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Flashcards in 11. Vascular Pathology Deck (55):

What is Polyarteritis nodosa (PAN)?

Segmental necrotizing vasculitis; it is a systemic vasculitis of small or medium-sized muscular arteries, typically involving renal and visceral vessels but sparing the pulmonary circulation. Symptoms result from ischemic damage to affected organs, often the skin, heart, kidneys, and nervous system. Generalised symptoms include fever, fatigue, weakness, loss of appetite, and weight loss. Muscle and joint aches are common.


What organ does polyarteritis nodosa avoid?

The lungs.


What are the three stages of damage that polyarteritis nodosa inflicts?

I. Acute lesion: fibrinoid necrosis and neutrophils. II. Healing lesions: fibroblast proliferation. III. Healed lesions: nodular fibrosis and loss internal elastic lamina.


What are possible complications of PAN?

Thrombosis, infarction, and aneurysms (kidney, heart, and GI tract).


What are some laboratory findings of PAN?

Perinuclear antineutrophil cytoplasmic autoantibodies (P-ANCA); they are autoantibodies against myeloperoxidase. It correlates with disease activity.


How is PAN diagnosed?

Arterial biopsy.


What is the treatment for PAN?

Corticosteroids and cyclophosphamide.


What is Churg-Strauss syndrome?

AKA allergic granulomatosis and angitis, it is a variant of polyarteritis nodosa, associated with bronchial asthma, systemic vasculitis with granulomas and eosinophilia, and involves the lung, splee, kidney, etc.


What is Wegener granulomatosis?

Necrotizing vasculitis with granulomas; classically involves the nose, sinuses, lungs, and kidneys. It affects small size arteries and veins.


What are clinical presentations of Wegener granulomatosis?

Bilateral pneumonitis with nodular and cavitary pulmonary infiltrates, chronic sinusitis, nasopharyngeal ulcerations, renal disease (Focal necrotizing glomerulonephritis, crescentic glomerulonephritis). Micro: fibrinoid necrosis, neutrophils, and granulomas.


What are some laboratory findings of Wegener granulomatosis?

Cytoplasmic antineutrophil cytoplasmic autoantibodies (C-ANCA); they are antibody against proteinase 3, correlates with disease activity. [Waggy Cunt]


What is the treatment for Wegener?

Immunosuppressive drugs (cyclophosphamide).


What is temporal arteritis?

Segmental granulomatous vasculitis of small and medium-seized arteries such as the cranial arteries (temporal, facial, and ophthalmic arteries), and rarely aortic arch (giant cell aortitis). Multinucleated giant cells and fragmentation of the internal elastic lamina and intimal fibrosis with luminal narrowing. AKA giant cell arteritis, it primarily affects the elderly population.


What are the symptoms of temporal arteritis?

Throbbing headache, tender and firm temporal arteries, visual disturbances (blurred or double vision, visual loss), facial pain, fever, malaise, weight loss, muscle aches, anemia. Polymyalgia rheumatica: systemic flu-like symptoms and joint involvement.


What are some laboratory findings of giant cell arteritis?

Elevated erythrocyte sedementation rate (ESR) and associated with HLA-DR4.


What is Takayasu arteritis?

Granulomatous vasculitis with massive intimal fibrosis, irregular fibrous thickening of the wall of the aortic arch, and narrowing of the orifices of the major arterial branches. Affects medium-sized to large arteries, aortic arch and major branches. AKA pulseless disease, affects young and middle-aged asian women.


What are the clinical presentations for Takayasu arteritis?

Loss of pulse in the upper extremeties, ocular manifestations (visual loss or field defect, retinal hemorrhages), and neurologic abnormalities.


What is Buerger disease?

Recurrent neutrophilic vasculitis with microabscesse, segmental thrombosis that leads to vascular insufficiency. Affects small and medium-sized arteries and veins; involves the extremeties.


What is Buerger disesase strongly associated with?



What are the clinical presentations for Buerger disease?

Severe pain (claudication) in the affected extremity, thrombophlebitis, Raynaud phenomenon, ulceration and gangrene.


What is the treatment for Buerger disease?

Smoking cessation.


What is mucocutaneous lymph node syndrome?

AKA Kawasaki disease, it is segmental necrotizing vasculitis, weakened vascular wall may undergo aneurysm formation. Affects all calibers of arteries (coronary artery commonly affected [70%]).


What are the symptoms of mycocutaneous lymph node syndrome?

