Cardio - Mechanisms of Disease - Vasculitides; Cardiac Inflammation; Vascular Tumors Flashcards by Jason Worley

Most cases of vasculitis ________ (are/are not) infectious.

Most cases of vasculitis are not infectious.

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Vasculitides (e.g. polyarteritis nodosa) are often associated with __________ necrosis.

Vasculitides (e.g. polyarteritis nodosa) are often associated with fibrinoid necrosis.

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Name two forms of large-vessel vasculitis.

Takayasu arteritis

Temporal (giant cell) arteritis

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Name three forms of medium-vessel vasculitis.

Polyarteritis nodosa

Kawasaki’s arteritis

Buerger’s disease (thromboangiitis obliterans)

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Name two forms of small-vessel vasculitis.

Granulomatosis with polyangiitis (Wegener’s granulomatosis)

Microscopic polyangiitis

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Takayasu arteritis is also colloquially known as ‘_________’ disease.

Takayasu arteritis is also colloquially known as ‘pulseless’ disease.

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Takayasu arteritis typically affects which arteries?

Branches of the aorta and pulmonary arteries

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Both Takayasu arteritis and temporal arteritis are characterized on histopathology by the presence of _______ cells.

Both Takayasu arteritis and temporal arteritis are characterized on histopathology by the presence of giant cells.

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Takayasu arteritis typically affects ________ (men/women) ___ (>) 50 years of age.

Temporal arteritis typically affects ________ (men/women) ___ (>) 50 years of age.

Takayasu arteritis typically affects women < 50 years of age.

Temporal arteritis typically affects women > 50 years of age.

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What is the most common presentation for Takayasu arteritis?

Young woman;

visual/neurological changes;

weak/absent pulse in upper extremities

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What is the most common presentation for temporal arteritis?

Older woman;

headache;

visual changes;

elevated ESR;

polymyalgia rheumatica

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An older woman presents with headache, visual changes, highly elevated ESR, and polymyalgia rheumatica.

What do you do?

Start high-dose corticosteroids immediately

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Why must a biopsy test for giant cell (temporal) arteritis take a fairly long section of artery?

The granulomatous inflammation is segmental and easily missed

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The blindness associated with giant cell (temporal) arteritis is due to what immediate cause?

Ophthalmic artery occlusion

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Polyarteritis nodosa is a _________-sized vasculitis associated with ______ and ______ (viruses).

Polyarteritis nodosa is a medium-sized vasculitis associated with HBV and HCV.

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How does polyarteritis nodosa typically present on histology?

Transmural, necrotizing lesions;

‘string-of-pearls’ vascular lesions in small-to-medium vessels

(associated with hypertension, abdominal pain with melena, neurologic disturbances, skin lesions, no involvement of heart or lungs)

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Describe the common clinical presentation of polyarteritis nodosa.

Hypertension (renal artery involvement), abdominal pain with melena, neurologic disturbances, skin lesions

(no involvement of heart or lungs)

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Polyarteritis nodosa is characterized by _________ (layers involved) inflammation of medium vessels.

Polyarteritis nodosa is characterized by transmural inflammation of medium vessels.

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Polyarteritis nodosa classically spares the ______s.

Polyarteritis nodosa classically spares the lungs.

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Which vasculitis is associated with hypertension and multiple skin lesions?

Polyarteritis nodosa

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Which medium-sized vessels are most often affected by polyarteritis nodosa?

Renal and mesenteric

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What population is affected by Kawasaki disease?

Children < 4 years of age

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Describe the S/Sy of Kawasaki disease:

CRASH and burn

Conjunctivitis

Rash

Adenopathy

Strawberry tongue

Swollen, erythematous Hands

Burn (5 days of fever)

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What disorder is a cause of AMI in children and one of the few situations in which aspirin is indicated for pediatric patients?

Kawasaki disease

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Which arteries are most affected by the aneurysms of Kawasaki's disease?

The coronary vessels

Which arteries are preferentially involved in Kawasaki's disease?

The coronary arteries

Buerger's disease is another name for what condition?

Thromboangiitis obliterans

Thromboangiitis obliterans (Buerger's disease) is associated with ___________ (activity) and __________ phenomenon.

Thromboangiitis obliterans (Buerger's disease) is associated with **_smoking_** and **_Raynaud's_** phenomenon.

Thromboangiitis obliterans is believed to be caused by direct toxicity against what cell type?

Endothelial cells

How is the mainstay treatment of thromboangiitis obliterans (Buerger's disease)?

Smoking cessation

C-ANCAntibodies typically target \_\_\_\_\_\_\_\_-3. P-ANCAntibodies typically target \_\_\_\_\_\_\_\_.

C-ANCAntibodies typically target **_proteinase_**-3 (***PR3-ANCA***). P-ANCAntibodies typically target **_myeloperoxidase_**.

What condition associated with smoking may cause auto-amputation of the digits?

Thromboangiitis obliterans | (Buerger's disease)

Describe the histopathology of thromboangiitis obliterans (Buerger's disease).

