Diseases of the Blood Vessels

I. Degenerative Diseases of the Arteries II. Inflammatory Diseases a) Syphylitic aortitis b) Polyarteritic nodosa c) Giant cell temporal arteritris d) Buergers disease e) Takayusus arteritis f) Kawasaki syndrome g) Wageners Granulomatosis
III. Neutrotropic Vascular Disorders a) Raynauds Disease b) Acrocyanosis IV. Aneurysm V. Diseases of the Veins

I. Degenerative Diseases of the Arteries Arteriosclerosis- hardening of the arteries or more specifically refers to a group of processes which have in common thickening and loss of elasticity of arterial walls.
3 Morphologic Variants: 1) Atherosclerosis 2) Medial calcific sclerosis or Monckebergs Sclerosis 3) Arteriosclerosis A. Atherosclerosis a lesion of large and medium sized arteries with deposits in the

intima of yellowish plaques containing cholesterol, lipoid material and lipophages. Predisposing Factors: a) Age b) Obesity c) Diet high caloric and high fat diet d) Hyperlipoproteinemia. Lipoproteins are implicated in atheroma formation. e) Cigarette Smoking substance in cigarettes that alter the metabolic balance and causes deposition of lipids in the arteries. f) HPN not a cause but it aggravates atherosclerosis in that it causes more filtration of lipids in the circulating blood.

g) Hormonal Factor explains why females before menopause are less prone to atherosclerosis because estrogens protect the intima of the blood vessels from lipid deposition. h) Hemodynamic Factors (Blood Flow) Most atheromas usually occur at the bifurcation or where primary vessels spring from the aorta.
i)

Inflammation following infection or from drugs or chemicals which renders the endothelium permeable to lipids.

Atherosclerosis affects the large and medium sized arteries with the lesions becoming less severe and less numerous as the smaller arteries are reached and disappearing entirely some distance proximal to the arterioles. Grossly, the arteries most commonly affected are the aorta, coronary arteries, cerebral arteries and the major divisions of the aorta such as the innominate, common carotid and iliac arteries. The essential lesions of atherosclerosis occur in the arterial intima although secondary changes maybe seen in the internal elastic lamina and media. The early atheromas

Appear as fine yellow flat flecking or streakingof the intima varying from 1 up to 3-4 mm in diameter. Later the lesions coalesce producing an elevation on the intima surface of the affected vessel known as an ATHEROMA. Section and staining with sudan dye reveals these streaks to be made up of mononuclear cells containing fatty droplets lying close to the endothelium. These streaks in itself are

Harmless, its importance lies in its potential to progress to more severe lesions.
CHANGES THAT OCCUR IN AN ATHEROMA FOLLOWING FURTHER LIPID DEPOSITION: a) Accumulation of calcium salts DYSTROPHIC CALCIFICATION, the intima of blood vessels become brittle and thickened. b) Laying down of collagen fibers to form fibrous plaques and becomes enlarged and more elevated. c) Invasion of granulation tissue. d) Necrosis in the depths of the affected areas and oftentimes a zone of necrotic tissue and debris is formed at the base of the thickened intima.

e) Plaque ulceration can occur resulting from softening of layers covering the fat pool and necrotic debris deep in the plaque.
CLINICAL SIGNIFICANCE: 1. Cause narrowing of the vascular lumens to cause ischemia. 2. Sudden occlusion of the lumens by superimposed thrombosis or hemorrhage. 3. Provides a foci for thrombosis and then embolism. 4. Weakening of the wall of a vessel may lead to formation of an aneurysm or may lead to rupture.

MONCKEBERGS MEDIAL CALCIFIC SCLEROSIS (MEDIAL CALCINOSIS) - Characterized by a ringlike or plate calcifications within the media of medium to small arteries giving a gooseneck lamp nodularity on palpation. - Commonly affected vessels are the femoral, tibial, radial and ulnar arteries although the
the coronary arteries may likewise be affected. - Rare in individuals below 50 years. - Oftentimes the condition is detected as an unexpected finding on x-ray of a limb and mistaken for suspected fracture. - It is in itself quite harmless though its detection causes some alarm.

- It may be accompanied by a coincidental intimal disease which maybe of more potential danger. ARTERIOSCLEROSIS - Refers to the thickening of the walls and narrowing of the lumens of small arteries and arterioles. Believed to be the anatomic
reflections of prolonged significant elevations of blood pressure. - 2 Types: 1) Hyaline Arteriosclerosis - Associated with a slowly rising moderate blood pressure usually encountered in the elderly people.

- Characterized by a pink collagenous fibrosis and thickening of the walls of the arterioles. - Causes an ischemic nephropathy called Benign Nephrosclerosis. 2) Hyperplastic Arteriosclerosis - characterized by an onion-skin appearance to the arterioles.
- The two forms may co-exist implying that there is a period of slow moderate elevation of blood pressure following a phase of rapid elevation. - The most striking lesions are usually found in the arterioles and small arteries of the kidneys, pancreas, gallbladder, small intestine and adrenals.

