RSL - Vasculature Flashcards

(52 cards)

1
Q

Temporal (Giant Cell) arteritis (Vessels)

A

Large cell vasculitis

Elderly females

Unilateral headache

Jaw or tongue claudication during meals

Opthalmic occlusion –> irreversible blindness

Associated to polymyalgia rheumatica

Focal granulomatous inflammation of media with intimal fibrosis (branches of carotid; more distal than takayasu)

Increased ESR

Treat high dose corticosteroids (prevent blindness)

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2
Q

Takayasu arteritis (Vessels)

A

Large cell vasculitis

Asian females <40

Pulseless disease (weak upper extremity pulse)

Fever, nightsweats, arthritis, myalgia, skin nodules, ocular disturbances

Granulomatous medial thickening and narrowing of aortic arch and proximal great vessels

Increased ESR

treat with corticosteroids

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3
Q

Polyarteritis Nodosa (Vessels)

A

Medium sized vasculitis

Young adults

Hep B positive (30%)

Fever, weight loss, malaise, headache

GI: abdominal pain, melena

Hypertension, neurologic dysfunction, cutaneous eruptions (Livedo reticularis), renal damage, palpable purpura

NOT pulmonary, typically renal and visceral (numerous microaneurisms and vasospasms)

Immune complex mediated; Transmural inflammation of arterial wall with fibrinoid necrosis

Treat with corticosteriods and cyclophosphamide

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4
Q

Kawasaki Disease (vessels)

A

Medium sized Vasculitis

Asian children <4yrs

Mucocutaneous lymph node syndrome: CRASH & burn

  • Conjunctival injection
  • Rash (polymorphous –> desquamating)
    • Begins on extremitites and moves to trunk
  • Adenopathy (cervical)
  • Strawberry tongue (oral mucositis)
  • Hand-foot changes (edema, erythema)
  • Fever

Coronary artery aneurysms; thrombosis and rupture

Treat: IV Ig, and aspirin

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5
Q

Buerger disease (thrombophlebitis obliterans)

(Vessels)

A

Medium sized vasculitis

Heavey smokers, males <40 yrs old

Direct hypersensitivity to nicotine –> endothelial/nervous damage

Intermittant claudication –> ulceration, gangrene, autoamputation of digits

Associated to Raynaud phenomenon

Segmental thrombosing vasculitis

Treat with smoking sessation

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6
Q

Granulomatosis with polyangitis

(Wegener)

Vessels?

A

Small vessel vasculitis

URT: perforation of nasal septum, chronic sinusitis, otitis media, mastoiditis

LRT: hemoptysis, cough, dyspnea

Renal: Hematuria, red cell casts (RPGN)

Triad:

  • focal necrotizing vasculitis
  • Necrotizing granulomas in lung and upper airway
  • necrotizing glomerulonephritis

PR3-ANCA / c-ANCA (antiproteinase 3)

CXR: large nodular densities

Treat: cyclophosphamide and corticosteroids

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7
Q

Microscopic polyangitis

Vessels?

A

Small vessel vasculitis

Necrotizing vasculitis of Lung, kidneys, and skin

Pauci-immune glomerulonephritis with palpable purpura

NO nasopharyngeal involvement, NO granulomas

MPO-ANCA/p-ANCA (antimyeloperoxidase)

Treat: cyclophosphamide and corticosteroids

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8
Q

Eosinophilic granulomatosis with polyangitis (Churg-strauss)

Vessels?

A

Small vessel vasculititis

Asthma, sinusitis, skin nodules, purpura, peripheral neuropathy (wrist/foot drop)

Heart, GI, kidneys (pauci-immune glomerulonephritis)

Granulomatous, necrotizing vasulitis with eosinophilia

MPO-ANCA/p-ANCA, increased IgE

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9
Q

Henoch-Schönlein purpura

(vessels)

A

Small vessel vasculitis

Follows URI (dt/ IgA) - few days

Classic triad:

  • Skin: palpable purpura on buttocks / legs
  • Arthralgias
  • GI: abdominal pain

Vasculitis 2o to IgA immune complex deposition

Associated with IgA nephropathy (Berger disease)

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10
Q

Trousseau syndrome (migratory thrombophebitis)

