Vasculitis

Question 1

————- refers to inflammation and necrosis of bld vessels

Answer 1

VASCULITIS

Question 2

These vessels may be damaged by

Answer 2

immune mechanisms,
infectious agents,
mechanical trauma,
radiation or toxins

Question 3

Majority are thought to involve immune mechanisms including;
• -deposition of ———-
• - direct attack on vessels by ———-
• - various forms of ———- immunity

Answer 3

• -immune complexes
• - circulating antibodies
• - cell mediated immunity

Question 4

Inciting agents may be associated with ———-

Answer 4

viral infection

Question 5

Some vasculitides are associated with ———— antibodies
• These can be detected by indirect ———— assays using the patient’s serum and ——- fixed ———

Answer 5

Somevasculitidesareassociatedwith antineutrophil cytoplasmic antibodies (ANCA)
• These can be detected by indirect immunoflourescence assays using the patient’s serum and ethanol fixed neutrophils

Question 6

POLYARTERITIS NODOSA
• PAN
• This acute, necrotizing, vasculitis affects mainly ———- -sized muscular arteries
• Male:Female = —:—
• — to —years
• — -ANCA immunoflourescence mainly against ————

Answer 6

POLYARTERITIS NODOSA
• PAN
• This acute, necrotizing, vasculitis affects mainly medium-sized muscular arteries
• Male:Female = 2:1
• 40-60 years
• P-ANCA immunoflourescence mainly against myeloperoxidase

Question 7

POLYARTERITIS NODOSA
• PAN
• Pathology
• Lesions are ——— and may not involve the entire circumference of the vessel
• Most prominent feature is an area of the ——— and ———, fused into an ———— mass
• Inflammatory response involves the entire ————
• B/C of thrombosis in the artery, ———- are commonly found in the involved organs
• Small ——— may form
• Healed lesions have a ——- media

Answer 7

POLYARTERITIS NODOSA
• PAN
• Pathology
• Lesions are patchy and may not involve the entire circumference of the vessel
• Most prominent feature is an area of the media and adventitia, fused into an eosinophilic mass
• Inflammatory response involves the entire adventitia (periarteritis)
• B/C of thrombosis in the artery, infarcts are commonly found in the involved organs
• Small aneurysm may form
• Healed lesions have a fibrotic media

Question 8

POLYARTERITIS NODOSA
• PAN
• Clinical Features
• ———-, ———, ————-, ———— and ———— are mostly involved
• ——— and ——— are common
• Without treatment PAN can be fatal
• ————— and ———— therapy can lead to remissions or cures.

Answer 8

POLYARTERITIS NODOSA
• PAN
• Clinical Features
• Kidneys, heart, skeletal muscle, skin and mesentery are mostly involved
• Fever and weight loss are common
• Without treatment PAN can be fatal
• Anti-inflammatory and immunosuppressive therapy can lead to remissions or cures.

Question 9

HYPERSENSITIVITY ANGIITIS
• HA
• This is a broad category of inflammatory vascular lesions representing a reaction to foreign materials such as ——— or ———-.
• It includes;
• —————
• —————

Answer 9

HYPERSENSITIVITY ANGIITIS
• HA
• This is a broad category of inflammatory vascular lesions representing a reaction to foreign materials such as bacterial products or drugs.
• It includes;
• Cutaneous vasculitis
• Systemic hypersensitivity angiitis

Question 10

HYPERSENSITIVITY ANGIITIS
• HA
• Cutaneous vasculitis
• May follow administration of drugs such as ———, ——- and ———
• ——— or ——— infections
• ———— purpura of the ———extremities is typical
•Superficial cutaneous venules display ———- necrosis and an ——— inflammatory reaction
• The disease is usually ———-

Answer 10

HYPERSENSITIVITY ANGIITIS
• HA
• Cutaneous vasculitis
• May follow administration of drugs such as aspirin, penicillin and thiazide diuretics
• Bacterial or viral infections
• Palpable purpura of the lower extremities is typical
•Superficial cutaneous venules display fibrinoid necrosis and an acute inflammatory reaction
• The disease is usually self limited

Question 11

HYPERSENSITIVITY ANGIITIS
• HA
• Systemic hypersensitivity angiitis
• Also called —————-
•May be an isolated entity or a feature of other vascular diseases
• May also exhibit —— lesions
•A feared complication is ——- involvement, characterized by rapidly progressive ———- and ———-
• It is associated with the presence of ———

