Usually affects patients aged over 55 years in age and is more common in women. It usually involves the extracranial arteries of the head, particularly the temporal and ophthalmic arteries, but it may involve the carotid, vertebral, and coronary arteries, and the aorta. Symptoms and signs relate to the territory of supply of the affected vessel; visual problems, including blindness, are particularly important. It is often part of a more generalised syndrome, including polymyalgia rheumatica, fever, weight loss, arthralgia and increased ESR.
Histopathological abnormalities are often focal and include thickening of the intima with focal mucoid degeneration and fragmentation of the internal elastica. The inflammatory infiltrate includes lymphocytes, plasma cells and multinucleate giant cells (Figure 14). In the late stages the vessel wall becomes fibrotic and occasionally an aneurysm may form.
| Figure 14.|
Giant cell arteritis (temporal arteritis) showing infiltration of the wall of the temporal artery by a mixture of inflammatory cell types, including multinucleate giant cells.
Most presents in adults and presents with headaches, encephalopathy and focal neurological signs. Angiography shows narrowed segments in cerebral arteries.
Mainly affects arteries but also affects veins. Inflammation is granulomatous in type, with lymphocytes, epithelioid cells and giant cells (Figure 15).
| Figure 15.|
Granulomatous angiitis. The inflammatory infiltrate is granulomatous in type and includes epithelioid cells and giant cells.
This is most common in Oriental races and usually in relatively young patients (15-45). Involves mainly the arch of the aorta and its main branches. Inflammation is granulomatous in type, affecting mainly the tunica media, causing destruction of the elastic lamina and the formation of multinucleate giant cells, with fibrosis in the late stages.
Approximately half of the patients with this syndrome develop neurological signs and symptoms and intracranial pathology is the main cause of death in about one fifth of cases. Pathological abnormalities include fibrinoid necrosis, deposition of immunoglobulins and complement in the vessel wall, inflammatory infiltrates in the wall and fibrous thickening. Patients may have circulating antiphospholipid antibodies which are associated with an increased incidence of thrombosis.
This is a chronic disease with fluctuating course and more common in men. It is associated with necrotising lesions in medium-sized and small arteries with a predilection for the branching points of vessels. It is regarded as an immune complex-mediated vasculitis associated with a variety of antigens including micro-organisms, drugs and autoantigens. It occurs in generalised and limited forms and nerve and muscle are involved in up to 80% of cases. Necrosis may affect the entire thickness of the vessel wall and the inflammatory infiltrate includes numerous polymorphs. Weakening of the wall may result in aneurysmal dilatation.
This is a chronic disease characterised by necritising lesions within the respiratory tract, frequently with systemic vasculitis that may also involve the cranial circulation. It is associated with anti-neutrophil cytoplasmic antibodies against proteinase 3. Neurological symptoms occur in about one third of cases.
Sjgren's syndrome is an autoimmune disorder with multiple abnormalities of cellular and humoral immunity with involvement of salivary and lacrimal glands. It is associated with vasculitis in a minority of cases, mainly affecting veins, but sometimes the picture can closely mimic polyarteritis nodosa. Involvement of the peripheral nervous system is well documented but there is some variation in figures quoted for involvement of the central nervous system.
Behet's syndrome is a rare vasculitic disorder affecting multiple organs with ulcers of the oral and genital mucous membranes, uveitis, and arthritis. The CNS is involved in about one third of cases.
There is an increased incidence of stroke amongst drug abusers and some of these, particularly in the case of cocaine, are associated with vasculitis. Cerebral haemorrhage is associated with cocaine, phenylpropanolamine and amphetamine abuse.