Flashcards in Cardiology - Blood vessels, aneurysms, veins, lymphatics, tumours Deck (106):
What is an aneurysm?
Aneurysms are defined as a weakening of the vessel wall, followed by dilation due to increased wall stress. Aneurysm affecting the abdominal aortia (AAA) is the most common type?
What patients are most commonly affected by abdominal aortic aneurysm?
AAA is the most common vessel aneurysm.
Usually occurs in men >60 years old (4:1 M:F).
They are usually located BELOW the renal artery orifices.
What is the pathogenesis of AAA?
Atherosclerosis weakens the arterial wall
- vessel wall stress increases with diameter (law of Laplace)
- vessel lumen fills with atheromatous debris and blood clots
Other factors play a role: familial, structural defects in connective tissue, absence of vasa vasorum in abdominal aorta (vessel that supplies the blood vessel)
What are the clinical findings associated with AAA?
Can present as a pulsatile epigastric mass that may or may not be tender.
Bruit (harsh sounds) is heard if renal artery stenosis or visceral arterial stenosis is present.
Atherosclerotic plaques can chip off and embolize to distal extremities. Rupture is the most common complication.
What are the clinical findings of a ruptured AAA?
There is a triad of:
1) Sudden onset of severe left flank pain (bleed is initially retroperitoneal)
2) Hypotension from blood loss into the retroperitoneum
3) Presence of pulsatile mass on physical examination
What is the greatest predictor of rupture in AAA?
Surgical repair is beneficial if the diameter of the aneurysm is >5.0-5.4 cm.
How are AAA diagnosed?
Ultrasound is 100% accurate (excellent initial screen)
CT scan can be used preoperatively to localise extent into renal vessels and evaluate the integrity of the vessel wall to exclude rupture.
Angiography gives detailed arterial anatomy.
Large diameter asymptomatic aneurysms (i.e. >5cm) are usually referred for repair, as diameter predicts rupture. Distal embolisation is also a factor.
What is the most common peripheral aneurysm?
Popliteal artery aneurysm:
- predominantly seen in males (>95%)
- pulsatile mass behind the knee
- treated surgically
What is a mycotic aneurysm?
This is a weakening of the vessel wall due to infection. It does NOT have to be fungal, but fungi are most common.
Fungi that commonly invade the arterial wall and weaken them include Candida, Aspergillus, and Mucor.
Bacteria that invade the vessel wall and weaken them include Bacteroides, Pseudomonas, and Salmonella.
Clinical findings include thrombosis with or without infarction and in some cases rupture. They are treated surgically.
What is a Berry aneurysm?
This is a saccular dilatation typically found around the circle of Willis and base of the brain.
What risk factors are associated with Berry aneurysms?
Normal haemodynamic stress
HTN of any cause
Coarctation of the aorta
Polycystic kidney disease
What artery is most commonly affected in Berry aneurysms?
Most common site is at the junction of the communicating branches with the anterior cerebral artery (ACA).
What is the pathogenesis of Berry aneurysms?
At the junction of the communicating branches with the main cerebral vessels, the vessels normally lacks an internal elastic lamina and smooth muscle. Rupture of the aneurysm releases blood into the subarachnoid space or into the brain parenchyma. Classic CT features are blood filled ventricles.
What are the clinical findings of a subarachnoid haemorrhage?
Sudden onset of severe occipital headache - described as the "worst I've ever had!".
Nuchal rigidity from irritation of the meninges.
How common are syphilitic aneurysms?
These occur as a complication of tertiary syphilis due to infection by Treponema pallidum (spirochete).
Aneurysms usually occur in men aged between 40 to 55 years of age.
What is the pathogenesis of syphilitic aneurysm?
T.pallidum infects the vasa vasorum of the ascending and transverse portions of the aortic arch:
- vasculitis is called endarteritis obliterans
- characteristic plasma cell infiltrate is present in the vessel wall
- inflammation is intense and often occludes the lumen of the vessel
Vessel ischaemia of the medial tissue leads to weakness and subsequent dilatation of the aorta and aortic valve ring.
What are the clinical findings of syphilitic aneurysm?
- left recurrent laryngeal nerve is stretched by the aneurysm
How is syphilitic aneurysm diagnosed?
There are linear calcifications seen in the aortic wall on a plain radiograph, but the definitive diagnosis is with aortography.
Treatment is with antibiotics for syphilis and surgery, if warranted.
Why is the pulse pressure increased in aortic regurgitation?
This is a problem in closing the AV valve. Because the AV valve closes in diastole, the murmur occurs in early diastole as blood leaks back into the ventricle. The increase in left ventricular end diastolic volume results in an increase in stroke volume (increased systolic pressure - due to Starlings Law). Blood rapidly draining back into the left ventricle decreases the diastolic pressure. The wide pulse pressure is manifested by a hyperdynamic circulation (e.g. pulsating uvula, bounding pulses).
What is an Ausin-Flint murmur?
Excessive blood dripping back onto the anterior mitral valve leaflet produces another diastolic mumur called the Austin-Flint murmur. This finding indicates the need for aortic valve replacement.
In what group of patients does aortic dissection occur most commonly?
This most often occurs in men (3:1 male/female ratio) with a mean age of 40-60 years and a history of antecedent HTN.
It can also occur in young people with connective tissue disorders, usually Marfan's syndrome or Ehlers-Danlos syndrome type 1.
What is Marfan's syndrome? What clinical features are often seen?
This is an autosomal dominant disorder resulting in the production of weak elastic tissue due to a defect in synthesizing fibrillin (missense mutation). Cardiovascular abnormalities dominate. Dilation of the ascending aorta may progress to aortic dissection and/ or AV regurgitation. Mitral valve prolapse (MVP) is the most common valvular defect and is often associated with conduction defects causing sudden death. Skeletal defects include hypermobile joints, eunuchoid proportions (lower body length > upper body length, arm span > height), and arachnodactyly (spider hands). Dislocation of the lens is another finding, because the suspensory ligament holding the lens is composed of elastic tissue.
What is the pathogenesis of aortic dissection?
Cystic medial degeneration (CMD)
- elastic tissue fragmentation in the media weakens the elastic artery
- degraded matrix material collects in areas of fragmentation in the media
Risk factors for CMD:
- Increased wall stress: HTN, pregnancy (increased plasma volume) and coarctation of the aorta
- Defects in connective tissue: Marfans syndrome (defects in elastin), EDS (defects in collagen)
Intimal tear in the aorta:
- tear is due to HTN or underlying structural weakness in the media
- usually occurs within 10cm of the aortic valve
- blood dissects under arterial pressure through areas of weakness
- blood dissects proximally and/or distally
What is aortic dissection classified?
There are 2 classification systems used to aortic dissection:
1) Stanford classification
- Type A = ascending aorta/ aortic root; treatment is with surgery - aortic root replacement
- Type B = descending aorta; medical therapy with anti-hypertensives
2) DeBakey classification
- Type I = ascending aorta, aortic arch, descending aorta
- Type II = ascending aorta only
- Type III = descending aorta distal to the left subclavian artery
What are the clinical features of aortic dissection?
Tearing, sudden onset chest pain (painless 10%)
Hypertension or Hypotension
A blood pressure difference (in each arm) greater than 20 mm Hg
Neurologic deficits (20%)
There is quite often loss of upper extremity pulses due to compression of the subclavian artery by blood in the false lumen.
Rupture is also a danger - sites include pericardium, pleural cavity and peritoneal cavity.
How is aortic dissection investigated?
CXR: widened mediastinum, abnormal aortic knob, ring sign, deviation of the trachea/oesophagus
CT angiography of the thoracic aorta
Conventional angiography (now rarely used diagnostically)
How is aortic dissection treated?
Beta-blockers: aim HR 60-80 bpm and systolic BP 100-120 mm Hg
For type A dissections the standard of care is aortic root replacement
What is peripheral arterial disease? What is meant by the term chronic lower limb ischaemia?
Almost all peripheral arterial disease (PAD) is due to atherosclerosis and it shares the same risk factors as CAD. Around 20% of UK adults aged 55–75 yrs have PAD, but only one-quarter have symptoms. PAD affects the leg eight times more often than the arm. The pres- ence and severity of lower limb ischaemia can be determined by clinical examination and measurement of the ankle:brachial pressure index, the ratio between systolic ankle and brachial BPs (ABPI, normal >1.0). Chronic lower limb ischaemia presents as two distinct clinical entities: intermittent claudication (IC; ABPI 0.5–0.9) and critical limb ischaemia (CLI; ABPI <0.5).
