Vasculitis Flashcards
(55 cards)
What are vasculitides?
Inflammation and subsequent necrosis of blood vessels leading to tissue ischaemia or infarction; any organ system may be involved.
When should vasculitis be suspected?
Cases of unexplained multiple organ ischaemia or systemic illness with no evidence of malignancy or infection
What is cANCA?
Circulating anti-neutrophil cystoplasmic antibody associated with anti PR3
What is pANCA?
Perinuclear anti-neutrophil cytoplasmic antibody associated with multiple antigens (e.g. lactoferrin in IBD; myeloperoxidase in microscopic polyangitis)
What are the features of small vessel vasculitis?
- Palpable purpura
- Vesicles
- Chronic urticaria
- Superficial ulcers
What is the Churg Strauss Triad?
- Allergic rhinitis and asthma
- Eosinophilic infiltrative disease resembling pneumonia
- Systemic vasculitis often mono neuritis multiplex / peripheral neuropathy and peripheral eosinophilia
What are the features of medium vessel vasculitis?
- Livedo reticularis
- Erythema nudism
- Raynaud’s phenomenon
- Nodules
- Digital infarcts
- Ulcers
What are the medium vessel vasculitides?
- Polyarteritis nodosa
- Kawasaki’s
What are the large vessel vasculitides?
- Giant cell /temporal arteritis
- Takayasu’s arteritis
How is small vessel vasculitis classified?
ANCA-associated and non-ANCA associated
What are the non-ANCA associated small vessel vasculitides?
- Predominately cutenaous vasculitis
- Henoch-Schoenlein purpura
- Essential cryoglobulinemic vasculitis
What are the ANCA associated small vessel vasculitides?
- Granulomatosis with polyangitis (GPA; aka Wegener’s)
- Eosniophilic granulomatosis with polyangitis (Churg Strauss Syndrome)
- Microangiopathic polyangitis
What is Henoch Schonlein Purport?
Vascular deposition of IgA causing systemic vasculitis (skin, GI, renal); usually self limiting; most common in childhood.
What is essential cryoglobulinemic vasculitis?
Systemic vasculitis caused by circulating cryoproteins forming immune complexes. May be associated with underlying disease (e.g. Hep C) or connective tissue disease.
What is granulomatosis with polyangitis? (GPA)
Aka Wegener’s: granulomatous inflammation of vessels of respiratory tract (upper = rhinitis, sinusitis; lungs = pulmonary nodules, infiltrates) and kidneys (glomerulonephritis, renal failure). Initially have URTI symptoms; most common in middle age.
What is eosinophilic granulomatosis with polyangitis (EPGA)?
Aka Churg Strauss;
Granulomatous inflammation of vessels with hypereosinophilia and eosinophilic tissue infiltration; frequent lung involvement (asthma, allergic rhinitis).
Other manifestations: coronary arteritis, myocarditis and neuropathy.
Which antigens is EPGA associated with?
MPO or pR3
Average age of EGPA onset?
EGPA/Churg Strauss onset = 40s.
What is microangiopathic polyangitis?
Pauci-immune necrotising vasculitis affecting kidneys (necrotising glomerulonephritis), lungs (capillaritis and alveolar haemorrhage), skin.
Most common in middle age.
What is polyarteritis nodosa?
Segmental, non-granulomatous necrotising inflammation. Unknown aetiology.
What is Takayasu’s arteritis?
“Pulseless disease”: unequal peripheral pulses, chronic inflammation; most often affecting the aorta and its branches. Risk of aortic aneurysm
Who does Takayasu’s most often affect?
Usually young adults of Asian descent, F>M.
What is Beurger’s disease and who does it affect?
Inflammation secondary to pathological clotting, affects small and medium size vessels of distal extremities, may lead to distal claudication and gangrene.
- Most common in young Asian males
- Most important etiologic factor is cigarette smoking.
What is Behcet’s disease?
Leukocytoclastic vasculitis; multi system disorder presenting with ocular involvement (uveitis), recurrent oral and genital ulceration, venous thrombosis, skin and joint involvement.
