Vasculitides Flashcards
Define vasculitides (incl. eosinophilic granulomatosis with polyangiitis, granulomatosis with polyangiitis, microscopic polyangitis, polyarteritis nodosa, Takayasu arteritis)
Vasculitis is the inflammation and necrosis of blood vessels. Primary vasculitides are classified according to the main vessel size affected:
- Large: Giant cell arteritis (GCA), Takayasus aortitis (TA).
- Medium: Polyarteritis nodosa (PAN), Kawasakis disease (KD).
- Small: Churg–Strauss syndrome (CSS), microscopic polyangiitis (MP), Henoch-Schonlein purpura (HSP), Wegeners granulomatosis (WG), mixed essential cryoglobulinaemia (MEC), relapsing polychondritis (RP).
Do these conditions affect small, medium or large vessels? - eosinophilic granulomatosis with polyangiitis, granulomatosis with polyangiitis, microscopic polyangitis, polyarteritis nodosa, Takayasu arteritis
Eosinophilic granulomatosis with polyangiitis = Churg-Strauss syndrome - SMALL
Granulomatosis with polyangiitis = Wegeners granulomatosis - SMALL
Microscopic polyangitis - SMALL
Polyarteritis nodosa - MEDIUM
Takayasu arteritis - LARGE
How common are vasculitides?
Small vessel vasculitis - incidence 1/10,000
TA more common in young Japanese females
PAN may affect any age (male:female ratio 2:1)
Which infection is PAN (polyarteritis nodosa) associated with?
Hep B
Which infection is mixed essential cyroglobulinaemia (MEC) associated with?
Hepatitis C infection
Which antibody is microscopic polyangiitis (MP) associated with?
pANCA
Which antibody is Wegener’s granulomatosis (WG) associated with?
c-ANCA
What is the aetiology of vasculitides?
- Unknown
- Postulated to be of autoimmune origin
- Immune complex deposition in vessel wall triggers classical complement activation and inflammation
What investigations would you do for vasculitides?
- ESR/CRP - ESR>100mm/hr, CRP raised
- ANCA -positive - ANCA is strongly correlated with certain forms of vasculitis (i.e., granulomatosis with polyangiitis, microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis)
- Serum urea and creatinine- N or raised
- Urinalysis - haematuria, proteinuria, RBC casts
- FBC - normocytic anaemia, platelets and neutrophils raised
Imaging:
- PET - demonstrates changes with vasculitis
- MRA - beading, aneurysm (in PAN) , smooth tapering vessel stenosis
- CXR - diffuse, nodular or fitting shadows, atelectasia
Invasive:
- Biopsy - vessel wall necrosis, fibrinoid necrosis, karyorrhexis (fragmentation of the nucleus and the break up of the chromatin into unstructured granules), and red blood cell extravasation
What is Churg-Strauss syndrome?
eosinophilic granulomatosis with polyangiitis
Which autoantibodies are found in these subtypes of vasculitides:
- granulomatosis with polyangiitis
- microscopic polyangiitis
- eosinophilis granulomatosis with polyangiitis
- granulomatosis with polyangiitis (WG) - c-ANCA
- microscopic polyangiitis - p-ANCA
- eosinophilis granulomatosis with polyangiitis (CSS) - p-ANCA
What are the signs/symptoms of vasculitides? (all types by system affected)
Possible features of all diseases:
- General: fever, night sweats, malaise, weight loss
- Skin: rash (vasculitis, purpuric, maculopapular, livedo reticularis)
- Joint: arthralgia or arthritis
- GI: abdominal pain, haemorrhage from mucosal ulceration, diarrhoea,
- Kidney: glomerulonephritis, renal failure
- Lung: dyspnoea, cough, chest pain, haemoptysis, lung haemorrhage
- CVS: pericarditis, coronary arteritis, myocarditis, HF, arrhythmias
- CNS: mononeurosis multiplex, infarctions, meningeal involvement
- Eyes: retinal haemorrhage, cotton wool spots
What are the features of TA?
- Constitutional upset
- head or neck pain
- tenderness over affected arteries (aorta and major branches)
- dizziness
- fainting
- reduced peripheral pulses
- hypertension
What are the features of polyarteritis nodosa?
- Microaneurysms
- Thrombosis
- Infarctions
- (e.g. causing GI perforations)
- Hypertension
- Testicular pain
What are the features of Churg-Strauss syndrome?
- Asthma
- Eosinophilia
