VASCULITIS Flashcards
What is vasculitis?
Inflammation of the blood vessels
Chapel Hill consensus definition of vasculitis?
Large vessel - Takayasu + GCA
Medium - PAN + Kawasaki
Small: ANCA-associated and immune-complex-associated
ANCA-associated vasculitis?
Microscopic polyangitis
Granulomatosis with polyangitis
Eosinophilic granulomatosis with polyangitis
Immune-complex-associated vasculitis?
Anti-GBM disease
Cryoglobulinaemic vasculitis
IgA vasculitis/HSP
Hypocomplementemic urticaria vasculitis (anti-C1q)
Examples of medium vessel vasculitis?
Polyarteritis nodosa
Kawasaki disease
Examples of large vessel vasculitis?
Giant cell arthritis
Takayasu’s arteritis
What is granulomatosis with polyangiitis also known as?
Wegeners granulomatosis
What is Eosinophilic granulomatosis with polyangiitis also known as?
Churg-Strauss syndrome
What is anti-glomerular basement membrane disease also known as?
Goodpastures syndrome
Which ANCA type is associated with granulomatosis with polyangiitis?
cANCA
Which ANCA type is associated with Eosinophilic granulomatosis with polyangiitis?
pANCA
Most commonly targeted protein in granulomatosis with polyangiitis?
Serine proteinase 3 (PR3)
Most commonly targeted protein in Eosinophilic granulomatosis with polyangiitis?
MPO
Conditions associated with cANCA?
Granulomatosis with polyangiiitis
40% of microscopic polyangiitis
Conditions associated with pANCA?
75% of microscopic polyangiitis
50% of Eosinophilic granulomatosis with polyangiitis
25% of granulomatosis with polyangiitis
70% of UC
70% of PSC
25% of anti-GBM disease
20% of Crohn’s disease
Features of granulomatosis with polyangiitis?
- upper respiratory tract: epistaxis, chronic sinusitis, nasal crusting, saddle-shape nose deformity
- lower respiratory tract: dyspnoea, haemoptysis, cough, wheeze, cavitating lesions
- rapidly progressive glomerulonephritis (pauci-immune)
also: vasculitic rash, eye involvement (e.g. proptosis), ear involvement (e.g. hearing loss), cranial nerve lesions, constitutional symptoms
Why does granulomatosis with polyangiitis cause a saddle nose deformity?
As it causes progressive loss of the cartilage in the nose
Investigtaions for granulomatosis with polyangiitis?
Urinalysis
FBC - anaemic, leukocytosis and thrombocytosis, raised CRP and ESR
ANCA antibodies - cANCA positive in >90% and pANCA positive in 25%
CXR - cavitating lesions, nodules, infiltrates
Renal biopsy - epithelial crescents in bowman’s capsule
Management of granulomatosis with polyangiitis?
steroids
cyclophosphamide (90% response)
plasma exchange
Prognosis of Granulomatosis with polyangiitis?
Median survival is 8-9 years
Clinical presentation of Eosinophilic granulomatosis with polyangiitis?
Severe, adult-onset asthma
Paranasal sinusitis
Mononeuritis multiplex
Vasculitis rash
Muscle and joint pain
Investigtaions for Eosinophilic granulomatosis with polyangiitis?
FBC - blood eosinophilia (>10%), anaemia, raised CRP and ESR
Serum ANCA - pANCA positive
Investigtaions for microscopic polyangitis?
ANCA - 75% have pANCA And 40% have cANCA
Renal biopsy - shows necrotising glomerulonephritis
Presentation of microscopic polyangiitis?
Renal failure due to glomerulonephritis
Vasculitis rash
Diffuse alveolar haemorrhage = haemoptysis
Mononeuritis multiplex
And constitutional symptoms
What is a vasculitic rash?
Palpable purpura
What is Henoch-schonlein purpura?
An IgA mediated small vessel vasculitis
Inflammation occurs due to IgA deposits in blood vessels
Who is HSP usually seen in?
Children under 10 following an infection (usually a viral RTI)
Presentation of HSP?
Palpable purpuric rash over buttocks and extensor surfaces of arms and legs - in 100%!
Abdominal pain that is severe and colicky
Polyarthritis
Features of IgA nephropathy e.g. haematuria or renal failure
Investigtaions for HSP?
Urinalysis - check for haematoproteinuria
BP - may be elevated in nephrotic syndrome
FBC
ESR&CRP raised
U&Es - elevated cr if renal involvement
Skin or renal biopsy can be performed for immunofluroresnece of IgA/C3 but this is rarely done!
Management of HSP?
Supportive & Analgesia for arthralgia
Prognosis of HSP?
Usually excellent, self-;limiting (esp in children without renal involvement)
1/3rd of pt have a relapse
Monitoring following HSP?
Bp and urinalysis to detect progressive renal involvement
What is goodpastures syndrome?
Anti-glomerular basement membrane disease
A rare type of small-vessel vasculitis
Cause of anti-GBM disease?
Anti-glomerular basement membrane antibodies against type 4 collagen present in kidneys and lung alveoli
Associated with HLA DR2
Who commonly gets anti-GBM disease?
Men
20-30s or 60-70s
Presentation of anti-GBm disease?
Pulmonary haemorrhage
Rapidly progressive glomerulonephritis - rapid onset AKI or nephritis
Investigations for anti-GBM disease?
Urinalysis
Bloods - U&Es, ABG/VBG, ANCA
CXR
Renal biopsy is gold standard
What will renal biopsy show in anti-GBm disease?
Focal and segmental necrotising crescents and linear IgG deposits along the basement membrane
Management of anti-GBM disease?
Plasmapheresis to remove circulating antibodies
Steroids or cyclophosphamide
Prognosis of anti-GBM disease?
If untreated it is fatal
If they have severe AKI or require dialysis it has a poor renal prognosis
Main complication of anti-GBm disease?
Pulmonary haemorrhage
What is cryoglobulinaemic vasculitis?
A types of small vessel vasculitis
Causes palpable purpura, Arthralgias in hands and knees, polyneuropathy, renal involvement
What is hypocomplementemic urticarial vasculitis?
A rare form of small vessel vasculitis
Causes recurrent episodes of urticaria on top of other vasculitis symptoms
What is polyarteritis nodosa?
Medium vessel vasculitis with necrotizing inflammation leading to aneurysm formation
Who is polyarteritis nodosa most common in?
Middle-aged men 40-60
Those with hep B infection
Presentation of polyarteritis nodosa?
Constitutional - fever, malaise, weight loss
Hypertension
Arthralgias
Mononeuritis multiplex or sensorimotor polyneuropathy
Testicular pain
Livedo reticularis or palpable purpura
Mesenteric arteritis causes abdominal angina
Haematuria or renal failure
Investigations for polyarteritis nodosa?
Urinalysis - blood/protein
CRP or ESR
FBC will show anaemia and thrombocytopenia
Hep B surface antigen may be positive
Biopsy of affected tissue
Treatment of PAN?
Mild-moderate - oral corticosteroids
Severe - high-dose pulsed IV corticosteroids
Additional immunotherapy is usually required e.g. methotrexate
Who does Kawasaki disease typically affect?
Children
Presentation of Kawasaki disease?
High-grade fever lasting >5 days and resistant to anti-pyretics
Conjunctival injection
Bright red cracked lips
Strawberry tongue
Cervical lymphadenopathy
Red palms of hands and soles which later peel
Investigtaions for Kawasaki disease?
It’s a clinical diagnosis
Bloods for CRP and ESR may be done
Echocardiogram is done due to risk of coronary artery aneurysm
Management of Kawasaki disease?
High dose aspirin
IV immunoglobulin
Prognosis of Kawasaki disease?
Usually takes a few weeks to heal
Complication of Kawasaki disease?
Coronary artery aneurysm
What is takayasu’s arteritis?
A chronic granulomatous vascultiis affecting the large arteries - primarily the aorta and its main branches
Who does takayasu’s arteritis affect?
Young Females 10-40
Asian people
Pathophysiology of takayasu’s arteritis?
Vascular inflammation causes stenosis, occlusion and aneurysm formation
What is takayasu’s arteritis also known as?
Pulseless disease
Presentation of takayasu’s arteritis?
systemic features of a vasculitis e.g. malaise, Arthralgias, headache
unequal blood pressure in the upper limbs
carotid bruit and tenderness
absent or weak peripheral pulses
upper and lower limb claudication on exertion
aortic regurgitation (around 20%)
Investigtaions for takayasu’s arteritis?
FBC - normocytic anaemia
Raised CRP/ESR
Magnetic resonance angiography or CTA are diagnostic
Management of takayasu’s arteritis?
Steroids
What is giant cell arteritis?
Temporal arteritis
A vasculitis of an unknown cause that affects medium and large0sized vessels
Who is temporal arteritis most common in?
> 60s
Most commonly females
Caucasians have highest rates
What condition is temporal arteritis associated with?
Polymyalgia rheumatica - 50% will have features of PMR
Presentation of temporal arteritis?
Weeks of headache, jaw claudication, tender palpable temporal artery
Scalp tenderness
May have amaurosis fugax or prememntn visual loss
Systemic features - lethargy, depression, low-grade fever, anorexia, night sweats
How does temporal arteritis affect the eyes?
It causes anterior ischaemic optic neuropathy resulting from occlusion of the posterior ciliary artery which is a branch of the ophthalmic artery
Causes temporary visual loss and then permenant visual loss
May also cause diplopia from involvement of any part of the oculomotor system
Most common serious complication of temporal arteritis?
Irreversible blindness due to optic nerve ischaemia
Investigtaions for temporal arteritis?
CPR/ESR
FBC - may have normochromic, normocytic anaemia
LFTs - 1/3rd may have midlly deranged
Vascular USS
Temporal artery biopsy if USS not possible
Findings on temporal artery biopsy in temporal arteritis?
Granulomatous inflammation of the inner half of the media with infiltration of inflammatory cells, including giant cells
May have skip lesions so 3-5cm of artery should be taken
Doppler USS findings for temporal arteritis?
Halo sign
Management of temporal arteritis?
Urgent high-dose glucocorticoids (before temporal artery biopsy)
Urgent ophthalmology review
Which steroids should be used for temporal arteritis?
No visual loss = high-dose prednisolone
Visual loss = IV methylprednisolone before starting high-dose prednisolone
What is Behçet’s syndrome?
A complex multisystem disorder with autoimmune mediated inflammation of arteries and veins
Epidemiology of Behçet’s syndrome?
Eastern Mediterranean
Men
Young adults 20-40
30% have positive FHx
Features of Behçet’s syndrome?
Oral ulcers
Genital ulcers
Anterior uveitis
Also: thrombophlebitis, DVTs, arthritis, neurological involvement, GI symptoms, erythema nodosum
What is pathergy?
What can it be seen in?
A skin condition in which a minor trauma e.g. a bump can lead to the development of skin lesions or ulcers
Seen in Behçet’s disease and pyoderma gangrenosum
Which antigen is associated with Behcets syndrome?
HLA B51