II. INFLAMMATORY A.Syphilitic Aortitis - commonest manifestation of the tertiary stage of syphilis. - Present in 80% of patients with tertiary lesions. - Particular predilection to the aortic arch which may be the result of heavy involvement of the mediastinal lymph nodes in the secondary stage of syphilis.

- In the latent stage, it is in these nodes that a few treponenes survive and years later when tissue sensitivity permits further activity they escape from the nodes and gain access to the lymphatic plexus around the aortic arch. The lesions are maximal in the ascending part of the arch and diminishes in severity below this region to cease at the level of the diaphragm. - Microscopic examination shows endarteritis and periarteritis of the vasa vasorum in the adventitia and media with growing collections of lymphocytes, plasma cells and macrophages along their course and in places expanding to form miliary gumma which may undergo necrosis.

- Effects of syphilitic aortitis are variable. May vary in from a small aortic dilatation or an aneurysm may result. If the syphilitic process spreads to the valve ring, aortic incompetence occurs due to involvement of the valvular cusps with intimal fibrosis and become thickened and shortened. B. POLYARTERITIS NODUS discussed under collagen diseases. C. GIANT CELL (TEMPORAL) ARTERITIS - chronic granulomatous reaction involving the whole circumference of the arterial wall with special predilection for the cranial arteries particularly the temporal arteries, however, the aorta and its branches may be occasionally

be involved. - rare before age 50. - In the affected artery we see neutrophils invading the wall of the vessel plus the presence of histiocytes, plasma cells, foreign body giant cells and lymphocytes. - Most cases run a benign course however with involvementof the ophthalmic arteries,
may lead to visual disturbances and possible blindness. D. THROMBOANGITIS OBLITERANS (BUERGERS DISEASE) - affects males ranging from 20-35 yrs. - patients usually are heavy cigarette smokers - etiology unknown It is believed that

nicotine in cigarettes produces vasospasm secondary to its effect on catecholamine metabolism. - affects mostly the blood vessels of the lower extremities and rarely those of the upper extremity. - starts as cramplike pain in the calf muscles occuring after exercise and disappearing at rest. This is a symptom complex of

intermittent claudication and after some time ischemic lesions of the toes develop and progress to gangrene requiring amputation. - The earliest change is an inflammatory reaction affecting the whole of the arterial wall and often spreading to neighboring veins and nerves. The exudate is composed mostly of neutrophils in the early stage. Later, there maybe lymphocytes, macrophages and a few

giant cells. There is proliferation of endothelial cells. The condition is further complicated by thrombus formation. E. TAKAYUSUS ARTERITIS - granulomatous vasculitis of medium and larger arteries described in 1908 by Takayusu. - clinical syndrome characterized principally by

ocular disturbances and marked weakening of the pulses in the upper extremities (pulseless disease) related to the fibrous narrowing or virtual obliteration of the origins of the great vessels arising in the arch. - common in Asia, predominantly females 1540 yrs old. - pathogenesis unknown.

- affects mostly the aortic arch (1/3) but may also affect the remainder of the aorta and its branches. - Grossly, there is irregular thickening of the aortic wall or intimal wrinkling thus become narrowed and sometimes obliterated.

F. KAWASAKI SYNDROME ( Musculocutaneous Lymph Node Syndrome ) - an arteritis involving large, medium sized and small arteries (often the coronary arteries) that is associated with the mucocutaneous lymph node syndrome usually seen in young children and infants (80%) younger than 4yrs of age.

- starts as a fever with conjunctival and oral erythema and erosion, edema of the hands and feet, erythema of the palms and soles, skin rashes often with desquamation and enlargement of the cervical lymph nodes. - approximately 20% of patients with deep cardiovascular sequelae e.g. Coronary artery atresia, coronary artery aneurysm.
- May rupture or have thromboses which may cause sudden death. - Leading cause of acquired heart disease in children in the U.S.

G. WEGENERS GRANULOMATOSIS - a form of necrotizing vasculitis characterized by: a) acute necrotizing granulomas of the upper and lower respiratory tract (nose, sinuses and lungs) b) focal necrotizing vasculitis affecting the small to medium sized vessels, capillaries,

venules, arterioles and arteries. Most prominent in the lungs and upper airways but may affect other sites as well (not common) c) renal disease in the form of focal or diffuse necrotizing glomerulitis. - may not manifest the full triad thus named limited Wegeners Granulomatosis.
- Peak incidence 5th decade. - Affects small arteries and veins in virtually every vessel and organ of the body. - Untreated, grim prognosis, die within 1 year. - Treated with immunosuppressive cytotoxic drugs as cyclophosphamide 90% improvement.

