Nonatherosclerotic Arterial Syndromes

Amjad AlMahameed, MD, MPH, FACP

Division of Cardiology
Beth Israel Deaconess Medical Center
 Occlusive Arterial Disease

90% atherosclerotic 10% Non-atherosclerotic

Vasculitidis, FMD,
Thromboangiitis Obliterans,
Thoracic Outlet Syndrome,
Vasospastic Disorders,
Other
Temporal Arteritis

Evidence of
temporal arteritis: A
painting by Jan Van
Eyck (c. 1385–
1440),
entitled “The Virgin”
with Canon Van der
Paele, 1436.
Notice the
prominent arteries
on the temples of
the bishop
.
 GCA: TA/Takayasu’s
Normal
medium-
sized
artery

Vessel
affected by
giant cell
arteritis

The inflammatory cells appear as

dark blue dots. The giant cells
are circled.
Arteries Commonly Affected by TA

Temporal Artery

Ophthalmic
Artery

Facial
Artery
Carotid Central Retinal Artery Occlusion
Artery
 Clinical Findings in Giant Cell Arteritis
ESR:      >50 90%
            >100 60%
Headache 70%
Tenderness of arteries 50%
Jaw claudication (predictive:ask) 50%
Bruits (listen to subclavian) 40%
PMR 50%
Alkaline Phosphatase 50%
Artery thickening 45%
No arterial pulse 40%
Visual symptoms:  Diplopia 10%
                         Vision loss 10%
                         Ultimate Blindness 15%
Weight Loss 40%
Claudication (non-jaw) 20%
Fever 20%
Takayasu’s is LARGE Vessel Dz
Types of Involvement in Takayasu’s Arteritis

Classical
Takayasu’s

Pulmonary
Arteries

Descending Asc. & Desc.

Aorta Aorta
Subclavian stenosis in a pt with GCA and atherosclerosis
 16 y/o Female with Takayasu.
Right and left renal art stenosis and SMA not present
Occluded L carotid artery from origin and severely
stenosed R subclavian artery
 (A) MRA patient with active TA at diagnosis. There is complete occlusion of the left SCA at its
origin (arrow) with numerous collaterals evident and an ostial stenosis of the left common
carotid artery. (B) MRA image from the same patient in remission. No significant progression of
the lesions found on the baseline MRA is seen.

Andrews, J et al. Ann Rheum Dis 2004;63:995-1000

 (A) [18F]FDG-PET scan of patient with active TA at diagnosis. Note the markedly abnormal
uptake of [18F]FDG in the aortic arch and carotid arteries (arrows). (B) [18F]FDG-PET scan of
the same patient in remission after treatment with prednisolone and intravenous
cyclophosphamide. Note almost complete resolution of abnormal [18F]FDG uptake in these
areas.

Andrews, J et al. Ann Rheum Dis 2004;63:995-1000

Copyright ©2004 BMJ Publishing Group Ltd.

 Polyarteritis Nodosa
• More common in adult males
• Spares the arterioles, capillaries, venules and glomeruli
• Associated with hepatitis B antigenemia
• Kidney (most frequently affected): 85%
• Multiple intrarenal aneurysms
• Aneurysms may thrombose and disappear
• Appear in new locations
GCA
PAN

prominent eosinophilic "fibrinoid" necrosis

involving the intima and media. In contrast to
GCA, there is no granulomatosis
inflammation. Notice also how the lumen has
been occluded by granulation tissue.
 Polyarteritis Nodosa: Presentation
Polyarteritis nodosa
• Abdominal pain
Low index of Headache HTN
• Systemic hypertension suspicion is Arthralgias
necessary & Stroke
• Anorexia and weight loss Myalgias
• No hematuria Myocardial
• Abdominal distention Ischemia
• 30% positive
Liver Mesenteric
• Hematemesis, melena for hepatitis B
Ischemia
surface antigen damage
Nephritis &
• Jaundice renal failure

• Painless hematuria
Digital
• Peripheral neuropathy ischemia or
Gangrene
• Tender subcutaneous nodules
• Gangrene of fingers and toes p-ANCA titers often
Neuropathy
are found but are not
diagnostic
Polyarteritis Nodosa
Micro “Berry” aneurysms
Angiogram : coeliac axis : demonstrating aneurysms
due to vasculitis (polyarteritis nodosa)
 Tender, hyperpigmented,
firm subcutaneous nodules
with a background of livedo
reticularis common in
cutaneous PAN

Nonspecific,
firm, tender
subcutaneous
nodules
without livedo
reticularis
and/or
systemic Tender erythematous
involvement nodules with central
"punched out" ulcerations
common in cutaneous PAN
Severe Skin Ulceration in PAN
 Buerger’s Disease
Buerger’s Thromboangiitis Obliterans
Pathology Specimen shows
INFLAMMED/SCARRED
NEUROVASCULAR BUNDLE
• The histopathological findings vary according to
the duration of the disease. The findings are
most likely to be diagnostic in the acute phase of
the disease
• The hallmark of the acute-phase lesion is an
occlusive, highly cellular, inflammatory
thrombus, with less inflammation in the walls of
the blood vessels. Polymorphonuclear
leukocytes, microabscesses, and multinucleated
giant cells may be present
• First reported by von Viniwalter in 1879
but first detailed description in 1908 by
Leo Buerger.
Self mutilation by smoking – this patient had
all four limbs amputated for a Buerger’s type
of arteritis. His cigarette holder was made out
of a coat hanger by one of his friends on the
ward
Buerger’s
 Clinical Presentation of
Thromboangiitis Obliterans
Kaniak Wysokinski Olin Ohta

No. of Patients 112 328 124 377

Mean age (years) 42 36 29 29.5
Intermittent 63 18 32 89
claudication %
Rest Pain % 81 50 85
Ischemic Ulcers % 76 50 88
38 51 59 62
Thrombophlebiti
s%
Raynauds 44 16 10
Phenomenon %
Coldness/cyanosis 69 11
/parathesia %
 example of how to perform the Allen’s test on the hands is shown in figure 2
and 3.

Collateralization
around areas of
Allen’s test occlusion (corkscrew
Source collaterals)
 Fibromuscular
Dysplasia

Medial 75 -8 0% String of Beads (beads

Fibroplasia larger than the artery)
Intimal <10% Long smooth stenosis
Fibroplasia
Perimedial 10 -1 5% Beads smaller than the
Fibroplasia diameter of the artery
String of Beads
 Classify the FMD Subtype

Medial Fibroplasia

Intimal Fibroplasia