9 Vascular Disorders

Lipoprotein Disorders
Lipoprotein fractions
1. Chylomicron
a. Transports diet-derived triglyceride in the blood
b. Synthesized in intestinal epithelium
Requires apolipoprotein -!" for assembly and secretion
c. #bsent during fasting
d. Source of fatty acids and glycerol
ii $sed to synthesize triglyceride in the liver and adipose
iii %ydrolysis by capillary lipoprotein lipase &C'L( leaves a chylomicron
remnant.
). *ery lo+-density lipoprotein &*L,L(
a. Transports liver-synthesized triglyceride in the blood
Requires apolipoprotein -1-- for assembly and secretion
b. Source of fatty acids and glycerol
ii $sed to synthesize triglyceride in the adipose
iii %ydrolysis by C'L produces intermediate-density lipoprotein &.,L( and
lo+-density lipoprotein &L,L(
). Lo+-density lipoprotein &L,L(
a. Transports cholesterol in the blood
b. Calculated L,L / cholesterol - high-density lipoprotein - triglyceride01
'resence of chylomicrons falsely lo+ers calculated L,L by increasing
diet-derived triglyceride.
c. 2unctions of cholesterol
ii Component of the cell membrane
iii Synthesis of vitamin ,3 adrenal corte4 hormones3 bile salts and acids
5. %igh-density lipoprotein &%,L(
a. 67ood cholesterol6
.ncreased by e4ercise3 +ine3 estrogen
d. Synthesized by the liver and small intestine
e. 2unctions of %,L
ii Source of apolipoproteins for other lipoprotein fractions
iii Removes cholesterol from atherosclerotic plaques
Lipoprotein fractions
1. Chylomicron
a. Transports diet-derived triglyceride in the blood
b. Synthesized in intestinal epithelium
Requires apolipoprotein -!" for assembly and secretion
c. #bsent during fasting
d. Source of fatty acids and glycerol
ii $sed to synthesize triglyceride in the liver and adipose
iii %ydrolysis by capillary lipoprotein lipase &C'L( leaves a chylomicron
remnant.
). *ery lo+-density lipoprotein &*L,L(
a. Transports liver-synthesized triglyceride in the blood
Requires apolipoprotein -1-- for assembly and secretion
b. Source of fatty acids and glycerol
ii $sed to synthesize triglyceride in the adipose
iii %ydrolysis by C'L produces intermediate-density lipoprotein &.,L( and
lo+-density lipoprotein &L,L(
). Lo+-density lipoprotein &L,L(
a. Transports cholesterol in the blood
b. Calculated L,L / cholesterol - high-density lipoprotein - triglyceride01
'resence of chylomicrons falsely lo+ers calculated L,L by increasing
diet-derived triglyceride.
c. 2unctions of cholesterol
ii Component of the cell membrane
iii Synthesis of vitamin ,3 adrenal corte4 hormones3 bile salts and acids
5. %igh-density lipoprotein &%,L(
a. 67ood cholesterol6
.ncreased by e4ercise3 +ine3 estrogen
d. Synthesized by the liver and small intestine
e. 2unctions of %,L
ii Source of apolipoproteins for other lipoprotein fractions
iii Removes cholesterol from atherosclerotic plaques
Lipoprotein fractions
1. Chylomicron
a. Transports diet-derived triglyceride in the blood
b. Synthesized in intestinal epithelium
Requires apolipoprotein -!" for assembly and secretion
c. #bsent during fasting
d. Source of fatty acids and glycerol
ii $sed to synthesize triglyceride in the liver and adipose
iii %ydrolysis by capillary lipoprotein lipase &C'L( leaves a chylomicron
remnant.
). *ery lo+-density lipoprotein &*L,L(
a. Transports liver-synthesized triglyceride in the blood
Requires apolipoprotein -1-- for assembly and secretion
b. Source of fatty acids and glycerol
ii $sed to synthesize triglyceride in the adipose
iii %ydrolysis by C'L produces intermediate-density lipoprotein &.,L( and
lo+-density lipoprotein &L,L(
). Lo+-density lipoprotein &L,L(
a. Transports cholesterol in the blood
b. Calculated L,L / cholesterol - high-density lipoprotein - triglyceride01
'resence of chylomicrons falsely lo+ers calculated L,L by increasing
diet-derived triglyceride.
c. 2unctions of cholesterol
ii Component of the cell membrane
iii Synthesis of vitamin ,3 adrenal corte4 hormones3 bile salts and acids
5. %igh-density lipoprotein &%,L(
a. 67ood cholesterol6
.ncreased by e4ercise3 +ine3 estrogen
d. Synthesized by the liver and small intestine
e. 2unctions of %,L
ii Source of apolipoproteins for other lipoprotein fractions
iii Removes cholesterol from atherosclerotic plaques
Selected lipoprotein disorders
page 11!
