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Flashcards in Vasculitis (Postlew) Deck (54)
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1
Q

Primary finding in vasculitis

A

inflammation within blood vessel walls

2
Q

What arteries does Polyarteritis Nodosa affect?

A

medium-sized

spares arterioles, venules, capillaries

3
Q

Polyarteritis Nodosa is associated with…

A

Hep B (30% of patients)

4
Q

Polyarteritis Nodosa: epidemiology

A

slight male predominance

5
Q

Polyarteritis Nodosa: Clinical Manifestations

A
  • -neuropathy
  • -kidney involvement (= HTN and renal failure)
  • -testicular tenderness
  • -cardiac involvement (MI, CHF, pericarditis)
  • -skin (livedo reticularis)
6
Q

Polyarteritis Nodosa: lab findings

A

↑ESR, ↑CRP, hematuria, ↑creatinine

7
Q

Cutaneous Polyarteritis Nodosum is associated with…

A

Hep C

8
Q

How is Buerger’s Disease characterized?

A

Granulomatous inflammation, which predominantly affects vascular supply to the lower limbs

9
Q

Buerger’s Disease: epidemiology

A

young adult M, tobacco smokers

10
Q

Which CT disorders can be associated with IC-mediated vasculitis?

A

SLE, Sjögren’s syndrome, and RA

11
Q

What is the most prominent feature in Immune Complex-Mediated Small Vessel Vasculitis?

A

Cutaneous involvement of small blood vessels

12
Q

What is the classic cutaneous symptom associated with Immune Complex-Mediated Small Vessel Vasculitis?

A

palpable purpura

pustules, vesicles, urticaria, small ulcerations may also be found

13
Q

Hypersensitivity Vasculitis usually occurs secondary to…

A

drug exposures or infections

14
Q

How is Hypersensitivity Vasculitis characterized?

A

IC deposition in capillaries, postcapillary venules and arterioles

15
Q

Clinical manifestations of Hypersensitivity Vasculitis?

A
  • -purpura
  • -arthritis
  • -glomerulonephritis
  • -colicky abdominal pain
16
Q

Henoch-Schonlein Purpura: epidemiology

A

90% in children

17
Q

What vessels does Henoch-Schonlein Purpura affect?

A

small vessels, primarily postcapillary venules

18
Q

What is characteristically present in Henoch-Schonlein Purpura?

A

IgA

19
Q

Henoch-Schonlein Purpura: classic tetrad

A

purpura, arthritis, abdominal pain, GN

20
Q

Henoch-Schonlein Purpura: lab findings

A

↑ESR, ↑CRP, hematuria, ↑creatinine, RBC casts present (if renal involvement)

21
Q

Cryoglobulinemia Vasculitis is most often associated with…

A

long-standing hep C infection

22
Q

How are types II/III of Cryoglobulinemia Vasculitis different than type I?

A

IgM and IgG (polyclonal)
associated with hep C, Sjogrens, SLE
result in IC-mediated vasculitis

23
Q

Cryoglobulinemia Vasculitis: clinical manifestations

A
  • -palpable purpura with ulcers
  • -systemic symptoms
  • -neurological involvement, peripheral neuropathy
  • -Raynaud’s
  • -Digital ischemia (often secondary to cold_
  • -Secondary Sjogren’s
24
Q

Cryoglobulinemia Vasculitis: lab findings

A

↑ESR, ↓C4, (+) RF

25
Q

What happens if heparin is in collection tubes when trying to dx Cryoglobulinemia Vasculitis?

A

can → false (+) cryofirinogen

26
Q

Cryoglobulinemia Vasculitis: pathology

A

leukocytoclastic vasculitis with IgM + C3 deposits in/around small and medium sized vessels

27
Q

What vessels does Giant Cell Arteritis (Temporal) affect?

A

large sized arteries originating from the arch of the aorta

28
Q

How is Giant Cell Arteritis characterized?

