RSL - Vasculature Flashcards
Temporal (Giant Cell) arteritis (Vessels)
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)
Takayasu arteritis (Vessels)
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
Polyarteritis Nodosa (Vessels)
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
Kawasaki Disease (vessels)
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
Buerger disease (thrombophlebitis obliterans)
(Vessels)
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
Granulomatosis with polyangitis
(Wegener)
Vessels?
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
Microscopic polyangitis
Vessels?
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
Eosinophilic granulomatosis with polyangitis (Churg-strauss)
Vessels?
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
Henoch-Schönlein purpura
(vessels)
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)
Trousseau syndrome (migratory thrombophebitis)
Cancer –> paraneoplastic –> hypercoaguablity
Small vessel hypersensititivy vasculitis
Adenocarcinomas; colon, pancreas, lung
Myxoma - symptoms
Ball valve obstruction –> Multiple postural sycopial episodes, hear tumor plop sound
Hepatic angiosarcoma
Blood vessel malignancy associated with vinyl chloride and arsenic exposures and thorotrast
Bacillary angiomatosis
Benign capillary skin papule in AIDS patients - caused by Bartonella henselae - Has neutrophilic infiltrate (vs. Kaposi with lymphocytic)
Cherry hemangioma
Elderly, does not regress
Cystic Hygroma (+ associatioN)
Cavernous lymphangioma of neck associated with Turners
Glomus tumor
Benign, painful, red-blue tumor under fingernails that arises from modified smooth muscle cells of thermoregulatory glomus body
Kaposi sarcoma
HHV-8 and HIV; lymphocytic infiltrate (vs. bacilliary angiomatosis which is neutrophillic)
Pyogenic granuloma
Polypoid capillary hemangioma; can ulcerate and bleed; associating with trauma and pregnancy
Strawberry hemangioma
Infants; grows rapidly and regresses spontaneously by 5-8 years
Raynaud phenomenon - pathophys
Vasospasm of skin blood vessels in response to cold or stress
White (ischemia), to blue (hypoxia), to red (reperfusion)
lymphangiosarcoma
Angiosarcome associated with radiationa nd chronic post-mastectomy lymphedema
Raynaud’s Rx
CCBs
Temporal (Giant Cell) arteritis - who
Elderly females
Takayasu arteritis - who
Asian females
Polyarteritis Nodosa - who
Medium sized vasculitis
Young adults
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
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
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
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
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
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)
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)
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
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)
Immune complex mediated; Transmural inflammation of arterial wall with fibrinoid necrosis
Treat with corticosteriods and cyclophosphamide
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
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
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
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
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
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)
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)
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
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
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
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
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
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
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)
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
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
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
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