Vasculitis

Question 1

in vasculitis, there is inflammation and necrosis of the ____

Answer 1

in vasculitis, there is inflammation and necrosis of the blood vessels, including arteries, veins and capillaries

Question 2

list the vasculitis disorders that are immune complex-mediated

Answer 2

• immune complex-mediated
  
  • hepatitis B or C virus-mediated
  • SLE and rheumatoid arthritis
  • drug induced

Question 3

describe the vasculitis disorders that are ANCA-mediated

Answer 3

• ANCA-mediated
  
  • Wegener’s granulomatosis
  • Churg-Strauss

Question 4

list the vasculitis disorders that are direct antibody attack-mediated

Answer 4

• direct antibody attack-mediated
  
  • Kawasaki disease (anti-endothelial cells antibodies)

Question 5

describe the vasculitis disorders that are cell-mediated

Answer 5

• cell-mediated
  
  • allograft organ rejection

Question 6

___ is the most common type of vasculitis

explain this vasculitis

Answer 6

giant cell (temporal) arteritis (GCA)is the most common type of vasculitis

• affects mainly the arteries in the head
  
  • aorta (giant cell aortitis)
  • temporal
  • vertebral
  • ophthalmic → can lead to blindness
• T-cell mediated immunologic rxn against elastin (?)

Question 7

describe the histology of giant cell arteritis

Answer 7

• focal, nodular thickening with reduction of lumen
• granulomatous inflammation of intima and inner media
• giant cells, mononuclear cells
• fragmentation of IEL

Question 8

describe diagnosis of GCA

Answer 8

• diagnosis:
  
  • elevated ESR
  • temporal artery biopsy
    
    • 2-3 cm. segment, multiple section
    • elastic trichrome stain
  • association with polymyalgia rheumatica

Question 9

describe the treatment of giant cell arteritis

Answer 9

• treatment:
  
  • steroids, analgesics

Question 10

describe Takayasu arteritis

Answer 10

• granulomatous vasculitis of medium and large arteries with narrowing and obliteration of lumen
• most commonly affects arch of aorta with narrowing or virtual obliteration of the origins of the great vessels arising in the arch
• can also involve pulmonary, coronary and renal arteries

Question 11

describe the clinical features of Takayasu arteritis

Answer 11

• most common in females < 40 yrs
• ocular changes: visual disturbances, retinal hemorrhages, blindness
• progressive diminution of upper limb pulses with coldness or numbness of fingers → pulseless disease
• low BP in upper limb
• neurologic defects → dizziness, focal weakness or complete hemiparesis

Question 12

which disease shows the following:

progressive diminution of upper limb pulses with coldness or numbness of fingers → pulseless disease

Answer 12

Takayasu arteritis

Question 13

describe what is seen in the image

Answer 13

Takayasu disease

aortic arch angiogram showing reduced flow of contrast material into the great vessels and narrowing of the brachiocephalic, carotid and subclavian arteries

Question 14

Answer 14

Takayasu arteritis

• cross-sections of the right carotid artery demonstrating marked intimal thickening and luminal narrowing

Question 15

describe what is seen in the image

Answer 15

Takayasu arteritis

• histo illustrating destruction and fibrosis of the arterial media associated with mononuclear infiltrates and giant cells (arrows)

Question 16

describe polyarteritis nodosa

Answer 16

• systemic vasculitis
• transmural necrotizing inflammation of small or medium-sized muscular arteries
• typically involves renal and visceral arteries and spares the pulmonary circulation (PAN = Pulmonary Arteries Not affected)
• arterioles, capillaries and venules are not affected

Question 17

30% of PAN patients also have _____

Answer 17

30% of PAN patients also have Hep B

Question 18

describe the gross morphology of PAN and the order in which organs are affected

Answer 18

• gross:
  
  • distribution of lesions in descending order of frequency → kidney, heart, liver, GIT (lungs NOT involved)
  • lesions have a predilection for branching points​

Question 19

describe the histo morphology of PAN

Answer 19

all stages of activity may coexist in different vessels or even within the same vessel which is a characteristic feature

• histo:
  
  • acute stage: transmural inflammation, fibrinoid necrosis of the inner half of the vessel wall
  • later stages: inflammation is replaced by fibrous thickening of the vessel wall

Question 20

describe the image

Answer 20

PAN

• associated with segmental fibrinoid necrosis and thrombotic occlusion of a small artery. note that part of the vessel (arrow) is uninvolved

Question 21

list complications of PAN

Answer 21

• weakening of arterial wall owing to inflammatory process may cause aneurysmal dilatation or localized rupture
• impairment of perfusion causing ulcerations, infarcts, ischemic atrophy, or hemorrhages in the areas supplied by these vessels

