Vasculitis Dan Flashcards
What are the major DIFFERENTIALs for the clinical appearance of a vasculitis?
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
Which drugs can cause vasculitis?
Anti BHP – biotics, hypertensives and psychotics NSAIDs, aspirin Derm drugs; Retinoids Dapsone tetracyclines MTX, CsA, AZA, cyclophos Anti-TNFs
What is chronic CSVV?
How common is it?
what are risk factors for recurrent or chronic disease?
chronic if >3 months 10% of cases risk factors - CASH Cryoglobulinaemia Arthralgia Strep cause Hep C cause
What are diagnostic criteria of HSP?
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
T/F
In HSP the presence of renal disease is major determinate of long term morbidity and mortality
True
T/F
HSP becomes chronic or recurrent in 25%
False
5-10%
T/F
Adults with IgA vasculitis are less likely to get skin ulceration than kids with HSP
False
more likely - 60% ulcerate
T/F
Adult IgA vasculitis is associated with lung carcinoma
True
more risk of solid organ tumours than haematologic and most are lung carcinoma
How long does HSP last?
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
T/F
Eosinophils are a feature of drug induced CSVV
True
often many eos on biopsy
also 80% have a peripheral eosinophilia
(nb 25% of non-drug CSVV also has periph eosinophilia)
What are the DIF findings of CSVV?
Perivascular IgM or C3 deposition of upper dermal papillary vessels in >80% of fresh lesions
Sometimes IgG or IgA
T/F
CSVV usually starts 7-10 days after the trigger
True except for drug causes - usually 1-3 weeks
may be delayed several months after starting a new regular drug
What are the causes of CSVV?
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
What is the prognosis of adult IgA vasculitis or HSP compared to kids?
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
What are the types of urticarial vasculitis?
Hypocomplementaemic urticarial vasculitis (almost always females)
Normocomplementaemic urticarial vasculitis (F>M)
