ANCA ass vasculitis Flashcards
What are ANCAs?
Predominantly IgG antibodies directed against components of primary granules of neutrophils and **monocyte lysosomes **
List the three subtypes of ANCA vasculitis?
GPA - granulmatosis with polyangitis
**MPA **- microscopic polyangitis
EGPA - Eosinophilic granulomatosis with polyangitis
What are the key cutanoues features of GPA?
Purpuric macules and papules
Friable, papular gingivae (strawberry gums), oral ulceration
Pyoderma like ulcers
Subcutaneous nodules
Palisaded neutrophilic and granulomatous dermatitis
What are the non-cutanoues features of GPA?
**Upper resp Tract: **
- sadle nose deformitiy,
- epistaxis,
- nasal crusting, sino-nasal congestions
- conductive or sensorineural hearing loss
- subglottic stenosis
Lower Resp tract:
- nodules
- mass
- cavitations
Renal:
- pauci immune glomerulonephritis
Ocular:
- proptosis, scleritis
Less common:
***Neuro
GI
Cardiac involvement *
Histology of GPA?
Histologic findings may be nonspecific (e.g. perivascular lymphocytic infiltrates), or they may demonstrate **LCV **and/or **granulomatous inflammation. **
What does a biopsy of a sub cut nodule in GPA show?
palisading neutrophilic dermatitis with areas of granulomatous inflammation
surrounding foci of basophilic necrobiosis, i.e. palisaded neutrophilic
and granulomatous dermatitis
How is GPA treated?
Oral corticosteroids (1mg / kg)
- tapered over 4 -6 months
In combination with Rituximab or Cyclophosphamide
Maintenance:
- Rituximab (1st line)
- Aza
- MMF
- MTx (careful with renal disease)
What antibodies are associated with GPA?
c-ANCA
anti PR3 antibodies
What antibodies are associated with Microscopic Polyangitis?
p-ANCA
anti - MPO
Clinial presentaiton of MPA?
Constitutional symptoms
20 - 70% develop cutanoues involvement
- most commonly petechiae
and purpura
- livedo reticularis and racemosa are more common in (MPA >GPA or EGPA)
- painful papules or nodules
- ulcers
- gangrene
Livedo reticularis and racemoa is more common in MPA than GPA?
True
Skin invovlement in MPA is associated with higher rates of renal and pulmonary complicaitons?
False - lower
Skin invovlement in MPA is associated with higher rates of MSK and neurological issues.
True
Extracutaneous manifestaion of MPA?
Renal involvement (90%)
- pauci immune crescentic** necrotising glomerulonephritis**
Pulmonary
- capiliritis
- alveolar haemorrhage (10%)
- ILD
Neurological
- peripheral neuropahty
- mononeuritis multiplex
Upper resp tract involvement
ILD is more common in GPA than MPA
False
MPA is progressive and often leads to renal failure and pulmonary haemorrhaeg
True
Recommended Ix for extracutanoues features of MPA?
CXR or CT
Nerve conduction studies
Lung, nerve or kidney biopsy
What are the histo findings of MPA?
segmental necrotising vasculitis of small blood vessels > medium BVs
No granulomatous inflammation
Treatment of MPA?
Oral corticosteroids
+ Ritux or cyclophosphamide
Maintenance:
- Rituximab (1st line)
- AZA
- MMF
- MTx
Patients with GPA have a lower risk of relapse than MPA?
False
What is EGPA?
An ANCA ass vasculitis, characterised by vascular and extravascular granulomas, eosinophil rich pulmonary infiltrates and necrotising vasculitis of small and medium vessels
EPGA is associated with?
Asthma
Eosinophillia