Answer 11

HYPERSENSITIVITY ANGIITIS
• HA
• Systemic hypersensitivity angiitis
• Also called microscopic polyarteritis
•May be an isolated entity or a feature of other vascular diseases
• May also exhibit skin lesions
•A feared complication is renal involvement, characterized by rapidly progressive glomerulonephritis and renal failure
• It is associated with the presence of ANCA

Question 12

ALLERGIC GRANULOMATOSIS AND ANGIITIS
• Also known as ———-
• It occurs in (young/old?)people with ——- and features ———-
• Male:Female = —:—
• — to —years
• Affects ——— to —— sized vessels
• —% exhibit ——— or ———

Answer 12

ALLERGIC GRANULOMATOSIS AND ANGIITIS
• Also known as CHURG-STRAUSS SYNDROME
• It occurs in young people with asthma and
features eosinophilia
• Male:Female = 2:1
• 40-60 years
• Affects small to medium sized vessels
• 70% exhibit C-ANCA or P-ANCA

Question 13

ALLERGIC GRANULOMATOSIS AND ANGIITIS
• Also known as ————
• There is widespread necrotizing vascular lesions of ——- and ——— sized arteries in multiple organs
• There is prominent involvement of the ———
• Other organs involved are the ———, ———, ——— and ——

Answer 13

ALLERGIC GRANULOMATOSIS AND ANGIITIS
• Also known as CHURG-STRAUSS SYNDROME
• There is widespread necrotizing vascular lesions of small and medium sized arteries in multiple organs
• There is prominent involvement of the lungs
• Other organs involved are the kidneys, heart, liver and CNS

Question 14

————- is the most common vasculitis

Answer 14

GIANT CELL ARTERITIS
(TEMPORAL ARTERITIS, GRANULOMATOUS ARTERITIS)

Question 15

GIANT CELL ARTERITIS TEMPORAL ARTERITIS, GRANULOMATOUS ARTERITIS
• The most common vasculitis
• Average age at onset is — years (— to —)
• It involves a focal, chronic, ———— inflammation
• Usually of the ———— arteries and there branches
• Male:Female = —:—

Answer 15

GIANT CELL ARTERITIS TEMPORAL ARTERITIS, GRANULOMATOUS ARTERITIS
• The most common vasculitis
• Average age at onset is 70 years (50-75)
• It involves a focal, chronic, granulomatous inflammation
• Usually of the temporal (large) arteries and there branches
• Male:Female = 1:3

Question 16

GIANT CELL ARTERITIS TEMPORAL ARTERITIS, GRANULOMATOUS ARTERITIS
• Pathology
• Xterised by granulomatous inflammation of the ——— and ——— with ——— cells at the internal elastic lamina and infiltrates of ——— and ——— cells
• Clinical features
• Patients presents with ———- and
————- pain
• ——— symptoms occur in almost ——— of the patients
• ———- and ———— are present in the skin overlying the affected artery

Answer 16

GIANT CELL ARTERITIS TEMPORAL ARTERITIS, GRANULOMATOUS ARTERITIS
• Pathology
• Xterised by granulomatous inflammation of the media and intima with giant cells at the internal elastic lamina and infiltrates of leukocytes and plasma cells
• Clinical features
• Patients presents with headache and throbbing temporal pain
• Visual symptoms occur in almost half of the patients
• Swelling and tenderness are present in the skin overlying the affected artery

Question 17

WEGENER GRANULOMATOSIS
• A systemic necrotizing vasculitis
•Xterized by lesions of the ——-,———,——— and ———— disease
• Aetiology is known T or F
• Male:Female=—:—
• — to — years
• Affects ——- arteries
• >—% have ANCA in their blood; of those —% are — -ANCA, which suggests that ——— are responsible for the attack

Answer 17

WEGENER GRANULOMATOSIS
• Asystemicnecrotizingvasculitis
•Xterized by lesions of the nose,sinuses,lungs and renal glomerular disease
F • Aetiology is unknown
• Male:Female=1:1
• 30-50 years
• Affects small arteries
• >90% have ANCA in their blood; of those 75% are C-ANCA, which suggests that activated neutrophils are responsible for the attack

Question 18

WEGENER GRANULOMATOSIS
• Pathology
• Features ———- necrosis and ————— inflammation composed of ———- and other leukocytes
• Individual lesions may be as large as —cm across
• Involves ——- to ——- sized vessels
• Mostly in the ———-, ——— and ———

