Unit 2 Vasculitis

Question 1

Define vasculitis and 3 etiological classifications

Answer 1

Inflammation of blood vessel wall. 1) Primary systemic vasculitis- specific cause unknown- presumed “immune mediated” (most of them). 2) Infectious (or direct) vasculitis—i.e. syphilis, RMSF, Gonorrhea, Mycoses (Aspergillus or Mucor). 3) Secondary systemic vasculitis—results from another disease process—i.e. HBV associated, Drug related HSR (ex: PCN, sulfa, etc…), Paraneoplastic, Lupus or RA immune complex mediated, Serum sickness

Question 2

Describe the 3 layers of the BV wall

Answer 2

Intima-innermost layer; Media-smooth muscle layer in middle; Adventitia—outermost connective tissue layer.

Question 3

Describe the nonspecific symptoms of vascular inflammation

Answer 3

Fever, wt loss, fatigue, myalgias

Question 4

Describe the organ specific symptoms of vascular inflammation

Answer 4

Luminal narrowing or thrombosis can lead to organ ischemia

Question 5

Large vessel vasculitides (LVV) include which vessels?

Answer 5

Aorta and major branches—Brachiocephalic, Subclavian, Common Carotid Arteries.

Question 6

Medium vessel vasculitides (MVV) include which vessels?

Answer 6

Muscular arteries supplying organs—i.e. renal artery

Question 7

Small vessel vasculitides (SVV) include which vessels?

Answer 7

Arterioles, capillaries and venules

Question 8

List 2 major large vessel vasculitides (LVV)

Answer 8

Giant Cell Arteritis, Takayasu Arteritis

Question 9

List 3 major medium vessel vasculitides (MVV)

Answer 9

Polyarteritis Nodosa, Kawasaki disease, Buerger disease

Question 10

List 4 major small vessel vasculitides (SVV)

Answer 10

Wegener Granulomatosis, Microscopic Polyangiitis, Churg-Strauss Syndrome, Henoch-Schonlein Purpura

Question 11

Describe the classic symptoms of Giant (Temporal) Cell Arteritis

Answer 11

Headache (HA)—temporal artery involved; Visual disturbance—opthalmic artery involved; Jaw claudication—maxillary artery; Flu-like symptoms, joint and muscle pain (polymyalgia rheumatica);

Question 12

Most commonly affected arteries in Giant Cell Arteritis

Answer 12

External Carotid Artery branches: Superficial Temporal Artery, Maxillary Artery; Internal Carotid branches: Opthalmic Artery; Subclavian branch: Vertebral Artery

Question 13

Most likely complication if GCA is NOT treated

Answer 13

Blindness

Question 14

Treatment for GCA

Answer 14

Corticosteroids

Question 15

GCA biopsy findings and interpretation

Answer 15

Inflamed vessel wall, giant cells, intimal fibrosis; long segments needed and negative biopsy does not exclude disease!

Question 16

Describe the symptoms of Takayasu Arteritis (TAK)

Answer 16

Visual and neurological symptoms; weak or absent pulse in upper extremeity (pulseless)

Question 17

Treatment for Takayasu arteritis (TAK)

Answer 17

Corticosteroids

Question 18

List the major vessels commonly affected in TAK

Answer 18

Aortic arch at branch points

Question 19

Name game: list another name for Wegeners Granulomatosis

Answer 19

Wegeners Granulomatosis = Granulomatosis with polyangiitis (GPA)

Question 20

Name game: list another name for Churg-Strauss Syndrome

Answer 20

Churg-Strauss Syndrome = Eosinophilic granulomatosis with polyangiitis (EGPA)

Question 21

Name game: list another name for Henoch-Schonlein Purpura

Answer 21

Henoch-Schonlein Purpura = IgA Vasculitis

Question 22

Describe the classic symptoms of Polyarteritis Nodosa (PAN)

Answer 22

Young adults with hypertension (renal artery), abdominal pain with melena (mesenteric artery), neurologic disturbances and skin lesions.

Question 23

Most likely complication if PAN is NOT treated

Answer 23

PAN is fatal if not treated

Question 24

Treatment for PAN

Answer 24

Corticosteroids and Cyclophosphamide

Question 25

Most commonly affected arteries in PAN

Answer 25

Medium sized, muscular arteries to all organ systems EXCEPT lungs.

Question 26

Describe PAN blood vessel pathological findings

Answer 26

Necrotizing lesions—demonstrate fibrinoid necrosis where medial muscle and adjacent tissues are fused into a structureless eosinophilic mass that stains for fibrin. Inflammatory response/cells cause infarcts that produce symptoms.

