Vasculitis Flashcards
Vasculitis : Definition
Normally due to autoimmune disease which causes inflammation of the blood vessels
Vaculitis : Pathophysiology
1 . Autoimmune reaction against antigens of blood vessels
* Immune system can begin to attack the antigens of the endothelial layer of the vessels
2 . Endothelium antibodies
* Antibodies produced against damaged endothelium
* Exposes underlying collagen and tissue factor
3 . Increased thrombosis risk
* Underlying tissue factor exposed - increases risk of blood coagulation
* Increases risk of aneurysms forming
4 . Vessel fibrosis
* As vessel heals, fibrin is deposited resulting ins car tissue making it harder and stiffer.
Large cell vasculitis : Types
- Temporal artery vasculitis
2 Takayasu arteritis
Temporal artery vasculitis : Risk factors
- aged > 50 years
- Association between temporal arteritis and polymyalgia
Temporal artery vasculitis : Pathophysiology
Most commonly affected arteries;
* external carotid artery
* ophthalmic artery
* temporal arteries
Temporal artery vasculitis :
* tender, palpable temporal artery and can lead to ischaemia resulting in;
* jaw claudication or pain while masticating,
Ophthalmic artery vasculitis
* occlusion of the posterior ciliary artery - branch of ophthalmic artery leading to ischaemia of the optic nerve ;
* Visual loss
Occulomotor cranial nerve
* diplopia
Temporal artery vasculitis : Clinical features
Requires early recognition and treatment to minimise risk of complications
- Rapid onset < 1 month
- Head ache
- Jaw claudication / pain while masticating
- Visual changes, diplopia or loss of vision
Systemic sx - muscle stiffness and weakness, leathery, fever, night sweats
Temporal artery vasculitis : Diagnosis
- Blood tests : Raised inflammatory markers (Raised ESR and CRP)
- Temporal artery biopsy - skip lesion may be present
Temporal artery vasculitis : Management
Urgent Ophthalmology review
1. High dose prednisolone if suspected - prior to biopsy
2. Evolving visual loss - IV methylprednisolone
3. Bone protection due to steroid use
4. Low dose aspirin - to thin blood, prevent clots
Takayasu arteritis : incidence
Rare condition in Asian females < 40 years of ages
Takayasu arteritis : Clinical features
- BP asymmetry on L compared to right, reduced peripheral pulses and limb claudication
- Affects the aorta - turbulent blood blow and bruits of any involved arteries
Takayasu arteritis : Investigations
CT angio- skip lesions where there is stenosis or aneurysms
Medium vessel vasculitis : Types
1 . Polyarteritis nodosa
2 . Kawasaki’s disease
Polyarteritis nodosa : Definition
- vasculitis affecting medium-sized arteries with necrotizing inflammation leading to aneurysm formation.
- multisystem disease affects any side of the body
Polyarteritis nodosa : Risk factors
PAN is more common in middle-aged men and is associated with hepatitis B infection.
Polyarteritis nodosa : Clinical features
Aneurysm formation compromises blood supply leading to ischaemia
Athralgia, Myalgia
High risk of bleeding
CVS disease - hypertension,
1 . Skin :
* Livedo reticular : lace-like purplish discolouration of the skin, mottled rash which can develop into large ulcers which can get infected
* 2nd to ischaemia and microaneurysm leading to areas of vasocontriction and vasodilation
2 . Peripheral neuropathy : pain, numbness and burning
Mononeuritis multiplex: Peripheral nerve damage affecting multiple nerves, leading to sensory and motor deficits.
* Caused by ischaemia to peripheral nerves
3 . Systemic : * fever, malaise, arthralgia, weight loss
Complications : internal ischaemia, kidney failure, hypertension or MI
Polyarteritis nodosa : Diagnosis
Ix : pANCA antibody +, Hep B serology +
Dx : CT angiography can lead to small aneurysms that look like a string of beads - Rosary sign
Biopsy - shows pattern of inflammation consistent with polyarteritis nodosa.
Kawasaki’s disease : Definition
systemic, medium-sized vessel vasculitis.
It affects young children, typically under 5 years. There is no clear cause or trigger.
Kawasaki’s disease : Clinical features
CRASH + burn :
1. conjunctivitis,
1. Rash,
1. lymphAdenopathy,
1. strawberry tongue,
1. Hands and feet oedema alongside fever
Complication : Coronary artery aneurysm
Small vessel vasculitis : Types
1 . Granulomatosis with polyangitis - Wegener’s disease
2 . Eosiophilic granulomatosis with polyangitis
3 . Microscopic polyangitis
Granulomatosis with polyangitis : Definition
- Systemic autoimmune vasculitis that involves inflammation of blood vessels leading to;
* Formation of granulomas particularly in the respiratory tract (nose, sinuses, lungs), kidneys, and sometimes other organs.
Granulomas are structures that form in response to chronic inflammation. They consist of immune cells, primarily macrophages, which aggregate to wall off and contain an offending agent or persistent inflammation
Granulomatosis with polyangitis : Clinical features
Granulamatous inflammation of blood vessels to ENT area, lungs are kidneys
1 . Eyes : granuloma posterior to eyeball causing painful exophthalmos, diplopia, uveitis/scleritis
2 .Ear : destruction of internal ear can leak to deafness
3 .Nose : ‘Saddle shape deformity’ 2nd to large granuloma that destroys bone structure of nose
4 .Throat : Laryngeal collapse, hoarse voice
5 . Renal : Glomerulonephritis - haematuria and proteinuria
Granulomatosis with polyangitis : Investigations
- Bloods
* Normocytic anaemia, * Thrombocytosis
* U+E may be deranged 2nd to renal involvement and raised inflammatory factors), - cANCA - most common in Wegener’s disease
- CXR may show fibrotic lesions
Eosiophilic granulomatosis with polyangitis - CF and Ix
- Eosiophilic granulomatosis with polyangitis - also causes granulomatous inflammation but with the predominance of eisenophils
2 . Clinical features - similar to Wegener’s however can cause chronic asthma, sinusitis and blood eosinophilia
3 . **Ix **:
* Positive for p- ANCA antibodies
* ECHO as high levels of eosinophils can damage cardiac muscle
Microscopic polyangitis : Definition
no granulomatous inflammation, instead is a type of cryoglbuliminemic vasculitis
