Vasculitis

Question 1

Definition

Answer 1

A group of immune-mediated disorders that cause damage to blood vessels through an inflammatory process

Question 2

Presenting features of CNS vasculitis

Answer 2

Systemically unwell patient

Headache/stroke like episodes

peripheral nerve involvement (1/3rd)

1/4 additional organ involvement - commonly kidneys

sometimes seizures

Question 3

Presenting features of neurological manifestations of CNS vasculitis

Answer 3

Stroke-like episodes with focal neurology

plus confusion, agitation, seizures, decreased level of consciousness or asymmetric rapidly progressive painful neuropathy (MMX)

Question 4

Commonest CNS vasculitis

Answer 4

Giant cell arteritis (temporal arteritis)

Symptoms: fever, generally unwell, weight loss, stiff muscles, persisting bi-temporal headache, jaw claudication, jaw pain, ear pain, double vision, tenderness of superficial temporal artery

If not treated - early risk of stroke

In patients only > 50

Treat with steroids - prednisolone

VZV - 74% with previous infection, may trigger inflammatory cascade

May coexist with polymyalgia rhematica

Pathological mechanisms - dendritic cells in the vessel wall recruit T cells and macrophages to form granulomatous infiltrates. T helper 17 cells play a critical role

Question 5

Wegner’s granulomatosis with polyangitis

Answer 5

Small and medium sized cells

commonly upper respiratory tract and kidneys

typical signs: varing - rhinitis usually first, nose bleeds, stuffy nose, crustiness of nasal secretions, inflamed uveal layer of the eye, can affect other organs too

granulomas - structure formed in inflammation, collection of macrophages. when immune system attempts to fight off foreign substances but can’t, associated with necrosis

polyangitis - inflammation of multiple blood vessels

Cause unknown

ANCA antibodies responsible for the inflammation (P-ANCA and C-ANCA)

C-ANCA (proteinase 3) - enzyme in neutrophil granylocytes

Question 6

Vasculitis secondary to connective tissue disease

Answer 6

Either small, medium or large vessels

poor prognosis

any organ system can be involved so presentation varies

examples - rhematoid arthitis

Question 7

Churg-Strauss syndrome

Answer 7

small and medium sized vessels

in a person with a history of airway allergic hypersensitivity

3 stages 1. asthma/allergic rhinitis, 2. abnormally high eosinophils - tissue damage to lungs and digestive tract 3. vasculitis - cell death

Question 8

Behcet’s disease

Answer 8

Affects multiple parts of the body: mouth sores, genital sores, inflamed eye, arthiritis, inflamed brain and spinal cord, blood clots, aneurysms or blindness

CNS involvement typically as a chronic meningoencephalitis

Leisons in BG, WM and resemble MS

Neuro invovlement - aseptic menigitis, vascular thrombosis, confusion, seziures, memory loss, sudden hearing loss