Vasulitis Flashcards
Definition of Vasculitis
A disease of blood vessels characterised by inflammation of the vessel wall
HIV vasculitis
A large variety of vasculitidies has been described in HIV involving all types of vessel
Causes of cutaneous vasculitis (4)
HIV
HCV (cryoglobulinaemia)
Drugs
Other
Takayasu’s arteritis
A large vessel disease effecting the aortic arch and major branches causing dilation or occlusion
9x more common in young (15-25) women – Fever, malaise, weight loss, mild leucocytosis & raised inflammatory markers
Erythema elevatum diutinum
A sometimes paraneoplastic vasculitis which presents with rounded, discoloured plaques on the backs of hands and extensor surfaces
Lesions occurring on the face are indistinguishable from granuloma faciale
Behcet’s disease
Onset 20-35yrs, M:F/1:1, associated with HLA B51 (mediterranean or far eastern)
Presents with recurrent oral & genital ulceration
Uveitis, skin involvement (pathergy reaction), CNS, arthritis, thrombus (arterial or venous)
Classification of blood vessels
Large – aorta & major branches (Giant cell/Takayasu’s arteritis)
Medium – main visceral arteries (Polyarteritis nodosa or kawaski disease)
Small – venules, capillaries & arterioles (many)
Polyarteritis nodosa (PAN)
M:F, 2:1 – 40-60yrs – peripheral nerves (neuropathy or mononeuritis), focal necrotising GN, palpable purpura, punched out skin lesions, infarctions, livedo reticularis (also joints, muscle, gut)
Raised inflammatory markers and HBsAg (hep B infection in 10-60% of cases)
Vasculitidies of the small vessels (6)
Cutaneous leukocytoclastic angiitis. Essential cryoglobulinaemia
Microscopic poylangitis (PGA) Wegener’s/granulomatosis+PA (GPA)
Churg-strauss syndrome (CSS) HSP
Churg-strauss syndrome (CSS) or Eosinophilic granulomatosis with polyangitis (EGPA)
M:F/2:1, onset 15-70 but mean 38yrs, Associated with atopy/eosinophilia – Migratory pulmonary infiltrates
Can cause mono- or peripheral neuropathy and cutaneous vasculitis – Treat with corticosteroids
Allergic phase>eosinophilic stage>vasculitic stage
Wegener’s granulomatosis/granulomatosis+polyangitis (GPA)
M:F/1:1, 97% white and mean age 41yrs
Affects upper (90%) and lower (50%) airways, eyes (50%) and kidneys (75%) - starts with rhinitis and saddle-nose deformity
cANCA - proteinase-3 positive
Treat with cyclophosphamide + high dose corticosteroids
Microscopic polyangitis (PGA)
Necrotising small vessel disease without granuloma formation
Most pts present with haematuria and proteuria due to progressive GM and may also have fever, malaise, weight loss etc
pANCA positive – treat with pred or cyclophosphamide/ azathioprine
Essential cryoglobulinaemia
Idiopathic condition of high levels of cryoglobulins in the blood
Has been associated with Hep C and can cause purplish vasculitis, nerve and kidney damage, splenic enlargement, arthritis and swelling
Symptoms of Large vessel vasculitis (4)
Visual symptoms
Dizziness and syncope
Arm claudication
Inflammation or aneurysm of the vessel
Symptoms of medium vessel vasculitis (5)
Angina or MIs (coronary artery) Kidney or bowel infarction Stroke Thrombus formation vessel wall oedema
Symptoms of small vessel vasculitis (4)
Glomerulonephritis
Cutaneous lesions (purpura or petechiae)
Peripheral neuropathy
Local inflammation and blockage
Blood tests in vasculitis
ESR or CRP, FBC, WCC and Hb LFT, urinalysis, CPK, RFT. Complement or Cryoglobulins Blood cultures or serology. ANA (anti-nuclear antibody) or ANCA
ANCA