1. Type .. hyperlipoproteinemia
a. .ncrease in serum L,L above 18-mg0dL &915- mg0dL(
b. #cquired causes
i. 'rimary hypothyroidism
,ecrease in L,L receptor synthesis
ii. :ephrotic syndrome
.ncrease in L,L correlates +ith the degree of hypoalbuminemia
c. 2amilial hypercholesterolemia
i. #utosomal dominant disorder
ii. ,eficiency of L,L receptors
iii. Clinical findings
'remature coronary artery disease and stro;e
Tendon 4anthomas
Cholesterol deposit located over tendons &e.g.3 #chilles(
and e4tensor surfaces of <oints
=anthelasma
>ello+3 raised plaque on the eyelid
). Type ... hyperlipoproteinemia
a. 2amilial dysbetalipoproteinemia &6remnant disease6(
b. ,eficiency of apolipoprotein ?
i. ,ecreased liver upta;e of chylomicron remnants and .,L
ii. .ncrease in serum cholesterol and triglyceride
c. Clinical findings
i. 'almar 4anthomas in fle4or creases
ii. .ncreased ris; for coronary artery disease
5. Type .* hyperlipoproteinemia
a. .ncrease in *L,L
,ue to increase in synthesis or decrease in catabolism
b. #cquired causes
i. ?4cess alcohol inta;e
ii. 'rogesterone in oral contraceptives
iii. ,iabetes mellitus
b. 2amilial hypertriglyceridemia
i. #utosomal dominant disorder
ii. Clinical findings
?ruptive 4anthomas
>ello+3 papular lesions
.ncreased ris; for coronary artery and peripheral vascular
disease
!. #polipoprotein deficiency &abetalipoproteinemia(
c. #utosomal dominant disorder
d. ,eficiency of apolipoprotein -!" and -1--
i. ,eficiency of chylomicrons3 *L,L3 and L,L
ii. ,ecrease in serum cholesterol and triglyceride
e. Clinical findings
i. @alabsorption
Chylomicrons accumulate in villi and prevent reabsorption of
micelles.
@ar;ed decrease in vitamin ?
ii. #ta4ia3 hemolytic anemia +ith thorny RCs &acanthocytes(
Arteriosclerosis
#rteriosclerosis is thic;ening and loss of elasticity of arterial +alls.
@edial calcification
1. ,ystrophic calcification in the +all of muscular arteries
o ?4amples-calcification in uterine and radial arteries
). :o clinical consequence unless associated +ith atherosclerosis
#therosclerosis
page 111
Serum C-reactive peptide is increased in patients +ith disrupted
&inflammatory( plaques. 'laques may rupture and produce vessel thrombosis3
+hich leads to acute myocardial infarction &@.(. C-reactive protein may be a
stronger predictor of cardiovascular events than L,L.
page 111
page 11A
1. 'athogenesis
a. ?ndothelial cell damage of muscular and elastic arteries
b. Causes of endothelial cell in<ury
%ypertension3 smo;ing tobacco3 homocysteine3 L,L
c. Cell response to endothelial in<ury
ii @acrophages and platelets adhere to damaged endothelium.
iii Released cyto;ines cause hyperplasia of medial smooth muscle cells.
iiii Smooth muscle cells migrate to the tunica intima.
ivi Cholesterol enters smooth muscle cells and macrophages &called foam
cells(.
vi Smooth muscle cells release cyto;ines that produce e4tracellular matri4.
@atri4 components include collagen3 proteoglycans3 and elastin.
b. ,evelopment of fibrous cap &plaque(
ii Components of fibrous cap
Smooth muscle3 foam cells3 inflammatory cells3 e4tracellular
matri4
iii 2ibrous cap overlies a necrotic center.
Cellular debris3 cholesterol crystals &slit-li;e spaces(3 foam cells
iiii ,isrupted plaques may e4trude underlying necrotic material leading to
vessel thrombosis
ivi 2ibrous plaque becomes dystrophically calcified and ulcerated.
