Flashcards in Week 224 - Vasculitis Deck (24)
What are the two more common forms of Primary Vasculitis?
Polymyalgia Rheumatica and Giant Cell Arteritis
Temporal Arteritis AKA Giant Cell Arteritis, Polymyalgia Rheumatica, Weger's Granulomatosis and Polyarteritis Nodosa are all forms of what?
How does Polymyalgia Rheumatica (the milder form) commonly present?
Pain and stiffness in neck, shoulders and pelvic girdle > worse in the morning, improves with activity
In middle aged and elderly (if presents
How does Giant Cell Arteritis (GCA - the more severe of the two) tend to present?
Fairly sudden onset and constant headache
Tenderness around scalp and temples
Which vessels are affected in GCA?
Large ones - aorta and branches coming off it
Limited to vessels with internal elastic
If left untreated what possible outcomes might there be?
- irreversible blindness
- stroke/MI/Tissue necrosis
EARLY DIAGNOSIS AND MANAGEMENT CRUCIAL
How do you diagnose GCA?
PET scan - radioactive isotope takes up glucose
Temporal artery biopsy (not everyone has them)
What is the immediate treatment for GCA?
Along with Wegner's Granulomatosis and Polyarteritis Nodosum name two more causes of 'small vessel vasculitis'
List the 3 main non-specific symptoms of small vessel vasculitis
3) weight loss
What is the reason that small vessel vasculitis can be mistaken for these other conditions?
The non-blanching rash (purpura / petichial rash)
List 4 things vasculitis can mimic
1) infectious endocarditis
3) left atrial myxoma
What causes purpura / petichae?
Leaky blood vessels
What is ANCA?
"Anti neutrophil cytoplasmic antibody"
A heterogenous group of antibodies:
- cANCA (cytoplasmic)
- pANCA (perinuclear)
- aANCA (atypical)
Not very specific but indictor of vasculitis!
ANCA together with what 2 other antibodies indicates Wegner's Granulomatosis?
MPO and PR3
Wegner's Granulomatosis is associated with what, pathologically?
Granulomas - can look like TB
What lung manifestations are associated with Wegner's?
SOB; Pleurisy; Haemoptysis due to Haemorrhagic alveolitis
What ENT manifestations are associated with Wegner's?
Sinusitis (most commonly); Otitis Media; Nasal Discharge; URT stenosis; Saddle Nose Deformity
What is associated with / characterised by aneurysms in medium sized vessels, wrist drop, foot drop and Hepatitis B?
Polyarteritis Nodosa (uncommon condition)
Necrotizing vasculitis of medium-sized vessels
What are the main aims of vasculitis (particularly small vessel) treatment?
To induce and then maintain remission
How do Polyarteritis Nodosa and Microscopic Polyangiitis differ?
Microscopic Polyangiitis - necrotizing vasculitis of small vessels; rapidly progressive glomerulonephritis usually features; associated with p-ANCA (to lesser ext. c-ANCA)
What are the 3 overarching drug types used to induce remission of primary vasculitis?
> Biologics e.g. Anti-TNFs / Rituximab
List 3 key recurrences that occur in Antiphospholipid Syndrome
> Recurrent vascular thrombosis
> Recurrent migraine
> Repeated miscarriages