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Aortitis
– Frequently affects ascending aorta and may result
in aneurismal dilatation and aortic regurgitation
(AR)
– It also can obstruct branch vessels of aorta
Types of aortitis
• Syphilitic aortitis – clinically signification in 10-15
years after infection, in older people
• Rheumatic aortitis – in young females
• Takayasu aortitis
• Giant cell aortitis – especially in 2nd half of life
Syphillitic aortitis
– Late variant of mycotic infection affecting proximal
ascending aorta especially aortic root causing
aortic dilatation and aneurysm formation
– May occasionally involve the aortic arch or the
descending aorta
– The aneurysm can be circular or fusiform and are
usually asymptomatic but compression and
erosion into adjacent structures may result in
symptoms
– Rupture also may occur
– Destruction of collagen and elastic tissues leads to
aortic dilatation, scar formation and calcification
– Disease presents typically in incidental radiological
finding within 15-30yo after initial infection
symptoms result from AR, narrowing of coronary
arteries with symptoms of angina (DDx with CAD),
compression of adjacent structures (e.g.
compression of esophagus lt dysphagia)
– Dx is done by serologic testing
– Rx :
○ Penicillin
○ Surgery in advance stage
– Arteriography can show absence of upper limb
pulses, stenoocclusive lesions
Rheumatic aortitis
> Systemic disease,
> ankylosing disease,
> psoriatic arteritis,
> Rieter’s syndrome,
> relapsing polyarthritis,
> inflammatory bowel syndrome,
> Inflammatory lesion involve ascending aorta and extend
to sinuses of Valsalva, mitral valve leaflets & adjacent
myocardium
> Late CM is aneurysm, AR, involvement of cardiac
conduction of system with different kinds of heart block
Takayasu’s Aortitis
– Inflammatory disease of aortic arch causing
obstruction of aorta and its major arteries
– Is also termed as pulseless disease because of Rx
of occlusion of large arteries are regenerating from
aorta
– It also may involve the descending thoracic and
abdominal aorta and occlude large branches such
as the renal arteries
– Aortic aneurysm may also occur
– The pathology is panarteriritis characterized by
mononuclear cells and occasionally giant cells
with marked intimal hyperplasia.
– Medial and adventitial thickening end in chronic
form of fibrotic occlusion
– Disease is prevalent in young female of Asian
descent
– During active stage of disease : fever, malaise,
LOW, myalgias and other general symptoms of
inflammation maybe evident
– ↑ ESR is common
– Lab : signs of autoimmune inflammation (high
level of circulating immune complexes and low
levels of complements, dysproteinemia with ↓
albumin &  globulin
– Chronic stage disease present with symptoms
related to large artery occlusion such as upper
extremity occlusion, cerebral ischemia, syncope
– The chronic disease is intermittently active
– Prognosis is grave because the process is
progressive & there’s no definitive therapy
– Involvement of renal arteries & their stenosis
cause vasorenal AHT often refractory to medical
treatment
– Systolic bruits over renal arteries & ________ are
symmetric in _______
– As the active phase of the disease,
immunosuppressive agents & GCS are effective
– Anti-coagulant for thrombosis & complete
occlusion of large arteries are useful (e.g. direct
HMW heparin, dextraparin, praxiparin)
– Disaggregants : aspirin, abxifimab
– Pt with high risk thromboembolism require LMW
heparin
– Advance stage of disease with ischemic
sign/symptoms in different localization due to
stenosis require surgery  intravascular stenting,
bypass
Giant Cell Arteritis
 This vasculitis occur in old patients, more often
women.
 Primarily large and medial sized arteries are
affected
 Pathology is that focal granulomatous lesions
involving the entire arterial wall
 It may be associated with polymyalgia rheumatica
 Obstruction of medium-sized artery (e.g. temporal,
ophthalmic artery & major branches of aorta &
development of aortitis & AR
 High dose GCS Rx (1 mg/kg prednisolone orally)
mb effective when given early
 The dx of vasculitis is a morphological diagnosis
 Temporal artery biopsy confirms this morphological
diagnosis
Marfan Syndrome
• Is a strong cause of aortic rupture
• Require diagnosis of ultrasonography (non-
invasive test done first)
• Rx : surgery
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Aortic occlusion has 3 features of RFS: ANTI-PHOSPHOLIPID SYNDROME & THROMBOPHILIA

