Vasculitis Flashcards
Tocilizumab is routinely used in the treatment of which form of vasculitis?
GCA
Is there any role for plasma exchange in ANCA vasculitis?
No
What agent is used for induction and maintainance in severe ANCA vasculitis (aside from steroids)?
Rituxumab
What are some cardinal features of GCA?
- Old pt, nil before Age of 50
- Vission distirbance (Painless loss of vision, unilateral)
- Diffuse headache (worse over temporal / parietal regions) / Tender scalp or pain when combing hair
- Jaw claudication
- PMR
- Constitutional upset (evidence of inflammation ie night sweats, LOW)
What is the next step in someone who has vision loss due to suspected GCA (not confirmed)? What is they dont have vission loss / symptoms?
High dose steroids - IV methyl pred
Give high dose pred in GCA but nil vision changes
What is the key feature of GCA?
- Mural inflamation (full thickness)
- Multinucleated giant cells (hence GCA)
- Thickening of the intima - this is what results in the steonsis
What is a steroid sparing regime for GCA treatment?
Add tocilizumab and wean steroids over 6 months
What feature is most likely to be associated with GCA (ie highest likelihood ratio)?
Jaw claudication
What size of vessel does GCA affect? Does it affect a particular part of the body or is it systemic?
Medium to Large vessel vasculitis
Systemic, manifestations are mainly GCA but affects every where equally
How does PMR present?
Morningt stiffness in the neck and shoulders, sometimes the hips too
- Mainly difficulty when reaching above the head
What should be given to a patient on high dose steroids?
High dose PPI
Calcium and vitamin D
What is the gold standard for Dx GCA? What are the other two diagnostic options?
Temporal artery Bx is still gold standard because can be done and interpreted by many people
Temporal artery ultrasound
- Needs to be done by experienced staff in high volume centre. Needs to scan temporal and axillia at a minimum to get enough info for Dx
Temporal artery PET scan
- FDG PET but focused on temporal, axilliary and aorta arteries
Why are there occasional false negatives with temporal artery Bx?
Specific Bx segmnent may not be involved, this is why need a descent length of vessel
May just be predominatly afecting the large vessels IE the aorta
What are teh USS features of GCA?
Hypoechoic wall thickening (intimal thickening) which looks like a halo (halo sign)
What is tocilizumab? what vasculiutis is it involved in management of?
IL6 receptrors antagonist
Used routinely unless contraindication as a steroid sparing agent in managment of GCA
Historial treatment (nil toc) involve 1-2 year pred wean in vunrable population
What are the vascular complication of GCA?
VIssion loss (permanent)
- anterior ischemic optic nephropathy
Aortic aneurysm and dissection
Stroke (mostly posterior circulaiton stroke due to vertebral artery disease)
Limb claudication (uncommon)
Pt Dx GCA 10 years ago, now presents with chest pain. Dx?
Dissection / aneurysm
What form of vasculitis is takayasu? Who doe it affect?
Large vessel vasculitis
Affects younger asian pts (unlike GCA that is a large vessel diverticulitis that affects older white pts)
What arteries are primarily involved in takayasu arteritis?
Aorta and primary branchs
- as opposed to GCA which is medium and large vessel
Young pt, asian, constitutionally unwell, presents with a stroke / loss of a pulse in left arm. Dx?
Takayasu arteritis
- Too young for atherosclerosis
- too young for FMD
What size of vessels does PAN affect?
Medium vessels only
What infection is PAN associated with?
hep B and C
Constitutional Sx, neuropathyrash, renal failure or infarcts with elevated ESR/CRP and ANCA negative. WHat Dx?
PAN
What are the features of PAN?
- Evidence of inflammation (constitutional Sx, CRP and ESR raised)
- often neuropathy
- Rash, painful and ulcerating
- renal involvment - renal failure or infarcts therefore nil active sediment
- Serology negative (ie nil ANCA)
How is Dx PAN confirmed?
Bx: skin (deep skin Bx to involve arteries), nerve/muscle, kidney
Imaging: distal mesenteric and intrartenal aneurysms
What are features of PAN on imaging?
Mostly small renal artery aneurysms, but can have aneurysms in the main renal arteries
How is PAN dif from FMD?
FMD:
- beading of the main renal arteries but NOT inflammatory (therefore nil constitutiuonal symptoms, nil esr or crp)
- Sapring of the proximal third of renal artery
- Usually HTN
- Pulsitile tinitus
- Nil sytemic symptoms
What are some examples of small vessel vasculitis?
- ANCA vasculitis (MPA, GPA, EGPA)
- Connective tissue disease related (RA, SLE, Sjogrens) - - Other immune complex: IgA vasculitis (HSP), cryoglob
- Infections: HepB/C, HIV
- Drug induced: Cocaine
- Malignancy: haematological most common
Suspect someone has GPA. What is the immidiate next step?
Urinalysis then renal Bx
- Dont need to give immediate steroids because unlike GPA, ANCA vasculitis progresses over several days not hours?