III. NEUROTROPIC VASCULAR DISEASE RAYNAUDS DISEASE - functional vasospastic disorder affecting the small arteries and arterioles of the extremities occuring primarily in young apparently healthy women. - most commonly affected are the fingers and hands but occasionally may affect the tip of

the nose and feet. - Cold and emotional stimuli trigger vasospasm. - Etiology unknown. - Differs from Raynauds phenomenon (refers to arterial insufficiency of the extremities secondary to the arterial narrowing induced by various conditions including SLE,
scleroderma, atherorsclerosis or Buergers disease because this condition is always secondary to an underlying disorder producing an organic lesion in the arterial wall.

IV. ANEURYSMS - is a localized abnormal dilatation of a blood vessel or the wall of the heart. - when an aneurysm is bounded by arterial wall components or the attenuated wall of the heart it is called a TRUE aneurysm. e.q. Atherosclerotic Syphilitic Congenital Vascular Aneurysm

 - Left Ventricular Aneurysm that follow a myocardial infarction. FALSE ANEURYSM ( PSEUDOANEURYSM ) - A breach in the vascular wall leading to an extravascular hematoma that freely communicates with the intravascular space (pulsating hematoma) - E.q. Post-myocardial infarction rupture that has been contained by a pericardial adhesion or a leak at the junction of the anastomosis of a vascular graft with a natural artery.

Causes:
a) Improper development of the coats of the vessel - congenital e.g. Berry Aneurysm these aneurysms arise at the base of the Circle of Willis and its major branches within the subarachnoid space. - most cases of intracranial hemorhage occurring in adolescence is due to a ruptured congenital berry aneurysm. b) Acquired defect in the vessel wall from a degenerative process (e.g. Atherosclerosis) or from an inflammation e.g. SY

MYCOTIC ANEURYSMS - occur in small sized arteries or even arterioles as a result of bacterial infection or some non-specific inflammation resulting in the dilatation of the affected vessel due to destruction of the vessel wall. - If there is no atherosclerosis, inflammation is due to bacterial infection or a hypersensitivity reaction.

3 Most important conditions causing aneurysms: 1. Arteriosclerosis 2. Syphilis 3. Cystic medionecrosis

Clinical significance of aneurysms. It can Cause: 1. compression 2. erosion 3. infection of structures 4. massive hemorrhage - death

DISEASES OF THE VEINS ACUTE PHLEBITIS - occurs in the small veins around any acute inflammatory process as seen in veins adjacent to a carbuncle or a suppurating wound. - thrombosis of the veins in an area of inflammation is called THROMBOPHLEBITIS.

- When the primary trouble subsides this thrombus may become organized and possibly recanalize, however there is great risk that this thrombus maybe invaded by pyogenic bacteria leading to softening of the thrombus and release into the circulation infected emboli with development of metastatic pyemic lesions. E.g. Portal pyemia as a complication of suppuration in the peritonel cavity.

PHLEBOTHROMBOSIS presence of a clot or thrombus in a vein not associated with any inflammation in the vein. VIRCHOWS TRIAD OF THE CAUSE OF THROMBOPHLEBITIS: a) damage to the vessel wall b) slowing of the blood stream c) increased coagulability of the blood

- Most frequent site where phlebothrombosis starts is in the small veins of the calf muscles. - they are also common after trauma and surgical operations and during the puerperium. - There is a great risk that fragments of these thrombus can be carried off by the blood stream into the pulmonary arteries where
they maybe massive enough to cause sudden death. VARICOSE VEINS - condition wherein the veins of a part are permanently dilated and tortous. - occur most often in the lower limbs, one of mans commonest minor ailments however

if severe may form a crippling disease. - Veins have a system of valves best developed at the lower extremities. The valve cusps are inserted in pairs in larger veins at distances varying from 1-6 cm apart and are often situated just distal to the point of entry of a tributary vein. These valves function to prevent retrograde venous flow as well as limit the hydrostatic pressure to which the lower parts of the vein are subjected to in the standing position.

- The essential structural changes in varicose veins are incompetence of the venous valves and dilatation of the veins. Once this has started, the process tends to be progressive for incompetence of one valve produces a double strain on the valve below and if this yields the transmitted strain on the next valve is further increased. Dilatation of the vessel is accompanied by elongation which in turn produces tortuosity. Dilatation is usually uneven and pouching (PHLEBECTASIA), results where thrombus formation is common.

- Commonly seen in women during pregnancy because of the pressure on the iliac vessels. - Elastic tissue degeneration and spotty calcifications within the media of the veins is termed PHLEBOSCLEROSIS. - In the legs, distention of the veins are most painful or some of the disabling sequelae are persistent edema in the extremities and trophic changes in the skin leads to stasis dermatitis and ulcerations. - Due to impaired circulation of the affected part it is extremely vulnerable to injury. Wounds heal slowly or tend to become

chronic ulcers. HEMORRHOIDS varicosities of the veins of the anal canal and lower rectum. THERAPEUTIC INTERVENTIONS IN VASCULAR DISEASE: 1. Thrombolysis dissolution of the clot. 2. Balloon angioplasty dilatation of an
atheromatous stenosis of an artery by a balloon catheter. 3. Vascular replacement using autologous saphenous veins. 4. Coronay artery bypass graft surgery.