1. DM Definition : a special kind of thrombophilia which is a result

2. Heavy smokers of thee circulation of antibodies which react with
3. AHT phospholipid determinants (structures of membranes of diff
– Require ultrasonography (non-invasive) then cells)
angiography
Synthesis of anti-phospholipid antibodies associated with
Stent Grafting for : auto-immune disturbances which lead to specific syndrome
characterized by residues of recurrences of venous-aortic
– Thoracic aortic pathology : aneurysm, thrombosis
pseudoaneurysm, dissection, penetrating ulcer
– Stent is one of the prophylaxis of aortic rupture Variants of APS :

Surgery of AATA 1. Secondary form of SLE

2. APS in lupus-like syndrome
1. Clamping 3. Primary APS – isolated form without any clinical
2. Removal of the dilated part
syndrome
3. Stenting
Primary APS

• Is of unknown origin
• Usually takes place in genetically predisposed
after exposure of provoking factors of
environment; mostly
○ infections,
○ trauma,
○ surgery,
○ tissue biopsy,
○ mental manipulation,
○ obstetrics manipulation,
○ in malignant tumors (paraneoplastic
syndrome)
• In ½ of all patients, provoking factors are
unidentified
• Mechanism of thrombogenic complication in APS
are difficult and multiple
• Most investigators state that circulation of AP Ab
lead to damage of membranes of thrombocytes
and endothelial cells and therefore endothelial
dysfunction
• In this case, vascular wall loses anti-thrombogenic
features, activate coagulation cascade and
depresses anti-coagulation dt imbalance &
proaction of prostacycline & thromboaxone A2.
• Imbalance due to activation of protein cascade
and depression of fibrinolysis due to activation of
tissue plasminogen inhibition.

Symptoms of APS

 Obstetric pathology
 Thrombocytopenia
 Different neurological, skin, CVS disturbances
associated with venous or arterial thrombosis

Venous thrombosis

1. DVT, phelibitis (veins of extremities), brain veins

thrombosis
2. HSM dt thrombosis of the small vessels,
hypereosinemia, Arnold-Chiari syndrome
(described in 1989, thrombosis of hepatic veins &
Vena Cava Inferior)
- suspect veins disturbances in recurrences even
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with treatment AHT in APS occurs as 2ndary HPT. It can run as vasorenal
- normally vein disturbances CM 1st is in GIT due to stenosis of main renal arteries, its branches or dt
- ulcer on extremities, painful ulcer intraglomerular thrombosis of Kidney (Renal Parenchymal
3. Thrombosis of renal veins with or w/o renal HPT), dt kidney infarction dt glomerular stenosis as a result
infarction of residues of microthrombosis.
4. Adrenal gland – thrombosis of central vein,
hemorrhages, infarction, adrenal insufficiency It is also possible to observe AHT dt thrombosis of abd aorta
5. Lungs – pulmonary thromboembolism, capilaritis, @ pseudo-coarctation
pulmonary HPT
6. Large veins – vena cava superior syndrome Pulmonary HPT
7. Skin nodules – livedo reticularis (lower & upper
extremities), purpura – Outcome : chronic cor pulmonale
8. Eyes – thrombosis of retinal veins with sudden – As a result of recurrences of thrombosis of lung
loss of vision vessels
– Mild pulmonary thrombosis mb undiagnosed
Arterial Thrombosis – Most cardiac manifestation is valve defect
formation, mostly defect of mitral valve and rarely
1. Extremities ischemia, gangrene aortic or tricuspid valves
2. Brain stroke – Secondary APS in SLE is associated with valvular
3. Heart heart defect relatively rare in comparison with
a. Large vessels : MI primary. Frequency is 33% in primary and 10% in
b. Small vessels : AHF, CMP 2ndary.
-in young patients without risk for ATS in MI, – Signs of differentiation with IE is necessary dt
search for other causes : vasculitis presence of failure, progression of HF and
4. Placenta – infarction, fetal death vegetation
5. Heart valves – vegetations, regurgitations,
stenosis Intracardiac Thrombosis
6. Aorta – aortic arch syndrome, bowel ischemia
7. Skin – digital gangrene, chronic leg ulcer  Relatively a rare manifestation which runs as a
8. Eyes – thrombosis of retinal arteries recurrence of thromboembolism, complication of
9. Bowel – aseptic necrosis different location
 MI dt pulmonary artery thrombosis causes death in
10% of cases esp aneurysm in SLE
– CM is polymorphic  In 1° APS, coronary artery thrombosis with
– Distal gangrene – severe pain, long term healing, symptoms of acute MI in young patients often
seen in APS & DM takes place in the absence of any signs of ATS
– In thrombosis, we can see purple spots on lower documented by coronography investigation
digits  MI can occur during child bearing aged women
w/o risk factor for ATS
Laboratory  APS can also manifest as cardiologic X-syndrome
(symptoms of myocardial ischemia, clinical ECG
• Thrombocytopenia and absence of vessel pathology on
• Hemolytic anemia coronography)
• False +ve (Wasserman’s reaction)  Acute or chronic thrombosis of small coronary
• Prolongation of APTT vessels can lead to myocardial pathology which
• Dyslipidemia runs as Dilated Cardiomyopathy with mild
• Immunodeficiency test (very specific) myocardial hypertrophy in combination with
○ High titers of AP Abs (the most dilatation of both heart chambers and progressive
informative for APS Abs to cardiolipine worsening of pump f(x)
& β2 glycoprotein I  In severe cases, attack of acute HF can occur as
○ Lupus anti-coagulant ○ Cardiogenic shock
○ Pulmonary edema
Multiple recurrent thrombosis of different location, pathology  Local cardiac pathology lead to diastolic
of obstructive heart diseases especially with pathology of dysfunction of heart by echocardiography
obstruction allow doctor to suspect APS  APS associated with rapid progression of ATS.
The main causes
Cardiologic manifestations: ○ Dysproteinemia
○ Hyperproduction of Abs to oxidated LDL
1. AHT  These factors can be assessed as independent &
2. Pulmonary HT strong risk factors of cardiovascular pathology
3. Intracardiac thrombosis  Oxidative LDL & hyperproduction of anti-oxidated
4. Thrombosis of coronary vessels LDL lead to adhesions & transformation of
5. Thrombotic occlusion after coronary artery bypass macrophageal cells to foamy cells stimulate
6. Rapid progression of atherosclerosis migration & proliferation of SM cells, aggregation
7. Aortic arch syndrome of thrombocytes & reduced vasodilatory effect of
8. Cardiomyopathy NO.
 Oxidated LDL are present in ATS plaques of diff
localization
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 Therefore its necessary to exclude APS & provide

additional examination : APS Abs in young
patients with CAD esp in the absence of signs of
ATS pathology proved with coronography
 In residues of thrombogenic complication, in
presence of clots in heart chambers, valve defects
& before planned surgical interventions of valves
or coronary vessels

Plan of the examination of patients with possible APS:

1) Anamnesis
a. Obstetric pathology
b. Previous thrombosis
c. Embolic complications of diff
localizations
2) CM
a. Skin disorders
3) Instrumental
a. Echo
b. ECG
c. USE
d. CT
e. MRI
4) Labs
a. CBC (TCP, APTT)
5) Immunologic test (specific test)

Treatment

 Drugs of choice dt high risk of thrombotic complication,

anti-coagulant all life
 In patients with higher titers of APS Abs and in the
absence of venous-arterio thrombosis
 In anamnesis, treatment with Aspirin
 Thrombogenic complications especially recurrences
require treatment with indirect anticoagulant
○ Warfarin
○ Control INR
 In high risk recurrence of arteriothrombosis, change dose
optimal til 10mg with goal of INR >3 IU
 In venous thrombosis, goal levels 2-3 IU
 In ineffectiveness of monotherapy (residual thrombosis),
use
○ Aspirin + other disaggregants
○ Aspirin, dipyridamole is indicated
 Additional risk factors of thrombosis in APS which require
adequate corrections, such as :
➢ AHT
➢ Hyperlipidemia
➢ Smoking
➢ Pregnancy
➢ OCP
➢ Infection
➢ Surgical manipulation
➢ Stress
➢ Hyperhomocysteinuria
➢ TCP
 Immunomodulators are still on experimental stages