). Sites for atherosclerosis &descending order(
a. #bdominal aorta
b. Coronary artery
c. 'opliteal artery
d. .nternal carotid artery
5. Complications of atherosclerosis
a. *essel +ea;ness &e.g.3 abdominal aortic aneurysm(
b. *essel thrombosis
ii #cute @. &coronary artery(
iii Stro;e &internal carotid artery3 middle cerebral artery(
iiii Small bo+el infarction &superior mesenteric artery(
c. %ypertension
Renal artery atherosclerosis may activate the renin-angiotensin-
aldosterone system.
b. 'eripheral vascular disease
ii .ncreased ris; of gangrene
iii 'ain in the buttoc;s and +hen +al;ing &claudication(
b. Cerebral atrophy
#therosclerosis involving circle of Billis vessels or internal carotid artery
#rteriolosclerosis
%ardening of arterioles
1. %yaline arteriolosclerosis
a. 'athogenesis
.ncreased protein is deposited in the vessel +all and occludes the lumen
b. #ssociated conditions
ii ,iabetes mellitus
,ue to nonenzymatic glycosylation of proteins in the basement
membrane
asement membrane lea;s protein into the vessel +all.
iii %ypertension
.ncreased intraluminal pressure pushes plasma proteins into the
vessel +all.
). %yperplastic arteriolosclerosis
a. 'athogenesis
ii Renal arteriole effect caused by an acute increase in blood pressure.
?4ample-malignant hypertension
iii Smooth muscle cell hyperplasia and basement membrane duplication
b. #rterioles have an 6onion s;in6 appearance.
Vessel Aneurysms
*essel aneurysms are due to +ea;ening of the vessel +all3 follo+ed by dilation and a
tendency to rupture.
#bdominal aortic aneurysm
page 11"
1. $sually located belo+ the renal artery orifices
). 'athogenesis
a. #therosclerosis +ea;ens vessel +all
i. *essel +all stress increases +ith vessel diameter.
ii. Lumen fills +ith atheromatous debris and blood clots
b. 2amilial factors3 structural defects in connective tissue
5. Clinical findings
a. $sually asymptomatic
b. Rupture is the most common complication.
i. Severe bac; pain is follo+ed by hypotension from blood loss in the
retroperitoneum.
ii. # pulsatile mass can be palpated.
@ycotic aneurysm
1. 'athogenesis
a. *essel +all +ea;ening due to an infection
b. 2ungi that invade vessels
Aspergillus, Candida, Mucor
c. acteria that invade vessels
Bacteroides fragilis, Pseudomonas aeruginosa, Salmonella species
). Clinical findings
a. Thrombosis +ith or +ithout infarction
b. Rupture
erry aneurysm of cerebral arteries
1. 'athogenesis
a. ,efect at the <unction of communicating branches +ith main cerebral vessels
*essel lac;s an internal elastic lamina and smooth muscle.
b. Ris; factors for developing the aneurysm
ii :ormal hemodynamic stress
iii 'resence of hypertension of any cause
iiii Coarctation of the aorta
b. Rupture releases blood into the subarachnoid space
). Clinical findings
a. Sudden onset of severe occipital headache
,escribed as the 6+orst headache . have ever had6
b. :uchal rigidity from irritation of the meninges
c. Complications
ii ,eath may occur shortly after the bleed.
iii Rebleed3 hydrocephalus3 neurologic deficits
Syphilitic aneurysm
page 11"
page 118
#ortic regurgitation is a problem in closing the aortic valve. ecause the aortic
valve closes in diastole3 the murmur occurs in early diastole as blood lea;s
bac; into the ventricle. The increase in left ventricular end-diastolic volume
results in an increase in stro;e volume &increased systolic pressure(. lood
rapidly draining bac; into the left ventricle produces a drop in the diastolic
pressure. The +ide pulse pressure &difference bet+een the systolic and
diastolic pressure( is manifested by a hyperdynamic circulation &e.g.3
pulsating uvula3 bounding pulses(.
1. Complication of tertiary syphilis due to Treponema pallidum
o $sually occurs in men !- to 11 years of age
). 'athogenesis
a. T. pallidum infects the vasa vasorum of the ascending and transverse portions of
aortic arch.
i. *asculitis is called endarteritis obliterans.
ii. Characteristic plasma cell infiltrate is present in the vessel +all.
b. *essel ischemia of the medial tissue leads to dilation of the aorta and aortic valve
ring.
). Clinical findings
a. #ortic valve regurgitation
b. rassy cough
Left recurrent laryngeal nerve is stretched by the aneurysm.
#ortic dissection
@arfan syndrome is an autosomal dominant disorder resulting in the
production of +ea; elastic tissue due to a defect in synthesizing fibrillin.
