Vasculitis Flashcards
What is polyarteritis nodosa?
a multisystem vasculitis characterized by segmental necrotizing vasculitis involving predominantly medium-sized blood vessels.
PAN is more common in males
T - ratio of 1.5:1 M :F
Associations with PAN
Infections
- HBV
- Group A strep
Inflammatory diseases (e.g. inflammatory bowel disease),
Malignancies (especially hairy cell leukemia)
Medications
- minocycline
VEXAS ( vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic)
Genetic associaiton for children with PAN?
CECR1 gene
Clinical presentation of PAN?
Constitutional symptoms - weight loss, fever
Arthralgia
Sensory and motor neuropahty (mononeuritis multiplex)
Myagias
Abdo pain
Renal involvement - renovascular HTN and renal failure
Orchitis
thrombotic microangiopathy or vasculitic arterial occlusion resulting in:
Stroke
Ischaemic cardiomyopathy
Mesenteric ischaemia –> bowel perforation
*lungs are spared
PAN is associated with glomerulonephritis
False - it is ass with renovascular HTN and renal failure
Poor prognostic factors for PAN
GI involvement, 1 year survival rate < 50%
Cutaneous findings with PAN?
Tender erythematous nodules
Livedo Racemosa
Retiform purpura
Ulceration
Palpable purpura
Atrophe blanche
A skin predominant form of Polyarteritis nodosa occurs in 30% of cases
false - only 10%
Cutaneous PAN often progresses to systemic PAN
False - this is rare.
Ix findings for PAN
No lab test is diagnostic
ANCA - negative
Leukocytosis
Thrombocytosis
ESR often elevated
HBV and HCV should be performed
Histology of PAN
segmental necrotizing vascu-
litis of medium-sized muscular arteries
medium-sized vessels are located within the deep dermis and subcutaneous fat,
although overlying small blood vessels
and nearby fat lobules may be secondarily involved.
DIF, if performed, may show deposits of C3, IgM, and fibrin within or around vessel walls, but this is of uncertain significance.
Differences between ANCA ass vasculitis and PAN?
ANCA = glomerulonephritis and pulmonary involvement, circulating ANCAs
PAN = renovascular HTN, lacks pulmonary involvement, ANCA negative
Treatment of systemic PAN?
1st Line:
Oral corticosteroids - 1mg / kg / day
tapered over 6 months
Or Oral corticosteroids + plasmaphoresis + entecavir
One half of patients will acheive remission or cure with corticosteroids alone
Steroid spating agents:
- AZA
- MTX
- IVIG
- cyclophosphamide
Rx of cutaneous PAN?
First Line:
- NSAIDs
- Corticosteorids (oral, topical, IL)
Second line:
- Dapsone
- Colchicine
- Aza
- MTX
Third line:
- pentoxyfyline
- HCQ
- IVIG
- MMF
- TNF inhibitors
Ix for suspected Vasculitis?
Lesional skin biopsies (early lesions)
- Histology
- DIF
For CSVV - punch biopsy
For mediusm vessel - Ideally incisional, or deep punch (telescope) of a subcut nodules > reiform purpura > edge of an ulcer
Bloods:
- FBC and film
- UEC
- LFTs
- C3 / C4
- ANCA
- Cryoglobulins
- ANA, ENA, dsDNA, RF
- Hep B / C / HIV
- ASOT, anti DNA-ase B titres
- SPEP, age app malignancy screening
- pre-immunosupression screen
- G6PD (consider HLA 13:01 in chinese)
- Urinalysis
Describe
Is PAN more common in men or women?
1.5 : 1
M : F
ANCA ass vasculitis - disease and antibody association
Acute haemorrhagic oedema is more common in Males (70%) compared to females (30%)
True
Who does acute haemorrhagic oedema of infancy affect?
Children < 2 years old
Boys
How can you distinguish between urticaria and urticarial vasculitis
Urticarial vasculitis:
Duration of lesion
Resolution with bruising
Pain
Brown lesion of diascopy
Ix for urticarial vasculitis?
ANA
ENA
C3 / C4 / C1q solid phase assay
FBC
UEC - urinalysis
ESR