A

Cancer –> paraneoplastic –> hypercoaguablity

Small vessel hypersensititivy vasculitis

Adenocarcinomas; colon, pancreas, lung

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11
Q

Myxoma - symptoms

A

Ball valve obstruction –> Multiple postural sycopial episodes, hear tumor plop sound

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12
Q

Hepatic angiosarcoma

A

Blood vessel malignancy associated with vinyl chloride and arsenic exposures and thorotrast

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13
Q

Bacillary angiomatosis

A

Benign capillary skin papule in AIDS patients - caused by Bartonella henselae - Has neutrophilic infiltrate (vs. Kaposi with lymphocytic)

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14
Q

Cherry hemangioma

A

Elderly, does not regress

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15
Q

Cystic Hygroma (+ associatioN)

A

Cavernous lymphangioma of neck associated with Turners

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16
Q

Glomus tumor

A

Benign, painful, red-blue tumor under fingernails that arises from modified smooth muscle cells of thermoregulatory glomus body

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17
Q

Kaposi sarcoma

A

HHV-8 and HIV; lymphocytic infiltrate (vs. bacilliary angiomatosis which is neutrophillic)

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18
Q

Pyogenic granuloma

A

Polypoid capillary hemangioma; can ulcerate and bleed; associating with trauma and pregnancy

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19
Q

Strawberry hemangioma

A

Infants; grows rapidly and regresses spontaneously by 5-8 years

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20
Q

Raynaud phenomenon - pathophys

A

Vasospasm of skin blood vessels in response to cold or stress

White (ischemia), to blue (hypoxia), to red (reperfusion)