Cardiovascular abnormalities dominate. ,ilation of the ascending aorta may
progress to aortic dissection or aortic regurgitation. @itral valve prolapse is
the most common valvular defect and is often associated +ith conduction
defects causing sudden death. S;eletal defects include eunuchoid
proportions &lo+er body length C upper body length3 arm span C height( and
arachnodactyly &spider hands(.
page 118
page 1A-
1. ?pidemiology
a. @en +ith a mean age of !- to A- years +ith antecedent hypertension
@ost common group
b. >oung patients +ith a connective tissue disorder
?4amples-@arfan syndrome3 ?hlers-,anlos syndrome &?,S(
). 'athogenesis
a. Cystic medial degeneration &C@,(
ii ?lastic tissue fragmentation
iii @atri4 material collects in areas of fragmentation in the tunica media
b. Ris; factors for C@,
ii .ncrease in +all stress
%ypertension3 pregnancy &increased plasma volume(3 coarctation
iii ,efects in connective tissue
@arfan syndrome &defect in elastic tissue(3 ?,S &defect in
collagen(
c. .ntimal tear
ii ,ue to hypertension or underlying structural +ea;ness in the media
iii $sually occurs +ithin 1-cm of the aortic valve
iiii lood dissects under arterial pressure through areas of +ea;ness.
ivi lood dissects pro4imally and0or distally.
). Clinical findings
a. #cute onset of severe retrosternal chest pain radiating to the bac;
b. #ortic valve regurgitation
ii ,ue to aortic valve ring dilation
iii # radiograph or echocardiogram sho+s +idening of the aortic valve root.
c. Loss of the upper e4tremity pulse
,ue to compression of the subclavian artery
b. Rupture
$sually into the pericardial sac3 pleural cavity3 or peritoneal cavity
Venous System Disorders
Saphenous venous system
1. Superficial veins drain blood into the deep veins via penetrating branches.
). *alves prevent reversal of blood flo+ into the superficial system.
5. ,eep veins direct blood to the heart.
Saphenous venous system
1. Superficial veins drain blood into the deep veins via penetrating branches.
). *alves prevent reversal of blood flo+ into the superficial system.
5. ,eep veins direct blood to the heart.
*aricose veins
1. #bnormally distended3 lengthened3 and tortuous veins
). Locations
a. Superficial saphenous veins &most common site(
b. ,istal esophagus &due to portal hypertension(
c. #norectal region &e.g.3 hemorrhoids(
d. Left scrotal sac &e.g.3 varicocele(
5. Superficial varicositiesD causesE
a. *alve incompetence
?4acerbated by pregnancy3 prolonged standing3 obesity3 oral
contraceptives3 advanced age
b. 2amilial tendency
c. Secondary to deep venous thrombosis
'hlebothrombosis
1. Thrombosis of a vein without inflammation
). Causes
a. Stasis of blood flo+
b. %ypercoagulability &e.g.3 antithrombin ... deficiency(
5. Location
a. @ost often occurs in the deep vein of the calf
b. Less common sites include portal vein3 hepatic vein3 dural sinuses
!. Clinical findings associated +ith deep vein thrombosis
a. 7eneral findings
i. S+elling
ii. 'ain on dorsifle4ion of foot and compression of calf
iii. 'itting edema distal to the thrombosis
b. 'ulmonary thromboembolism
Fccurs +hen the thrombus e4tends into the femoral vein
b. ,eep venous insufficiency
i. Stasis dermatitis
Frange discoloration &hemosiderin( around the an;les
Caused by rupture of the penetrating branches
ii. *aricosities develop in the superficial system
,ue to retrograde blood flo+ into the superficial system
Thrombophlebitis
page 1A-
page 1A1
1. 'ain and tenderness along the course of a superficial vein
). 'athogenesis
a. .ntravenous cannulation of veins
b. .nfection &Staphylococcus aureus(
c. Carcinoma of the pancreatic head
i. 'roduces superficial migratory thrombophlebitis
ii. ,ue to the release of thrombogenic substances by the cancer
5. Clinical findings
a. Tender and palpable cord
b. ?rythema and edema of the overlying s;in and subcutaneous tissue
Superior vena cava syndrome
1. 'athogenesis
a. ?4trinsic compression of the superior vena cava
b. ,ue to a primary lung cancer &8-G of cases(
$sually a small cell carcinoma of the lung
). Clinical findings
a. 6'uffiness6 and blue to purple discoloration of the face3 arms3 and shoulders
b. Retinal hemorrhage3 stro;e
Thoracic outlet syndrome
1. 'athogenesis
a. Compression of the neurovascular compartment in the nec;
b. Causes include cervical rib3 spastic anterior scalene muscles3 or positional
change in the nec; and arms
). Clinical findings
a. *ascular signs &e.g.3 arm 6falls asleep6 +hile person is sleeping(
b. :erve root signs &e.g.3 numbness3 paresthesias(
c. 'ositive #dson test
'ulse disappears +hen the arm is outstretched and the patient loo;s to
the side of the outstretched arm.