A

Granulomatous inflammation

29
Q

Giant Cell Arteritis: epidemiology

A

Age >50, Females 3:1, Northern European ancestry

30
Q

Giant Cell Arteritis: potentially serious complication

A

thoracic aortic aneurysms

31
Q

Clinical Manifestations of GCA?

A
  • -Systemic symptoms (fever, weight loss, fatigue)
  • -Temporal headaches
  • -Visual disturbances (can progress to sudden blindness; due to ↓blood to ophthalmic a)
  • -Jaw or tongue claudication
  • -Arthralgias
  • -Tender, swollen temporal artery
32
Q

GCA: lab findings?

A

markedly elevated ESR

33
Q

If you think a patient may have Giant Cell Arteritis, what is the first thing you should do?

A

begin trx to prevent blindness; do NOT wait for biopsy

34
Q

What vessels does Takayasu’s Arteritis affect?

A

large-sized arteries + involvement of aortic arch + branches (+ coronary/pulm arteries)

35
Q

How is Takayasu’s Arteritis characterized?

A

Granulomatous inflammation

36
Q

Takayasu’s Arteritis: epidemiology

A

adolescent girls and young women of Asian descent

37
Q

What vessels does Kawasaki Disease affect?

A

small and medium-sized muscular arteries, especially coronary arteries

38
Q

Kawasaki Disease: epidemiology

A

Children, Asian ancestry

39
Q

Kawasaki Disease is known to cause…

A

acquired heart disease (**esp in US and Japan)

40
Q

What causes most of the mortality in Kawasaki Disease?

A

Coronary lesions

diseases causes pericardial effusion, myocarditis, aneurysms, MI

41
Q

Clinical Manifestations of Kawasaki Disease?

A

fever, rash, strawberry tongue, erythema of palms/soles, edema of hands/feet, conjunctival injection…

42
Q

What occurs in the second week of Kawasaki Disease?

A

profound lymphocytosis

43
Q

Cutaneous vasculitis in Connective Tissue Diseases is associated with…

A

hypocomplementemia and ↑↑ANAs

44
Q

Pathology associated with Connective Tissue Disease Associated Vasculitis?

A

IgG and C3 deposition in/around dermal blood vessels +/- IgM

45
Q

What are the hallmarks of Rheumatoid Vasculitis?

A
  • -Pupuric lesions +/- concomitant medium vessel vasculitis

- -Deep cutaneous ulcer over malleoli

46
Q

Rheumatoid Vasculitis typically occurs in patients with what condition?

A

nodular, joint-destructive, RF(+) RA

47
Q

Clinical manifestations associated with Microscopic Polyangiitis?

A
  • -glomerulonephritis
  • -lung involvement + pulm hemorrhage
  • -skin involvement
  • -systemic symptoms
48
Q

Microscopic Polyangiitis: lab findings

A
↑ ESR
↑ CRP
RBC casts in urine
↑creatinine
ANCA positive (usually p-ANCA)
49
Q

Granulumatosis With Polyangiitis: Clinical Manifestations (classic triad?)

A

**ENT, respiratory tract and kidney involvement

glomerulonephritis, sinusitis, nasal ulcerations, saddle nose, +/- pulm infiltrates or nodules

50
Q

Most notable lab finding associated with Granulumatosis with Polyangiitis?

A

C-ANCA

51
Q

Allergic Granulomatosis With Polyangiitis: lab findings

A

↑ESR, eosinophilia, eosinophiluria, RBC casts, proteinuria

52
Q

Allergic Granulomatosis With Polyangiitis: Clinical manifestations

A

Systemic symptoms (fever, weight loss, fatigue)
Lungs: dyspnea, cough
Neurological: peripheral neuropathy
GU: eosinophilic granulomas of urinary tract and/or prostate

53
Q

Bechet’s Disease: clinical manifestations

A
  • -Oral and genital ulcers
  • -ocular lesions
  • -Cutaneous lesions (pyoderma gangrenousum-like lesions, pathergy lesions)
  • -arthritis
54
Q

Bechet’s Disease: epidemiology

A

20’s and 30’s, M=F

in US/Korea/China F>M, in Middle East M>F