Diseases Of The Vessel Wall

VASCULITIS AND COLLAGEN VASCULAR DISEASES (contd.) ALLERGIC ANGIITIS (Hypersensitivity vasculitis)

Intercurrent illnesses (infection or neoplasia) trigger immune complex deposition on basement membranes of capillaries and venules. Systemic symptoms - rash, fever and arthralgia are associated with multiorgan involvement. Neurological features - neuropathy, stroke-like syndromes - occur in 30% of patients. Investigations suggest systemic upset - elevated ESR, anaemia, leukopaenia. Skin biopsy confirms peri-venular inflammation. Treatment of underlying infection and steroids produce rapid improvement.

TAKAYASU'S (PULSELESS) DISEASE

A giant cell arteritis involving the aorta and its major branches. Predominantly affects Asian females in third or fourth decades.

Symptoms:

- Non-specific - fever, arthralgias and myalgia

- Vascular - myocardial ischaemia, peripheral vascular disease

- Neurological vascular TIAS (including subclavian steal), strokes and dementia.

Diagnosis:

Steroids are useful initially. The role of surgical reconstruction of occluded vessels is uncertain

ISOLATED ANGIITIS OF CENTRAL NERVOUS SYSTEM

Systemic symptoms and laboratory evidence of generalised vasculitis are absent. Presentation with headaches/seizures, encephalopathy and stroke

Diagnosis:

Condition should be borne in mind in atypical stroke

- CSF shows lymphocytes

- MRI, multiple ischaemic changes

- Angiography, beading (multiple narrow segments) on intracranial arteries

- Meningeal biopsy.

Treatment:

Prognosis often dismal. Steroids and cyclophosphamide may produce remission.

GIANT CELL ARTERITIS (see page 69) CHURG STRAUSS ANGIITIS

A distinctive syndrome of eosinophilia, pulmonary infiltrates, neuropathy and encephalopathy or stroke. Related to polyarteritis nodesa, steroid responsive. Other immunosuppressants e.g. cyclophosphamide in resistant cases.

GRANULOMATOUS VASCULITIS/WEGENERS GRANULOMATOSIS

A rare disorder, most frequent in males aged 20-50 years.

Upper or lower respiratory tract granuloma is associated with glomerulonephritis Small arteries and capillaries are affected

Upper or lower respiratory tract granuloma is associated with glomerulonephritis Small arteries and capillaries are affected

Neurological involvement

- direct granulomatous invasion of skull base (cranial nerve palsies, visual failure from chiasmal compression)

- Stroke-like symptoms from vasculitis.

Diagnosis:

- Elevated ESR and C-reactive protein (CRP)

- Elevated immunoglobulins

- Impaired renal function

- Radiological findings: Chest and sinuses: granuloma mass

MRI (cranium): granuloma mass or vasculitis.

Treatment:

- Immunosuppression: steroids and cyclophosphamide

- Surgical decompression of granulomas occasionally required.

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