Lymphatic Disorders
Structure of lymphatic vessels
Lymphatic vessels have incomplete basement membranes3 +hich predisposes them to
infection and tumor invasion.
Structure of lymphatic vessels
Lymphatic vessels have incomplete basement membranes3 +hich predisposes them to
infection and tumor invasion.
#cute lymphangitis
1. .nflammation of lymphatics &6red strea;6(
2. $sually due to cellulitis caused by Streptococcus pyogenes
Lymphedema
page 1A1
page 1A)
1. Collection of lymphatic fluid in interstitial tissue or body cavities
). Fbstructive lymphedema
o Radiation damage follo+ing radical mastectomy
5. TurnerHs syndrome
a. Lymphedema of hands and feet in ne+borns caused by defective lymphatics
b. ,ilated lymphatic channels in the nec; &cystic hygroma( produce +ebbed nec;.
). Chylous effusions &e.g.3 pleural cavity(
a. Contain chylomicrons +ith triglyceride plus mature lymphocytes
b. ,amage to thoracic duct
Causes include malignant lymphoma3 trauma
Vascular Tumors and Tumor-like Conditions
Tumors
@ost tumors derive from small vessels or arteriovenous anastomoses in glomus bodies
Tumors
@ost tumors derive from small vessels or arteriovenous anastomoses in glomus bodies.
*essel tumors and tumor-li;e conditions
Table 9-1. Vascular Tumors and Tumor-like Conditions
Tumor/Condition Clinical indin!s
#ngiomyolipoma Iidney hamartoma composed of blood vessels3 muscle3 and mature adipose
tissue
#ssociation +ith tuberous sclerosis
#ngiosarcoma Liver angiosarcoma associated +ith e4posure to polyvinyl chloride3 arsenic3
thorium dio4ide
acillary angiomatosis enign capillary proliferation involving s;in and visceral organs in #.,S
patients
Simulates Iaposi sarcoma in #.,S
Caused by Bartonella henselae3 a gram-negative bacillus
Capillary hemangioma 2acial lesion in ne+borns that regresses +ith age
Cavernous hemangioma @ost common benign tumor of liver and spleen
@ay rupture if large
Cystic hygroma Lymphangioma in the nec; associated +ith TurnerHs syndrome
7lomus tumor ,erive from arteriovenous shunts in glomus bodies
'ainful red subungual nodule in a digit
%ereditary telangiectasia &#,( ,ilated vessels on s;in and mucous membranes in mouth and gastrointestinal
tract
Chronic iron deficiency anemia
Iaposi sarcoma @alignant tumor arising from endothelial cells or primitive mesenchymal cells
#ssociated +ith human herpesvirus type "
Raised3 red-purple discoloration that progresses from a flat lesion to a plaque
to a nodule that ulcerates
Common sites include s;in3 mouth3 and gastrointestinal tract
Lymphangiosarcoma @alignancy of lymphatic vessels
#rises out of longstanding chronic lymphedema after modified radical
mastectomy
'yogenic granuloma *ascular3 red pedunculated mass that ulcerates and bleeds easily
'ost-traumatic or associated +ith pregnancy &relation to estrogen(D usually
regress postpartum
Spider telangiectasia #rteriovenous fistula &disappears +hen compressed(
#ssociated +ith hyperestrinism &e.g.3 cirrhosis3 pregnancy(
Sturge-Beber syndrome &#,( :evus flammeus &6birthmar;6( on face in distribution of ophthalmic branch of
cranial nerve * &trigeminal(
.psilateral malformation of pia mater vessels overlying occipital and parietal
lobes
*on %ippel-Lindau syndrome
&#,(
Cavernous hemangiomas in cerebellum and retina
.ncreased incidence of pheochromocytoma and bilateral renal cell carcinomas
#,3 autosomal dominant.
Vasculitic
Disorders
.nflammation of small vessels &arterioles3 venules3 capillaries(3 medium-sized vessels
&muscular arteries(3 large vessels &elastic arteries(3 or combinations of these vessel types.
