Car 23 - Vascular Disease Flashcards

1
Q

What is Microscopic polyangiitis?

A

A small vessel vasculitis, it affects the kidneys, causing Pauci-immune crescentic glomerulonephritis. Usually (+) P-ANCA.

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2
Q

What is the difference b/w p-ANCA vs c-ANCA?

A

ANCA is Anti-Neutrophil Cytoplasmic Antibodies. Perinuclear-ANCA (p-ANCA) is made up of Ab against antigen Myeloperoxidase (MPO), therefore sometimes called MPO-ANCA. Cytoplasmic ANCA (c-ANCA) is made up of Ab against Proteinase 3 (PR3), therefore AKA PR3-ANCA.

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3
Q

What is Granulomatosis w/ Polyangiitis (GPA Or Wegener’s granulomatosis)?

A

A small vessel vasculitis, it has a Classic Triad: Focal necrotizing vasculitis, granulomas in lung and upper airway, glomerulonephritis. Granulomas in soft palate, oropharynx, nasal cavity, sinuses. Can also cause collapse of the bridge of the nose (saddle nose). Dyspnea, hemoptysis. Hematuria. (+) c-ANCA.

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4
Q

What disease affects both the lungs and kidney?

A

Granulomatosis w/ polyangiitis (Wegener’s). Goodpasture syndrome.

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5
Q

What is the treatment for Granulomatosis w/ polyangiitis (GPA)?

A

Cyclophosphamide. Corticosteroids.

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6
Q

What is Churg-Strauss syndrome?

A

A small vessel vasculitis, it has vasculitis with eosinophilia. Associated w/ asthma, sinusitis, skin lesions, peripheral neuropathy. (+) p-ANCA.

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7
Q

What is Henoch-Schonlein purpura?

A

A small vessel vasculitis, it is the most common form of systemic vasculitis in childhood. Classic tetrad: Causes rash on legs and buttocks made up of palpable purpura. Arthralgias. Abdominal pain (intestinal hemorrhage). Renal disease (IgA complexes in the kidney). Associated w/ recent URI.

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8
Q

What is Polyarteritis Nodosa (PAN)?

A

A medium-sized vasculitis, it is pretty nonspecific. Involves kidneys and visceral vessels (skin, GI tract, heart). However, it spares the lungs. Strongly associated w/ hepatitis B/C and (-) ANCA.

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9
Q

What is the treatment for Polyarteritis nodosa (PAN)?

A

Corticosteroids. +/- cyclophosphamide.

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10
Q

What is Kawasaki disease?

A

A medium-sized vasculitis, AKA Mucocutaneouse lymph node syndrome. Causes necrotizing vasculitis in infants, especially in Asian descent. Fever that last for at least 5 days and 4 of the following 5 symptoms: [CRASH] Conjunctivitis (bilateral nonexudative), Rash (trunk), Adenopathy (cervical), Strawberry tongue, Hands and feet (erethyma and descamation). Can cause coronary aneurysm resulting in rupture or thrombosis.

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11
Q

What is the treatment for Kawasaki disease?

A

IV immunoglobulin (IVIG). High-dose aspirin (to prevent thrombosis of coronary artery aneurysms).

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12
Q

What is Buerger disease?

A

A small and medium-sized veins, seen in male smokers in their 40s. AKA thromboangiitis obliterans. Causes Intermitten claudication. Superficial nodular phlebitis. Cold sensitivity.

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13
Q

What is the treatment for Buerger disease?

A

Stop smoking.

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14
Q

What is Temporal Arteritis?

A

A large-sized vasculitis, AKA Giant cell arteritis. Most common form of vasculitis. Causes inflammation of branches of external carotid artery, typically seen in elderly women. Causes unilateral headache and jaw claudication. Can progress to occlusion of the ophthalmic artery, causing vision loss and irreversible blindness. Causes elevation in ESR. Associated w/ PMR (pain and joint stiffness, affecting the proximal joints).

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15
Q

How do we diagnose Temporal arteritis?

A

Temporal artery biopsy.

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16
Q

What is the treatment for temporal arteritis?

A

High-dose steroids for several months.

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17
Q

What is Takayasu arteritis?

A

A large-sized arteritis, AKA pulseless disease. Causes granulomatous inflammation of arteries off the aortic arch, leading to poor pulses in extremities. Seen in young Asian women (teens to 20s). Causes elevation of ESR.

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18
Q

What vasculitis can you suspect in 2 y.o Asian female?

A

Kawasaki.

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19
Q

What vasculitis can you suspect in 20 y.o Asian female?

A

Takayasu.

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20
Q

What vasculitis can you suspect in someone with asthma?

A

Churg-Strauss.

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21
Q

What vasculitis can you suspect in young male smoker?

A

Buerger.

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22
Q

What vasculitis can you suspect that is associated w/ polymyalgia rheumatica?

A

Giant cell.

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23
Q

What vasculitis can you suspect that is associated w/ IgA nephropathy?

A

Henoch-Scholen purpura.

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24
Q

What vasculitis can you suspect that is associated w/ hepatitis B/

A

PAN.

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25
Q

What vasculitis can you suspect in elderly woman w/ jaw claudication and vision loss?