What is the typical neruological finding in a small vessel vasculitis?
Mononeuritis multiplex (painful multifocal peripheral neuropathy)
What are the varies clinical findings in small vessel vasculitis grouped by organ system?
- Constitutional: fever, weight loss, sweats
- Skin: palpable purpura
- Joint: non errosive inflamatory arthropathy
- Nerves: painful multifocal peripheral neuropathy
- ENT: blood discharge, bony distruction
- Kidneys: active sediment, HTN, HTN
- Lungs: Alveolar haemorhage, lung mass
- Eyes: scleritis, orbital masses
What are features of small vessel vasculitis on skin Bx (On LM and IF)?
Fibrinoid necrosis and leukocytoclastia
- Basically fibrin in the wall and neutrophil dust =/-0 extrapolated red cells
IF
- If IF shows IgA staining then this is HSP (IgA vasculitis)
Where are palpable purpura typically found. Why are they found here?
Legs
this is because of the effect of gravity. Friable vessels + gravity = more likely to burst and cause palpable purpura
It is palpable because of the leucytoclastic, it is non blanching because red cell have extravasated. It is non painful because very small vessel affected
If large leision ie ulcers or painful rash then more likely to be a medium, vessel vasculitis ie PAN
What blood tests are used to work up a small vessel vasculitis?
- ANCA: ANCA (PR3, MPO), GBM (if renal and lung inv)
- CTD: ANA, ENA, dsDNA, C3, C4, RF, CCP
- Infection: HIV, HBV, HCV +/- strep serology and BC
- Cryoglobulin: Cryoglobulin
- Malignancy: FBE, consider SPEP and FLC
All types need urinalysis, ACR, PCR
Baseline bloods
GPA vs MPA vs EGPA?
GPA:
- cANCA (PR3)
- Granulomatous inflamation
-> Necrotising sinusoidal disease
-> Orbital masses
- Vasculitis
-> GN
-> Pul haemorhage
-> skin findings, neuropathy
MPA:
- pANCA and MPO+
- Typically just the vasculitis change, nil granulomatous inflamation
EGPA (This is a cross over condition between vasculitis and eosinophilic asthma (eosinophilic asthma Plus syndrome). Pts with have asthma and eosinophils PLUS:
- Nasal polyps
- Eosinophilic rich rash
- ANCA vasculitis changes
WHat is PR3 and MPO associated with?
PR3
- GPA
MPO
- MPA, EGPA
Treatment of ANCA vasculitis involving major organ?
Induction:
- Ritux OR cyclophosphamide
- Steroid (low dose PEXIVAS protocol)
- No role for PLEX in ANCA (would use in anti GBM)
Maintainance:
- Ritux
Pt with severe ANCA, contraindication to sateroids. WHat is used for induction?
Ritux and avacopan (C5a receptor inh)
Treatment for EGPA?
If severe, major organ involvement then treat as for GPA
But if more of a relapsing asthma picture or recurrent mild EGPA then consider steroid + IL5i:
- Mepolizumab
- Benralizumab
This is a similar approach for hard to control eosinophilic asthma
What are the three conective tissue disease associated with vasculitis?
RA
SLE
Sjogren
Rare in all three of these conditions
How is CTD associated vasculitis treated?
Treateds as a severe version of the underlying CTD
- ie treat lupus diverticulitis as would lupus nephritis
Pt has ANCA vasculitis with urine sediment but normal RF. What is next step?
Renal Bx and then treat if positive
- will progress to have renal failure if not treated promptly
Pt has IgA vasculitis with urine sediment but normal RF. What is next step?
Repeat urinalysis in 1 week, usually self limiting
What is the clinical tetrad assocaited with HSP?
- Palpable purpura
- Arthritis
- Nephritis
- Abdo pain
HSP is typically self limiting. WHen would immunosupression be indicated?
Rapidly progressive disease (ie with declining renal function)
What is a cryoglobulin? How do they cause vasculitis?
This is an antibody that forms precipitate in the cold
They can bind complement and activate the immune system to cause an inflammatory vasculitic syndrome. THis is called mixed cryoglobulinaemia syndrome)
If very high concerntrations, then can form vascular occlusion via precipitates in cold extremities
What are disease associations with Cryoglobuliuns?
- HCV - mixed pattern
- Autoimmune conditions (esp Sjogrens) - mixed pattern
- Haem malignancy (typically monoclonal cryoglobulin - ie type 1 cryoglobulin)
Pt with long standing sjogrens, now has palpable purpura. Also has low complement , RF positive. What Dx?
Cryoglobulinaemic vascultis
Typical clinical picture in cryoglobulin?
CLinical:
- looks like systemic small vessel vasculitis with skin, nerve and kindey inv
BLoods:
Low complement (C4)
Typically RF positive
Cryoglobulins
Treatment of cryoblobulinaemic vasculitis?
HCV:
- treat Hep C
Idiopathic:
- steroids +/- ritux, plasma exchange