Answer 18

WEGENER GRANULOMATOSIS
• Pathology
• Features parenchymal necrosis and granulomatous inflammation composed of neutrophils and other leukocytes
• Individual lesions may be as large as 5cm across
• Involves small to medium sized vessels
• Mostly in the respiratory tract, kidney and spleen

Question 19

WEGENER GRANULOMATOSIS
• Clinical Features
• ————— and ——— are prominent
• ————- and ————- indicate glomerular involvement
• Untreated disease is ——
• Immunosuppressive treatment with ——- leads to striking improvement

Answer 19

WEGENER GRANULOMATOSIS
• Clinical Features
• Persistent bilateral pneumonitis and chronic sinusitis are prominent
• Haematuria and proteinuria indicate glomerular involvement
• Untreated disease is fatal
• Immunosuppressive treatment with cycloP leads to striking improvement

Question 20

TAKAYASU ARTERITIS
• Refers to an inflammatory disorder of the ———— and its major branches
• Affects ——— (<— years), — to —
• An ——— basis has been proposed
• Male:Female=—:—
• Pathology
• Aorta is thickened and has ————
• Branches of the aorta display stenosis and occlusion, interfering with bld flow and accounting for the synonym ‘———-’

Answer 20

TAKAYASU ARTERITIS
• Refers to an inflammatory disorder of the aortic arch and its major branches
• Affects young women (<30 years), 15-25
• An autoimmune basis has been proposed
• Male:Female=1:9
• Pathology
• Aorta is thickened and has focal raised plaques
• Branches of the aorta display stenosis and occlusion, interfering with bld flow and accounting for the synonym ‘pulseless dx’

Question 21

TAKAYASU ARTERITIS
• Clinical features
• Constitutional symptoms
• -
• -
• -
• Assymetric differences in ——— may develop, and the pulse in one extremity may ————

Answer 21

TAKAYASU ARTERITIS
• Clinical features
• Constitutional symptoms
• - dizziness
• - visual disturbance
• - dyspnoea
• Assymetric differences in blood pressure may develop, and the pulse in one extremity may disappear

Question 22

KAWASAKI DISEASE (also called ———— SYNDROME)
• An acute necrotizing vasculitis of ——- and early ——— xterized by
• -
• -
• -
• -
• Male:Female = —:—
• — to — years

Answer 22

KAWASAKI DISEASE (MUCOCUTANEOUS LYMPH NODE SYNDROME)
• An acute necrotizing vasculitis of infancy and early childhood xterized by
• - fever
• - rash
• - conjunctival and oral lesions
• - lymphadenitis
• Male:Female = 1:5
• 1-5 years

Question 23

KAWASAKI DISEASE (MUCOCUTANEOUS LYMPH NODE SYNDROME)
• Affects —— to —— sized vessels
• May affect the —— arteries and cause
aneurysms in —%
• An infectious cause is suspected
• Disease is usually ———

Answer 23

KAWASAKI DISEASE (MUCOCUTANEOUS LYMPH NODE SYNDROME)
• Affects large to medium sized vessels
• May affect the coronary arteries and cause
aneurysms in 70%
• An infectious cause is suspected • Disease is usually self-limited

Question 24

THROMBOANGIITIS OBLITERANS (also called——— DISEASE)
• Occlusive inflammatory dx of ——— and ——- arteries in the ——- and ——
• Exacerbated by ——— which plays an aetiological role in the disease
• An increased prevalence of HLA—- and HLA- — haplotypes among patients with the disease lends credence to the idea that a genetic hypersensitivity to ——— is involved in the pathogenesis.

Answer 24

THROMBOANGIITIS OBLITERANS (BUERGER DISEASE)
• Occlusive inflammatory dx of medium and small arteries in the distal arms and legs
• Exacerbated by smoking which plays an aetiological role in the disease
• An increased prevalence of HLA-A9 and HLA- B5 haplotypes among patients with the disease lends credence to the idea that a genetic hypersensitivity to tobacco is involved in the pathogenesis.