What are the clinical features of chronic lower limb ischaemia?
Pulses - diminished or absent
Bruits - denote turbulent blood flow but bear no relationship to severity of the underlying disease
Reduced skin temperature
Pallor on elevation and rubor on dependency (Buerger's sign)
Superficial veins that fill sluggishly and empty ("gutter") upon minimal elevation
Loss of hair
What is intermittent claudication?
IC refers to ischaemic pain of the leg muscles precipitated by walking and relieved by rest. It is usually felt in the calf muscles (superficial femoral artery disease) but may occur in the thigh or buttock (iliac artery disease). Typically, the pain comes on after a reasonably constant ‘claudication distance’ and rapidly resolves on stopping.
How is intermittent claudication managed?
All patients with PAD should receive ‘best medical therapy’ (BMT), which comprises standard secondary prevention measures for atherosclerosis (smoking cessation, regular exercise, low-dose aspirin, reduction of cholesterol, diagnosis and treatment of hyper- tension and diabetes) and optimisation of exacerbating conditions (e.g. heart failure, anaemia). Peripheral vasodilators such as cilostazol can improve walking distance. Intervention (angioplasty, stent- ing, endarterectomy or bypass) is usually only considered after 6 mths of BMT, and then only in patients whose disability is severe or threatens their livelihood. Provided patients comply with BMT, only 1–2%/yr will require amputation and/or revascularisation, but the annual mortality rate exceeds 5%, reflecting a high incidence of MI and stroke.
What is the definition of critical limb ischaemia?
CLI is defined as rest (night) pain, requiring opiate analgesia, and/or tissue loss (ulceration or gangrene), present for >2 wks, in the presence of an ankle BP <50 mmHg. Whereas IC is usually due to single-segment plaque, CLI is always due to multi-level disease. Patients are at risk of losing their limb (or life) in a matter of weeks or months without surgical bypass or endovascular revascularisa- tion, but treatment is difficult because they are often elderly with significant multisystem comorbidity and have severe multi-level disease.
How is critical limb ischaemia investigated?
Imaging is performed using duplex ultrasonography. More detailed non-invasive imaging can be provided by contrast MRI or CT. Intra-arterial digital subtraction angiography is indicated for patients suitable for endovascular revascularisation.
How is diabetic vascular disease managed?
Diabetes is present in 30–40% of those with severe limb ischaemia. It does not cause obstructive capillary microangiopathy; revascularisation is therefore effective when used together with control of infection and protection from pressure.
What is buerger's disease?
This inflammatory obliterative arterial disease usually affects male smokers aged 20–30 yrs, causing claudication and finger pain, with absent wrist and ankle pulses. Smoking cessation is essential, and sympathectomy and prostaglandin infusions may help.
What is acute limb ischaemia?
Acute limb ischaemia (ALI) is most frequently caused by acute thrombotic occlusion of a pre-existing arterial stenosis or throm- boembolism (often secondary to AF). All suspected acutely ischaemic limbs must be discussed immediately with a vascular surgeon. If there are no contraindications, an IV bolus of heparin (3000–5000 U) should be given to limit thrombus propagation and protect the collateral circulation. Distinguishing thrombosis from embolism is frequently difficult.
Evidence of chronic lower limb ischaemia (e.g. previous IC symptoms, bruits, diminished contralat- eral pulses) favours thrombosis, while the absence of such features and the presence of AF favour embolism.
How is acute limb ischaemia treated?
This depends on whether the occlusion is caused by thrombosis or embolism. ALI due to thrombosis can often be treated medically with IV heparin (target APTT 2.0–3.0), antiplatelet agents, high-dose statins, IV fluids and oxygen. ALI due to embolus (no collateral circulation) normally results in extensive tissue necrosis within 6 hrs unless the limb is revascularised. Irreversible ischaemia mandates early ampu- tation or palliative therapy.
What features may suggest acute limb ischaemia?
* More reliable signs of ALL and suggest irreversible ischaemia
How is the saphenous venous system organised?
