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Rheumatology - Rutvi > Vasculitis > Flashcards

Vasculitis Flashcards

1
Q

What is vasculitis?

A

inflammation of blood vessels w/ necrosis of blood vessel wall, narrowing, and occlusion

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2
Q

what are the major signs and symptoms for vasculitis?

A
  • fever, myalgia, athralgia, malaise

- palpable purpura, focal skin necrosis, livedo reticularis, urticartria, myositis, etc

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3
Q

What happens in livedo reticularis?

A

blood vessels are dilated

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4
Q

How are the different types of vasculitis classified?

A

size of blood vessel involved

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5
Q

What are the large vessel vasculitis?

A

Giant cell temporal arteritis and Takayasu Arteritis - both granulomatous, Giant cell is older people while Takayasu is less than 50 yr olds

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6
Q

What are the medial vessel vasculitis?

A
  1. PAN
  2. Kawakasi Dz
  3. Buerger
  4. Cutaneous PAN
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7
Q

What are the small vessel vasulitis?

A
  1. Wegners - GPA, lung, kidney, and URT ( C-ANCA)
  2. Churg Strauss - eosinophils and asthma (p-ANCA)
  3. MPA - no URT but yes to lung and kidney (pANCA)
  4. HSP - skin, gut, and kidney – IgA
  5. Essential Cryoglobulinemia - skin and kidney
  6. Leukocytoclastic vasculitis - HSR vasculitis
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8
Q

What vasculitis have granulomas?

A
  1. giant cell
  2. takayasu
  3. cogan
  4. wegners
  5. churg strauss
  6. Buerger
  7. Rheumatoid vasculitis
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9
Q

What is PAN?

A
  1. necrotizing, medium
  2. spares small vessels
  3. no GN or granuloma
  4. MEN
  5. Hep B
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10
Q

What vasculitis have GN?

A
  1. MPA
  2. HSP
  3. Wegner
  4. Churg Strauss
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11
Q

What are the clinical manifestations of PAN?

A
  1. systemic symptoms
  2. peripheral neuropathy
  3. HTN and renal failure
  4. GI problems -
  5. testicular involvement
  6. cadiac involvement
  7. skin — LIVEDO RETICULARIS
  8. SPARES LUNG!
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12
Q

What is the histology of PAN?

A

Fibrinoid Necrosis, thrombosis, acute inflammatory cell exudates, aneurysm formation

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13
Q

What will be see in lab tests for PAN?

A
  1. A, T, E, C
  2. hematuria
  3. elevated creatinine
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14
Q

what happens in the acute phase and late phase of PAN on histo?

A
  1. acute - PMN and fibrinoid necrosis

2. late - chronic inflammation, fibroblasts, and scarring

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15
Q

What phase are all the lesions in PAN on histo?

A

different phases – pearl on a string appearance!

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16
Q

What is cutaneous PAN?

A

PAN that is limited to the skin, associated w/ Hep C. Myalgia and peripheral neuropathy seen.

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17
Q

What is Buerger’s dz? (thomboangitis obliterans)

A
  1. affects the vascular supply to the lower limbs in young adult male tobacco smokers - South Asians
  2. Abs including ANCAS against MPO have been associated w/ sever dz
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18
Q

What symptoms do Buerger’s pts have?

A

begins w/ bilateral pain and ischemia in lower extermities, pain on exposure to cold may exist

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19
Q

How does one stop Beurger’s?

A

STOP SMOKING - helps with the rapid evolution of the disease to ischemia

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20
Q

What signs can be presents for Beurger’s?

A

limb pain, digital cyanosis, splinter hemorrhages, and skin/digital ulcers

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21
Q

What is seen for Buerger’s for histo?

A

segmental, transmural w/out necrosis; Granuloma present

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22
Q

What is Cogan’s syndrome?

A
  1. rare, young adults w/ red/painful eyes and/or hearing loss within 4 months
  2. rarely you get widespread vasculitis w/ purpura and gangrene
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23
Q

What is Susac’s syndrome?

A
  1. rare, endotheliopathy, wide age range
  2. sudden onset of sensorineural hearing loss w/ encelphalopathy and branch retinal artery occlusion
  3. DDx includes - Cogan’s syndrom and GPA
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24
Q

What is Microscopic Polyangitis? MPA

A
  1. necrotizing - small vessels
  2. GN
  3. p-ANCA
  4. more common than PAN
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25
Q

What are the clinical manifestations of MPA?

A
  1. systemic symptoms
  2. GN
  3. lung - pulmonary hemorrhage
  4. neuropathy
  5. skin
26
Q

Lab findings for MPA

A
  1. A, T, E, C
  2. RBC casts
  3. elevated creatinine
  4. p-ANCA
27
Q

What are the most common type of IC-mediated vasculitis?

A
  1. HSR vasculitis
  2. HSP
  3. mixed cryoglobulinemia
28
Q

What is the most prominent feature of IC-mediated vasculitis?

A

cutaneous involvement of small blood vessels

29
Q

What is the classic cutaneous finding in small vessel vasculitis?

A

palpable purpura

30
Q

What is HSP associated w/ in terms of symptoms/signs?

A
  1. purpura
  2. arthritis
  3. GN
  4. colicky ab pain
31
Q

What is cryoglobulinemic vasculitis most often associated w/?

A

long standing Hep C. Also you have both IgM and IgG

32
Q

What is HSR vasculitis?