Neutrophils; microabcesses

What organs are affected by granulomatosis with polyangitiis (Wegener's disease)?

The **nasopharynx**; the **lungs**; the **kidneys** (think of a We**c**kner C connecting the nose through the lungs to the kidneys)

Wegener's granulomatosis (granulomatosis with polyangiitis) is associated with increased levels of serum \_\_\_\_\_\_\_\_.

Wegener's granulomatosis (granulomatosis with polyangiitis) is associated with increased levels of serum **_C-ANCA_**. (Think We**c**kner's)

Granulomatosis with polyangiitis (Wegener's disease) is typically treated with what two medications/classes?

Corticosteroids + cyclophosphamide

The histopathology of granulomatosis with polyangiitis (Wegener's disease) includes the presence of _______ cells.

The histopathology of granulomatosis with polyangiitis (Wegener's disease) includes the presence of **_giant_** cells.

Granulomatosis with polyangiitis (Wegener's disease) most commonly affects what patient population?

Men around age 40

Granulomatosis with polyangiitis (Wegener's disease) typically causes what renal pathology?

Focal necrotizing and crescentic glomerulonephritis

What kidney pathology is associated with granulomatosis with polyangiitis (Wegener's disease)?

Necrotizing glomerulonephritis

Which small-vessel vasculitis is not associated with granuloma formation?

Microscopic polyangiitis

What are the major differences between microscopic polyangiitis and granulomatosis with polyangiitis (Wegener's disease)?

**Microscopic polyangiitis**: no nasopharyngeal involvement; no granulomas; P-ANCA instead of C-ANCA

How is microscopic polyangiitis treated?

Corticosteroids + cyclophosphamide

Microscopic polyangiitis is associated with _________ infiltrate.

Microscopic polyangiitis is associated with **_neutrophilic_** infiltrate.

Microscopic polyangiitis (hypersensitivity vasculitis) most commonly affects which small vessels?

Those in **cutaneous** and **mucous** **membranes** (may include pulmonary capillitis and necrotizing glomerulonephritis)

Which vasculitis is associated with a necrotizing capillaritis in multiple organs but NO GRANULOMAS?

Microscopic polyangiitis

Which vasculitis may be drug-induced?

**Microscopic polyangiitis** (hypersensitivity vasculitis)

Which vasculitis may be associated with smoking and Raynaud's phenomenon?

**Thromboangiitis obliterans** (Buerger's disease)

Which vasculitis may be associated with HBV and/or HCV?

Polyarteritis nodosa

Which small-vessel vasculitis is characterized by concurrent asthma, eosinophilia, and granulomatous disease?

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)

Churg-Strauss (eosinophilic granulomatosis with polyangiitis) typically shows a characteristic increase in \_\_\_-ANCA.

Churg-Strauss (eosinophilic granulomatosis with polyangiitis) typically shows a characteristic increase in **_P_**-ANCA.

Name two vasculitides associated with elevated P-ANCA.

Microscopic polyangiitis Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)

Name the most common pediatric vasculitis.

Henoch-Shlönlein purpura

Henoch-Schlönlein purpura usually occurs following what inciting event?

Upper respiratory tract infection

Henoch-Schlönlein purpura is entirely a disorder of ______ deposition.

Henoch-Schlönlein purpura is entirely a disorder of **_IgA_** deposition.

Name a small-vessel vasculitis most likely to affect the kidneys and skin of children (leading to IgA nephropathy and palpable purpura over the buttocks and legs).

Henoch-Shönlein purpura

Isolated IgA nephropathy is also known as _________ disease.

Isolated IgA nephropathy is also known as **_Berger's_** disease.

Roughly 15% of individuals with cryoglobulinemia (may be associated with HCV, *Mycoplasma pneumoniae*, and other causes) will develop what small-vessel vasculitis?

Cryoglobulinemic vasculitis

**True/False**. SLE is commonly associated with an SLE-induced medium-vessel vasculitis.

**False**. SLE is commonly associated with an SLE-induced ***small***-vessel vasculitis.

Endocarditis is usually a result of what?

Microbial infection

Non-infectious endocarditis is caused by damage to the endocardium which causes tiny _________ that form vegetations on valves and this is called Nonbacterial Thrombotic Endocarditis or NBTE.

Non-infectious endocarditis is caused by damage to the endocardium which causes tiny **_thrombi_** that form vegetations on valves and this is called Nonbacterial Thrombotic Endocarditis or NBTE.

Name a few causes of non-bacterial thrombotic endocarditis.

Hypercoagulable states; malignancies; SLE

Infective endocarditis is usually caused by low-virulence _________ streptococci which attack valves that have had some previous damage.

Infective endocarditis is usually caused by low-virulence **_viridans_** streptococci which attack valves that have had some previous damage.

Most cases of endocarditis are due to what infectious agent? What about in IV drug use? And on prosthetic valves?

