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ANEURYSM AND
VASCULITIS
DR. ASHUTOSH SINGH
ASSISTANT PROFESSOR
• An aneurysm is an outward bulging, likened to a bubble or balloon,
caused by a localized, abnormal, weak spot on a blood vessel wall
• Aneurysms may be a result of a hereditary condition or an acquired
disease
• Aneurysms can also be a nidus (starting point) for clot formation
(thrombosis) and embolization
• As an aneurysm increases in size, the risk of rupture, which leads to
uncontrolled bleeding, increases
• Although they may occur in any blood vessel, particularly lethal
examples include aneurysms of the Circle of Willis in the
brain, aortic aneurysms affecting the thoracic aorta, and abdominal
aortic aneurysms
• Aneurysms can arise in the heart itself following a heart attack,
including both ventricular and atrial septal aneurysms
True and false aneurysms
• A true aneurysm is one that involves all three
layers of the wall of an artery
(intima, media and adventitia)
• True aneurysms include atherosclerotic, syphilitic,
and congenital aneurysms, as well as ventricular
aneurysms that follow transmural myocardial
infarctions (aneurysms that involve all layers of
the attenuated wall of the heart are also
considered true aneurysms)
• A false aneurysm, or pseudoaneurysm, is a collection of
blood leaking completely out of an artery or vein but
confined next to the vessel by the surrounding tissue.
This blood-filled cavity will eventually
either thrombose (clot) enough to seal the leak or
rupture out of the surrounding tissue
• Pseudoaneurysms can be caused by trauma that
punctures the artery, such as knife and bullet wounds,as
a result of percutaneous surgical procedures such
as coronary angiography or arterial grafting, or use of
an artery for injection
Morphology
• Aneurysms can also be classified by their macroscopic shapes and
sizes and are described as either saccular or fusiform. The shape of
an aneurysm is not specific for a specific disease
• Saccular aneurysms, or "berry" aneurysms, are spherical in shape
and involve only a portion of the vessel wall; they usually range
from 5 to 20 cm (2.0 to 7.9 in) in diameter, and are often filled,
either partially or fully, by a thrombus
• Saccular aneurysms have a "neck” that connects the aneurysm to its
main ("parent") artery, a larger, rounded area, called the dome
• Fusiform aneurysms ("spindle-shaped" aneurysms) are variable in
both their diameter and length; their diameters can extend up to
20 cm (7.9 in). They often involve large portions of the ascending
and transverse aortic arch, the abdominal aorta, or, less frequently,
the iliac arteries
Location
• Aneurysms can also be classified by their location:
• Arterial and venous, with arterial being more common
• The heart, including coronary artery aneurysms, ventricular
aneurysms, aneurysm of sinus of Valsalva, and aneurysms following
cardiac surgery.
• The aorta, namely aortic aneurysms including thoracic aortic
aneurysms and abdominal aortic aneurysms
• The brain, including cerebral aneurysms, berry aneurysms
• The legs, including the popliteal arteries
• The kidney, including renal artery aneurysm and intraparechymal
aneurysms
• Capillaries, specifically capillary aneurysms.
• The Large vessels such as external and internal jugular veins
• Cerebral aneurysms, also known as intracranial
or brain aneurysms, occur most commonly in
the anterior cerebral artery, which is part of
the circle of Willis
• This can cause severe strokes leading to death
• The next most common sites of cerebral
aneurysm occurrence are in the internal carotid
artery
Signs and symptoms
• Aneurysm presentation may range from life-
threatening complications of hypovolemic
shock to being found incidentally on X-ray
• Symptoms will differ by the site of the
aneurysm and can include
Cerebral aneurysm
• Fatigue
• Loss of perception
• Loss of balance
• Speech problems
• Severe headaches
• Loss of vision
• Double vision
• Neck pain or stiffness
• Pain above or behind the eyes
ABDOMINALANEURYSM
• Abdominal aneurysms are
usually asymptomatic but in rare cases can
cause lower back pain or lower limb ischemia
Renal (kidney) aneurysm
• Flank pain and tenderness
• Hypertension
• Haematuria
• Signs of hypovolemic shock
Risk factors
• Risk factors for an aneurysm
include diabetes, obesity, hypertension, tobacco
use, alcoholism, high cholesterol, copper deficiency,
increasing age, and tertiary syphilis infection
• Connective tissue disorders such as Loeys-Dietz
syndrome, Marfan syndrome, and certain forms of
Ehlers-Danlos syndrome are also associated with
aneurysms
• Aneurysms, dissections, and ruptures in individuals
under 40 years of age are a major diagnostic criteria of
the vascular form of Ehlers-Danlos syndrome
Pathophysiology
• Multiple factors, including factors affecting a blood vessel wall and the blood through the vessel,
contribute.