'athogenesis
1. Type ... hypersensitivity &immunocomple4(
o ?4ample-%enoch-SchJnlein purpura
). Type .. hypersensitivity &antigen-antibody(
o ?4ample-7oodpasture syndrome &anti-basement membrane antibodies(
5. #ntineutrophil cytoplasmic antibodies &#:C#(
a. #ctivate neutrophils causing release of their enzymes and free radicals resulting
in vessel damage
b. c-#:C# type
i. #ntibodies are directed against proteinase 5 in cytoplasmic granules.
ii. ?4ample-BegenerHs granulomatosis
c. p-#:C# type
i. #ntibodies are directed against myelopero4idase.
ii. ?4amples-microscopic polyangiitis3 Churg-Strauss syndrome
). ,irect invasion by all classes of microbial pathogens
Clinical findings
'urpura due to thrombocytopenia or vessel instability &e.g.3 scurvy( is not
palpable3 because acute inflammation is not involved.
page 1A5
page 1A!
1. Small vessel vasculitis
a. Called leu;ocytoclastic venulitis or hypersensitivity vasculitis
b. 7ross appearance
i. S;in overlying the vasculitis is hemorrhagic3 raised3 and painful to
palpation
Called palpable purpura
ii. ?4amples-%enoch-SchJnlein purpura3 microscopic polyangiitis
c. @icroscopic appearance
*essel is disrupted and contains a neutrophilic infiltrate associated +ith
nuclear debris and fibrinoid necrosis.
ii @edium-sized vessel vasculitis
a. @uscular artery vasculitis
b. 'resents +ith vessel thrombosis and infarction or aneurysms
c. ?4amples-polyarteritis nodosa3 Ia+asa;i disease
ii Large vessel vasculitis
a. ?lastic artery vasculitis
b. 'resents +ith loss of a pulse or stro;e
c. ?4amples-Ta;ayasu arteritis3 giant cell &temporal arteritis(
ii Summary table of vasculitides
Table 9-". Vasculitic Disorders# $lastic Artery% &uscular Artery% and Small
Vessel Disease
Disorder Vasculitis $pidemiolo!y/$tiolo!y
Clinical/Laboratory
indin!s
Ta;ayasuHs
arteritis
&6pulseless
disease6(
7ranulomatous large
vessel vasculitis
involving aortic arch
vessels
>oung #sian +omen and children #bsent upper e4tremity pulse
*isual defects3 stro;e
7iant cell
&temporal( arteritis
7ranulomatous large
vessel vasculitis
involving superficial
temporal and
ophthalmic arteriesD
#dults C 1- years of age Temporal headache3 <a+
claudication &pain +hen che+ing(
lindness on ipsilateral side
'olymyalgia rheumatica &muscle
and <oint painD normal serum
thrombi contain
microabscesses
creatine ;inase(
.ncreased ?SR
'olyarteritis
nodosa
:ecrotizing medium-
sized vessel vasculitis
involving renal3
coronary3 mesenteric
arteries &spares
pulmonary arteries(
@iddle-aged men
#ssociation +ith %s#g &5-G(
*essels at all stages of acute and
chronic inflammation
2ocal vasculitis produces
aneurysms &detected +ith
angiography(
Frgan infarction in ;idneys &renal
failure(3 heart &acute @.(3 bo+els
&bloody diarrhea(3 s;in &ischemic
ulcer(
Ia+asa;i disease :ecrotizing medium-
sized vessel vasculitis
involving coronary
arteries &e.g.3
thrombosis3
aneurysms(
Children 9 ! years of age ,esquamating rash3 s+elling of
hands and feet3 cervical
adenopathy3 oral erythema
#bnormal ?C7 &e.g.3 acute @.(
Corticosteroids contraindicated
&danger of vessel rupture(
Thromboangiitis
obliterans
&uergerHs
disease(
@edium-sized vessel
vasculitis +ith digital
vessel thrombosis
@en )1-1- years of age +ho
smo;e cigarettes
2oot claudication3 RaynaudHs
phenomenon3 ulceration3
gangrene
RaynaudHs
disease
@edium-sized vessel
vasculitis involving
digital vessels in
fingers and toes
>oung +omen
?4aggerated vasomotor
response to cold or stress
'aro4ysmal digital color changes
&+hite-blue-red sequence(
$lceration and gangrene in
chronic cases
RaynaudHs
phenomenon
@edium-sized vessel
vasculitis involving
digital vessels in
fingers and toes
#dult men and +omen
Secondary to other diseases
&e.g.3 systemic sclerosis3 CR?ST
syndrome(
Systemic sclerosis and CR?ST
syndromeE digital vasculitis +ith
vessel fibrosis3 dystrophic
calcification3 ulceration3 gangrene
BegenerHs
granulomatosis
:ecrotizing medium-
sized and small
vessel vasculitis
involving upper
respiratory tract3 lung3
renal vessels
Childhood to middle age :ecrotizing vasculitis in upper
respiratory tract &nasopharyn43
sinuses3 trachea(3 lo+er
respiratory tract &pulmonary
vesselsD infarction3 pneumonia(3
;idneys &crescentic
glomerulonephritis(
:ecrotizing granulomas in upper
respiratory tract &saddle nose
deformity(3 lungs c-#:C#
antibodies &C8-G of cases(
TreatmentE corticosteroids3
cyclophosphamide
@icroscopic
polyangiitis
Small vessel
vasculitis involving
s;in3 lung3 brain3 7.