Acute febrile illness, conjunctivitis (erythema and erosions of the oral mucosa), generalized maculopapular skin rash, lymphadenopathy.


What is the difference between Raynaud disease and Raynaud phenomenon?

The phenomenon is arterial insufficiency due to an underlying disease while the disease is due to episodic small artery vasospasm in the extremities, nose or ears.


What is arteriosclerosis?

A group of diseases that result in arterial wall thickening; Monckeberg, arteriolosclerosis, atherosclerosis.


What is Monckeberg medial calcific sclerosis?

Medial calcification of medium-sized arteries that may affect femoral, tibial, and ulnar arteries. It is asymptomatic anad may be deceted by x-ray.


What is arteriolosclerosis?

Hardening of small arteries and arterioles. Occurs in diabetes, hyperstension, and aging.


What is hyaline arteriolosclerosis?

Pink, glassy arterial wall thickening with luminal narrowing.


What is hyperplastic arteriolosclerosis?

Smooth-muscle proliferation resulting in concentric ("onin-skin") wall thickening and luminal narrowing.


What is atherosclerosis?

Lipid deposition and intimal thickening of large and medium-sized arteries, resulting in fatty streaks and atheromatous plaques. Distribution of disease: aorta, coronary, carotid, cerebral, iliac, and popiteal arteries.


What is hypertension?

A sustained diastolic pressure >90 mmHg and/or systolic pressure >140 mmHg.


What is primary hypetensions and percentage does it make up of total cases of hypertension?

Idiopathic; essential. 95%.


What are some microscopic changes seen in benign hypertension?

Hyaline arteriolosclerosis.


What is an aneurysm?

Congenital or acquired weakness of the vessel wall media, resulting in a localized dilatation or outpouching.


What happens to aortic aneurysms > 6 cm in diameter?

Half of them will rupture within 10 years.


What is a syphilitic aneurysm?

Involves the ascending aorta; syphilitic (leutic) aortitis causes an obliterative endarteritis of the vasa vasorum, leading to ischemia and smooth-muscle artrophy of the aortic media. May dilate the aortic valve ring causing aortic insufficiency.


What is an aortic dissecting aneurysm?

Blood from the vessel lumen enters and intimal tear and dissects through the layers of the media, caused by degeneration (cystic medial necrosis) of the tunica media. May compress and obstruct the aortic branches (e.g., renal or coronary arteries).


What is a Berry aneurysm?

A congenital aneurysm of the circle of Willis, may burst and cause a subarachnoid hemorrhage. May present as the wrose headche of his/her life.


What disease is associated with Berry aneurysm?

Adult polycystic kidney disease.


What are mycotic aneurysms?

Aneurysms due to BACTERIAL (not fungal) infections.


What is an arteriovenous (AV) fistula?

A direct communication between a vein and an artery without an intervening capillary bed.


How are arteriovenous fistulas acquired?



Where do most deep vein thrombosis (DVT) form?

Deep leg veins (90%): iliac, femoral, popiteal veins.


What is Homan sign?

A positive sign is present when there is pain in the calf on forceful and abrupt dorsiflexion of the patient's foot at the ankle while the knee is extended.


What is phleothrombosis?

Venous thrombosis without inflammation or infection.


What is thrombophlebitis?

Venous thrombosis due to inflammation and bacterial infection.


How are deep vein thrombosis detected?

By doppler "duplex" ultrasound.


What are varicose veins?

Dilated, tortuous veins caused by increased intraluminal pressure. It affects superficial veins of the lower extremities the most.


What are hemangioblastomas?

Multiple tumors involving the cerebellum, brain stem, spinal cord and retina.


Which disease are hemangioblastomas associated with?

von Hippel-Lindau disease.


What are glomangioma?

Glomus tumor, which are benign small, painful tumor of the glomus body in the dermis. Usually occur under fingernails.


Describe the African form of Kaposi sarcoma.

Affects African children and young men. Generalized lymphatic spread common.


Describe the Classic European form of Kaposi sarcoma.

Affects older men of Eastern European or Mediterranean origin. Causes red-purple plaques on the low extremities.


What is a Kaposi sarcoma?

Low-grade malignant tumor of endothelial cells, associated with (HHV8). Gross: Multiple red-purple plaques or nodules. Micro: Proliferation of spindle-shaped endothelial cells, slit-like vascular spaces, extravasaded RBCs. May remain confined to the skin or may disseminate.


What is another name for angiosarcoma?