GU1 - Nephrology > Systemic Vasculitis > Flashcards

Systemic Vasculitis Flashcards

(46 cards)

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1
Q

vasculitis def

A
  • group of clinical syndromes characterized by inflammation of blood vessels
  • can affect many diff organ systems
  • hard to dx
  • life-threatening
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2
Q

When to suspect vasculitis

A
  • multisystem inflammatory dz
  • significant constitutional sx
  • rapidly progressive organ dysfunction
  • elevated ESR
  • Severe anemia
  • platelets >500,000
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3
Q

Clinical features particularly suggestive of small vessel inflammation

A
  • palpable purpura
  • pulmonary infiltrates/hemoptysis
  • active urinary sediment
  • foot drop
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4
Q

Tests to order when suspect vasculitis

A
  • CBC
  • Chems
  • LFTs
  • UA/micro
  • CXR
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5
Q

Serologic tests for vasculitis

A
  • ANCA
  • HBsAg
  • Hep C
  • C3 and C4
  • HIV
  • ANA
  • ACA, “lupus” anticoagulant panel
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6
Q

Imaging tests for vasculitis

A
  • sinus CT
  • chest CT
  • mesenteric angiogram
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7
Q

Tissue biopsies to perform in vasculitis

A
  • temporal artery
  • sural nerve
  • muscle
  • lung
  • renal

** biopsy is the ultimate diagnostic tool

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8
Q

Common clinical manifestations of vasculitis

A
  • systemic: fever, sweat, weight loss
  • skin: palpable purpura
  • neuro: mononeuritis multiplex (foot drop, eye droop, etc.)
  • Musculoskeletal: arthralgia, arthritis, muscle pain, claudication
  • respiratory: sinusitis, epistaxis, pulm infiltrate
  • GI: abd pain, bloody stools
  • Renal: glomerulonephritis, HTN
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9
Q

Two common skin manifestations in vasculitis

A
  • palpable purpura
  • livedo reticularis (vascular inflammation of the skin)
  • splinter hemorrhages
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10
Q

Genitourinary manifestations of vasculitis

A
  • glomerulonephritis
  • HTN
  • hematuria
  • RBC casts
  • testicular pain (esp. PAN)
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11
Q

GI manifestations of vasculitis

A
  • mesenteric ischemia (after eating, bloody diarrhea, bowel perf)
  • Hepatitis
  • pancreatitis
  • cholecystitis
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12
Q

Ocular manifestations of vasculitis

A
  • scleritis
  • retinal vasculitis
  • iritis
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13
Q

Vasculitis common lab findings

A

Inflammatory markers:

  • elevated ESR
  • elevated CRP
  • leukocytosis
  • thrombcytosis
  • anemia
  • low albumin
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14
Q

Vasculitis mimics

A
  • infectious diseases: Endocarditis, HIV
  • drugs: cocaine, meth
  • cholesterol emboli
  • antiphospholipid antibody syndrome
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15
Q

Giant cell arteritis

  • criteria
  • how many needed for positive
A

3 of the 5:

  • Age > 50
  • new onset HA
  • ESR > or = 50
  • abnormal artery biopsy
  • temporal artery abnormality (necrosis, granulomata, WBC infiltrate)
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16
Q

Giant cell arteritis other manifestations

A
  • visual loss
  • jaw claudication
  • scalp tenderness
  • weight loss
  • muscle pain
  • large vessel involvement in 10%
  • blindness
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17
Q

GCA tx

A

high dose steroids (before biopsy to confirm!)

