Delays in recognition and management of giant cell arteritis: results from a retrospective audit.

Prompt institution of corticosteroids (CS) can prevent devastating neuro-ophthalmic complications (NOC) in patients with giant cell arteritis (GCA). Guidelines on managing GCA place emphasis on early recognition of symptoms and prompt treatment of the disease where there is a high index of clinical suspicion. The aims of this study are to review the clinical findings in patients with GCA, evaluate the baseline practice in diagnosis and treatment and to identify delays in treating patients with NOC. The study utilised retrospective case notes review of patients diagnosed with GCA between 2003 and 2008. Sixty-five patients were identified (47 females, 18 males, mean age, 75 years). A significant minority presented with constitutional, polymyalgic and ischaemic symptoms. Mean time from symptom onset to diagnosis of GCA was 35 days. CS were not delayed in those diagnosed with GCA. Recognition of ischaemic symptoms was slow. Visual loss at presentation occurred in 16 patients (24.6%). Ten patients (15.4%) presented with NOC in the absence of headache, seven (70%) of whom developed permanent visual impairment. Five (7.7%) patients had cerebrovascular complications. There are major delays in the recognition and treatment of GCA. There is a high incidence of irreversible ischaemic complications which may partly result from diagnostic and treatment delay.

Current understanding and management of giant cell arteritis and polymyalgia rheumatica.

Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) are linked conditions that occur in the elderly. GCA is a vasculitis of large- and medium-sized vessels causing critical ischemia. It is a medical emergency owing to the high incidence of neuro-ophthalmic complications. PMR is an inflammatory disease characterized by abrupt-onset pain and stiffness of the shoulder and pelvic girdle muscles. Both conditions are associated with a systemic inflammatory response and constitutional symptoms. The pathogeneses are unclear. The initiating step may be the recognition of an infectious agent by activated dendritic cells. The key cell type involved is CD4(+) T cells and the key cytokines are IFN-γ (implicated in granuloma formation) and IL-6 (key to the systemic response). The pathogenesis of PMR may be similar to that of GCA, however, PMR exhibits less clinical vascular involvement. The mainstay of therapy is corticosteroids, and disease-modifying therapy is indicated in relapsing disease. This article reviews recent guidelines on early recognition, investigations and management of these diseases, as well as advances in imaging.

Peripheral arterial disease in polymyalgia rheumatica.

Patients with polymyalgia rheumatica have been shown to have an increased risk of peripheral arterial disease on longitudinal follow-up. Possible explanations for this include premature atherosclerosis related to chronic inflammation, as with other inflammatory rheumatological conditions. Alternatively, polymyalgia rheumatica can be associated with vasculitis, even in the absence of clinical giant cell arteritis, and peripheral vascular disease may represent subclinical vasculitis. Further work is required to elucidate the reasons for this increased risk. Currently, it would remain reasonable to aggressively control modifiable atherosclerotic risk factors.

Treatment and outcomes of large vessel arteritis.

Giant cell arteritis (GCA) and Takayasu's arteritis (TA) are characterised by systemic inflammation and critical ischaemia. GCA is a medical emergency. Neuro-ophthalmic complications occur early, with permanent vision loss in up to a fifth of patients, resulting mainly from failure of prompt recognition and treatment. Diagnosis of large vessel vasculitis is often delayed due to poor recognition of early, often non-specific symptoms. Laboratory inflammatory markers are often discordant with disease activity. Modern imaging techniques show promise in diagnosis and disease monitoring, improving our understanding of major artery involvement in large vessel vasculitis. However, in practice, their role is still unclear. The mainstay of therapy remains corticosteroids. Experience using conventional disease-modifying drugs is mixed, and biological therapies require further evaluation for their steroid-sparing potential.

Neuro-ophthalmic complications in giant cell arteritis.

Giant cell arteritis (GCA) is a medical emergency characterized by systemic inflammation and critical ischemia. Neuro-ophthalmic complications occur early, with permanent vision loss in up to one fifth of patients. This mainly results from failure of prompt recognition and treatment. Diagnosis of GCA is often preceded by unrecognized symptoms, including constitutional upset and jaw claudication. Features predictive of permanent visual loss include jaw claudication and temporal artery abnormalities on physical examination. These patients often do not mount high inflammatory responses. Modern imaging techniques show diagnostic promise, and have led to an increased recognition of major artery involvement in GCA. However, temporal artery biopsy remains the gold standard for investigation. Intimal hyperplasia on histologic examination is associated with neuro-ophthalmic complications. The mainstay of therapy remains corticosteroids. Experience using conventional disease-modifying drugs has been mixed, and biologic therapies require further evaluation for their steroid-sparing potential.

Syndromes and complications of interferon therapy.

PURPOSE OF REVIEW: Interferons are used to treat a variety of medical conditions. They are integral players in immunity and a number of immune-mediated complications can arise during interferon therapy. We have reviewed the occurrence of these complications, and the mechanisms behind them. RECENT FINDINGS: Case reports and follow-up studies of large cohorts of patients on interferon therapy have confirmed that immune-mediated complications are uncommon but can occur in a number of different organ systems. IFNalpha production is induced by specific autoantibody-nuclear antigen immune complexes, and has a key role in the development and maintenance of autoimmunity in systemic lupus erythematosus. SUMMARY: Interferon therapy can precipitate immune-mediated abnormalities de novo or can exacerbate an existing autoimmune tendency. This is manifest in the rise in titre of existing antibodies and in the development of clinical disease in patients with preexisting antibodies. Type I interferons have a key role in the development of systemic lupus erythematosus.