Vasculitis

Question 1

What is vasculitis?

Answer 1

Vasculitis = inflammation of the vessel wall characterised by 2 main features:

i. size of the blood vessels involved:

=> large vessel vasculitis i.e. aorta and major tributaries

=> medium vessel vasculitis i.e. medium and small sized arteries and artieroles

=> small vessel vasculitis i.e. small arteries, arterioles, venules and capillaries

ii. presence/absence of anti-neutrophil cytoplasmic antibodies (ANCA)

Question 2

Large vessel vasculitis

What is polymyalgia rheumatica?

What are the presenting features?

Answer 2

1. Sudden onset of severe pain and stiffness of the shoulders and neck, hips and lumbar spine (limb girdle pattern)

=> worse in the morning

=> lasts 30mins to several hours

2. Inflammation of peripheral joints in 25%
3. Systemic features i.e. tiredness, fever, weight, depression and nocturnal sweats in 1/3 patients
4. > 50yrs old
   * clinical hx is diagnostic

Question 3

Large vessel vasculitis

What are the investigations for polymyalgia rheumatica (PMR)?

Answer 3

1. Raised ESR and/or CRP (hallmark of this PMR - rare to see without an acute phase response)
2. Serum alkaline phosphatase and gamma-glutamyl-transpeptidase
   => may be raised as markers of inflammation
3. Anaemia
   => mild normochromic / normocytic
4. Temporal artery biopsy
   => giant cell arteritis in 10-30% (only performed if GCA suspected)

Question 4

Large vessel vasculitis

What is giant cell arteritis?

What are the clinical signs?

Answer 4

Inflammatory granulomatous arteritis of large cerebral arteries - occurs in assoc. with polymyalgia rheumatica affecting those >50yrs

Presenting symptoms:

1. Severe headaches, tenderness of the scalp or temple (combing hair is painful)
2. Claudication of jaw when eating + tenderness & swelling one or more temporal or occipital arteries. Facial pain on facial, maxillary and lingual inflammation

Most feared manisfestation:
3. Sudden, painless temporary or permanent loss of vision in one eye due to involvement of ophthalmic artery

=> amaurosis fugax may precede permanent blindness

4. Systemic manifestation = severe malaise, tiredness and fever

Question 5

Large vessel vasculitis

What is the investigation for giant cell arteritis?

Answer 5

1. Normochromic, normocytic anaemia
2. ESR raised ; very high CRP
3. Abnormal LFT ; low albumin => as in PMR
4. Temporal artery biopsy from the affected side (taken before steroids)
5. Ultrasound scanning of temporal arteries
6. Histological features of GCA:
   => cellular infiltrates of CD4+ T lymphocytes, macrophages and giant cells in the vessel wall

=> granulomatous inflammation of the intima and media

=> Breaking up of the internal elastic lamina

=> Giant cells, lymphocytes, plasma cells in the internal elastic lamina

Question 6

Large vessel vasculitis

What is the management of polymyalgia rheumatica / giant cell arteritis?

Answer 6

1. Corticosteroids = massive reduction in inflammation within 24-48h

=> corticosteroids reduce risk of GCA in patients with PMR

*NSAIDs not affective - should not be used

=> corticosteroids in GCA mandatory - sig. reduce risk of irreversible visual loss

=> if GCA is suspected treatment should not be delayed esp. if there has been visual episodes / stroke - don’t wait for ultrasound/temporal artery biopsy

PMR : 15mg prednisolone as single dose in the morning

GCA : 60-100 mg prednisolone

2. Calcium + vitamins D supplements and sometimes bisphosphonates to prevent osteoporosis

Question 7

Large vessel vasculitis

What is takayasu’s arteritis?

Answer 7

Granulomatous inflammation of the aorta - very rare except Japan

Question 8

Medium-sized vessel vasculitis

What is polyarteritis nodosa?

Answer 8

Polyarteritis nodosa - very rare ; occurs in middle aged men

Fibrinoid necrosis of vessel walls with micro-aneurysm formation, thrombosis and infarction

Question 9

Medium-sized vessel vasculitis

What are the clinical features of polyarteritis nodosa?

Answer 9

Systemic features: fever, malaise, weight loss and myalgia

Acute features due to organ infarction:

1. Neurological: mononeuritis
2. Abdominal: arterial involvement of the abdominal viscera => mimics acute cholecystitis, pancreatitis, appendicitis

=> gastrointestinal haemorrhage occurs due to mucosal ulceration

3. Renal: haematuria and proteinuria. Hypertension and acute/chronic kidney disease
4. Cardiac: coronary arteritis causes MI, heart failure and pericarditis
5. Skin: subcutaneous haemorrhage and gangrene.
   => persistent livedo reticularis in chronic disease
   => cutaneous & subcutaneous palpable nodules

Question 10

Medium-sized vessel vasculitis

What is the investigation and management of polyarteritis nodosa?

Answer 10

Blood count: anaemia, leucocytosis, raised ESR

Biopsy

Angiography: microaneurysm in hepatic, intestinal or renal vessels

If needed: ECG, abnormal ultrasound,

Treatment: corticosteroids with immunosuppressants i.e. azathioprine

Question 11

Medium-sized vessel vasculitis

What is Kawasaki disease?

Answer 11

Acute systemic vasculitis involving medium-sized vessels

Affects mainly children <5yrs

Common in Japan

Infective trigger?

Question 12

Medium-sized vessel vasculitis

What are the clinical features of Kawasaki’s disease?

Answer 12

Fever >4days

Bilateral conjunctival congestion

Dry red lips & oral cavities

Cervical lymphadenopathy

Rash & redness of palms and soles

CVS changes in acute stage = pancarditis and coronary arteritis => aneurysms or dilation visible on echo, MRI or angiography

Question 13

Medium-sized vessel vasculitis

What is the management for Kawasaki’s disease?

Answer 13

Single dose of high-dose IV immunoglobulin => prevents coronary artery disease

After acute phase => aspirin 200-300mg daily

Question 14

Small vessel vasculitis

What are the 2 categories of small vessel disease?

Answer 14

1. ANCA-associated vasculitis including:

=> granulomatosis with polyangiitis

=> eosinophilic granulomatosis with polyangiitis

=> microscopic polyangiitis

2. Immune complex (ANCA negative) small vessel vasculitis with vessel wall immunoglobulin including:

=> anti-glomerular basement membrane (anti-GBM) disease

=> IgA vasculitis i.e. Henoch-Schönlein)

=> cutaneous leucocytoclastic vasculitis

Question 15

Small vessel vasculitis

What is cutaneous leucocytoclastic vasculitis?

Answer 15

Small vessel vasculitis only affecting skin characterised by:

Signs:
=> palpable purpuric lesion involving dermal post-capillary venules e.g. on legs

=> nodules, ulcers, livedo reticularis

=> may be accompanied by arthralgia and glomerulonephritis

Causes:
=> drugs i.e. penicillin, sulphonamides, thiazides

=> idiopathic

=> neoplasia

=> systemic vasculitis i.e. polyarteritis nodosa, Henoch-Schonlein purpura (vasculitic rash on legs/buttocks ± arthralgia, abdo pain & glomerulonephritis)

=> Wegener’s granulomatosis

Question 16

Small vessel vasculitis

What is the management of small vessel vasculitis?

Answer 16

Depends on organs involved

Vasculitis confined to skin may not require systemic treatment

Major organ involvement = high dose corticosteroids, immunosuppression and sometimes plasma exchange