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Pathology > Unit 2 Vasculitis > Flashcards

Unit 2 Vasculitis Flashcards

Vasculititides

1
Q

Define vasculitis and 3 etiological classifications

A

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

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2
Q

Describe the 3 layers of the BV wall

A

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

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3
Q

Describe the nonspecific symptoms of vascular inflammation

A

Fever, wt loss, fatigue, myalgias

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4
Q

Describe the organ specific symptoms of vascular inflammation

A

Luminal narrowing or thrombosis can lead to organ ischemia

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5
Q

Large vessel vasculitides (LVV) include which vessels?

A

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

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6
Q

Medium vessel vasculitides (MVV) include which vessels?

A

Muscular arteries supplying organs—i.e. renal artery

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7
Q

Small vessel vasculitides (SVV) include which vessels?

A

Arterioles, capillaries and venules

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8
Q

List 2 major large vessel vasculitides (LVV)

A

Giant Cell Arteritis, Takayasu Arteritis

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9
Q

List 3 major medium vessel vasculitides (MVV)

A

Polyarteritis Nodosa, Kawasaki disease, Buerger disease

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10
Q

List 4 major small vessel vasculitides (SVV)

A

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

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11
Q

Describe the classic symptoms of Giant (Temporal) Cell Arteritis

A

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

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12
Q

Most commonly affected arteries in Giant Cell Arteritis

A

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

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13
Q

Most likely complication if GCA is NOT treated

A

Blindness

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14
Q

Treatment for GCA

A

Corticosteroids

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15
Q

GCA biopsy findings and interpretation

A

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

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16
Q

Describe the symptoms of Takayasu Arteritis (TAK)

A

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

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17
Q

Treatment for Takayasu arteritis (TAK)

A

Corticosteroids

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18
Q

List the major vessels commonly affected in TAK

A

Aortic arch at branch points

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19
Q

Name game: list another name for Wegeners Granulomatosis

A

Wegeners Granulomatosis = Granulomatosis with polyangiitis (GPA)

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20
Q

Name game: list another name for Churg-Strauss Syndrome

A

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

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21
Q

Name game: list another name for Henoch-Schonlein Purpura

A

Henoch-Schonlein Purpura = IgA Vasculitis

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22
Q

Describe the classic symptoms of Polyarteritis Nodosa (PAN)

A

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

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23
Q

Most likely complication if PAN is NOT treated

A

PAN is fatal if not treated

24
Q

Treatment for PAN

A

Corticosteroids and Cyclophosphamide

25
Q

Most commonly affected arteries in PAN

A

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

26
Q

Describe PAN blood vessel pathological findings

A

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.

27
Q

Viral infections associated with PAN-like lesions

A

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

28
Q

Imaging: PAN findings

A

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

29
Q

Compare/contrast age, gender for GCA vs TAK

A

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

30
Q

Vasculitis commonly affecting children

A

Kawasaki disease (usually Asian child <4)

31
Q

Describe classic symptoms of Kawasaki disease (KD)

A

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

32
Q

Most commonly affected arteries in Kawasaki disease (KD)

A

Coronary arteries

33
Q

Major complications of Kawasaki disease (KD)

A

1)Thrombosis with MI or 2) Aneurysm with rupture

34
Q

Treatment for Kawasaki disease (KD)

A

Usually self-limited; May treat with IVIG and aspirin

35
Q

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

A

Kawasaki disease = Mucocutaneous Lymph Node Syndrome

36
Q

Describe the classic symptoms of Buerger disease

A

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

37
Q

Major risk factor for Buerger disease

A

Smoking (heavy)

38
Q

Treatment for Buerger disease

A

Smoking cessation

39
Q

Name game: list another name for Buerger disease

A

Buerger disease = Thromboangiitis Obliterans

40
Q

Describe the pathological mechanism of Buerger disease

A

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.

41
Q

Describe classic symptoms of Wegener Granulomatosis

A

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

42
Q

Most commonly affected arteries/organs in Wegeners Granulomatosis

A

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

43
Q

Most likely complication if Wegeners Granulomatosis is NOT treated

A

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

44
Q

Treatment for Wegeners Granulomatosis

A

Cyclophosphamide and steroids; Relapses occur

45
Q

Biopsy: Wegeners Granulomatosis

A

Large necrotizing granulomas with adjacent necrotizing vasculitis

46
Q

Lab marker: Wegener Granulomatosis

A

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

47
Q

Describe classic symptoms of Microscopic Polyangiitis

A

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

48
Q

Lab marker: Microscopic Polyangiitis

A

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

49
Q

Treatment for Microscopic Polyangiitis

A

Cyclophosphamide and corticosteroids. Relapse common

50
Q

Describe classic patient type with Churg-Strauss Syndrome

A

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

51
Q

Most commonly affected arteries/organs in Churg-Strauss Syndrome

A

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

52
Q

Describe pathology of Churg-Strauss Syndrome

A

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

53
Q

Treatment for Churg-Strauss Syndrome

A

Corticosteroids; poor prognosis if untreated

54
Q

Lab marker: Churg-Strauss Syndrome

A

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

55
Q

Describe classic symptoms of Henoch-Schonlein Purpura (HSP)

A

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

56
Q

Describe pathology of HSP

A

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

57
Q

Treatment for HSP

A

Self-limited; Steroids for severe cases; may recur

Pathology (3 decks)

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