tract3 and
postcapillary venules
and glomerular
capillaries
Children and adults
'recipitated by drugs &e.g.3
penicillin(3 infections &e.g.3
streptococci(3 immune disorders
&e.g.3 SL?(
*essels at same stage of
inflammation
'alpable purpura3
glomerulonephritis p-#:C#
antibodies &C"-G of cases(
Churg-Strauss
syndrome
Small vessel
vasculitis involving
s;in3 lung3 heart
vessels
Children and adults #llergic rhinitis3 asthma
p-#:C# antibodies &K-G of
cases(3 eosinophilia
%enoch-SchJnlein
purpura
Small vessel
vasculitis involving
s;in3 7. tract3 renal3
<oint vessels
Children and young adults
@ost common vasculitis in
children
.g# immunocomple4es
'alpable purpura of buttoc;s and
lo+er e4tremities
'olyarthritis3 glomerulonephritis3
7. bleeding
Cryoglobulinemia Small vessel
vasculitis involving
s;in3 7. tract3 renal
vessels
#dults
#ssociation +ith %C*3 type .
@'7:
CryoglobulinsE immunoglobulins
that gel at cold temperatures
'alpable purpura3 acral cyanosis
of nose and ears and RaynaudHs
phenomenon &reverses +hen in
+arm room(
Serum sic;ness Small vessel
vasculitis involving
immunocomple4
Children and adults
Complication of treatment of
rattlesna;e envenomation +ith
2ever3 urticaria +ith vasculitis3
arthralgia3 7. pain +ith melena
deposition in s;in
vessels
horse-or sheep-based antivenin
.nfectious
vasculitis
Small vessel
vasculitis involving
s;in vessels
Children and adults
.nvolves all microbial pathogens
Roc;y @ountain spotted feverE
tic; transmission of Rickettsia
rickettsiae organisms invade
endothelial cellsD petechiae on
palms spread to trun;
,isseminated meningococcemia
due to !eisseria meningitidis
Capillary thrombosis produces
hemorrhage into s;in and
confluent ecchymoses
c-#:C#3 cytoplasmic antineutrophil cytoplasmic antibodiesD ?C73 electrocardiogramD ?SR3 erythrocyte sedimentation rateD
7.3 gastrointestinalD %s#g3 hepatitis surface antigenD %C*3 hepatitis C virusD @.3 myocardial infarctionD @'7:3
membranoproliferative glomerulonephritisD p-#:C#3 perinuclear antineutrophil cytoplasmic antibodiesD SL?3 systemic lupus
erythematosus.
'ypertension
%ypertension is defined as systolic blood pressure above 1!-mm%g and diastolic blood
pressure above 8-mm%g for a sustained period.
'athophysiology of hypertension
*asodilators include nitric o4ide3 prostaglandins3 histamine3 L-bloc;ers3 and
calcium-channel bloc;ers.
2actors that contract arteriole smooth muscle cells causing vasoconstriction
include M-adrenergic stimuli3 catecholamines3 angiotensin ..3 vasopressin3 and
increased total body sodium.
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1. Systolic blood pressure
a. Correlates +ith stro;e volume
b. ,etermination of stro;e volumeE
i. lood volume &equates +ith sodium homeostasis(
ii. 2orce of contraction
iii. %eart rate
). ,iastolic blood pressure
a. ,etermination of diastolic blood pressure
i. *olume of blood in the arteries +hile the heart is filling in diastole
,epends on the vascular tone of the peripheral resistance
arterioles
ii. ?lastic recoil of the aorta
b. Role of the total peripheral resistance &T'R( arterioles
i. #bbreviated 'oiseuilleHs equation
T'R / viscosity of blood0&radius of arteriole(
!

ii. *asodilation decreases T'R
,ecreases diastolic blood pressure
.ncreases venous return to the heart
iii. *asoconstriction increases T'R
.ncreases diastolic blood pressure
5. Role of sodium in hypertension
a. ?4cess sodium increases plasma volume
.ncreases stro;e volume and systolic blood pressure
b. ?4cess sodium produces vasoconstriction of T'R arterioles
i. Sodium enters arteriole smooth muscle cells and opens calcium
channels3 causing vasoconstriction.
ii. .ncreases diastolic blood pressure
?ssential hypertension
1. #ccounts for 81G of cases of hypertension
). 'athogenesis
a. 7enetic factors reduce renal sodium e4cretion.
b. $n;no+n factors cause vasoconstriction of arterioles.
c. Fbesity3 stress
Reduced renal sodium e4cretion is the primary mechanism of essential
hypertension in blac; #mericans and the elderly. .ncreased plasma volume
suppresses renin release from the <u4taglomerular apparatus.
Secondary hypertension
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1. #ccounts for 1G of cases of hypertension
). Renovascular hypertension
a. Causes
i. ?lderly men
#therosclerotic plaque partially bloc;s blood flo+ at the renal
artery orifice.
ii. >oung to middle-aged +omen
2ibromuscular hyperplasia occurs in multifocal areas of the renal
artery.
b. 'athogenesis
i. ,ecreased renal arterial blood flo+ activates the renin-angiotensin-
aldosterone &R##( system.
ii. #ngiotensin .. vasoconstricts T'R arterioles.
iii. #ldosterone increases sodium retention.
c. Clinical findings
i. Severe3 uncontrollable hypertension
ii. .ncreased plasma renin activity &'R#(
iii. .nvolved ;idney has increased 'R# in the renal vein.
iv. $ninvolved ;idney has decreased 'R#.
.ncreased plasma volume due to aldosterone e4cess suppresses
R## system in normal ;idney
v. ?pigastric bruit
Sound is due to turbulence of blood flo+ through the narro+ renal
artery.
vi. #ngiography
.nvolved ;idney sho+s diminished size &atrophy( and delayed
emptying.
Renal artery has 6beaded6 appearance in fibromuscular
hyperplasia.
d. Fther causes of secondary hypertension
Table 9-(. Causes o) Secondary 'ypertension
System or
Source Description
#drenal Cushing syndromeE increased mineralocorticoids
'heochromocytomaE increased catecholamines
:euroblastomaE increased catecholamines
11-%ydro4ylase deficiencyE increased mineralocorticoids &i.e.3 deo4ycorticosterone(
'rimary aldosteronism &Conn syndrome(E increased aldosterone
#orta 'ostductal coarctationE activation of R## system
?lderlyE systolic hypertension due to decreased elasticity of the aorta
C:S .ntracranial hypertensionE release of catecholamines
,rugs Fral contraceptiveE increased synthesis of angiotensinogenD most common cause of
hypertension in young +omen
CocaineE increased sympathetic activity
'arathyroid 'rimary hyperparathyroidismE calcium increases peripheral resistance arteriole smooth
muscle cell contraction
'regnancy 'reeclampsiaE increased angiotensin ..
Renal Renovascular diseaseE atherosclerosis &elderly men(3 fibromuscular hyperplasia &+omen(
Renal parenchymal diseaseE e.g.3 diabetic nephropathy3 adult polycystic ;idney disease3
glomerulonephritis
Thyroid 7ravesH diseaseE systolic hypertension from increased cardiac contraction
%ypothyroidismE diastolic hypertension due to retention of sodium
C:S3 central nervous systemD R##3 renin-angiotensin-aldosterone.
Complications of hypertension
Table 9-*. Complications o) 'ypertension
System Complications
Cardiovascular Left ventricular hypertrophyE most common overall complication
#cute myocardial infarctionE most common cause of death
#therosclerosis
C:S .ntracerebral hematomaE due to rupture of Charcot-ouchard aneurysms
erry aneurysmE rupture produces a subarachnoid hemorrhage
Lacunar infarctsE small infarcts due to hyaline arteriolosclerosis
Renal enign nephrosclerosisE ;idney disease of hypertensionD due to hyaline arteriolosclerosisD
atrophy of tubules and sclerosis of glomeruliD progresses to renal failure
@alignant hypertensionE rapid increase in blood pressure accompanied by renal failure and
cerebral edema
?yes %ypertensive retinopathyE arteriovenous nic;ing3 hemorrhage of retinal vessels3 e4udates
&increased vessel permeability3 retinal infarction(3 papilledema