Viridans streptococci (e.g. *Streptococcus sanguinis*); * Staphylococcus aureus*; * Staphylococcus epidermidis*

You diagnose a patient with a case of endocarditis caused by *Streptococcus gallolyticus*. What is the next step in management?

**Colonoscopy** (*Streptococcus gallolyticus* is the new name for *S. bovis*)

What microbes are part of the HACEK causes of culture-negative endocarditis?

***H**aemophilus* ***A**ggregatibacter* ***C**ardiobacteria* ***E**ikenella* ***K**ingella*

What do HACEK, *Bartonella*, *Candida*, and *Coxiella* have in common as causes of endocarditis?

They are all culture-negative.

What mnemonic can be used to remember the signs/symptoms of endocarditis?

FROM JANE

What are the signs/symptoms of endocarditis? (Use the **FROM JANE** mnemonic.)

**F**ever **R**oth's spots **O**sler's nodes **M**urmur **J**aneway lesions **A**nemia **N**ail-bed emboli **E**mboli

The anemia associated with endocarditis is what type of anemia?

Anemia of chronic disease

Identify the basic mechanism by which each of the following are caused by endocarditis: **Osler's nodes** **Janeway lesions** **Roth's spots** **Pulmonary damage** **Cerebral damage** **Splinter hemorrhages** **Glomerulonephritis**

Identify the basic mechanism by which each of the following are caused by endocarditis: **Osler's nodes** (type III hypersensitivity) **Janeway lesions** (septic emboli) **Roth's spots** (type III hypersensitivity) **Pulmonary damage** (septic emboli) **Cerebral damage** (septic emboli) **Splinter hemorrhages** (septic emboli) **Glomerulonephritis** (type III hypersensitivity)

Which signs/symptoms of endocarditis are results of type III hypersensitivity?

**Osler's nodes** **Roth's spots** **Glomerulonephritis**

Which signs/symptoms of endocarditis are results of septic emboli?

**Splinter hemorrhages** **Janeway lesions** (also infarcts of other organs; e.g. the lungs and brain)

What are the two main diagnostic features of endocarditis?

(1) **Blood cultures** and (2) **echocardiography**

What is the most common cause of myocarditis in the U.S.?

*Coxsackievirus*

Paradoxical emboli occur when emboli cross from the right heart to left through what?

A patent **ASD**

Name a common cause of myocarditis in South America.

Chagas disease

Name a few immunological causes of myocarditis.

SLE; polymyositis; drug reactions

How might myocarditis appear on an electrocardiogram?

**Sinus tachycardia**; **T-wave inversions** and “saddle-shaped” **ST-segment elevation**

Name a few examples of immunological pericardial disease.

SLE; rheumatoid arthritis

In high-income countries, constrictive pericarditis is typically either _________ or caused by the ____________ virus.

In high-income countries, pericarditis is typically either **_idiopathic_** or caused by the **_Coxsackie B_** virus.

Name (1) an ischemic cardiac pathology and (2) a renal pathology that are both associated with pericarditis.

(1) **Dressler** syndrome (2) **U****remic** syndrome

In low-income countries, constrictive pericarditis is typically caused by \_\_\_\_\_\_\_\_\_\_\_\_\_.

In low-income countries, constrictive pericarditis is typically caused by **_Tuberculosis_**.

Constrictive pericarditis is associated with what type of cancer treatment?

Radiation therapy

Kussmaul's sign: during inspiration, the jugular venous pressure will paradoxically \_\_\_\_\_.

Kussmaul's sign: during inspiration, the jugular venous pressure will paradoxically **_rise_**.

**Kussmaul's** **sign** is characterized by a(n) 10 mmHg __________ (increase/decrease) in _jugular venous pressure_ upon inspiration. **Pulsus** **paradoxus** is characterized by a(n) 10 mmHg __________ (increase/decrease) in _systolic blood pressure_ upon inspiration.

**Kussmaul's** **sign** is characterized by a(n) 10 mmHg **_increase_** in _jugular venous pressure_ upon inspiration. **Pulsus** **paradoxus** is characterized by a(n) 10 mmHg **_decrease_** in _systolic blood pressure_ upon inspiration.

On ECG, constrictive pericarditis often manifests as what?

Widespread **ST segment elevation** in several leads

Hemangiomas are most commonly found in which organs?

Skin; liver

Do hemangiomas blanch with pressure?

Yes. (The blood is still within vessels that can be compressed.)

Name a few common sites of angiosarcoma development.

Skin; breast; liver

Hepatic angiosarcomas are associated with what three substances?

**PVC**; **arsenic**; **Thorotrast** (thorium contrast)

Do the lesions associated with Kaposi sarcoma blanch with pressure?

No. (The blood is within the cutaneous interstitium, NOT within vessels that can be compressed.)

Do purpura blanch with pressure?

No. (The blood is within the cutaneous interstitium, NOT within vessels that can be compressed.)

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Cardio - Mechanisms of Disease - Vasculitides; Cardiac Inflammation; Vascular Tumors Flashcards

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