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21
Q

lymphangiosarcoma

A

Angiosarcome associated with radiationa nd chronic post-mastectomy lymphedema

22
Q

Raynaud’s Rx

23
Q

Temporal (Giant Cell) arteritis - who

A

Elderly females

24
Q

Takayasu arteritis - who

A

Asian females

25
Polyarteritis Nodosa - who
Medium sized vasculitis Young adults
26
Kawasaki Disease - Symptoms
Asian children Mucocutaneous lymph node syndrome: **CRASH & burn** * **Conjunctival injection** * **Rash (polymorphous --\> desquamating)** * **Begins on extremitites and moves to trunk** * **Adenopathy (cervical)** * **Strawberry tongue (oral mucositis)** * **Hand-foot changes (edema, erythema)** * **Fever** Coronary artery aneurysms; thrombosis and rupture Treat: IV Ig, and **aspirin**
27
Buerger disease (thrombophlebitis obliterans) - pathophys
Heavey smokers, males **Direct hypersensitivity to nicotine --\> endothelial/nervous damage** Intermittant claudication --\> ulceration, gangrene, autoamputation of digits Associated to Raynaud phenomenon Segmental thrombosing vasculitis Treat with smoking sessation
28
Granulomatosis with polyangitis (Wegener) - Symptoms
Small vessel vasculitis **URT: perforation of nasal septum, chronic sinusitis, otitis media, mastoiditis** **LRT: hemoptysis, cough, dyspnea** **Renal: Hematuria, red cell casts (RPGN)** Triad: * focal necrotizing vasculitis * Necrotizing granulomas in lung and upper airway * necrotizing glomerulonephritis PR3-ANCA / c-ANCA (antiproteinase 3) CXR: large nodular densities Treat: cyclophosphamide and corticosteroids
29
Microscopic polyangitis - Histology
Small vessel vasculitis **Necrotizing vasculitis of Lung, kidneys, and skin** Pauci-immune glomerulonephritis with palpable purpura NO nasopharyngeal involvement, NO granulomas MPO-ANCA/p-ANCA (antimyeloperoxidase) Treat: cyclophosphamide and corticosteroids
30
Eosinophilic granulomatosis with polyangitis (Churg-strauss) - Symptoms
Small vessel vasculititis **Asthma, sinusitis, skin nodules, purpura, peripheral neuropathy (wrist/foot drop)** Heart, GI, kidneys (pauci-immune glomerulonephritis) Granulomatous, necrotizing vasulitis with eosinophilia MPO-ANCA/p-ANCA, increased IgE
31
Henoch-Schönlein purpura - Symptoms
Small vessel vasculitis **Follows URI (dt/ IgA) - few days** **Classic triad:** * **Skin: palpable purpura on buttocks / legs** * **Arthralgias** * **GI: abdominal pain** Vasculitis 2o to IgA immune complex deposition Associated with IgA nephropathy (Berger disease)
32
Temporal (Giant Cell) arteritis - Symptoms
Large cell vasculitis Elderly females **Unilateral headache** **Jaw or tongue claudication during meals** **Opthalmic occlusion --\> irreversible blindness** **Associated to polymyalgia rheumatica** Focal granulomatous inflammation of media with intimal fibrosis (branches of carotid; more distal than takayasu) Increased ESR Treat high dose corticosteroids (prevent blindness)
33
Takayasu arteritis - symptoms
Large cell vasculitis Asian females **Pulseless disease (weak upper extremity pulse)** **Fever, nightsweats, arthritis, myalgia, skin nodules, ocular disturbances** Granulomatous medial thickening and narrowing of _aortic arch_ and _proximal_ great vessels Increased ESR treat with corticosteroids
34
Polyarteritis Nodosa - SYmptoms
Medium sized vasculitis Young adults **Hep B positive (30%)** **Fever, weight loss, malaise, headache** **GI: abdominal pain, melena** Hypertension, neurologic dysfunction, cutaneous eruptions (Livedo reticularis), renal damage, palpable purpura **_NOT pulmonary,_** typically **renal and visceral** (numerous microaneurisms and vasospasms) **I**mmune complex mediated; **Transmural** inflammation of arterial wall with **fibrinoid necrosis** Treat with corticosteriods and cyclophosphamide
35
Kawasaki Disease - treat
Asian children Mucocutaneous lymph node syndrome: CRASH & burn * Conjunctival injection * Rash (polymorphous --\> desquamating) * Begins on extremitites and moves to trunk * Adenopathy (cervical) * Strawberry tongue (oral mucositis) * Hand-foot changes (edema, erythema) * Fever Coronary artery aneurysms; thrombosis and rupture **Treat: IV Ig, and aspirin**
36
Buerger disease (thrombophlebitis obliterans) - Associations
Heavey smokers, males Direct hypersensitivity to nicotine --\> endothelial/nervous damage Intermittant claudication --\> ulceration, gangrene, autoamputation of digits **Associated to Raynaud phenomenon** Segmental thrombosing vasculitis Treat with smoking sessation
37
Granulomatosis with polyangitis (Wegener) - Investigations
Small vessel vasculitis URT: perforation of nasal septum, chronic sinusitis, otitis media, mastoiditis LRT: hemoptysis, cough, dyspnea Renal: Hematuria, red cell casts (RPGN) Triad: * focal necrotizing vasculitis * Necrotizing granulomas in lung and upper airway * necrotizing glomerulonephritis **PR3-ANCA / c-ANCA (antiproteinase 3)** **CXR: large nodular densities** Treat: cyclophosphamide and corticosteroids
38
Microscopic polyangitis - Symptoms
Small vessel vasculitis Necrotizing vasculitis of Lung, kidneys, and skin **Pauci-immune glomerulonephritis with palpable purpura** **NO nasopharyngeal involvement, NO granulomas** MPO-ANCA/p-ANCA (antimyeloperoxidase) Treat: cyclophosphamide and corticosteroids
