Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Rheumatology Exam > Vasculitis > Flashcards

Vasculitis Flashcards

1
Q

vasculitis - defined

A

*inflammation of the blood vessels
*causes destruction of the vessel integrity:
-narrowed artery, organ ischemia, aneurysm / hemorrhage, thrombus
*multi-system
*also associated with inflammation outside vessel

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

general symptoms to suggest vasculitis

A

*fever
*weight loss
*anorexia
*malaise
*failure to thrive
*evidence of end-organ damage

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

more specific symptoms that may suggest vasculitis

A

*hemoptysis
*regional ischemia
*glomerulonephritis
*rash (palpable purpura, ulceration)
*mononeuritis multiplex
*bruits
*arthritis/arthralgia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

examples of large-vessel vasculitis

A

*giant cell arteritis
*polymyalgia rheumatica
*Takayasu arteritis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

examples of medium-vessel vasculitis

A

*polyarteritis nodosa
*Kawasaki disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

examples of small-vessel vasculitis

A

*granulomatosis with polyangiitis (GPA)
*microscopic polyangiitis (MPA)
*eosinophilic granulomatosis with polyangiitis (EGPA)
*IgA vasculitis
*cryoglobulinemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

giant cell arteritis - epidemiology

A

*age: > 50 yo
*prevalence increases with age
*females > males (2:1)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

giant cell arteritis - symptoms

A

*systemic symptoms: fever, weight loss, fatigue
*temporal headaches
*scalp tenderness
*jaw claudication
*changes in vision (amaurosis fugax, diplopia, visual loss; if missed, can lead to blindness)
*can be associated with PMR (40-50%)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

giant cell arteritis - diagnosis

A

*biopsy of the temporal artery:
-granulomatous inflammation
-multinucleated giant cells
-predominantly involves media and elastic lamina fragmentation
*if one side negative and suspicion high, biopsy the contralateral side
*often have an elevated ESR

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

giant cell arteritis - treatment

A

*high dose steroids (prednisone) before anything else (including before biopsy)
*steroids do not effect the biopsy results for a few weeks (try to biopsy within 7 days of starting steroids)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

giant cell arteritis - new advances

A
  1. imaging:
    -temporal artery ultrasound
    -PET imaging
  2. medications:
    -methotrexate has been used historically
    -toclizumab (IL-6 inhibitor)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

giant cell arteritis - associated with what other condition?

A

*polymyalgia rheumatica

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

giant cell arteritis - important complication

A

*can develop permanent vision loss (blindness) if left untreated

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

polymyalgia rheumatica (PMR) - epidemiology

A

*age > 50 yo
*prevalence increases with age

recall: associated with giant cell arteritis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

polymyalgia rheumatica (PMR) - symptoms

A

*pain & stiffness in symmetric proximal muscles (shoulder and hip girdle muscles)
*ABSENCE OF TRUE WEAKNESS (stop because it hurts)
*elevated ESR or CRP
*diagnosis = clinical

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

polymyalgia rheumatica (PMR) - treatment

A

*low dose prednisone
*improves almost overnight

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

overlap between giant cell arteritis (GCA) and polymyalgia rheumatica (PMR)

A

*40% of pts with GCA will have PMR
*15% of pts with PMR will have GCA
*ALWAYS screen for the other during each visit

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Takayasu’s arteritis - epidemiology

A

*vasculitis of aorta and major branches
*fairly rare
*YOUNG ( < 40 yo)
*female > male (8:1)
*Asian descent (highest prevalence)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Takayasu’s arteritis - symptoms

A

*vasculitis of aorta and major branches
*fever, fatigue, weight loss
* upper extremity claudication (classically, young female swimmer having trouble finishing laps b/c arms hurt)
*“pulseless disease” = absent or weak upper extremity pulses
*elevated ESP/CRP
*HTN (renal artery stenosis)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Takayasu’s arteritis - physical exam

A

*bruits over carotid, subclavian, or renal
*may become pulseless (late)
*discordant blood pressure from one arm to the other

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Takayasu’s arteritis - diagnosis

A

*angiography (conventional, CTA, or MRA)
*biopsy is not generally feasible but would show granulomatous inflammation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Takayasu’s arteritis - treatment

A

*high dose steroids + steroid sparing agent

23
Q

polyarteritis nodosa (PAN) - epidemiology

A

*age: 40-60 yo
*seen in children as well
*slight male predominance
*often associated with Hepatitis B

24
Q

polyarteritis nodosa (PAN) - pathogenesis

A

*immune complex mediated (type III hypersensitivity reaction)

25
Q

polyarteritis nodosa (PAN) - symptoms

A

*fever, arthralgia, weight loss
*peripheral nerve involvement (mononeuritis multiplex)
*renal involvement (hypertension)
*testicular pain
*palpable purpura
*micro-aneurismal formation:
-abdominal pain / intestinal angina

overall: affects the skin, kidneys, intestines, and nerves; SPARES the LUNGS

26
Q

polyarteritis nodosa (PAN) - diagnosis

A

*biopsy of skin, muscle or sural nerve:
-shows transmural inflammation and fibrinoid necrosis
*abdominal angiogram showing vasculitis

27
Q

polyarteritis nodosa (PAN) - treatment

A

*if Hep B positive: high dose steroids and antivirals

*if Hep B negative: high dose steroids and immunosuppression

28
Q

Kawasaki disease - epidemiology

A

*pediatric disease typically (children < 5 years old)
*rare in adults (consider HIV if present in adults)
*aka mucocutaneous lymph node syndrome