Superficial saphenous veins drain blood into the deep veins via perforating branches.
Valves in the posterior branches prevent reversal of blood flow into the superficial system.
Deeps veins direct blood back to the heart.
What are varicose veins?
These are veins that are abnormally distended (>3 mm) and often tortuous underneath the skin surface.
Most commonly located:
- superficial saphenous venous system
- distal oesophagus (due to portal hypertension)
- anorectal region (due to portal hypertension)
- left scrotal sac (varicocele)
What risk factors are associated with superficial varicosities in the lower extremities?
Most common clinical manifestation of chronic venous insufficiency.
- famale gender
- family history of varicose veins
- multiple pregnancies, jobs with prolonged standing, obesity, advanced age
What is the pathogenesis of lower leg varicosities?
Valve incompetence of the perforator branches leads to reversal of blood flow from the high pressure deep venous system into the superficial system.
They may be secondary to DVT. Retrograde blood flow through the perforating branches into the superficial system causes increased pressure and dilatation of vessels.
What causes venous thromboses?
1) Stasis of blood (e.g. prolonged immobilisation, post operative state, CCF)
2) Hypercoagulability (e.g. antithrombin III deficiency, oral contraceptives, pancreatic cancer, factor V deficiency, protein C and S deficiencies)
3) Endothelial damage
Where are venous thromboses most commonly located?
Most often occurs in the deep veins of the lower extremity (e.g. veins in the calf [anterior, posterior, peroneal veins; calf venous sinusoids]; popliteal vein; femoral vein).
Less common sites include the periprostatic plexus, ovarian and periuterine veins, portal vein, hepatic vein and dural venous sinuses in the brain.
What signs are associated with acute DVTs in the calf?
Swelling of the affected leg relative to the other (>3 cm in circumference).
Pain on dorsiflexion of the foot (Homans sign) and compression of the calf.
Pitting oedema distal to the site of thrombosis due to increased hydrostatic pressure.
DVTs can be chronic. What signs are associated with chronic DVTs?
1) Stasis dermatitis:
- haemorrhagic or orange discolouration of the skin and ischaemic ulcers (poor O2 defusion) located around the medial malleolus of the ankles (organe discolouration is due to haemosiderin deposited in the skin from ruptured blood vessels)
- caused by rupture of the perforating branches due to pressure backup (retrograde flow) and chronic deep vein insufficiency (related to DVT, trauma and pregnancy)
- Rx: topic high potency corticosteroids and antibiotics if infection is present
2) Secondary varicosities may develop in the superficial system
How should a patient with a suspected DVT be investigated?
Patients suspected of having a DVT a two level Wells score should be performed (note this is different to the PE Wells score). Everything in this scoring system gets 1 point except an alternative diagnosis being more likely which gets -2 points:
1) Active cancer (treatment ongoing, within 6 months, or palliative)
2) Paralysis, paresis or recent plaster immobilisation of the lower extremities
3) Recently bedridden for 3 days or more or major surgery within 12 weeks requiring general or regional anaesthesia
4) Localised tenderness along the distribution of the deep venous system
5) Entire leg swollen
6) Calf swelling at least 3 cm larger than asymptomatic side
7) Pitting oedema confined to the symptomatic leg
8) Collateral superficial veins (non-varicose)
9) Previously documented DVT
10) An alternative diagnosis is at least as likely as DVT
What is the clinical probability of a DVT based on the Wells score?
Wells score >2 = DVT likely
Wells score <1 = DVT unlikely
What investigations should be performed if the Wells score indicates a DVT is likely?
A proximal leg vein ultrasound scan should be carried out within 4 hours and, if the result is negative, a D-dimer test.
If a proximal leg vein ultrasound scan cannot be carried out within 4 hours a D-dimer test should be performed and low-molecular weight heparin administered whilst waiting for the proximal leg vein ultrasound scan (which should be performed within 24 hours).
What investigations should be performed if the Wells score indicates a DVT is unlikely?
- perform a D-dimer test and if it is positive arrange:
i) a proximal leg vein ultrasound scan within 4 hours
ii) if a proximal leg vein ultrasound scan cannot be carried out within 4 hours low-molecular weight heparin should be administered whilst waiting for the proximal leg vein ultrasound scan (which should be performed within 24 hours)
How is a DVT managed?