Question 22

describe Kawasaki disease

Answer 22

• arteritis involving the large, medium and small arteries (often coronary arteries)
• also called mucocutaneous lymph node syndrome (MCLNS)
• usually in young children and infants
• leading cause of acquired heart disease in children in the US

Question 23

in Kawasaki disease there are ___ against ____ leading to acute vasculitis

Answer 23

in Kawasaki disease there are autoantibodies against endothelium, smooth muscle cells leading to acute vasculitis

Question 24

describe the clinical features of Kawasaki disease

Answer 24

Question 25

describe the treatment of Kawasaki disease

Answer 25

Question 26

describe the morphology of Kawasaki disease

Answer 26

* lesions may range from: * severe destruction of all constituents of the wall by a segmental necrotizing process, with moderate fibrinoid change and dense infiltrate of inflammatory cells, to * mild changes involving intima only * acute vasculitis may lead to **coronary aneurysm formation** and associated thrombosis with MI

Question 27

describe Buerger disease (Thromboangiitis obliterans)

Answer 27

* Buerger disease (thromboangiitis obliterans) * characterized by segmental, thrombosing, acute and chronic inflammation of medium-sized and small arteries * mainly the tibial and radial arteries and sometimes secondarily extending to veins and nerves of the extremities

Question 28

in Buerger disease (thromb. obliterans), the ___ and ___ arteries are mainly affected

Answer 28

in Buerger disease ( thromb . obliterans), the **tibial** and **radial** arteries are mainly affected

Question 29

the main predisposing factor for Buerger disease is _____ and mainly affects ____ (3 populations)

Answer 29

the main predisposing factor for Buerger disease is **cigarette smoking (HS reaction to tobacco → direct endothelial injury)** and mainly affects **people from India, Israel and Japan**

Question 30

describe the clinical features of Buerger disease

Answer 30

* affects males most commonly (\<35 yrs) * superficial nodular phlebitis, cold insensitivity (Reynaud's), claudication pain on the instep, severe progressive pain (due to neural involvement) on exercise and at rest * chronic ulceration of the toes, feet or finger may appear followed by gangrene

Question 31

describe the morphology seen in Buerger disease

Answer 31

* sharply segmental acute and chronic vasculitis: medium and small-sized arteries of extremities * acute and chronic inflammation permeating the arterial walls, accompanied by thrombosis of the lumen

Question 32

describe microscopic polyangiitis (hypersensitivity or leukocytoclastic vasculitis)

Answer 32

* necrotizing vasculitis of capillaries, arterioles and small venules * segmental fibrinoid necrosis of media with focal transmural necrotizing lesions - PMNs * no infarcts - small size vessels * absence of granulomatous response * all organs affected including lungs * all lesions same age

Question 33

microscopic polyangiitis may be caused by hypersensitivity to drugs, like ____ and infections by \_\_\_\_\_

Answer 33

microscopic polyangiitis may be caused by hypersensitivity to drugs, like **penicillin** and infections by **Strep**

Question 34

patients with microscopic polyangiitis test positive for ____ aka _____ and there is absence of \_\_\_\_

Answer 34

patients with microscopic polyangiitis test positive for **MPO-ANCA** aka **p-ANCA** and there is absence of **IgG (pauci-immune)**

Question 35

describe the clinical features of microscopic polyangiitis

Answer 35

* hemoptysis, hematuria, proteinuria * blood in the stool * cutaneous purpura * respond to removal of the offending agent

Question 36

describe the triad seen in granulomatosis with polyangiitis (Wegener Granulomatosis)

Answer 36

* triad: * acute necrotizing granulomas of upper and lower resp. tract * focal necrotizing or granulomatous vasculitis affecting small to medium-sized vessels * renal disease in the form of focal or necrotizing, often crescentic glomerulonephritis

Question 37

patients with GPA/Wegeners test positive for \_\_\_\_

Answer 37

patients with GPA/ Wegeners test positive for **c-ANCA**

Question 38

describe the clinical features of GPA/WG

Answer 38

* M\>F * persistent pneumonitis with bilateral nodular and cavitary infiltrates * hemoptysis * chronic sinusitis * mucosal ulceration of the nasopharynx * evidence of renal disease (hematuria, acute renal failure) * 80% of untreated patients die within 1 year

Question 39

describe Churg-Strauss Syndrome

Answer 39

* allergic granulomatosis and angiitis * allergic rhinits, bronchial asthma, peripheral eosinophilia * positivity for p-ANCA

Question 40

summarize the small blood vessel vasculitis conditions

Answer 40