Vascular Flashcards
Aneurysm
Artery with dilatation >50% of original diameter
True vs false aneurysms
True: involve all layers of the arterial wall
False (pseudoaneurysms): collection of blood in adventitia which communicates with the lumen
Common aneurysm sites
Aorta (infrarenal most common)
Iliac
Femoral
Popliteal
Complications of aneurysm
Rupture Thrombosis Embolism Fistulae Pressure on other structures
Symptoms and signs of ruptured AAA
Intermittent or continuous abdominal pain, radiating through to back, iliac fossae or groin
Syncope
Expansile abdominal mass
Haemodynamic instability and shock
Symptoms and signs of unruptured AAA
Often no symptoms but may cause abdominal/back pain
RFs for AAA rupture
HTN
Smoking
Female
Strong FHx
Mx of ruptured AAA
Contact vascular surgeon and experienced anaesthetist, advice theatre
IV access with 2 large-bore cannulae
ECG, bloods (lipase, Hb, crossmatch)
Keep SBP
Features of polymyalgia rheumatica (PMR)
Age >50 years
Subacute onset (less than 2 weeks) of bilateral aching, tenderness and morning stiffness in shoulders and proximal limb muscles +/- mild polyarthritis, tenosynovitis and CTS
May be associated with fatigue, fever, LOW, anorexia, depression
NOT associated with true weakness
Ix in PMR
CRP
ESR >40 but may be normal
CK normal (cf myositis, myopathy)
Mx of PMR
Prednisolone 15mg/day (wean over months, according to symptoms and ESR; most need steroids for >2 years so give gastric and bone protection)
Expect a dramatic response within 1 week
Steroid SEs
GIT: pancreatitis, candidiasis, ulceration
MSK: myopathy, OP, #, growth suppression
Endocrine: adrenal suppression, Cushing’s syndrome (HTN, hyperglycaemia)
CNS: aggravated epilepsy, depression, psychosis
Eye: cataracts, glaucoma, papilloedema
Immune: increased susceptibility to and severity of infections
Fever, increased WCC (rarely cause leucopenia)
DDx for PMR
Recent onset RA Polymyositis Hypothyroidism Primary muscle disease Occult malignancy or infection OA (esp cervical spondylosis, shoulder OA) Neck lesions Bilateral subacromial impingement Spinal stenosis
What is vasculitis?
Inflammatory disorder of blood vessel walls resulting in destruction (aneurysm/rupture) or stenosis
May be primary or secondary
Classified according to main size of blood vessel affected
Causes of secondary vasculitis
SLE
RA
Hepatitis B and C
HIV
Large vessel vasculitis
GCA
Takayasu’s arteritis
Medium vessel vasculitis
Polyarteritis nodosa
Kawasaki disease
ANCA+ small vessel vasculitis
p-ANCA associated microscopic polyangiitis
Churg-Strauss syndrome
c-ANCA associated with Wegener’s granulomatosis
ANCA- small vessel vasculitis
Henoch-Schonlein purpura
Goodpasture’s syndrome
Cryoglobulinaemia
Other causes of vasculitis
Malignancy-associated vasculitis
Sx of vasculitis
Differs depending on affected organs; should be considered in any unidentified multisystem disorder
May presents just as overwhelming fatigue with increased ESR or CRP
Is a severe vasculitis flare a medical emergency? Why/why not?
Yes
Organ damage may occur rapidly (e.g. critical renal failure within 24 hrs)
Epidemiology of GCA
Elderly (if under 55 consider Takayasu’s)
Associated with PMR in 50%
Sx of GCA
Headache
Temporal artery and scalp tenderness (e.g. when combing hair)
Jaw claudication
Amaurosis fugax or sudden blindness (typically unilateral)
Extracranial Sx: dyspnoea, morning stiffness, unequal or weak pulses
Mx of suspected GCA
Order ESR and commence prednisolone 60mg/day PO IMMEDIATELY
Risk is irreversible bilateral vision loss which can occur suddenly (some advocate IV methylpred for 3 days if there are visual Sx)
Ix findings in GCA
Increased ESR and CRP
Increased platelets, decreased Hb
Increased ALP
Definitive: temporal artery biopsy (within 7 days of starting steroids; skip lesions occur so don’t be put off by negative biopsy)
Prognosis of GCA
Typically a 2 year course then complete remission
Reduce prednisolone once Sx have resolved and ESR has decreased (increase if Sx recur)
Main cause of death and morbidity is long-term steroid treatment so consider risks and benefits, provide gastric and bone protection (PPI and bisphosphonate)
What is polyarteritis nodosa (PAN)?