• The pressure of blood within the expanding aneurysm may also injure the blood vessels supplying
the artery itself, further weakening the vessel wall
• Without treatment, these aneurysms will ultimately progress and rupture
• Infection. A mycotic aneurysm is an aneurysm that results from an infectious process that involves
the arterial wall
• A person with a mycotic aneurysm has a bacterial infection in the wall of an artery, resulting in the
formation of an aneurysm
• One of the causes of mycotic aneurysms is infective endocarditis
• The most common locations include arteries in the abdomen, thigh, neck, and arm
• A mycotic aneurysm can result in sepsis, or life-threatening bleeding if the aneurysm ruptures
• Less than 3% of abdominal aortic aneurysms are mycotic aneurysms
• Syphilis. The third stage of syphilis also manifests as aneurysm of the aorta, which is due to loss of
the vasa vasorum in the tunica adventitia
• Copper deficiency. A minority of aneurysms are caused by copper deficiency, which results in a
decreased activity of the lysyl oxidase enzyme, affecting elastin, a key component in vessel walls
Treatment
• Endovascular or minimally invasive
techniques have been developed for many
types of aneurysms. Aneurysm clips are used
for surgical procedure i.e. clipping of
aneurysms
VASCULITIS
• Vasculitis is inflammation of blood vessels, often
with ischemia, necrosis, and organ inflammation
• Vasculitis can affect any blood vessel—arteries,
arterioles, veins, venules, or capillaries
• Clinical manifestations of specific vasculitic
disorders are diverse and depend on the size and
location of the involved vessels, the extent of the
organ involvement, and the degree and pattern of
extravascular inflammation
• Vasculitis affects all ages, although some
types are restricted to certain age groups
• Vasculitis tends to affect Caucasians,
although many African-Americans are affected
• Vasculitis has a genetic component, but is
not heritable
• Vasculitis is a chronic relapsing disease,
although some patients experience prolonged
remission
Inflammatory destruction of blood vessels
• Infiltration of vessel wall with inflammatory
cells
– Leukocytoclasis
– Elastic membrane disruption
• Fibrinoid necrosis of the vessel wall
• Ischemia, occlusion, thrombosis
• Aneurysm formation
• Rupture, hemorrhage
Vasculitis: Classification
• Large-vessel vasculitis
– Aorta and the great vessels (subclavian, carotid)
– Claudication, blindness, stroke
• Medium-vessel vasculitis
– Arteries with muscular wall
– Mononeuritis multiplex (wrist/foot drop), mesenteric
ischemia, cutaneous ulcers
• Small-vessel vasculitis
– Capillaries, arterioles, venules
– Palpable purpura, glomerulonephritis, pulmonary
hemorrhage
• Large-vessel vasculitis
– Giant cell arteritis, Takayasu’s arteritis
– Behcet’s disease, Cogan’s syndrome
• Medium-vessel vasculitis
– Polyarteritis nodosa
– Buerger’s disease, Central nervous system vasculitis,
Kawasaki’s disease, Rheumatoid vasculitis
• Small-vessel vasculitis
– Wegener’s, microscopic polyangiitis, Churg-Strauss
– Cryoglobulinemic vasculitis, Henoch-Schönlein
purpura
ANCA-associated vasculitis
• Wegener’s granulomatosis: granulomatous
inflammation involving the respiratory tract and
necrotizing vasculitis affecting small to medium-
sized vessels
• Microscopic polyangiitis: Necrotizing vasculitis
affecting the small vessels
• Churg-Strauss Syndrome: Eosinophil-rich and
granulomatous inflammation involving the
medium-sized vessels, and associated with asthma
and eosinophilia
Wegener’s
Churg-
Strauss
MPA
Necrotizing Granuloma
Hypereosinophilia
•Sinusitis
•Subglottic stenosis
•Pulmonary nodules
•Orbital pseudotumor
•Asthma
•Pulmonary infiltrates
•Myocarditis
•Pulmonary capillaritis
•Glomerulonephritis
•Sensory neuropathy
•Mononeuritis multiplex
• Diagnosis of a systemic vasculitis is often a diagnosis of
exclusion, based on recognition of the clinical syndrome
– e.g. Churg-Strauss: adult onset asthma x 2 years, followed by
atypical pneumonias, followed by peripheral nerve involvement
• Biopsy of involved organ is the most straightforward
method of establishing a diagnosis
– Biopsy may be helpful to exclude infection/malignancy
• Other tests may be suggestive, but not diagnostic
– ESR, CRP
– CT: pulmonary hemorrhage, cavitary lesions
– Bronchoscopy: pulmonary hemorrhage (hemosiderosis)
– Urinalysis: for patients with kidney vasculitis
– ANCA (antineutrophil cytoplasmic antibodies)
– Angiogram (including MRA, CT-angiogram)
Vasculitis: Diagnosis
THANK YOU

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ANEURYSM AND VASCULITIS.pptx

  • 1. ANEURYSM AND VASCULITIS DR. ASHUTOSH SINGH ASSISTANT PROFESSOR
