Seminar G Vasculitides Flashcards Preview

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Flashcards in Seminar G Vasculitides Deck (36):
1

Definition

multisystem inflammatory diseases that are characterised by inflammation of blood vessel walls (=vasculitis).

2

Classification

The vasculitides represent an extremely heterogeneous group, but classification is generally by size of vessel wall affected. There have been many attempts at classification over the years, and none is entirely satisfactory.

3

Think of vasculitis when patient presents with

• unexplained pyrexia with normal cultures
• high inflammatory response
• unexplained weight loss
• unusual rashes (especially ulceration and "palpable purpura")
• new onset renal disease (especially microscopic haematuria)
• widespread musculoskeletal symptoms
• mouth ulcers, abdominal pain, bloody diarrhoea
• dyspnoea, haemoptysis
• sensory or motor loss

4

Large arteries




disease

Giant cell (temporal) arteritis
Takayasu’s arteritis

5

Medium Vessels disease

Polyarteritis nodosa
Kawasaki disease

6

Small Vessel disease

Wegener’s granulomatosis
Chur-Struss syndrome
Microscopic polyangiitis
Henoch-Schonlein purpura
Cutaneous leukocytoclastic angiitis

7

Arteries and veins disease

Behcet’s Syndrome
Secondary Vasculitis

8

Giant cell (temporal) arteritis



A granulomatous arteritis of the aorta and its major branches, with a predeliction for the extracranial branches of the carotid. A disease of the elderly, it is often associated with polymyalgia rheumatica

9

Takayasu’s arteritis

A granulomatous arteritis of the aorta and its major branches. Usually occurs under age 50.

10

Polyarteritis nodosa

Necrotizing inflammation of medium- or small-sized arteries, leading to aneurysm formation and organ infarction. Glomerulonephritis is usually absent.

11

Kawasaki disease

Usually affects infants and young children. Acute vasculitis of large-, medium- and small-sized arteries. Often affects coronary arteries. Also known as mucocutaneous lymph node syndrome - conjunctival injection, desquamation of hands and feet, cervical lymphadenopathy, oropharyngeal changes.

12

Wegener’s granulomatosis

Necrotizing granuloma of upper and lower respiratory tract, necrotizing vasculitis of capillaries, venules, arterioles and arteries. Necrotizing glomerulonephritis is common. Strongly associated with antineutrophil cytoplasmic antibodies (ANCA).

13

Chur-Struss syndrome

Granulomatous inflammation of respiratory tract, with necrotizing vasculitis of small- to medium-sized vessels. Associated with asthma. Eosinophilia a prominent feature. Strongly associated with ANCA.

14

Microscopic polyangiitis

Necrotizing vasculitis of capillaries, venules, and arterioles. Necrotizing glomerulonephritis is very common. Pulmonary capillaritis (with haemorrhage) often occurs. Strongly associated with ANCA.

15

Henoch-Schonlein purpura

Vasculitis of small vessels (capillaries, venules, arterioles) with IgA-dominant immune deposits. Typically involves skin ("Palpable purpura"), gut (abdominal pain, bloody diarrhoea), and glomeruli (microscopic haematuria), and is associated with arthritis or arthralgias.

16

Cutaneous leukocytoclastic angiitis

Isolated cutaneous small-vessel vasculitis, associated with immune-complex deposition. "Palpable purpura" usually seen. Systemic features absent. Often caused by drug hypersensitivity or malignancy.

17

Behcet’s Syndrome

A systemic vasculitis affecting virtually any vessel type. Oral and genital ulceration, and eye lesions (uveitis and retinal vasculitis) are characteristic. Arthritis, rashes, and internal organ involvement also occur.

18

Secondary Vasculitis

Vasculitis may occur secondary to other rheumatic disease. The most common examples are rheumatoid arthritis and systemic lupus erythematosus.

19

Differential Diagnosis →

• Infective endocarditis
• Atrial myxoma
• Antiphospholipid antibody syndrome
• Acquired Immune Deficiency Syndrome
• Sarcoidosis
• Secondary syphilis

20

Giant-cell (temporal) arteritis (GCA)

is a granulomatous arteritis of the aorta and large blood vessels.

21

Giant-cell (temporal) arteritis (GCA) Clinical Features

Severe headaches
Scalp tenderness
Visual disturbances
Jaw claudication

22

Giant-cell (temporal) arteritis (GCA) Investigations show

An acute inflammatory response with a very high ESR

23

Giant-cell (temporal) arteritis (GCA) Temporal artery biopsy shows

Disruption of the elastic lamina, monoculear cell infiltrates with formation of giant cells and granulomata

24

Giant-cell (temporal) arteritis (GCA) Most important complication

Irreversible blindness due to central retinal artery occlusion

25

Giant-cell (temporal) arteritis (GCA) Treatment

Prompt treatment of glucocorticoids (prednisolone 1mg/kg)

26

Polymyalgia Rheumatica (PMR)

• Pain and intractable early morning stiffness around the shoulder and hip girdles, without obvious synovitis in the joints
• Malaise, weight loss and a low-grade fever may also occur.

27

Polymyalgia Rheumatica (PMR) Mean age of onset

70 years (extremely rare under age of 50 yrs)

28

Polymyalgia Rheumatica (PMR) Investigations show

Raised ESR or plasma viscosity

29

Polymyalgia Rheumatica (PMR) Treatment

Prompt treatment of glucocorticoids (prednisolone 1mg/kg)

30

Polymyalgia Rheumatica (PMR) Concomitant treatment with

bisphosphonate

31

Behcet’s Syndrome: Definition

A systemic vasculitis

32

Behcet’s Syndrome: Female to Male:

1:1

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Behcet’s Syndrome:Association with

HLA-B51

34

Behcet’s Syndrome: Clinical Features

Orogenital ulceration
Rashes
Arthritis
Eye involvement
Panuveitis with hypopyon
Retinal vasculitis

35

Behcet’s Syndrome: Pathergy reaction

Characteristics pustules form at the site of simple trauma such as a needle puncture would (28-48hr)

36

Behcet’s Syndrome: Treatment

Colchicine
Glucocorticoids
Azathioprine

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