A

Giant cell.

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26
Q

What vasculitis can you suspect in Strawberry tongue?

A

Kawaski.

27
Q

What vasculitis can you suspect in desquamation of the hands/feet?

A

Kawasaki.

28
Q

What vasculitis can you suspect in poor pulses in the arms?

A

Takayasu.

29
Q

What vasculitis can you suspect in palpable purpura on the legs?

A

HSP.

30
Q

What vasculitis can you suspect in vasculitis of the kidney and GI tract, spares the lung?

A

PAN.

31
Q

What vasculitis can you suspect in vasculitis of the kidney, upper airway and lungs?

A

GPA.

32
Q

What is Raynaud phenomenon? What is this triggered by?

A

Arterial vasospasm that causes poor blood flow to the skin of the fingers. Triggered by cold temperature and emotional stress causing pallor or cyanosis. When it stops, the fingers get very red fro reperfusion.

33
Q

In what five disease can we see Raynaud phenomenon?

A

Raynaud disease. Mixed connective tissue disease. Buerger disease. Lupus. CREST scleroderma.

34
Q

What is the treatment for Raynaud phenomenon?

A

Dihydropyridine calcium channel blockers and low-dose aspirin.

35
Q

What is Sturge-Weber disease?

A

A congenital disorder that affects capillaries and causes a type of intracraneal AV malformation, called Leptomeningeal angioma affecting the brain and eyes. Causes port-wine stain in opthalmic division of trigeminal nerve. Can cause seizures, early onset glaucoma, and intellectual disability and hemiplegia.

36
Q

What is a Strawberry hemangioma?

A

A bening hemangioma of cutaneous surface capillaries. There is spontaneous regression.

37
Q

What is a Cherry hemangioma?

A

Benign hemangioma of cutaneous surface capillaries, They do not regress.

38
Q

What is a pyogenic granuloma?

A

Raised capillary hemangioma. Associated w/ trauma and pregnancy. Can ulcerate and bleed.

39
Q

What is Cystic hygroma?

A

A cavernous lymphangioma of neck. Associated w/ Turner syndrome.

40
Q

What is a glomus tumor?

A

A benign, painful reddish tumor on the hands, often in the fingernails.

41
Q

What is Bacillary angiomatosis?

A

Caused by Bartonella henselae. It is benign but can be mistaken for Kaposi sarcoma.

42
Q

What are the three different types of Kaposi sarcoma (KS)?

A

Indolent (older men, mediterranean descent). Endemic (sub-saharan). HHV-8 (AIDS).

43
Q

What is Angiosarcoma?

A

A blood vessel malignancy associated w/ prior radiation therapy. Usually found in face, scalp, neck and breast. Like to metastasize to brain and bone.

44
Q

What is lymphangiosarcoma?

A

Lymphatic malignancy associated w/ persistent lymphadema.

45
Q

What type of vasculitis is associated w/ necrotizing granulomas of lung and necrotizing glomerulonephritis?

A

Granulomatosis w/ polyangiitis.

46
Q

What type of vasculitis is associated w/ necrotizing immune complex inflammation of visceral/renal vessels?

A

Polyarteritis nodosa.

47
Q

What type of vasculitis is associated w/ young asthmatics?

A

Churg-Strauss.

48
Q

What type of vasculitis is associated w/ infants and young children; involves the coronary arteries.

A

Kawasaki disease.

49
Q

What is the most common vasculitis?

A

Temporal arteritis.

50
Q

What type of vasculitis is associated w/ hepatitis B infection?

A

Polyarteritis nodosa.

51
Q

What type of vasculitis is associated w/ occlusion of ophthalmic artery can lead to blindness?

A

Temporal arteritis.

52
Q

What type of vasculitis is associated w/ perforation of nasal septum?

A

Wegener’s granulomatosis.

53
Q

Which vascular tumor is a Benign, raised, red lesion about the size of a mole in older patients?

A

Cherry hemangioma.

54
Q

Which vascular tumor is a raised, red area presents at birth, increases in size initially then regresses over months to years?

A

Strawberry hemangioma.

55
Q

Which vascular tumor is a lesion caused by lymphoangiogenic growth factors in an HIV patient?

A

Kaposi sarcoma.

56
Q

Which vascular tumor is a polypoid red lesion found in pregnancy or after trauma?

A

Pyogenic granuloma.

57
Q

Which vascular tumor is a benign, painful, red-blue tumor under the fingernails?

A

Glomus tumor.

58
Q

Which vascular tumor is a cavernous lymphagioma associated w/ Turner syndrome?

A

Cystic hygroma.

59
Q

Which vascular tumor is a skin papule in AIDS patients cause by Bartonella?

A

Bacillart angiomatosis.

60
Q

RFF: Cold, pale painful digits.

A

Raynaud phenomenon.

61
Q

RFF: c-ANCA.

A

Granulomatosis w/ polyangiitis.

62
Q

RFF: p-ANCA.

A

Microscopic polyangiitis, Churg-Strauss.

63
Q

RFF: Tx for Buerger disease.

A

Stop smoking.

64
Q

RFF: Tx for temporal arteritis.

A

High-dose steroids.