  • 2. • An aneurysm is an outward bulging, likened to a bubble or balloon, caused by a localized, abnormal, weak spot on a blood vessel wall • Aneurysms may be a result of a hereditary condition or an acquired disease • Aneurysms can also be a nidus (starting point) for clot formation (thrombosis) and embolization • As an aneurysm increases in size, the risk of rupture, which leads to uncontrolled bleeding, increases • Although they may occur in any blood vessel, particularly lethal examples include aneurysms of the Circle of Willis in the brain, aortic aneurysms affecting the thoracic aorta, and abdominal aortic aneurysms • Aneurysms can arise in the heart itself following a heart attack, including both ventricular and atrial septal aneurysms
  • 3. True and false aneurysms • A true aneurysm is one that involves all three layers of the wall of an artery (intima, media and adventitia) • True aneurysms include atherosclerotic, syphilitic, and congenital aneurysms, as well as ventricular aneurysms that follow transmural myocardial infarctions (aneurysms that involve all layers of the attenuated wall of the heart are also considered true aneurysms)
  • 4. • A false aneurysm, or pseudoaneurysm, is a collection of blood leaking completely out of an artery or vein but confined next to the vessel by the surrounding tissue. This blood-filled cavity will eventually either thrombose (clot) enough to seal the leak or rupture out of the surrounding tissue • Pseudoaneurysms can be caused by trauma that punctures the artery, such as knife and bullet wounds,as a result of percutaneous surgical procedures such as coronary angiography or arterial grafting, or use of an artery for injection
  • 5. Morphology • Aneurysms can also be classified by their macroscopic shapes and sizes and are described as either saccular or fusiform. The shape of an aneurysm is not specific for a specific disease • Saccular aneurysms, or "berry" aneurysms, are spherical in shape and involve only a portion of the vessel wall; they usually range from 5 to 20 cm (2.0 to 7.9 in) in diameter, and are often filled, either partially or fully, by a thrombus • Saccular aneurysms have a "neck” that connects the aneurysm to its main ("parent") artery, a larger, rounded area, called the dome • Fusiform aneurysms ("spindle-shaped" aneurysms) are variable in both their diameter and length; their diameters can extend up to 20 cm (7.9 in). They often involve large portions of the ascending and transverse aortic arch, the abdominal aorta, or, less frequently, the iliac arteries
  • 6. Location • Aneurysms can also be classified by their location: • Arterial and venous, with arterial being more common • The heart, including coronary artery aneurysms, ventricular aneurysms, aneurysm of sinus of Valsalva, and aneurysms following cardiac surgery. • The aorta, namely aortic aneurysms including thoracic aortic aneurysms and abdominal aortic aneurysms • The brain, including cerebral aneurysms, berry aneurysms • The legs, including the popliteal arteries • The kidney, including renal artery aneurysm and intraparechymal aneurysms • Capillaries, specifically capillary aneurysms. • The Large vessels such as external and internal jugular veins
  • 7. • Cerebral aneurysms, also known as intracranial or brain aneurysms, occur most commonly in the anterior cerebral artery, which is part of the circle of Willis • This can cause severe strokes leading to death • The next most common sites of cerebral aneurysm occurrence are in the internal carotid artery
  • 8. Signs and symptoms • Aneurysm presentation may range from life- threatening complications of hypovolemic shock to being found incidentally on X-ray • Symptoms will differ by the site of the aneurysm and can include
  • 9. Cerebral aneurysm • Fatigue • Loss of perception • Loss of balance • Speech problems • Severe headaches • Loss of vision • Double vision • Neck pain or stiffness • Pain above or behind the eyes
  • 10. ABDOMINALANEURYSM • Abdominal aneurysms are usually asymptomatic but in rare cases can cause lower back pain or lower limb ischemia
  • 11. Renal (kidney) aneurysm • Flank pain and tenderness • Hypertension • Haematuria • Signs of hypovolemic shock
  • 12. Risk factors • Risk factors for an aneurysm include diabetes, obesity, hypertension, tobacco use, alcoholism, high cholesterol, copper deficiency, increasing age, and tertiary syphilis infection • Connective tissue disorders such as Loeys-Dietz syndrome, Marfan syndrome, and certain forms of Ehlers-Danlos syndrome are also associated with aneurysms • Aneurysms, dissections, and ruptures in individuals under 40 years of age are a major diagnostic criteria of the vascular form of Ehlers-Danlos syndrome
  • 13. Pathophysiology • Multiple factors, including factors affecting a blood vessel wall and the blood through the vessel, contribute. • The pressure of blood within the expanding aneurysm may also injure the blood vessels supplying the artery itself, further weakening the vessel wall • Without treatment, these aneurysms will ultimately progress and rupture • Infection. A mycotic aneurysm is an aneurysm that results from an infectious process that involves the arterial wall • A person with a mycotic aneurysm has a bacterial infection in the wall of an artery, resulting in the formation of an aneurysm • One of the causes of mycotic aneurysms is infective endocarditis • The most common locations include arteries in the abdomen, thigh, neck, and arm • A mycotic aneurysm can result in sepsis, or life-threatening bleeding if the aneurysm ruptures • Less than 3% of abdominal aortic aneurysms are mycotic aneurysms • Syphilis. The third stage of syphilis also manifests as aneurysm of the aorta, which is due to loss of the vasa vasorum in the tunica adventitia • Copper deficiency. A minority of aneurysms are caused by copper deficiency, which results in a decreased activity of the lysyl oxidase enzyme, affecting elastin, a key component in vessel walls
  • 14. Treatment • Endovascular or minimally invasive techniques have been developed for many types of aneurysms. Aneurysm clips are used for surgical procedure i.e. clipping of aneurysms
  • 15. VASCULITIS • Vasculitis is inflammation of blood vessels, often with ischemia, necrosis, and organ inflammation • Vasculitis can affect any blood vessel—arteries, arterioles, veins, venules, or capillaries • Clinical manifestations of specific vasculitic disorders are diverse and depend on the size and location of the involved vessels, the extent of the organ involvement, and the degree and pattern of extravascular inflammation
  • 16. • Vasculitis affects all ages, although some types are restricted to certain age groups • Vasculitis tends to affect Caucasians, although many African-Americans are affected • Vasculitis has a genetic component, but is not heritable • Vasculitis is a chronic relapsing disease, although some patients experience prolonged remission
  • 17. Inflammatory destruction of blood vessels • Infiltration of vessel wall with inflammatory cells – Leukocytoclasis – Elastic membrane disruption • Fibrinoid necrosis of the vessel wall • Ischemia, occlusion, thrombosis • Aneurysm formation • Rupture, hemorrhage
  • 18. Vasculitis: Classification • Large-vessel vasculitis – Aorta and the great vessels (subclavian, carotid) – Claudication, blindness, stroke • Medium-vessel vasculitis – Arteries with muscular wall – Mononeuritis multiplex (wrist/foot drop), mesenteric ischemia, cutaneous ulcers • Small-vessel vasculitis – Capillaries, arterioles, venules – Palpable purpura, glomerulonephritis, pulmonary hemorrhage
  • 19. • Large-vessel vasculitis – Giant cell arteritis, Takayasu’s arteritis – Behcet’s disease, Cogan’s syndrome • Medium-vessel vasculitis – Polyarteritis nodosa – Buerger’s disease, Central nervous system vasculitis, Kawasaki’s disease, Rheumatoid vasculitis • Small-vessel vasculitis – Wegener’s, microscopic polyangiitis, Churg-Strauss – Cryoglobulinemic vasculitis, Henoch-Schönlein purpura
  • 20. ANCA-associated vasculitis • Wegener’s granulomatosis: granulomatous inflammation involving the respiratory tract and necrotizing vasculitis affecting small to medium- sized vessels • Microscopic polyangiitis: Necrotizing vasculitis affecting the small vessels • Churg-Strauss Syndrome: Eosinophil-rich and granulomatous inflammation involving the medium-sized vessels, and associated with asthma and eosinophilia
  • 21. Wegener’s Churg- Strauss MPA Necrotizing Granuloma Hypereosinophilia •Sinusitis •Subglottic stenosis •Pulmonary nodules •Orbital pseudotumor •Asthma •Pulmonary infiltrates •Myocarditis •Pulmonary capillaritis •Glomerulonephritis •Sensory neuropathy •Mononeuritis multiplex
  • 22. • Diagnosis of a systemic vasculitis is often a diagnosis of exclusion, based on recognition of the clinical syndrome – e.g. Churg-Strauss: adult onset asthma x 2 years, followed by atypical pneumonias, followed by peripheral nerve involvement • Biopsy of involved organ is the most straightforward method of establishing a diagnosis – Biopsy may be helpful to exclude infection/malignancy • Other tests may be suggestive, but not diagnostic – ESR, CRP – CT: pulmonary hemorrhage, cavitary lesions – Bronchoscopy: pulmonary hemorrhage (hemosiderosis) – Urinalysis: for patients with kidney vasculitis – ANCA (antineutrophil cytoplasmic antibodies) – Angiogram (including MRA, CT-angiogram) Vasculitis: Diagnosis
  • 23.