Card - Path (Part 6: Vascular Disorders) Flashcards
Pg. 294-297 in First Aid 2014 Pg. 276-278 in First Aid 2013 Sections include: -Raynaud's phenomenon -Vasculitis -Vascular tumors
What is Raynaud’s phenomenon? What is the mechanism, and to what is it a response?
Decreased blood flow to the skin due to arteriolar vasospasm in response to cold temperature or emotional stress
Where is Raynaud’s phenomenon most often found on the body? What type of vessels does it affect?
Most often in the fingers and toes; Affects small vessels
What are the 2 different names given to Raynaud’s phenomenon, and in what contexts?
(1) Raynaud’s disease: primary (idiopathic) (2) Raynaud’s syndrome: secondary to a disease process (such as mixed connective tissue disease, SLE, or CREST [limited form of systemic sclerosis] syndrome)
What is Raynaud’s syndrome? What are some disease processes with which it may be associated?
Raynaud’s phenomenon (decreased blood flow to skin due to arteriolar vasospasm in response to cold temperature of emotional stress) secondary to a disease process (such as mixed connective tissue disease, SLE, or CREST [limited form of systemic sclerosis] syndrome)
Give 9 examples of vascular tumors.
(1) Strawberry hemangioma (2) Cherry hemangioma (3) Pyogenic granuloma (4) Cystic hygroma (5) Glomus tumor (6) Bacillary angiomatosis (7) Angiosarcoma (8) Lymphangiosarcoma (9) Kaposi sarcoma
What is a Strawberry hemangioma? When does it appear, and what is its incidence? How does it develop and/or resolve over time?
Benign capillary hemangioma of infancy. Appears in first few weeks of life (1/200 births); grows rapidly and regresses spontaneously at 5 to 8 years old
What is a Cherry hemangioma? Does it regress? How does it change with age?
Benign capillary hemangioma of the elderly. Does not regress. Frequency increased with age.
What defines a pyogenic granuloma? With what 2 conditions is it associated?
Polypoid capillary hemangioma that can ulcerate and bleed. Associated with trauma and pregnancy.
What is a cystic hygroma? With what condition is it associated?
Cavernous lymphangioma of the neck. Associated with Turner syndrome.
What is a glomus tumor? From where does it arise?
Benign, painful, red-blue tumor under fingernails. Arises from modified smooth muscle cells of glomus body.
What defines bacillary angiomatosis? What causes this? For what is this frequently mistaken?
Benign capillary skin papules found in AIDS patients. Caused by Bartonella henselae infections. Frequently mistaken for Kaposi sarcoma.
What is angiosarcoma? Where does it typically occur, and in what patient population?
Rare blood vessel malignancy typically occurring in the head, neck, and breast areas. Usually in elderly, on sun-exposed areas.
With what 2 exposures is angiosarcoma associated?
Associated with radiation therapy and arsenic exposure.
Briefly discuss the progression and resection of angiosarcoma.
Very aggressive and difficult to resect due to delay in diagnosis.
What is lymphangiosarcoma, and with what is it associated? Give an example of a context in which this may occur.
Lymphatic malignancy associated with persistent lymphedema (e.g., post-radical mastectomy)
What is Kaposi sarcoma, and where in the body is it most commonly found? Where else it found?
Endothelial malignancy most commonly of the skin, but also mouth, GI tract, and respiratory tract
With what 2 pathogens is Kaposi sarcoma associated?
Associated with HHV-8 and HIV
For what is Kaposi sarcoma frequently mistaken?
Frequently mistaken for bacillary angiomatosis
What are the 3 major categories of vasculitis?
(1) Large-vessel vasculitis (2) Medium-vessel vasculitis (3) Small-vessel vasculitis
What are 2 types of large-vessel vasculitis?
(1) Temporal (giant cell) arteritis (2) Takayasu arteritis
What is another name for temporal arteritis? What patient population is generally affected by it?
Temporal (giant cell) arteritis; Generally elderly females
What are 3 symptoms/signs in the presentation of Temporal (giant cell) arteritis? Where applicable, give the cause. With what other condition is Temporal (giant cell) arteritis associated?
(1) Unilateral headache (temporal artery), (2) Jaw claudication (3) May lead to irreversible blindness due to opthalmic artery occlusion.; Associated with polymyalgia rheumatica
Which vessels are most commonly affected by Temporal (giant cell) arteritis?
Most commonly affects branches of carotid artery
What are 2 histological/lab findings that characterize Temporal (giant cell) arteritis?
(1) Focal granulomatous inflammation (2) High ESR
How is Temporal (giant cell) arteritis treated, when, and why?
Treat with high-dose corticosteroids prior to temporal artery biopsy to prevent vision loss
What patient population does Takayasu arteritis affect?
Asian females < 40 years old
What are 7 signs/symptoms that characterize the presentation of Takayasu arteritis?
(1) “Pulseless disease” (weak upper extremity pulses), (2) fever, (3) night sweats, (4) arthritis, (5) myalgias, (6) skin nodules, (7) ocular disturbances
What are 2 histologic/lab findings that characterize Takayasu arteritis?
(1) Granulomatous thickening and narrowing of aortic arch and proximal great vessels (2) High ESR
How is Takayasu arteritis treated?
Treat with corticosteroids
What are 3 examples of medium-vessel vasculitis?
(1) Polyarteritis nodosa (2) Kawasaki disease (3) Buerger disease (thromboangiitis obliterans)
What patient population does polyarteritis nodosa affect?
