Vasculitis

Question 1

Vasculitis : Definition

Answer 1

Normally due to autoimmune disease which causes inflammation of the blood vessels

Question 2

Vaculitis : Pathophysiology

Answer 2

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.

Question 3

Large cell vasculitis : Types

Answer 3

1. Temporal artery vasculitis

2 Takayasu arteritis

Question 4

Temporal artery vasculitis : Risk factors

Answer 4

1. aged > 50 years
2. Association between temporal arteritis and polymyalgia

Question 5

Temporal artery vasculitis : Pathophysiology

Answer 5

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

Question 6

Temporal artery vasculitis : Clinical features

Answer 6

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

Question 7

Temporal artery vasculitis : Diagnosis

Answer 7

1. Blood tests : Raised inflammatory markers (Raised ESR and CRP)
2. Temporal artery biopsy - skip lesion may be present

Question 8

Temporal artery vasculitis : Management

Answer 8

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

Question 9

Takayasu arteritis : incidence

Answer 9

Rare condition in Asian females < 40 years of ages

Question 10

Takayasu arteritis : Clinical features

Answer 10

1. BP asymmetry on L compared to right, reduced peripheral pulses and limb claudication
2. Affects the aorta - turbulent blood blow and bruits of any involved arteries

Question 11

Takayasu arteritis : Investigations

Answer 11

CT angio- skip lesions where there is stenosis or aneurysms

Question 12

Medium vessel vasculitis : Types

Answer 12

1 . Polyarteritis nodosa

2 . Kawasaki’s disease

Question 13

Polyarteritis nodosa : Definition

Answer 13

1. vasculitis affecting medium-sized arteries with necrotizing inflammation leading to aneurysm formation.
2. multisystem disease affects any side of the body

Question 14

Polyarteritis nodosa : Risk factors

Answer 14

PAN is more common in middle-aged men and is associated with hepatitis B infection.

Question 15

Polyarteritis nodosa : Clinical features

Answer 15

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

Question 16

Polyarteritis nodosa : Diagnosis

Answer 16

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.

Question 17

Kawasaki’s disease : Definition

Answer 17

systemic, medium-sized vessel vasculitis.

It affects young children, typically under 5 years. There is no clear cause or trigger.

Question 18

Kawasaki’s disease : Clinical features

Answer 18

CRASH + burn :
1. conjunctivitis,
1. Rash,
1. lymphAdenopathy,
1. strawberry tongue,
1. Hands and feet oedema alongside fever

Complication : Coronary artery aneurysm

Question 19

Small vessel vasculitis : Types

Answer 19

1 . Granulomatosis with polyangitis - Wegener’s disease

2 . Eosiophilic granulomatosis with polyangitis

3 . Microscopic polyangitis

Question 20

Granulomatosis with polyangitis : Definition

Answer 20

1. 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

Question 21

Granulomatosis with polyangitis : Clinical features

Answer 21

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

Question 22

Granulomatosis with polyangitis : Investigations

Answer 22

1. Bloods
   * Normocytic anaemia, * Thrombocytosis
   * U+E may be deranged 2nd to renal involvement and raised inflammatory factors),
2. cANCA - most common in Wegener’s disease
3. CXR may show fibrotic lesions

Question 23

Eosiophilic granulomatosis with polyangitis - CF and Ix

Answer 23

1. 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

Question 24

Microscopic polyangitis : Definition

Answer 24

no granulomatous inflammation, instead is a type of cryoglbuliminemic vasculitis

Question 25

Microscopic polyangitis : Pathophysiology

Answer 25

1. Caused by cryoglobulins which are seem proteins that precipitate out in cold temperatures
2. Cryoglobulin proteins form immune complexed with complement proteins and deposit in walls of small vessels
3. Assoc with - Hepatitis c, Hepatitis B and HIV,

Question 26

Microscopic polyangitis : Clinical features

Answer 26

Clinical features - Purpura, joint pain and muscle pain

Question 27

Microscopic polyangitis : Investigations

Answer 27

Ix :
* Bloods show c-Anca and pAnca positive, low levels of complement factors due to complex formation and tissue deposition.

• Biopsy of skin lesion - shows precipitated cryoglobulins