39
Eosinophilic granulomatosis with polyangitis (Churg-strauss) - histology
Small vessel vasculititis Asthma, sinusitis, skin nodules, purpura, peripheral neuropathy (wrist/foot drop) Heart, GI, kidneys (pauci-immune glomerulonephritis) **Granulomatous**, **necrotizing vasulitis** with **eosinophilia** MPO-ANCA/p-ANCA, increased IgE
40
Henoch-Schönlein purpura - Associations
Small vessel vasculitis Follows URI (dt/ IgA) - few days Classic triad: * Skin: palpable purpura on buttocks / legs * Arthralgias * GI: abdominal pain **Vasculitis 2o to IgA immune complex deposition** **Associated with IgA nephropathy (Berger disease)**
41
Temporal (Giant Cell) arteritis - histology
Large cell vasculitis Elderly females Unilateral headache Jaw or tongue claudication during meals Opthalmic occlusion --\> irreversible blindness Associated to polymyalgia rheumatica **Focal granulomatous inflammation of media with intimal fibrosis (branches of carotid; more distal than takayasu)** Increased ESR Treat high dose corticosteroids (prevent blindness)
42
Takayasu arteritis - histology
Large cell vasculitis Asian females Pulseless disease (weak upper extremity pulse) Fever, nightsweats, arthritis, myalgia, skin nodules, ocular disturbances **Granulomatous medial thickening and narrowing of _aortic arch_ and _proximal_ great vessels** Increased ESR treat with corticosteroids
43
Polyarteritis Nodosa - histology
Medium sized vasculitis Young adults Hep B positive (30%) Fever, weight loss, malaise, headache GI: abdominal pain, melena Hypertension, neurologic dysfunction, cutaneous eruptions (Livedo reticularis), renal damage, palpable purpura _NOT pulmonary,_ typically renal and visceral (numerous microaneurisms and vasospasms) **Immune complex mediated; Transmural inflammation of arterial wall with fibrinoid necrosis** Treat with corticosteriods and cyclophosphamide
44
Buerger disease (thrombophlebitis obliterans) - histology
Heavey smokers, males Direct hypersensitivity to nicotine --\> endothelial/nervous damage Intermittant claudication --\> ulceration, gangrene, autoamputation of digits Associated to Raynaud phenomenon **Segmental thrombosing** vasculitis Treat with smoking sessation
45
Granulomatosis with polyangitis (Wegener) - treat
Small vessel vasculitis URT: perforation of nasal septum, chronic sinusitis, otitis media, mastoiditis LRT: hemoptysis, cough, dyspnea Renal: Hematuria, red cell casts (RPGN) Triad: * focal necrotizing vasculitis * Necrotizing granulomas in lung and upper airway * necrotizing glomerulonephritis PR3-ANCA / c-ANCA (antiproteinase 3) CXR: large nodular densities **Treat: cyclophosphamide and corticosteroids**
46
Microscopic polyangitis - investigations
Small vessel vasculitis Necrotizing vasculitis of Lung, kidneys, and skin Pauci-immune glomerulonephritis with palpable purpura NO nasopharyngeal involvement, NO granulomas **MPO-ANCA/p-ANCA (antimyeloperoxidase)** Treat: cyclophosphamide and corticosteroids
47
Eosinophilic granulomatosis with polyangitis (Churg-strauss) - investigations
Small vessel vasculititis Asthma, sinusitis, skin nodules, purpura, peripheral neuropathy (wrist/foot drop) Heart, GI, kidneys (pauci-immune glomerulonephritis) Granulomatous, necrotizing vasulitis with eosinophilia **MPO-ANCA/p-ANCA, increased IgE**
48
Temporal (Giant Cell) arteritis - treat
Large cell vasculitis Elderly females Unilateral headache Jaw or tongue claudication during meals Opthalmic occlusion --\> irreversible blindness Associated to polymyalgia rheumatica Focal granulomatous inflammation of media with intimal fibrosis (branches of carotid; more distal than takayasu) Increased ESR **Treat high dose corticosteroids (prevent blindness)**
49
Takayasu arteritis - treat
Large cell vasculitis Asian females Pulseless disease (weak upper extremity pulse) Fever, nightsweats, arthritis, myalgia, skin nodules, ocular disturbances Granulomatous medial thickening and narrowing of _aortic arch_ and _proximal_ great vessels Increased ESR **treat with corticosteroids**
50
Polyarteritis Nodosa - treat
Medium sized vasculitis Young adults Hep B positive (30%) Fever, weight loss, malaise, headache GI: abdominal pain, melena Hypertension, neurologic dysfunction, cutaneous eruptions (Livedo reticularis), renal damage, palpable purpura _NOT pulmonary,_ typically renal and visceral (numerous microaneurisms and vasospasms) Immune complex mediated; Transmural inflammation of arterial wall with fibrinoid necrosis **Treat with corticosteriods and cyclophosphamide**
51
Buerger disease (thrombophlebitis obliterans) - treat
Heavey smokers, males Direct hypersensitivity to nicotine --\> endothelial/nervous damage Intermittant claudication --\> ulceration, gangrene, autoamputation of digits Associated to Raynaud phenomenon Segmental thrombosing vasculitis **Treat with smoking sessation**
52
Microscopic polyangitis - treat
Small vessel vasculitis Necrotizing vasculitis of Lung, kidneys, and skin Pauci-immune glomerulonephritis with palpable purpura NO nasopharyngeal involvement, NO granulomas MPO-ANCA/p-ANCA (antimyeloperoxidase) **Treat: cyclophosphamide and corticosteroids**