Vasulitis Flashcards

1
Q

Definition of Vasculitis

A

A disease of blood vessels characterised by inflammation of the vessel wall

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2
Q

HIV vasculitis

A

A large variety of vasculitidies has been described in HIV involving all types of vessel

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3
Q

Causes of cutaneous vasculitis (4)

A

HIV
HCV (cryoglobulinaemia)
Drugs
Other

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4
Q

Takayasu’s arteritis

A

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

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5
Q

Erythema elevatum diutinum

A

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

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6
Q

Behcet’s disease

A

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)

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7
Q

Classification of blood vessels

A

Large – aorta & major branches (Giant cell/Takayasu’s arteritis)
Medium – main visceral arteries (Polyarteritis nodosa or kawaski disease)
Small – venules, capillaries & arterioles (many)

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8
Q

Polyarteritis nodosa (PAN)

A

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)

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9
Q

Vasculitidies of the small vessels (6)

A

Cutaneous leukocytoclastic angiitis. Essential cryoglobulinaemia
Microscopic poylangitis (PGA) Wegener’s/granulomatosis+PA (GPA)
Churg-strauss syndrome (CSS) HSP

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10
Q

Churg-strauss syndrome (CSS) or Eosinophilic granulomatosis with polyangitis (EGPA)

A

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

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11
Q

Wegener’s granulomatosis/granulomatosis+polyangitis (GPA)

A

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

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12
Q

Microscopic polyangitis (PGA)

A

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

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13
Q

Essential cryoglobulinaemia

A

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

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14
Q

Symptoms of Large vessel vasculitis (4)

A

Visual symptoms
Dizziness and syncope
Arm claudication
Inflammation or aneurysm of the vessel

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15
Q

Symptoms of medium vessel vasculitis (5)

A
Angina or MIs (coronary artery)
Kidney or bowel infarction
Stroke
Thrombus formation
vessel wall oedema
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16
Q

Symptoms of small vessel vasculitis (4)

A

Glomerulonephritis
Cutaneous lesions (purpura or petechiae)
Peripheral neuropathy
Local inflammation and blockage

17
Q

Blood tests in vasculitis

A
ESR or CRP, FBC, WCC and Hb
LFT, urinalysis,  CPK, RFT. 
Complement or Cryoglobulins
Blood cultures or serology. 
ANA (anti-nuclear antibody) or ANCA
18
Q

ANCA

A

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

19
Q

Sub components of ANA screen

A

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)

20
Q

pANCA

A

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

21
Q

cANCA

A

Principally due to antibodies against proteinase 3 and best associated with GPA – low sensitivity but very specific

22
Q

Cutaneous Polyarteritis nodosa (CPAN)

A

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

23
Q

Pathergy reaction

A

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)

24
Q

Henoch-schonlein purpura (HSP)

A

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

25
Q

Giant cell arteritis (GCA) (also temporal arteritis or Horton’s disease)

A

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)

26
Q

Causes of GCA

A

Arguments for environmental agents and even an infective cause (Chlamydia, parovirus B19, RSV)

27
Q

Symptoms of GCA

A

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

28
Q

Investigations in GCA

A
Raised ESR/CRP, 
Raised platelets
Raised alk phos
Nomochromic anaemia
Temporal artery biopsy is gold standard (beware skip lesions)
29
Q

ACR criteria for GCA

A

Three or more of:
Over 50 at onset New headache
Temporal artery abnormality Elevated ESR
Abnormal artery biopsy

30
Q

Treatments of GCA

A

40-60mg daily predisolone for 8 weeks –> drop by 5mg every 3-4wks to 10mg –> drop by 1mg per month
Give PPI and bisphosphonate

31
Q

Medications for the treatment of vasculitis (4)

A

Corticosteroids
Methotrexate
Azathioprine
Intravenous Ig (kawaski, early ACNA postive vasculitis)

32
Q

Polymyalgia rheumatica (demographics, associations and causes)

A

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

33
Q

Clinical features of PMR (4)

A

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

34
Q

Blood tests in PMR (5)

A

Raised ESR and CRP
Low CD8 cells and cortisol, high IL-6, Negative autoantibody profile
Can have abnormal LFTs or TFTs

35
Q

Treatment and prognosis of PMR

A

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)

36
Q

Use of ANA screen

A

Essentially it means you have SLE

37
Q

Treatment for PAN

A

Control BP
Corticosteroids and cyclophosphamide
Treat Hep B after steroid course