29
Q

Kawasaki disease - symptoms

A

*FEVER > 5 days
*conjunctivitis
*diffuse erythematous rash
*oral mucositis, Strawberry tongue
*rash on palms & soles, desquamating
*complications:
-acute coronary syndrome
-coronary artery aneurysms

30
Q

Kawasaki disease - important complication

A

*can develop coronary artery aneurysms

31
Q

Kawasaki disease - treatment

A

*high dose salicylates (aspirin)
*IVIG
*echo to evaluate for aneurysms (initially + 2 weeks + 6-8 weeks)

32
Q

ANCA vasculitis

A

*small vessel vasculitis
*p-ANCA = MPO
*c-ANCA = PR3

33
Q

granulomatosis with polyangiitis (GPA) - symptoms

A

*upper & lower respiratory tract + kidneys
*sinusitis, nasal, or ear involvement; saddle nose deformity
*cough, hemoptysis, pleurisy; cavitary lesions, often diagnosed with “multifocal pneumonia”
*pauci-immune glomerulonephritis (RBCs, casts, protein)
*eye involvement = uveitis
*palpable purpura

34
Q

granulomatosis with polyangiitis (GPA) - diagnosis

A

*clinical history
*lung or kidney biopsy:
-granulomas in tissue
-pauci-immune glomerulonephritis
*c-ANCA, anti-PR3+

35
Q

granulomatosis with polyangiitis (GPA) - treatment

A

*high dose steroids
*cyclophosphamide or rituximab

36
Q

microscopic polyangiitis (MPA) - symptoms

A

*lower respiratory tract + kidneys (no upper airway involvement)
*fever, arthralgia, palpable purpura
*rapidly progressive glomerulonephritis (pauci-immune)
*pulmonary hemorrhage

37
Q

microscopic polyangiitis (MPA) - diagnosis

A

*biopsy skin, lung, or kidney (necrotizing but not granulomas)
*p-ANCA, anti-MPO+

38
Q

microscopic polyangiitis (MPA) - treatment

A

*high dose steroids, cyclophosphamide or rituximab
*relapse rate higher than GPA

39
Q

eosinophilic granulomatosis with polyangiitis (EGPA) - symptoms

A

*history of asthma or allergic rhinitis (may develop as adult)
*multi-organ involvement possible
*lung, skin, heart, and nerves:
-pulmonary infiltrates
-palpable purpura
-mononeuritis multiplex

40
Q

eosinophilic granulomatosis with polyangiitis (EGPA) - diagnosis

A

*biopsy: granulomatous, necrotizing vasculitis with eosinophilic tissue involvement
*p-ANCA + (anti-MPO) (only in 40%)
*peripheral eosinophilia (>10%)

41
Q

eosinophilic granulomatosis with polyangiitis (EGPA) - treatment

A

*high dose steroids
*maybe cyclophosphamide or rituximab if severe

42
Q

IgA vasculitis - epidemiology

A

*previously Henoch-Schonlein Purpura
*most common CHILDHOOD vasculitis:
-ages 3 to 15 years; peak = 4-6 years old
-males > females (2:1)
-30 to 50% have a preceding URI
*rare in adults (associated with malignancy; more renal involvement)

43
Q

IgA vasculitis - clinical presentation

A

TETRAD:
1. purpura or petechiae with lower limb predominance
2. arthritis or arthralgias (large joints, lower extremity, oligoarticular)
3. abdominal pain (periumbilical pain, N/V, intussusception)
4. renal involvement (hematuria or proteinuria)

44
Q

IgA vasculitis - treatment

A

*supportive
*NSAIDs
*prednisone if severe renal involvement

45
Q

Behcet’s syndrome

A

*a small vessel vasculitis
*common in Mediterranean descent
*oral and genital ulcerations
*other manifestations: uveitis, rash, pulmonary hemorrhage

46
Q

types of cryoglobulins

A

*type 1: monoclonal IgM or IgG; associated with lympho-, myeloproliferative disease
*type 2: monoclonal IgM RF plus polyclonal IgG
*type 3: polyclonal IgM RF plus polyclonal IgG

*“mixed” cryoglobulins = types 2 & 3:
-type 2 = associated with Hep C
-type 3 = associated with autoimmune disease

47
Q

type 2 cryoglobulinemic vasculitis - associated with…

A

hepatitis C

48
Q

type 3 cryoglobulinemic vasculitis - associated with…

A

autoimmune disease

49
Q

cryoglobulinemic vasculitis - symptoms

A

* purpura - lower extremity/dependent areas
*mononeuritis multiplex
*immune complex glomerulonephritis

50
Q

cryoglobulinemic vasculitis - labs

A

*low complements (C4)
*Hep B, Hep C, HIV
*RF + but negative CCP

51
Q

treatment of cryoglobulinemic vasculitis

A

*Hep C associated (type 2): treat hep C, short course steroids if severe manifestations
*non Hep C associated: rituximab or cyclophosphamide

52
Q

cocaine-induced vasculitis

A

* levamisole is causative agent (70% of US cocaine contaminated)
*skin necrosis is predominate feature: ear lobe or nose necrosis
*clinically looks a lot like GPA, but worse cutaneous destruction, decreased systemic features

53
Q

cocaine-induced vasculitis - labs

A

*p-ANCA positive
*MPO negative
*can be PR3 positive
*anti-human neutrophil elastase positive
*agranulocytosis

54
Q

cocaine-induced vasculitis - treatment

A

*supportive
*stop cocaine

Rheumatology Exam (20 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions