
Induction of remission
High dose steroids + Cyclophosphomide or Rituximab
Plasma exchange if life threatening/Cr >500
(if no organ threatening involvement)
High dose steroids + Methotrexate or Mycophenolate
Maintenance of remission
Methotrexate + pred + folic acid
or Azathioprine
Which vasculitis is microscopic polyangitis similar to?
Difference?
Churg straus
pANCA +ve
No granuloma formation
Predominantly affects kidneys
Main features of HSP
Children
IgA
Purpuric rash
Arthritis
Abdominal pain
Glomerulonephritis
How to remember main feature of polyarteritis nodosa ?
Other features ?
Nodosa means knots (aneurysms)
Medium vessel
Common co-infection with Hep B
Hypertension
Epididymitis
constitutional Sx
Mx of Giant cell arteritis if visual disturbance? W/O disturbance?
What else do you give?
Prednisolone 40mg if no visual disturbance
Prednisolone 60mg if visual disturbance
Dramatic response normally seen within 48 hours
May require steroid sparing agents
-PPI
-Osteoporosis prophylaxis
-Aspirin
What condition is linked to temporal arteritis ?
Polymyalgia rheumatica
Sx / signs of GCA
Headache, severe, sharp dull throbbing, wouldn’t improve without steroids
TA swollen, tender, pulseless
Diplopia, ptosis, ischaemic optic neuropathy (sudden painless monocular visual loss)
Papilloedema, haemorrhages, optic atrophy later
Features of PMR
Shoulder / pelvic pain
morning stiffness
Weakness on exertion due to pain (rather than intrinsic muscle disease)
Can start asymmetrically then becomes bilateral
Decreased appetite can lead to weight loss
3 Ix in PMR ? What is this in part for?
mX?
ESR/CRP - THESE WILL BE HIGH
RhF/ACCP
ANA
CK - THIS WILL BE NORMAL AS MUSCLES ARE ACTUALLY SPARED. THIS DISTINGUISHES IT FROM OTHER MUSCLE PATHOLOGIES SUCH AS polymyositis.
TFT
Myeloma screen
CXR
(ruling out other conditions that can present similary
RA, SLE, polymyositis, hypo/hyperthyroid, myeloma
Pred 15mg
Bone protection
PPI
The main complication is steroid induced osteoporosis
Is GCA only in temporal?
name 2 Constitutional sx
Leading question - Obvs not
15% - carotid, subclavian, axillary
Arm claudication
Paraesthesia
Digital ischaemia
Raynaud’s
Absent pulses
Aortic aneurysm & dissection
Aortic insufficiency