Nonatherosclerotic Arterial Syndromes Amjad Al Mahameed MD MPH

Nonatherosclerotic Arterial Syndromes Amjad Al. Mahameed, 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 mediumsized 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 Artery Central Retinal Artery Occlusion

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 Vision loss Ultimate Blindness 10% 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 Aorta Asc. & Desc. 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) [18 F]FDG-PET scan of patient with active TA at diagnosis. Note the markedly abnormal uptake of [18 F]FDG in the aortic arch and carotid arteries (arrows). (B) [18 F]FDG-PET scan of the same patient in remission after treatment with prednisolone and intravenous cyclophosphamide. Note almost complete resolution of abnormal [18 F]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 • Systemic hypertension • Anorexia and weight loss Low index of suspicion is necessary • Abdominal distention • No hematuria • Hematemesis, melena • Jaundice • 30% positive for hepatitis B surface antigen • Painless hematuria • Peripheral neuropathy • Tender subcutaneous nodules • Gangrene of fingers and toes Headache Arthralgias & Myalgias HTN Stroke Myocardial Ischemia Liver damage Mesenteric Ischemia Nephritis & renal failure Digital ischemia or Gangrene p-ANCA titers often are found but are not diagnostic Neuropathy

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 involvement Tender erythematous 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 No. of Patients 112 328 124 377 Mean age (years) 42 36 29 29. 5 Intermittent claudication % 63 18 32 89 Rest Pain % 81 50 Ischemic Ulcers % 76 50 88 Thrombophlebiti s% 38 51 59 62 Raynauds Phenomenon % 44 16 10 Coldness/cyanosis/ parathesia % 69 11 Olin Ohta 85

example of how to perform the Allen’s test on the hands is shown in figure 2 and 3. Allen’s test Source Collateralization around areas of occlusion (corkscrew collaterals)

Fibromuscular Dysplasia Medial Fibroplasia 75 -8 0% String of Beads (beads larger than the artery) Intimal Fibroplasia <10% Perimedial Fibroplasia 10 -1 5% Beads smaller than the diameter of the artery Long smooth stenosis

String of Beads

Classify the FMD Subtype Medial Fibroplasia Intimal Fibroplasia