Systemic Vasculitis Flashcards
vasculitis def
- group of clinical syndromes characterized by inflammation of blood vessels
- can affect many diff organ systems
- hard to dx
- life-threatening
When to suspect vasculitis
- multisystem inflammatory dz
- significant constitutional sx
- rapidly progressive organ dysfunction
- elevated ESR
- Severe anemia
- platelets >500,000
Clinical features particularly suggestive of small vessel inflammation
- palpable purpura
- pulmonary infiltrates/hemoptysis
- active urinary sediment
- foot drop
Tests to order when suspect vasculitis
- CBC
- Chems
- LFTs
- UA/micro
- CXR
Serologic tests for vasculitis
- ANCA
- HBsAg
- Hep C
- C3 and C4
- HIV
- ANA
- ACA, “lupus” anticoagulant panel
Imaging tests for vasculitis
- sinus CT
- chest CT
- mesenteric angiogram
Tissue biopsies to perform in vasculitis
- temporal artery
- sural nerve
- muscle
- lung
- renal
** biopsy is the ultimate diagnostic tool
Common clinical manifestations of vasculitis
- 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
Two common skin manifestations in vasculitis
- palpable purpura
- livedo reticularis (vascular inflammation of the skin)
- splinter hemorrhages
Genitourinary manifestations of vasculitis
- glomerulonephritis
- HTN
- hematuria
- RBC casts
- testicular pain (esp. PAN)
GI manifestations of vasculitis
- mesenteric ischemia (after eating, bloody diarrhea, bowel perf)
- Hepatitis
- pancreatitis
- cholecystitis
Ocular manifestations of vasculitis
- scleritis
- retinal vasculitis
- iritis
Vasculitis common lab findings
Inflammatory markers:
- elevated ESR
- elevated CRP
- leukocytosis
- thrombcytosis
- anemia
- low albumin
Vasculitis mimics
- infectious diseases: Endocarditis, HIV
- drugs: cocaine, meth
- cholesterol emboli
- antiphospholipid antibody syndrome
Giant cell arteritis
- criteria
- how many needed for positive
3 of the 5:
- Age > 50
- new onset HA
- ESR > or = 50
- abnormal artery biopsy
- temporal artery abnormality (necrosis, granulomata, WBC infiltrate)
Giant cell arteritis other manifestations
- visual loss
- jaw claudication
- scalp tenderness
- weight loss
- muscle pain
- large vessel involvement in 10%
- blindness
GCA tx
high dose steroids (before biopsy to confirm!)
Granulomatous polyangiitis (GPA)
- aka
- describe
- Wegener’s
- necrotizing vasculitis that affects small vessels of renal system and respiratory tract (pulmonary-renal syndrome)
Criteria for GPA
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
GPA respiratory involvement
- sinusitis: nasal septal ulceration
- pneumonitis: usually late sx, nodules can cavitate
GPA renal involvement
- 85% of pts
- focal/segmental necrotizing glomerulonephritis
- usually progressive, within days
GPA
- antibody
- C ANCA
- proteinase 3 (C is 3rd letter)
- very sensitive and specific
GPA
- biopsy yield
- lung: highest yield
- sinus: 40% yield
- renal: vasculitis rarely seen, will see focal proliferative GN
GPA
Tx
- cyclophosphamide
- IV CYTOXAN
- Rituximab for induction and maintenance
- Azathioprine for maintenance
What are major ADRs for cyclophosphamide?
- bladder hemorrhage
- cancer
- affects ovaries
Microscopic Polyangiitis (MPA) - describe
- systemic vasculitis with predominant small vessel involvement
- usually RPGN
- sometimes with pulmonary hemorrhage
Which is more common: MPA or PAN
MPA but both are rare
MPA clinical manifestations
- renal
- weight loss
- skin
- the list goes on
- pulmonary is less likely
MPA antibodies
- P ANCA
- myeloperoxidase antibodies
- sensitivity/specificity is unclear…
MPA tx
- cyclophosphamide
- IV CYTOXAN
- Rituximab for induction and maintenance
- Azathioprine for maintenance
(same as GPA)
Polyarteritis Nodosa (PAN)
- necrotizing vasculitis of medium and small arteries
- vasculitis can be variable in distribution which can make dx difficult
PAN constitutional sx
common!
- fever
- weight loss
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
PAN manifestations
- mononeuritis multiplex
- renal involvement
- arthralgia, myalgia, myositis
(pulm involvement rare)
PEARLS PAN
- associated with HBsAG
- NOT associated with ANCA (unlike MPA and GPA)
Cryoglubulinemia
- describe
- paradigm of small vessel vasculitis
- damage is immune complex mediated
Cryoglubulinemia
- associated with what
- cryoprecipitate
- complement?
- associated with Hep C
- cryoprecipitate: Hep C Ag-Ab
- complement C4 level very low
Cryoglubulinemia
- organ involvement
- constitutional sx
- cutaneous
- articular
- vascular
- neuro
Cryoglubulinemia
- lab findings
- rheumatoid factor (check first, faster)
- complement C4 (low)
- cryoglobulin (cryocrit): have to keep warm!!
Cryoglubulinemia
- tx
treat hep C with antivirals
Vasculitis of small to medium sized vessels
- 4 main types
- drug-induced small vessel vasculitis
- Henoch-Schonlein purpura
- ANCA-associated vasculitis
- infection-related vasculitis
Drug-induced small vessel vasculitis
hypersensitivity vasculitis
Henoch-Schonlein purpura
IgA vasculitis
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
infection related vasculitis
- bacterial endocarditis
- post-streptococcal vasculitis
- glomerulonephritis
- Hep C related cryoglobulinemia
Vasculitis of medium sized vessel
PAN