Question 27

Viral infections associated with PAN-like lesions

Answer 27

Hepatitis B Virus (associated with HBsAg), HCV, HIV

Question 28

Imaging: PAN findings

Answer 28

“String of Pearls” appearances due to fibrotic nodes resulting from healed areas of necrosis in BV

Question 29

Compare/contrast age, gender for GCA vs TAK

Answer 29

GCA patients > 50 yo and female; most common vasculitis. TAK patients < 50 yo and female (Asian)

Question 30

Vasculitis commonly affecting children

Answer 30

Kawasaki disease (usually Asian child <4)

Question 31

Describe classic symptoms of Kawasaki disease (KD)

Answer 31

Non-specific: fever, conjunctivitis, erythematous rash of palms and soles, oral lesions, enlarged cervical LN in a child

Question 32

Most commonly affected arteries in Kawasaki disease (KD)

Answer 32

Coronary arteries

Question 33

Major complications of Kawasaki disease (KD)

Answer 33

1)Thrombosis with MI or 2) Aneurysm with rupture

Question 34

Treatment for Kawasaki disease (KD)

Answer 34

Usually self-limited; May treat with IVIG and aspirin

Question 35

Name game: list another name for Kawasaki disease (KD)

Answer 35

Kawasaki disease = Mucocutaneous Lymph Node Syndrome

Question 36

Describe the classic symptoms of Buerger disease

Answer 36

Ulceration, gangrene, auto-amputation of fingers and toes; Raynauds phenomenon is often present.

Question 37

Major risk factor for Buerger disease

Answer 37

Smoking (heavy)

Question 38

Treatment for Buerger disease

Answer 38

Smoking cessation

Question 39

Name game: list another name for Buerger disease

Answer 39

Buerger disease = Thromboangiitis Obliterans

Question 40

Describe the pathological mechanism of Buerger disease

Answer 40

Necrotizing vasculitis of digits: Smoking elicits pro-inflammatory antibodies; PMNs infiltrate arteries, neighboring veins while endothelium becomes inflamed leading to thrombosis and obliteration of BV lumen. Lesions become severe and progress to gangrene.

Question 41

Describe classic symptoms of Wegener Granulomatosis

Answer 41

Middle aged male; Sinusitis or nasopharyngeal ulceration, hemoptysis with bilateral nodular lung infiltrates (pneumonitis) and hematuria, proteinuria due to rapidly progressive glomerulonephritis (RPGN).

Question 42

Most commonly affected arteries/organs in Wegeners Granulomatosis

Answer 42

Small arteries, veins or lung (respiratory tract), kidney and spleen.

Question 43

Most likely complication if Wegeners Granulomatosis is NOT treated

Answer 43

Wegeners Granulomatosis is FATAL if untreated. Most will die within 1 year (avg: 5-6 months)

Question 44

Treatment for Wegeners Granulomatosis

Answer 44

Cyclophosphamide and steroids; Relapses occur

Question 45

Biopsy: Wegeners Granulomatosis

Answer 45

Large necrotizing granulomas with adjacent necrotizing vasculitis

Question 46

Lab marker: Wegener Granulomatosis

Answer 46

Serum c-ANCA (aka anti-proteinase 3 or PR3-ANCA) correlates with disease activity; ANCA = antineutrophil cytoplasmic antigens

Question 47

Describe classic symptoms of Microscopic Polyangiitis

Answer 47

Sinusitis, hemoptysis with nodular infiltrates, hematuria, proteinuria (presentation similar to Wegeners). No granulomas or nasopharyngeal involvement.

Question 48

Lab marker: Microscopic Polyangiitis

Answer 48

Serum p-ANCA (aka anti-myeloperoxidase or MPO-ANCA) correlates with disease activity

Question 49

Treatment for Microscopic Polyangiitis

Answer 49

Cyclophosphamide and corticosteroids. Relapse common

Question 50

Describe classic patient type with Churg-Strauss Syndrome

Answer 50

Asthma patients; CSS is a systemic vasculitis of young patients with asthma.

Question 51

Most commonly affected arteries/organs in Churg-Strauss Syndrome

Answer 51

Small vessels of lungs and heart; liver, kidney, spleen, CNS and other organs

Question 52

Describe pathology of Churg-Strauss Syndrome

Answer 52

Necrotizing lesions of small vessels (granulomas) with intense eosinophilic infiltrates;

Question 53

Treatment for Churg-Strauss Syndrome

Answer 53

Corticosteroids; poor prognosis if untreated

Question 54

Lab marker: Churg-Strauss Syndrome

Answer 54

Serum p-ANCA (aka anti-myeloperoxidase or MPO-ANCA) correlates with disease activity

Question 55

Describe classic symptoms of Henoch-Schonlein Purpura (HSP)

Answer 55

Child with palpable purpura on buttocks and legs, GI pain and bleeding, and hematuria (IgA nephropathy) following a upper respiratory tract infection. Most common vasculitis in children

Question 56

Describe pathology of HSP

Answer 56

Respiratory infection leads to IgA immune complex deposition in vessels—> vasculitis.

Question 57

Treatment for HSP

Answer 57

Self-limited; Steroids for severe cases; may recur