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18
Q

Granulomatous polyangiitis (GPA)

  • aka
  • describe
A
  • Wegener’s

- necrotizing vasculitis that affects small vessels of renal system and respiratory tract (pulmonary-renal syndrome)

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19
Q

Criteria for GPA

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A

3 of the 5:

  • nasal or oral inflammation: oral ulcers, bloody nasal drainage
  • abnormal CXR: nodules, infiltrates, cavities
  • urinary sediment: RBC and casts*
  • biopsy: vasculitis
  • proteinase-3 antibodies
20
Q

GPA respiratory involvement

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A
  • sinusitis: nasal septal ulceration

- pneumonitis: usually late sx, nodules can cavitate

21
Q

GPA renal involvement

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A
  • 85% of pts
  • focal/segmental necrotizing glomerulonephritis
  • usually progressive, within days
22
Q

GPA

- antibody

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A
  • C ANCA
  • proteinase 3 (C is 3rd letter)
  • very sensitive and specific
23
Q

GPA

- biopsy yield

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A
  • lung: highest yield
  • sinus: 40% yield
  • renal: vasculitis rarely seen, will see focal proliferative GN
24
Q

GPA

Tx

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A
  • cyclophosphamide
  • IV CYTOXAN
  • Rituximab for induction and maintenance
  • Azathioprine for maintenance
25
What are major ADRs for cyclophosphamide?
- bladder hemorrhage - cancer - affects ovaries
26
``` Microscopic Polyangiitis (MPA) - describe ```
- systemic vasculitis with predominant small vessel involvement - usually RPGN - sometimes with pulmonary hemorrhage
27
Which is more common: MPA or PAN
MPA but both are rare
28
MPA clinical manifestations
- renal - weight loss - skin - the list goes on - pulmonary is less likely
29
MPA antibodies
- P ANCA - myeloperoxidase antibodies - sensitivity/specificity is unclear...
30
MPA tx
- cyclophosphamide - IV CYTOXAN - Rituximab for induction and maintenance - Azathioprine for maintenance (same as GPA)
31
Polyarteritis Nodosa (PAN)
- necrotizing vasculitis of medium and small arteries | - vasculitis can be variable in distribution which can make dx difficult
32
PAN constitutional sx
common! - fever - weight loss
33
Criteria for PAN
3 of the 10: - Hep B Virus *** - weight loss > 4 kg - livedo reticularis - testicular pain - myalgia, weakness, leg tenderness - mono- or polyneuropathy - diastolic BP > 90 - increased BUN or Cr - arteriographic abnormality - biopsy of small/med artery contains PAN
34
PAN manifestations
- mononeuritis multiplex - renal involvement - arthralgia, myalgia, myositis (pulm involvement rare)
35
PEARLS PAN
- associated with HBsAG | - NOT associated with ANCA (unlike MPA and GPA)
36
Cryoglubulinemia | - describe
- paradigm of small vessel vasculitis | - damage is immune complex mediated
37
Cryoglubulinemia - associated with what - cryoprecipitate - complement?
- associated with Hep C - cryoprecipitate: Hep C Ag-Ab - complement C4 level very low
38
Cryoglubulinemia | - organ involvement
- constitutional sx - cutaneous - articular - vascular - neuro
39
Cryoglubulinemia | - lab findings
- rheumatoid factor (check first, faster) - complement C4 (low) - cryoglobulin (cryocrit): have to keep warm!!
40
Cryoglubulinemia | - tx
treat hep C with antivirals
41
Vasculitis of small to medium sized vessels | - 4 main types
1. drug-induced small vessel vasculitis 2. Henoch-Schonlein purpura 3. ANCA-associated vasculitis 4. infection-related vasculitis
42
Drug-induced small vessel vasculitis
hypersensitivity vasculitis
43
Henoch-Schonlein purpura
IgA vasculitis
44
ANCA-associated vasculitis
- GPA (Wegener's) - MPA - Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) * * these are the Pauci-immune glomerulonephritis types from Dr. V's lecture
45
infection related vasculitis
- bacterial endocarditis - post-streptococcal vasculitis - glomerulonephritis - Hep C related cryoglobulinemia
46
Vasculitis of medium sized vessel
PAN
GU1 - Nephrology
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