Question 25

THROMBOANGIITIS OBLITERANS (BUERGER DISEASE) • Pathology • Acute inflammation of the ——- and ———— arteries, with involvement of the ——— • May lead to ——— and obliteration of the lumen

Answer 25

THROMBOANGIITIS OBLITERANS (BUERGER DISEASE) • Pathology • Acute inflammation of the medium and small arteries, with involvement of the endothelium • May lead to thrombosis and obliteration of the lumen

Question 26

THROMBOANGIITIS OBLITERANS (BUERGER DISEASE) • Symptoms usually begins btw — to —years • Cramping pains in muscle during —— • Male:Female = —:— • Painful ischaemic disease can lead to —— of the extremity with possible necessity of ———

Answer 26

THROMBOANGIITIS OBLITERANS (BUERGER DISEASE) • Symptoms usually begins btw 25-40 years • Cramping pains in muscle during exercise • Male:Female = 1:9 • Painful ischaemic disease can lead to gangrene of the extremity with possible necessity of amputation

Question 27

RAYNAUD’S DISEASE • Refers to paroxysmal ——- and ——- of the digits of the ——-, ——, tip of the —— and —— • It is due to intense local ——- in (young/old?) healthy (women/men?) • It is often accompanied by ——— and ——- • Usually no organic change in vessel wall except later on when there is————— • Usually ———- and ——— and may lead to ——— and ——— of the tips of the digits

Answer 27

RAYNAUD’S DISEASE • Refers to paroxysmal pallor and cyanosis of the digits of the hands, feet, tip of the nose and ears • It is due to intense local vasospasm in young healthy women • It is often accompanied by paraesthesia and pain • Usually no organic change in vessel wall except later on when there is intimal proliferation • Usually bilateral and symmetrical and may lead to ulcers and gangrene of the tips of the digits

Question 28

RAYNAUD’S DISEASE • RAYNAUD’S PHENOMENON • Refers to ———— secondary to arterial narrowing induced by various conditions such as • - • - • -

Answer 28

RAYNAUD’S DISEASE • RAYNAUD’S PHENOMENON • Refers to arterial insufficiency secondary to arterial narrowing induced by various conditions such as • -SLE • -SCLERODERMA • -BUEGER’S DX

Question 29

VASCULITIS ASSOCIATED WITH OTHER DISORDERS • - those associated with connective tissue disorders (——-,———), ———— and ———— and malignancies may resemble —— •rheumatoid vasculitis tends to affect——- organs . Therefore, it is life-threatening. It occurs in —— with ———— and high titres of ——- factor • - most malignancies associated with vasculitis are of the ————type.

Answer 29

VASCULITIS ASSOCIATED WITH OTHER DISORDERS • - those associated withconnective tissue disorders (RA,SLE), cryoglobulinaemia and Henoch-Schonlein purpura and malignancies may resemble PAN • -rheumatoid vasculitis tends to affect visceral organs. Therefore, it is life-threatening. It occurs in RA with hypocomplimentaemia and high titres of rheumatoid factor • - most malignancies associated with vasculitis are of the lympho-proliferative type.

Question 30

BEHCET DISEASE • Widespread vasculitis of many organs • Xterized by —— and ——- ulcers; —— inflammation; occasional lesions in the ——, —— & —— • —— and ——- vessels are involved • — to —years • Male:Female = —:—

Answer 30

BEHCET DISEASE • Widespread vasculitis of many organs • Xterized by oral and genital ulcers; ocular inflammation; occasional lesions in the CNS, GIT & CVS • Large and small vessels are involved • 20-35 years • Male:Female = 1:1

Question 31

RADIATION VASCULITIS • It has an —— phase with injury to the endothelium and • A ——- phase with fibrosis of the vessel wall • Radiation damage predisposes to accelerated ———-

Answer 31

RADIATION VASCULITIS • It has an acute phase with injury to the endothelium and • A chronic phase with fibrosis of the vessel wall • Radiation damage predisposes to accelerated artherosclerosis

Question 32

INFECTIOUS ARTERITIS • Caused by direct invasion • ——— or ———, commonly • In bacterial/TB ———, bacterial ——- or pulmonary —- • It is important b/c it induces ——— leading to infarct, worsening the condition

Answer 32

INFECTIOUS ARTERITIS • Caused by direct invasion • Aspergillosis or mucormycosis, commonly • In bacterial/TB meningitis, bacterial pneumonia or pulmonary TB • It is important b/c it induces thrombosis leading to infarct, worsening the condition

Question 33

RICKETTSIAL VASCULITIS • ——— are intracellular parasites that produce a xteristic vasculitis • Different rickettsial dxs affect different types of ——- vessels in extent and severity.

Answer 33

RICKETTSIAL VASCULITIS • Rickettsia are intracellular parasites that produce a xteristic vasculitis • Different rickettsial dxs affect different types of small vessels in extent and severity.