Low molecular weight heparin (LMWH) or fondaparinux should be given initially after a DVT is diagnosed.
A vitamin K antagonist (i.e. warfarin) should be given within 24 hours of the diagnosis. The LMWH or fondaparinux should be continued for at least 5 days or until the international normalised ratio (INR) is 2.0 or above for at least 24 hours, whichever is longer, i.e. LMWH or fondaparinux is given at the same time as warfarin until the INR is in the therapeutic range.
How long should a patient take warfarin as treatment for a DVT?
warfarin should be continued for at least 3 months. At 3 months, NICE advise that clinicians should 'assess the risks and benefits of extending treatment'.
NICE add 'consider extending warfarin beyond 3 months for patients with unprovoked proximal DVT if their risk of VTE recurrence is high and there is no additional risk of major bleeding'. This essentially means that if there was no obvious cause or provoking factor (surgery, trauma, significant immobility) it may imply the patient has a tendency to thrombosis and should be given treatment longer than the norm of 3 months. In practice most clinicians give 6 months of warfarin for patients with an unprovoked DVT/PE. For patients with active cancer NICE recommend using LMWH for 6 months.
What dose of enoxaparin is used to treat DVT?
The dose differs for prophylaxis or treatment.
Prophylaxis (e.g. prior to surgery) = 20-40mg
- in preoperative patients, give the first dose 2 hours before surgery
Treatment = 1.5mg/kg for a minimum of 5 days and until adequate oral anticoagulation is established. Warfarin should be started on day 1 using dosage titration to achieve a stable INR of 2.0-3.0
What is the pathogenesis of superficial thrombophlebitis?
10-20% of superficial thrombophlebitis are associated with occult DVT. They tend to occur as a result of:
1) Intravenous cannulation
2) Infection - Staph. aureus is most common organism
3) Carcinoma of the pancreatic head - pancreatic cancers produce superficial migratory thrombophlebitis (Trousseau's sign) due to the release of procoagulants by the cancer
What are the clinical findings in superficial thrombophlebitis?
Pain a tenderness along the course and distribution of the affected vein.
Erythema and oedema of the overlying skin and subcutaneous tissue.
How is thrombophlebitis treated?
Warm, moist compress.
NSAIDS, flucloxacillin, or cephalexin
What is superior vena cava syndrome?
Extrinsic compression of SVC due to primary lung cancer (90% of cases). Small cell carcinoma of the lung is the most common cause.
What are the clinical findings associated with SVC syndrome?
"Puffiness" and blue, purple discolouration of the face, arms and shoulders.
Retinal haemorrhages, stroke.
What is the pathogenesis of thoracic outlet syndrome?
Compression of the neurovascular compartment of the neck. Causes include:
- cervical rib
- spastic anterior scalene muscle
- positional change in the neck and arms
What are the clinical findings associated with TOS?
Vascular signs (e.g. arms fall "asleep" while the person is sleeping)
Nerve root signs (e.g. numbness, paraesthesia)
Positive Adson test
- pulse disappears/ diminished when the arm is outstretched and the patient looks to the side of the outstretched arm
What is acute lymphangitis?
This means inflammation of the lymphatics.
Cellulitis is most often caused by streptococcus pyogenes.
Treatment is with clindamycin or erythromycin.
What causes nodular lymphangitis?
Nodular lymphangitis is caused by sporotrichosis ( a fungal skin infection). It is a subcutaneous mycotic infection and occurs as a result of traumatic implantation of the fungus - e.g. rose gardening, splinters from carpentry work, landscapers, berry pickers. It causes a chain of suppurating lymphocutaneous nodules.
What is lymphedema?
This is a collection of lymphatic fluid in the interstitial tissue or body cavities.
What is the most common cause of lymphedema?
In the Western world, the most common cause is post radical mastectomy followed by radiation to the axilla.
Other important causes to think about include:
- filariasis (causes elephantiasis)
- congenital origin
- Turner's syndrome
What are the clinical findings associated with lymphedema?
In early interstitial oedema, there is pitting with compression; however, in advanced cases it is non pitting due to increased fibrosis.
It is usually painless and non progressive.