Young adults
What kind of seropositivity is associated with polyarteritis nodosa, and what percentage of polyarteritis nodosa patients have it?
Hepatitis B seropositivity in 30% of patients
What are 10 signs/symptoms that characterize the presentation of polyarteritis nodosa?
(1) Fever, (2) Weight loss, (3) Malaise, (4) Headache. GI: (5) Abdominal pain, (6) Melena. (7) Hypertension, (8) Neurologic dysfunction, (9) Cutaneous eruptions, (10) Renal damage.
What vessels does polyarteritis nodosa typically involve versus not involve?
Typically involves renal and visceral vessels, not pulmonary arteries
What is the underlying mechanism of polyarteritis nodosa?
Immune complex mediated
What are 2 histologic/imaging findings associated with Polyarteritis nodosa?
(1) Transmural inflammation of the arterial wall with fibrinoid necrosis (2) Innumerable microaneurysms and spasm on arteriogram
How is Polyarteritis nodosa treated?
Treat with corticosteroids, cyclophosphamide
What patient population does Kawasaki disease affect?
Asian children < 4 years old
What 6 signs/symptoms characterize the presentation of Kawasaki disease?
(1) Fever, (2) Cervical lymphadenitis, (3) Conjunctival injection, (4) Changes in lips/oral mucosa (“strawberry tongue”), (5) Hand-foot erythema, (6) Desquamating rash
What cardiac complication(s) may arise from Kawasaki disease?
May develop coronary artery aneurysms, thrombosis => MI, rupture.
How is Kawasaki disease treated?
Treat with IV immunoglobulins and aspirin
What is another name for Buerger disease? What patient population does it affect?
Buerger disease (thromboangiitis obliterans); Heavy smokers, males < 40 years old
What are 3 main sign/symptoms/consequences of Buerger disease (thromboangiitis obliterans)? What is the underlying pathophysiology leading to these? What other condition is often present?
Intermittent claudication may lead to (1) gangrene, (2) autoamputation of digits, (3) superficial nodular phlebitis; Raynaud phenomenon is often present
Besides it being a medium-vessel vasculitis, what else characterizes the kind of vasculitis that occurs in Buerger disease?
Segmental thrombosing vasculitis
How is Buerger disease (thromboangiitis obliterans) treated?
Treat with smoking cessation
What are 4 examples of small-vessel vasculitis?
(1) Granulomatosis with polyangitis (Wegener) (2) Microscopic polyangitis (3) Churg-Strauss syndrome (4) Henoch-Schonlein purpura
What is another name for Granulomatosis with polyangitis?
Granulomatosis with polyangitis (Wegener)
What are 3 broad areas affected by Granulomatosis with polyangitis (Wegener)? What are the 9 signs/symptoms spread across these broad areas that characterize the presentation of Wegener?
UPPER RESPIRATORY TRACT: perforation of nasal septum, chronic sinusitis, otitis media, mastoiditis; LOWER RESPIRATORY TRACT: hemoptysis, cough, dyspnea; RENAL: hematuria, red blood cell casts
What is the triad that characterizes Granulomatosis with polyangitis (Wegener)?
Triad: (1) Focal necrotizing vasculitis (2) Necrotizing granulomas in the lung and upper airway (3) Necrotizing glomerulonephritis
What are 2 histologic/imaging findings that characterize Granulomatosis with polyangitis (Wegener)?
(1) PR3-ANCA/c-ANCA (anti-proteinase (2) CXR: large nodular densities
How is Granulomatosis with polyangitis (Wegener) treated?
Treat with cyclophosphamide, corticosteroids
What is microscopic polyangitis? What 3 organs does it commonly involve, and how so?
Necrotizing vasculitis commonly involving lung, kidneys, and skin with pauci-immune glomerulonephritis and palpable purpura
To what other vasculitis does microscopic polyangiitis present similarly? What distinguishes it from this other vasculitis?
Presentation similar to granulomatosis with polyangiitis but without nasopharyngeal involvement. Also, No granulomas.
What histologic finding characterizes microscopic polyangiitis?
MPO-ANCA/p-ANCA (anti-myeloperoxidase)
How is microscopic polyangiitis treated?
Treat with cyclophosphamide and corticosteroids
What are 4 major signs/symptoms of Churg-Strauss syndrome? What are 3 other organs that it can also involve?
Asthma, Sinusitis, Palpable purpura, Peripheral neuropathy (e.g., wrist/foot drop); Can also involve heart, GI, kidneys (pauci-immune glomerulonephritis).
How does Churg-Strauss syndrome impact the kidneys?
Kidneys (pauci-immune glomerulonephritis)
What are 3 histologic/lab findings that characterize Churg-Strauss syndrome?
(1) Granulomatous, necrotizing vasculitis with eosinophilia (2) MPO-ANCA/p-ANC (3) High IgE level
What is the most common childhood systemic vasculitis?
Henoch-Schonlein purpura
What condition does Henoch-Schonlein purpura often follow?
Often follows URI
What is the classic triad of Henoch-Schonlein purpura?
Classic triad: (1) Skin: palpable purpura on buttocks/legs (2) Arthralgias (3) GI: abdominal pain, melena, multiple lesions of same age
To what is the vasculitis in Henoch-Schonlein purpura secondary? With what condition is HSP associated?
Vasculitis secondary to IgA complex deposition; Associated with IgA nephropathy