A

small vessel vasculitis of skin that spares internal organs and usually following drug exposures – withdraw drugs in adults

33
Q

What is HSP?

A

necrotizing vasculitis of small vessels. Most common in children after an URT infection and usually resolves. IgA deposition!

34
Q

What is seen on histo for HSP in early and late stages?

A
  1. neutrophils w/ breakdown - dispersing nuclear dust
  2. later - lymphocytic infiltration

– lesions will be in the same stage due b/c of immune mediated by exposure

35
Q

Lab for HSP

A
  1. A, T, C, E

2. hematuria, elevated creatinine, RBC casts if renal involvement

36
Q

What is cryoglobulinemia vasculitis?

A

cryoglobulins that preciptate from serum under conditions of cold and resolubilize upon rewarming.
Associated w/ Hep C

37
Q

What are the different type of cryoglobulins?

A

Type 1: monoclonal, RF negative, associated w/ malignancy - cause is hyperviscosity syndrome not vasculitis
Type 2: polyclonal IgG, monoclonal IgM, RF positive, IC mediated vasculitis is the cause
Type 3: polyclonal, RF positive, IC mediated vasculitis is the casue

38
Q

What are the clinical presentations of Cryoglobulinemic vasculitis?

A
  1. palpable purpura w/ ulcers
  2. peripheral neuropathy
  3. Raynaud’s
  4. Digital ischemia
  5. Secondary Sjogrens
39
Q

What are the lab findings for Cryoglobulinemic?

A
  1. A, T, E,

2. low C4 levels, RF positive

40
Q

Rheumatoid vasculitis

A
  1. medium and small blood vessels
  2. deep cutaneous ulcer over malleoli are hallmark of RV
  3. biopsy shows granular IgM and C3 deposition in vessels.
41
Q

What organs does GPA usually affect?

A
  1. upper and lower RT
  2. kidneys
  3. lungs
  4. pts can get saddle nose deformity, ulcerations
42
Q

What is the antibody in GPA?

A

c-ANCA for proteinase 3 antigen

43
Q

What is AGPA?

A

prior history of adult onset asthma or allergic rhinitis and tissue eosinophilia w/ necrotizing vasculitis and extravascular granuloma formation

44
Q

What is the pathology of GPA?

A

necrotizing granulomatosis, geographic area of necrosis w/ basophilic debris and palisaded histocytes – like Rheumatoid nodule

45
Q

What antibody can be found in AGPA?

A

pANCA

46
Q

What are the clinical manifestations of AGPA?

A
  1. systemic symptoms
  2. lungs - dyspnea and cough
  3. peripheral neuropathy
  4. GU involvement
47
Q

What is giant cell arteritis?

A
  1. large sized - necrotizing inflammation
  2. granuloma
  3. AM stiffness, pain in pelvic girdle, shoulders
  4. old white women
48
Q

What are the clinical presentations of giant cell arteritis?

A
  1. temporal headaches
  2. visual disturbances
  3. jaw/tongue claudication
  4. tender, swollen, temporal artery
49
Q

Pathology of giant cell arteritis?

A
  1. segemental, transmural
  2. lymphocytes that affects internal or external elastic membrane,
  3. granuloma w/ multinucleated giant cells
  4. inflammation is most marked near elastic membrane
50
Q

What is crucial about giant cell in terms of Rx?

A

give corticosteroids to prevent the blindness, don’t even wait for biopsy just give them meds!

51
Q

What is Takaysu’s arteritis?

A

necrotizing, large vessels (aortic arch and its branches affects), granuloma. Young Asians

52
Q

Clinical manifestations of Takaysu’s?

A
  1. renal - renovascular HTN
  2. GI - N/V/IBD
  3. cardiac
  4. CNS
  5. Muscoskeletal symptoms
53
Q

What is seen on pathology of Takaysu’s?

A
  1. segmental, necrotizing, lymphocytes
  2. intimal and adventital thickening
  3. medial loss of elastic fibers = thinning = perforation likely
54
Q

What is Kawaski?

A

necrotizing, coronary arteries – give Aspiring and IvIg

55
Q

Clinical manifestation of Kawaksi?

A
  1. Rash
  2. bilateral conjunctival injection
  3. cervical LAD
  4. extremity changes
  5. mucus membrane changes - strawberry tongue
56
Q

Pathology for Kawaksi?

A

endothelial necrosis, transmural w/ PMN, lymphocytes, wall necrosis, Aneurysm, thrombosis — MEDIA Thickened

57
Q

What is primary angitis of CNS?

A
  1. vasculitis confined to brain, meninges, or spinal cord
  2. dx based on spinal fluid, brain/vascular imaging, biopsy
  3. many subsets exists such as granulomatosis PACNS
58
Q

What is GPACNS

A
  1. affects middle aged med, present w/ chronic insidious headaches along w/ diffuse and focal neurologic deficits
  2. commonly have infarcts, high T2 mediated fluid attentuation on MRI
  3. dont’ do a cerebral angiogram
59
Q

What is Behcet’s dz?

A
  1. complex multisystem - oral and genital ulcers
  2. cutaneous lesions - PMN vascular
  3. highest prevalence in silk route!
  4. HLA B51, think heat shock proteins
60
Q

What is the Dx of Behcet’s based on?

A

oral aphthae, genital aphthae, ocular lesions, cutaneous lesions, and a positive pathergy test

Rheumatology - Rutvi (7 decks)

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