What is an angiomyolipoma?
Kidney hamartoma: composed of blood vessels, muscle and mature adipose tissue. It is associated with tuberous sclerosis.
What causes liver angiosarcoma?
These are associated with exposure to PVC, arsenic and thorium.
What is bacillary angiomatosis?
This is a benign capillary proliferation involving the skin and visceral organs in AIDs patients.
Simulates Kaposi sarcoma in AIDs.
Caused by Bartonella henselae; gram negative bacillus (also causes cat scratch fever).
What blood vessel tumour can rupture and cause haemoperitoneum?
Cavernous haemangioma. Most common benign tumour of the liver and spleen. If large they may rupture.
What is a cystic hygroma?
This is a lymphatic cyst in the neck that may be associated with Turner syndrome (responsible for the webbed neck)
What is Kaposi sarcoma?
A malignant tumour arising from endothelial cells or primitive mesenchymal cells. It is an AIDS defining lesion, and the most common cancer in AIDS.
It is associated with the human herpesvirus type 8.
Presents with red-purple discolouration that progresses from a flat lesion to a plaque to a nodule that ulcerates. Common sites include the neck, mouth and GIT.
What is Sturge-Weber syndrome?
Nevus flammeus (birth mark) on the face in the distribution of the ophthalmic branch and/or maxillary branch of cranial nerve V (trigeminal).
Some cases show ipsilateral malformation of pia mater vessels overlying the occipital and parietal lobes. These can bleed and produce a subarachnoid haemorrhage.
What is Von Hippel-Lindau syndrome?
This produces cavernous hemangiomas in the cerebellum and retina.
There is also an increased incidence of bilateral pheochromocytoma and bilateral renal cell carcinomas.
What is vasculitis?
Vasculitis literally means inflammation of blood vessels. It is classified based on the size of the vessel affected - e.g. small, medium and large or combinations.
What is the pathogenesis of vasculitis?
Vasculitis can either a hypersensitivity reaction or caused by antineutrophil cytoplasmic antibodies (ANCA)
- type III (immunocomplex); e.g. HSP
- type II (antigen-antibody); e.g. Goodpastures
- antibodies activate neutrophils causing release of their enzymes and free radicals resulting in vessel damage
- c-ANCA types of vasculitis
antibodies are directed against proteinase 3 in cytoplasmic granules
e.g. Wegener's granulomatosis
- p-ANCA types of vasculitis
antibodies are directed against myeloperoxidase in neutrophils
e.g. microscopic polyangitis, Churg-Strauss, PAN (20%)
Vasculitis can also be caused by direct invasion of the vessel wall by ALL types of pathogens.
What vasculitides affect large vessels?
Giant cell arteritis
What vasculitides affect medium sized vessels?
What vasculitides affect small sized vessels?
ANCA associated vasculitis (e.g. Wegener's, microscopic polyangitis, Churg-Strauss)
What are the causes of vasculitis?
Think of "PAINT"
Primary - with or without granuloma; divided based on size of artery
Autoimmune: RA, SLE, scleroderma
Neoplasia: lymphoma, carcinoma (bronchus, ovary, renal)
Toxins: propylthiouracil, amphetamine, leflunomide
What infections can cause vasculitis?
Hepatitis B virus is associated with PAN; Hepatitis C virus causes a "essential mixed cryoglobulinaemia"
VZV, CMV, syphilis can all cause isolated CNS vasculitis
HIV and parvovirus B19 can also cause vasculitis.
Streptococcus (esp. Behcets, "erythema elevatum diutium"), TB, rickettsia, histoplasmosis can also cause vasculitis.
What are the clinical features of vasculitis?
This depends on the size of the artery affected:
1) Small vessel vasculitis
- called leukocytoclastic venulitis or hypersensitivity vasculitis
- skin overlying the vasculitis is haemorrhagic, raised and painful to palpation (called palpable purpura) - e.g. HSP, microscopic polyangitis
- microscopically, vessels are disrupted and contain neutrophilic infiltrate associated with nuclear debris and fibrinoid necrosis
2) Medium sized vessel vasculitis
- muscular artery vasculitis
- presents with vessel thrombosis and infarction or aneurysm
- e.g. PAN, Kawasaki disease
3) Large vessel vasculitis
- elastic artery vasculitis
- presents with loss of pulse or stroke
- e.g. Takayasu and GCA
What is Takayasu arteritis?
Otherwise known as "pulseless disease", it is a granulomatous large vessel vasculitis involving the aortic arch and its vessels. It affects young Asian women and children.
How does Takayasu arteritis present?
Absent upper extremity pulses
Discrepency in blood pressure between the arms is > 10mmHg
Visual defects, stroke
Treatment is with corticosteroids
What is giant cell arteritis?
This is a granulomatous large vessel vasculitis involving superficial temporal and ophthalmic arteries. It tends to affect adults over the age of 60.
How do patients with GCA present?
Temporal headache, jaw claudication
Blindness on the ipsilateral side due to ophthalmic artery vasculitis
Polymyalgia rheumatica (muscle and joint pain, with normal serum creatinine kinase) is commonly associated with PR
Increased ESR (>60)
Treatment is with high dose steroids (>60mg) and urgent ophthalmology review.
What is polyarteritis nodosa?
This is a NECROTISING medium sized vessel vasculitis involving renal, coronary, mesenteric arteries (spares the pulmonary arteries).
It usually affects middle aged men.
Associated with HBsAg (30% of cases), HCV less commonly. Hepatitis B associated polyarteritis is an immunecomplex disease (type III hypersensitivity).
How does PAN present? How is it treated?
Vessels are at ALL stages of acute and chronic inflammation
Fever is commonly present (often presents as fever of unknown origin)
Focal vasculitis produces aneurysms (detected on angiography)
Organ infarction in kidneys (renal failure, haematuria) heart (acute MI), bowels (blood diarrhoea) skin (ischaemic ulcer), testicle (testicular pain)
Angiography and biopsy of the lesions confirms the diagnosis.
Treatment is with corticosteroids and cyclophosphamide in resistant cases.
What is Kawasaki disease?
A necrotising medium sized vessel vasculitis involving coronary arteries (e.g. thrombosis, aneurysms).
Which patients are affected by Kawasaki disease?
Typically children <5 years of age
Boys > girls
Cause unknown (probably infection precipitating an immune reaction in genetically susceptible individuals)
Children of Asian descent are at highest risk
How does Kawasaki disease present? How is it treated?
Fever (>5 days, resistant to antipyretics), erythema, and oedema of the hands and feet convalescing with desquamated rash; cervical lymphadenopathy, oral erythema, cracking of the lips , strawberry appearing tongue (glossitis).
ECG may be abnormal (MI).
Treatment is with IVIg and aspirin.
Corticosteroids are only indicated if 2 courses of IVIg have been unsuccessful.
What is thromboangitis obliterans?
Otherwise known as Buerger's disease, this is a medium sized vessel vasculitis with digital vessel thrombosis and damage to neurovascular compartment.
Genetic mechanism has been proposed. Most commonly occurs in Ashkenazi Jews, with a high morbidity in India, Japan and Korea. Typically occurs in men aged 25-50 years who are smokers.
How does Buerger's disease present?
Lower extremity is involved in 100% of cases: resting pain on the forefoot is characteristic, with possible ischaemic ulcers and gangrene of foot/toes.
Upper extremity is involved in 40-50% of cases: upper limb ischaemia with ulceration and gangrene (amputation is common); Raynaud phenomenon
- smoking cessation ESSENTIAL
- IV iloprost (prostaglandin analogue)
- vasodilators (alpha blockers, calcium channel blockers)
What is the difference between Raynaud's disease and Raynaud's phenomenon?
Raynaud's phenomenon may be primary (Raynaud's disease) or secondary (Raynauds phenomenon).
Raynaud's disease is more likely to present in young women as symmetrical attacks due to exaggerated vasomotor responses to cold or stress.
The presentation is with paroxysmal digital colour changes (white-blue-red sequence). Ulceration and gangrene can occur in chronic cases.
Treatment is to avoid cold temperatures, calcium channels blockers (e.g. nifedipine)
What other conditions can cause Raynaud's phenomenon?
Raynaud's phenomenon can present in both men and women and is usually secondary to other diseases, e.g.
- connective tissue disorders: scleroderma (most common), rheumatoid arthritis, SLE
- type I cryoglobulinaemia, cold agglutinins
- use of vibrating tools
- drugs: oral contraceptive pill, ergot
- cervical rib
Features that suggest an underlying connective tissue disease are:
- onset after 40 years
- unilateral symptoms
- presence of autoantibodies
- features which may suggest rheumatoid arthritis or SLE, for example arthritis or recurrent miscarriages
- digital ulcers, calcinosis
- very rarely: chilblains
What is Wegener's?
Granulomatosis with polyangiitis is now the preferred term for Wegener's granulomatosis. It is a necrotizing autoimmune condition associated with a necrotizing granulomatous vasculitis, affecting both the upper and lower respiratory tract as well as the kidneys.
Mean age 41 years Incidence equal in men and women
Approximately 90% present with symptoms involving the upper or lower airways or both.
What are the features of Wegener's?
upper respiratory tract: epistaxis, sinusitis, nasal crusting
lower respiratory tract: dyspnoea, haemoptysis
rapidly progressive glomerulonephritis ('pauci-immune', 80% of patients)
saddle-shape nose deformity
also: vasculitic rash, eye involvement (e.g. proptosis), cranial nerve lesions
How is Wegener's investigated?
cANCA positive in > 90%, pANCA positive in 25%
chest x-ray: wide variety of presentations, including cavitating lesions
renal biopsy: epithelial crescents in Bowman's capsule
How is Wegener's treated?
The 3 C's
Median survival is 8-9 years
What type of vessel does microscopic polyangitis affect?
Microscopic polyangitis is a small vessel vasculitis involving the skin, lung, brain, GIT and kidneys (post capillary venules and glomerular capillaries).
It affects children and adults.
It is precipitated by drugs (e.g. penicillin), infections (e.g. streptococci) and immune disorders (e.g. SLE).
How does microscopic polyangitis present?
Histologically, vessels are at the SAME stage of inflammation.
Patient have palpable purpura, and glomerulonephritis (crescentic type)
Associated with p-ANCA antibodies (>80% of cases)
Treatment is with corticosteroids and cyclophosphamide.
What is Churg-Strauss syndrome?
A small vessel vasculitis involving skin, lung, and heart vessels.
It usually occurs at a mean age of 51 years and is probably autoimmune in nature.
Patients present with allergic rhinitis, asthma and p-ANCA antibodies are present in over 70% of cases. Eosinophilia is common.
Treatment is with corticosteroids, 1 year survival is 90% , 5 year survival is approx. 60%.
What is HSP?
Small vessel vasculitis involving the skin, GIT, renal and joint vessels.
It is usually seen in children aged 1-15 years; some cases are in young adults.
Peak incidence is in the spring, rarely in the summer.
Most common vasculitis in children
IgA- anti-IgA immunocomplexes (type III hypersensitivity)
How does HSP present?
Often follows a viral URI, group A streptococcal pharyngeal infection. Pathogens may act as an antigen trigger that causes antibody formation leading to immunocomplex formation (type III hypersensitivity)
Palpable purpura of buttocks and lower extremities is a characteristic finding (95%–100%).
Polyarthritis (80%), glomerulonephritis (80%), abdominal pain and vomiting (85%), and GI bleeding may occur.
Recurrence occurs in one third of cases.
Most have spontaneous recovery in 4 months without
Treatment: corticosteroids are mainly used if severe GI
disease or renal disease are present
What is cryoglobulinaemia? What diseases are associated with it?
Small vessel vasculitis involving skin, GI tract, renal vessels
Different types of cryoglobulinemia (mixed, monoclonal, polyclonal)
Adults; female more common than male (3:1 ratio)
Association with HCV (>50%), type 1 MPGN, multiple myeloma (monoclonal type), lymphoproliferative disorders, connective tissue disorders.
How does cryoglobulinaemia present?
Cryoglobulins: they are proteins in plasma that gel at cold temperatures, particularly in those areas that are exposed to the cold temperature (i.e., nose, fingers, ears).
Palpable purpura, acral cyanosis of nose and ears and Raynaud phenomenon (reverses when in warm room); glomerulonephritis (crescentic type); arthritis; abdominal pain.
Treatment: immunosuppressive agents