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GU1 - Nephrology > Systemic Vasculitis > Flashcards

Systemic Vasculitis Flashcards

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

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

A
  • sinusitis: nasal septal ulceration

- pneumonitis: usually late sx, nodules can cavitate

21
Q

GPA renal involvement

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

GPA

- antibody

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

GPA

- biopsy yield

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

GPA

Tx

A
  • cyclophosphamide
  • IV CYTOXAN
  • Rituximab for induction and maintenance
  • Azathioprine for maintenance
25
Q

What are major ADRs for cyclophosphamide?

A
  • bladder hemorrhage
  • cancer
  • affects ovaries
26
Q 
Microscopic Polyangiitis (MPA)
- describe
A
  • systemic vasculitis with predominant small vessel involvement
  • usually RPGN
  • sometimes with pulmonary hemorrhage
27
Q

Which is more common: MPA or PAN

A

MPA but both are rare

28
Q

MPA clinical manifestations

A
  • renal
  • weight loss
  • skin
  • the list goes on
  • pulmonary is less likely
29
Q

MPA antibodies

A
  • P ANCA
  • myeloperoxidase antibodies
  • sensitivity/specificity is unclear…
30
Q

MPA tx

A
  • cyclophosphamide
  • IV CYTOXAN
  • Rituximab for induction and maintenance
  • Azathioprine for maintenance
    (same as GPA)
31
Q

Polyarteritis Nodosa (PAN)

A
  • necrotizing vasculitis of medium and small arteries

- vasculitis can be variable in distribution which can make dx difficult

32
Q

PAN constitutional sx

A

common!

  • fever
  • weight loss
33
Q

Criteria for PAN

A

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
Q

PAN manifestations

A
  • mononeuritis multiplex
  • renal involvement
  • arthralgia, myalgia, myositis
    (pulm involvement rare)
35
Q

PEARLS PAN

A
  • associated with HBsAG

- NOT associated with ANCA (unlike MPA and GPA)

36
Q

Cryoglubulinemia

- describe

A
  • paradigm of small vessel vasculitis

- damage is immune complex mediated

37
Q

Cryoglubulinemia

  • associated with what
  • cryoprecipitate
  • complement?
A
  • associated with Hep C
  • cryoprecipitate: Hep C Ag-Ab
  • complement C4 level very low
38
Q

Cryoglubulinemia

- organ involvement

A
  • constitutional sx
  • cutaneous
  • articular
  • vascular
  • neuro
39
Q

Cryoglubulinemia

- lab findings

A
  • rheumatoid factor (check first, faster)
  • complement C4 (low)
  • cryoglobulin (cryocrit): have to keep warm!!
40
Q

Cryoglubulinemia

- tx

A

treat hep C with antivirals

41
Q

Vasculitis of small to medium sized vessels

- 4 main types

A
  1. drug-induced small vessel vasculitis
  2. Henoch-Schonlein purpura
  3. ANCA-associated vasculitis
  4. infection-related vasculitis
42
Q

Drug-induced small vessel vasculitis

A

hypersensitivity vasculitis

43
Q

Henoch-Schonlein purpura

A

IgA vasculitis

44
Q

ANCA-associated vasculitis

A
  • GPA (Wegener’s)
  • MPA
  • Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)
    • these are the Pauci-immune glomerulonephritis types from Dr. V’s lecture
45
Q

infection related vasculitis

A
  • bacterial endocarditis
  • post-streptococcal vasculitis
  • glomerulonephritis
  • Hep C related cryoglobulinemia
46
Q

Vasculitis of medium sized vessel

A

PAN

GU1 - Nephrology (16 decks)

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