Nonatherosclerotic Arterial Syndromes Amjad AlMahameed, MD, MPH, FACP Division of Cardiology Beth Israel Deaconess Medic

1. Nonatherosclerotic Arterial Syndromes Amjad AlMahameed, MD, MPH, FACP Division of Cardiology Beth Israel Deaconess Medical Center

2. Occlusive Arterial Disease 90% atherosclerotic 10% Non-atherosclerotic Vasculitidis, FMD, Thromboangiitis Obliterans, Thoracic Outlet Syndrome, Vasospastic Disorders, Other

3. 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 Temporal Arteritis .

4. Normal medium-sized artery Vessel affected by giant cell arteritis The inflammatory cells appear as dark blue dots. The giant cells are circled. GCA: TA/Takayasu’s

5. Arteries Commonly Affected by TA Temporal Artery Ophthalmic Artery Facial Artery Carotid Artery Central Retinal Artery Occlusion

6. Clinical Findings in Giant Cell Arteritis

7. Takayasu’s is LARGE Vessel Dz

8. Types of Involvement in Takayasu’s Arteritis Classical Takayasu’s Pulmonary Arteries Descending Aorta Asc. & Desc. Aorta

9. Subclavian stenosis in a pt with GCA and atherosclerosis

10. 16 y/o Female with Takayasu. Right and left renal art stenosis and SMA not present

11. Occluded L carotid artery from origin and severely stenosed R subclavian artery

12. (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

13. (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.

14. 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

15. PAN GCA 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.

16. Polyarteritis nodosa Low index of suspicion is necessary Headache Arthralgias & Myalgias Stroke Myocardial Ischemia Mesenteric Ischemia Nephritis & renal failure • No hematuria • 30% positive • for hepatitis B • surface antigen Liver damage Digital ischemia or Gangrene p-ANCA titers often are found but are not diagnostic Neuropathy Polyarteritis Nodosa: Presentation • Abdominal pain • Systemic hypertension • Anorexia and weight loss • Abdominal distention • Hematemesis, melena • Jaundice • Painless hematuria • Peripheral neuropathy • Tender subcutaneous nodules • Gangrene of fingers and toes HTN

17. Polyarteritis Nodosa Micro “Berry” aneurysms

18. Angiogram : coeliac axis : demonstrating aneurysms due to vasculitis (polyarteritis nodosa)

19. 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

20. Severe Skin Ulceration in PAN

21. Buerger’s Disease Buerger’s Thromboangiitis Obliterans Pathology Specimen shows INFLAMMED/SCARRED NEUROVASCULAR BUNDLE

22. 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

23. First reported by von Viniwalter in 1879 but first detailed description in 1908 by Leo Buerger.

24. 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

25. Buerger’s

26. Clinical Presentation of Thromboangiitis Obliterans

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

28. Fibromuscular Dysplasia

29. String of Beads

30. Classify the FMD Subtype Medial Fibroplasia Intimal Fibroplasia