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)
T/F
urticarial vasculitis affects women more than men
True
What are the triggers of urticarial vasculitis?
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.
What are diagnostic criteria for Hypocomplementaemic urticarial vasculitis syndrome?
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
What clinical and lab features differentiate Hypocomplementaemic urticarial vasculitis or HUV syndrome from SLE?
Occular inflammation, asthma/COPD and angio-oedema
Lack of anti-dsDNA and anti-Sm Abs
NB granular DEJ +ve DIF suggests lupus (lupus band)
Which types of vasculitis have many eos?
drug induced vasculitis eosinophilic small vessel vasculitis Granuloma faciale Churg-strauss eosinophilic angiocentric fibrosis - rare mucosal variant of granuloma faciale
What is the classical triad of Wegners granuloamtosis?
WG=V double G
Systemic small/medium vessel vasculitis
Glomerulonephritis
Necrotizing granulomatous inflammation of upper and lower respiratory tracts
What is Meltzer’s triad?
clinical features of cryoglobulinaemic vasculitis 'Meltzy the cat has PAWs" Purpura Arthralgia Weakness (asthenia)
What are the key laboratory findings in cryoglobuliaemic vasculitis?
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
What drugs are associated with urticarial vasculitis?
MANS Coke MTX AntiTNFα NSAIDs SSKI Cocaine
What are the associations of erythema induratum?
TB other infections eg) mycobacteria, strep Drugs esp propylthiouracil Autoimmune colitis (IBD) Colon cancer
