Vasculitis Dan Flashcards

1
Q

What are the major DIFFERENTIALs for the clinical appearance of a vasculitis?

A
PPPP VV W Amy TESTS
PPD
Actinic purpura
Purpura fulminans
PLEVA
Vaso-occlusive disease - many causes
Livedoid vasculopathy
Hypergammaglobulinameic purpura of Waldenstrom 
Amyloidosis
Trauma
Erythema multiforme
Sweets
Thrombocytopenia or plt dysfunction
Scurvy
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2
Q

Which drugs can cause vasculitis?

A
Anti BHP – biotics, hypertensives and psychotics
NSAIDs, aspirin
Derm drugs;
Retinoids
Dapsone
tetracyclines
MTX, CsA, AZA, cyclophos
Anti-TNFs
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3
Q

What is chronic CSVV?
How common is it?
what are risk factors for recurrent or chronic disease?

A
chronic if >3 months
10% of cases
risk factors - CASH
Cryoglobulinaemia
Arthralgia
Strep cause
Hep C cause
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4
Q

What are diagnostic criteria of HSP?

A
Palpable purpura + at least one of; RADA
Renal involvement (haematuria/proteinuria)
Arthralgia or acute Arthritis of any joint.
Diffuse abdo pain
DIF showing predominant IgA deposition
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5
Q

T/F

In HSP the presence of renal disease is major determinate of long term morbidity and mortality

A

True

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6
Q

T/F

HSP becomes chronic or recurrent in 25%

A

False

5-10%

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7
Q

T/F

Adults with IgA vasculitis are less likely to get skin ulceration than kids with HSP

A

False

more likely - 60% ulcerate

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8
Q

T/F

Adult IgA vasculitis is associated with lung carcinoma

A

True

more risk of solid organ tumours than haematologic and most are lung carcinoma

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9
Q

How long does HSP last?

A

skin diseas lasts 6-16 weeks
renal disease can ccur late but always onset in first 3 months
resolves over weeks-months but 5-10% chronic esp renal effects

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10
Q

T/F

Eosinophils are a feature of drug induced CSVV

A

True
often many eos on biopsy
also 80% have a peripheral eosinophilia
(nb 25% of non-drug CSVV also has periph eosinophilia)

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11
Q

What are the DIF findings of CSVV?

A

Perivascular IgM or C3 deposition of upper dermal papillary vessels in >80% of fresh lesions
Sometimes IgG or IgA

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12
Q

T/F

CSVV usually starts 7-10 days after the trigger

A

True except for drug causes - usually 1-3 weeks

may be delayed several months after starting a new regular drug

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13
Q

What are the causes of CSVV?

A

o Infection
Strep – v important cause
Hep C (most common hep), HepB, A, HIV, CMV
Others – guided by Hx and exam
o AI CTD / inflammatory diseases
RA, seroneg arthritis, SLE, IBD, Sjogren’s, Behcets, CF, sarcoid, PBC, coeliac
do ANA, ENA, ANCAs, RF, C3, C4
o Drugs – see below section but esp;
Anti BHP – biotics, hypertensives and psychotics
NSAIDs, aspirin
Derm drugs;
Retinoids
Dapsone
tetracyclines
MTX, CsA, AZA, cyclophos
Anti-TNFs
o Cancers – myeloma, monoclonal gammopathies, lymphoproliferative, myeloproliferative, solid carcinomas are unusual but increased risk if IgA +ve DIF
o Periodic fever syndromes are very rare cause

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14
Q

What is the prognosis of adult IgA vasculitis or HSP compared to kids?

A
Adults get;
more skin necrosis
more chronic renal failure
more diarrhoea
high WCC more often
Need more aggressive therapy and longer hospital stay
adult HSP assoc w/ coeliac
adult IgA vasculitis assoc w/ solid organ carcinoma esp lung
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15
Q

What are the types of urticarial vasculitis?

A

Hypocomplementaemic urticarial vasculitis (almost always females)
Normocomplementaemic urticarial vasculitis (F>M)

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16
Q

T/F

urticarial vasculitis affects women more than men

A

True

17
Q

What are the triggers of urticarial vasculitis?

A
AI-CTD very important cause
 - Sjogrens, SLE
Infection
 - Hep B, C
Malignancy
 - IgM/IgA gammopathies, bowel cancer
Drugs - similar to CSVV
Serum sickness
Physical urticaria - May rarely be precipitated by exercise/cold/UV light.
18
Q

What are diagnostic criteria for Hypocomplementaemic urticarial vasculitis syndrome?

A
Need both major + at least 2 minor;
Major
1.	Urticaria lasting >6 months
2.	Hypocomplementaemia
•	Minor (GRAVEL)
1.	Glomerulonephritis
2.	Recurrent abdo pain
3.	Arhralgia or arthritis
4.	Vasculitis on skin biopsy
5.	Episcleritis or Uveitis (can also get iritis or conjunctivitis)
6.	Low C1q leven and +ve C1q precipitin test
19
Q

What clinical and lab features differentiate Hypocomplementaemic urticarial vasculitis or HUV syndrome from SLE?

A

Occular inflammation, asthma/COPD and angio-oedema
Lack of anti-dsDNA and anti-Sm Abs
NB granular DEJ +ve DIF suggests lupus (lupus band)

20
Q

Which types of vasculitis have many eos?

A
drug induced vasculitis
eosinophilic small vessel vasculitis
Granuloma faciale
Churg-strauss
eosinophilic angiocentric fibrosis - rare mucosal variant of granuloma faciale
21
Q

What is the classical triad of Wegners granuloamtosis?

A

WG=V double G
Systemic small/medium vessel vasculitis
Glomerulonephritis
Necrotizing granulomatous inflammation of upper and lower respiratory tracts

22
Q

What is Meltzer’s triad?

A
clinical features of cryoglobulinaemic vasculitis
'Meltzy the cat has PAWs"
Purpura
Arthralgia
Weakness (asthenia)
23
Q

What are the key laboratory findings in cryoglobuliaemic vasculitis?

A

Low C4 - 90%
RF +ve - 70%
IgM band on EPP (if type 2 mixed cryoglobulinaemia)
2 out of 3 of above needed for lab part of criteria
Hepatitis serology, esp HepC

24
Q

What drugs are associated with urticarial vasculitis?

A
MANS Coke
MTX
AntiTNFα
NSAIDs
SSKI
Cocaine
25
Q

What are the associations of erythema induratum?

A
TB 
other infections eg) mycobacteria, strep
Drugs esp propylthiouracil
Autoimmune colitis (IBD)
Colon cancer