Necrotising vasculitis that causes aneurysms and thrombosis in medium-sized arteries, leading to infarction in affected organs and severe systemic symptoms
Epidemiology of PAN
More common in males
May be associated with hepatitis B
Sx of PAN
Typically systemic
Predominantly skin: rash and “punched out” ulcers
Renal, cardiac, GI and GU involvement
Kawasaki disease
Childhood PAN variant (may get coronary aneurysms)
Ix findings in PAN
Increased WCC, mild eosinophilia, anaemia
Increased ESR/CRP
ANCA -ive
Renal or mesenteric angiography (showing multiple aneurysms) or renal biopsy can be diagnostic
Mx of PAN
Control BP meticulously
Specialist r/v
Most respond to corticosteroids and cyclophosphamide
Any HBV should be managed with an antiviral after initial treatment with steroids
What is microscopic polyangiitis?
Necrotising vasculitis affecting small- and medium-sized vessels
Sx of microscopic polyangiitis
Rapidly progressive glomerulonephritis (RPGN) usually features
Pulmonary haemorrhage in up to 30%
Other features rare
Ix findings in microscopic polyangiitis
p-ANCA (MPO) positive
Mx of microscopic polyangiitis
As for PAN (specialist involvement; most respond to corticosteroids and cyclophosphamide)
Features of vasculitis by organ system
Systemic: fever, malaise, LOW, arthralgia, myalgia
Skin: purpura, ulcers, livedo reticularis, nailbed infarcts, digital gangrene
Eyes: episcleritis, scleritis, visual loss
ENT: epistaxis, nasal crusting, stridor, deafness
Pulmonary: haemoptysis and dyspnoea secondary to pulmonary haemorrhage
Cardiac: angina or MI (due to coronary arteritis), HF, pericarditis
GI: pain or perforation (infarcted viscus), malabsorption (chronic ischaemia)
Renal: HTN, haematuria, proteinuria, casts, renal failure (renal cortical infarcts; glomerulonephritis in ANCA +ive vasculitis)
Neurological: stroke, fits, chorea, psychosis, confusion, impaired cognition, altered mood, arteritis of vasa nervorum (arterial supply to peripheral nerves) may cause mononeuritis multiplex or sensorimotor polyneuropathy
GU: orchitis (testicular pain or tenderness)
What is Takayasu’s arteritis?
AKA aortic arch syndrome, pulseless disease
Systemic vasculitis affecting the aorta and its major branches
Granulomatous inflammation causes stenosis, thrombosis and aneurysms
Epidemiology of Takayasu’s arteritis
Rare outside of Japan!
Often affects women aged 20-40 years
Sx of Takayasu’s arteritis
Dependent on arteritis involved
Aortic arch often involved, with cerebral, opthalmological and upper limb Sx (e.g. dizziness, visual changes, weak arm pulses)
Systemic features common: fever, LOW, malaise
HTN often a feature due to renal artery stenosis
Complications of Takayasu’s arteritis
Aortic valve regurgitation
Aortic aneurysm and dissection
Ischaemic stroke (due to HTN or thrombus)
IHD
Ix findings in Takayasu’s arteritis
Increased ESR and CRP
MRI/PET allows earlier Dx than standard angiography
Mx of Takayasu’s arteritis
Predinisolone (1mg/kg/day PO)
Methotrexate or cyclophosphamide have been used in resistant cases
Angioplasty +/- stenting or CABG is performed for critical stenosis
What is Henoch-Schonlein purpura (HSP)? Epidemiology, presentation, complications, Mx
Small vessel vasculitis, presenting with purpura often over buttocks and extensor surfaces, typically affecting young males
Complications: glomerulonephritis, arthritis, abdominal pain +/- intussusception (which may mimic “acute abdomen”)
Mx: mostly supportive
What are purpura?
Non-blanching purple papules due to intradermal bleeding
What is Churg-Strauss syndrome?
Triad of adult-onset asthma, eosinophilia and vasculitis (+/- vasospasm +/- MI +/- DVT) affecting lungs, nerves, heart and skin
Complications of Churg-Strauss syndrome
Can get septic-shock picture (systemic inflammatory response syndrome, SIRS), may be accompanied by glomerulonephritis/renal failure (esp ANCA +ive)
Mx of Churg-Strauss syndrome
Steroids
Biological agents if refractory disease (e.g. rituximab)
What is Wegener’s granulomatosis?
Granulomatosis with polyangiitis (GPA)
Multisystem disorder of unknown cause characterised by necrotising granulomatous inflammation and vasculitis of small and medium vessels
Has a predilection for the upper respiratory tract, lungs and kidneys
Sx of Wegener’s granulomatosis
Upper airways disease: nasal obstruction, ulcers, epistaxis, destruction of the nasal septum causing characteristic “saddle-nose” deformity, sinusitis
Renal disease: RPGN with crescent formation, haematuria and proteinuria
Pulmonary involvement: cough, haemoptysis, pleuritis
Others: skin purpura or nodules, peripheral neuropathy, mononeuritis multiplex, arthritis/arthralgia, ocular involvement
Ix for Wegener’s granulomatosis
c-ANCA directed against PR3 is most specific
Increased ESR/CRP
Urinalysis: proteinuria, haematuria
CXR: nodules +/- fluffy infiltrate of pulmonary haemorrhage
Mx for Wegener’s granulomatosis
Severe: corticosteroids and cyclophosphamide (or rituximab) to induce remission
Maintenance: azothioprine, methotrexate
Co-trimoxazole for prophylaxis against PJP and staphylococcal colonisation
General principles of vasculitis Mx
Large vessels: steroids in most cases
Medium/small: steroids and IV cyclophosphamide (15mg/kg)
Azathioprine may be useful as steroid-sparing maintenance treatment
Goodpasture’s syndrome
Pulmonary-renal syndrome
Characterised by acute glomerulonephritis + lung symptoms (haemoptysis/diffuse pulmonary haemorrhage) caused by anti-GBM Abs binding the kidney’s BM and alveolar membrane
Ix findings in Goodpasture’s syndrome
CXR: infiltrates due to pulmonary haemorrhage, often in lower zones
Renal biopsy: crescenteric GN
Mx of Goodpasture’s syndrome
Treat shock
Vigorous immunosuppressive treatment and plasmapheresis
Raynaud’s syndrome
Peripheral digital ischaemia due to paroxysmal vasospasm, precipitated by cold or emotion
Fingers or toes ache and change colour: pale (ischaemia) to blue (deoxygenation) to red (reactive hyperaemia)
Raynaud’s disease vs Raynaud’s phenomenon
Raynaud’s disease: idiopathic
Raynaud’s phenomenon: underlying cause
Mx of Raynaud’s syndrome
Keep warm (gloves)
Stop smoking
Nifedipine
Evening primrose oil
Sildenafil
Prostacyclin for severe attacks/digital gangrene
In severe disease, consider chemical or surgical (lumbar or digital) sympathectomy
Conditions in which Raynaud’s phenomenon may be exhibited
CTDs: systemic sclerosis, SLE, RA, dermatomyositis/polymyositis
Occupational: using vibrating tools
Obstructive: thoracic outlet obstruction, Buerger’s disease, atheroma
Blood: thrombocytosis, cold agglutinin disease, polycythaemia rubra vera, monoclonal gammopathies
Drugs: B-blockers
Endocrine: hypothyroidism
Behcet’s disease
Systemic inflammatory disorder of unknown aetiology, common in Mediterranean and Chinese
Sx of Behcet’s disease
Recurrent oral and genital ulceration Uveitis Skin lesions (e.g. erythema nodosum) Arthritis (non-erosive large joint oligoarthropathy) Thrombophlebitis Vasculitis Myo/pericarditis CNS involvement (pyramidal signs) Colitis
Mx of Behcet’s disease
Colchicine for orogenital ulceration
Azathioprine or cyclophosphamide