anti-neutrophil cytoplasmic antibodies - a heterogenous group of Ab which can either be cytoplasmic (cANCA), perinuclear (pANCA) or atypical (aANCA)
pANCA is in some ways an artefact, binding to cationic proteins
Sub components of ANA screen
Anti-RO – SLE or sjogren’s (anti-La)
Anti-Sm – specific but not sensitive for SLE
Anti-dsDNA – SLE (specifically lupus nephritis)
Anti-histone - SLE or drug induced lupus
Anti-Scl 70 – diffuse cutaneous systemic sclerosis (dcSSc)
pANCA
Caused by antibodies against myeloperoxidases, elastase, cathepsin G, lactoferrin & lysozyme
Associated with microscopic polyangitis (40-80%) but is also seen in other rheumatic diseases, IBD, HIV or drug reaction
cANCA
Principally due to antibodies against proteinase 3 and best associated with GPA – low sensitivity but very specific
Cutaneous Polyarteritis nodosa (CPAN)
A less severe form of PAN – tender subcut nodules, livedo reticularis, cutaneous ulcers & necrosis
often associated with strep infection. Rarely it will progress to PAN
Pathergy reaction
The reactive formation of a skin lesion at the site of trauma
considered an important positive predictive sign for Behcet’s disease
Similar to Koebner phenomenon (psoriasis, lupus etc)
Henoch-schonlein purpura (HSP)
Presents in child (M:F/2:1) after a URTI with widespread (lower limbs and buttocks), palpable purpura – can cause nephritis or arthritis
Risk of GI involvement
IgA mediated
Giant cell arteritis (GCA) (also temporal arteritis or Horton’s disease)
Vasculitis mainly effecting the temporal cranial arteries
Commonest vasculitis – 20/100,000 over 50yr, M:F/2:1
Most common in northern europeans (HLA-DR4)
Causes of GCA
Arguments for environmental agents and even an infective cause (Chlamydia, parovirus B19, RSV)
Symptoms of GCA
Temporal headache and visual loss (amaurosis fugax)
Cough or dyspnoea
Jaw claudication (tongue, arm, angina) CVA or TIA
Tender, thickened Temporal artery with scalp tenderness
Investigations in GCA
Raised ESR/CRP, Raised platelets Raised alk phos Nomochromic anaemia Temporal artery biopsy is gold standard (beware skip lesions)
ACR criteria for GCA
Three or more of:
Over 50 at onset New headache
Temporal artery abnormality Elevated ESR
Abnormal artery biopsy
Treatments of GCA
40-60mg daily predisolone for 8 weeks –> drop by 5mg every 3-4wks to 10mg –> drop by 1mg per month
Give PPI and bisphosphonate
Medications for the treatment of vasculitis (4)
Corticosteroids
Methotrexate
Azathioprine
Intravenous Ig (kawaski, early ACNA postive vasculitis)
Polymyalgia rheumatica (demographics, associations and causes)
3/400 people/year, Most patients >50yrs, M:F/1:2
Associated with GCA (occurs in 50% of GCA, 20% of PMR develop GCA)
Can occur paraneoplastically
Clinical features of PMR (4)
Even pts without GCA often have abnormal TA biopsy or shoulder bursitis
Abrupt onset of pain (shoulder or neck mostly) – 1/3 will have systemic symptoms – good response to low dose steroids
Blood tests in PMR (5)
Raised ESR and CRP
Low CD8 cells and cortisol, high IL-6, Negative autoantibody profile
Can have abnormal LFTs or TFTs
Treatment and prognosis of PMR
15mg/day pred for 1/12 – down by 2.5mg to 10mg/day – can use depot. Azathioprine or methotrexate can be used as steroids sparing agents (recently biologics)
Bone protection required
Usually resolves in 2-4yrs (more commonly longer if PMR+GCA)
Use of ANA screen
Essentially it means you have SLE
Treatment for PAN
Control BP
Corticosteroids and cyclophosphamide
Treat Hep B after steroid course
