Vasculitis Flashcards
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.
Who does Behcet’s commonly affect?
More common in Mediterranean and Asia; average age = 30s; M>F
What is predominately cutaneous vasculitis subdivided into?
- Drug induced vasculitis
- Serum sickness reaction
- Vasculitis associated with other underlying primary disease
Which drugs are linked to predominately cutaneous vasculitis?
- Allopurinol
- Gold
- Sulfonamides
- Penicillin
- Phenytoin
Aetiology of predominately cutaneous vasculitis?
- Drug exposure
- Viral or bacterial infection
- Idiopathic causes
Which vessels are involved in predominately cutaneous vasculitis?
Small vessels; most frequently the post capillary vessels
What are the signs and symptoms of primarily cutaneous vasculitis?
- Palpable purpura +/- vesicles and ulceration
- Urticaria
- Macules
- Papules
- Bullae
- Subcutaneous nodules
Ix in ?Small vessel vasculitis?
-FBE/UEC/LFTs
-ESR / CRP
-RF
-ACCP
-ANA/ENA (if ANA +ve)
-Complement (C3/4)
Bx: vascular involvement (both arteriole and venule)
Rx predominately cutaneous vasculitis?
- Stop offending drug
- Corticosteroids +/- immunosuppressives
- Usually self limiting
Which antibody is GPA most strongly associated with?
c-ANCA
What are the classic features of GPA?
- Necrotising granulomatous vasculitis of lower and upper respiratory tract
- Focal segmental glomerulonephritis
What are the criteria for GPA diagnosis?
2+ criteria for diagnosis:
- Nasal or oral involvement (inflammation, ulcers, epistaxis)
- Abnormal CXR (nodules, cavitations etc)
- Urinary sediment (protein, RBC casts)
- Biopsy of tissue involved (lungs = granulomas, kidneys = necrotising segment glomerulonephritis)
What are the systemic S/S of GPA?
- Malaise
- Fever
- Weakness
- LoW
What are the HEENT S/S of GPA?
- Sinusitis or rhinitis, nasal crusting and bloody nasal discharge, nasoseptal perforation, saddle nose deformity
- Proptosis
- Hearing loss due to CNVIII involvement
What are the pulmonary S&S of GPA?
Cough, haemoptysis
CXR findings in GPA?
- Pneumonitis
- Lung nodules
- Infiltrations
- Cavitary lesions
Treatment of GPA?
- Prednisone 1mg/kg/d PO 3-6/12
- +/- cyclophosphamide 2mg/kg/d PO 3-6/12
Followed by - High dose methotrexate (25mg PO weekly) or azathioprine (2g/kg/d PO OD)
ALSO: consider biologics (rituximab, IVIg) and plasmapheresis
What was demonstrated by the RAVE trial?
NEJM 2010: rituximab equivalent or superior to cyclophosphamide with less toxicity in GPA Rx
What is PAN?
Systemic necrotising vasculitis of medium size vessels
ANCA associated with PAN?
No. PAN is ANCA negative
What is PAN often associated with?
Hep B +ve
What is livedo reticularis?
Mottled, reticular pattern over skin
Biopsy features of PAN?
Presence of granulocytes and / or mononuclear leukocytes in the artery wall
Pathophysiology of PAN?
Focal, panmural necrotising inflammatory lesions in small and medium sized arteries; thrombosis, aneurysm or dilation at lesion site may occur. Healed lesions show fibrosis and endothelial proliferation –> may lead to luminal occlusion
Rx PAN?
-Prednisone 1mg/kg/d PO
-Cyclophosphamide 2mg/kg/d PO
+/- antivirals for HepB clearance
Ix in PAN?
- Bloods: FBE/UEC/LFTs, ESR, pANCA, HepB serology
- Imaging: angiography (beading)
- Arterial biopsy
Epidemiology of GCA?
- Most frequent vasculitis
- Patients >50y
- 2F:1M
Diagnostic criteria for GCA?
Diagnosed if 3+ present:
- Age >50
- New headache
- Temporal artery abnormality
- Elevated ESR
- Abnormal artery biopsy
Biopsy features of GCA?
Mononuclear cell infiltration or granulomatous inflammation, usually with multinucleate giant cells
S&S of GCA?
- New onset temporal headache +/- scalp tenderness
- Sudden painless loss of vision / diplopia
- Tongue/jaw claudication
- Polymyalgia rheumatica
- Aortic arch syndrome
How is GCA diagnosed?
- Clinical suspicion
- Increased ESR/CRP
- Temporal artery Bx within 14d of steroids
Rx of GCA?
High dose prednisone 1mg/kg for approximately 4/52
