Epidemiology of giant cell arteritis and polymyalgia rheumatica.
TL;DR: Giant cell arteritis and Polymyalgia rheumatica are relatively common diseases in the elderly due to the progressive aging of the population in Western countries.
Abstract: Giant cell arteritis (GCA), also called Horton or granulomatous arteritis, is a largeand medium-sized blood vessel systemic vasculitis characterized by the granulomatous involvement of the aorta and its major branches (1,2). Polymyalgia rheumatica (PMR) is a disease characterized by severe bilateral pain and aching involving the neck, shoulder, and pelvic girdles associated with morning stiffness (1). GCA and PMR mainly occur in white individuals age 50 years. Due to the progressive aging of the population in Western countries, both conditions have emerged as relatively common diseases in the elderly (1,2).
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TL;DR: One in 12 women and 1 in 20 men will develop an inflammatory autoimmune rheumatic disease during their lifetime, which can serve as useful guides in counseling patients regarding their lifetime risk of these conditions and have important implications regarding disease awareness campaigns.
Abstract: Objective Understanding of the personal risks for rheumatoid arthritis (RA) and other rheumatic diseases remains poor, despite advances in knowledge with regard to their pathogenesis, therapeutics, and clinical impact, in part because the personal lifetime risk of developing these diseases is unknown. This study was undertaken to estimate the lifetime risk of RA, as well as other inflammatory autoimmune rheumatic diseases, including systemic lupus erythematosus, psoriatic arthritis, polymyalgia rheumatica (PMR), giant cell arteritis, ankylosing spondylitis, and Sjogren's syndrome, and to provide an overall estimate of the risk of developing inflammatory autoimmune rheumatic disease over a lifetime. Methods Using the incidence rates obtained from our population-based studies of rheumatic diseases among residents of Olmsted County, Minnesota, and mortality rates from life tables for the general population, we estimated the sex-specific lifetime risk of rheumatic disease. Results The lifetime risk of RA developing in US adults was 3.6% for women and 1.7% for men, and the lifetime risk of rheumatoid factor-positive RA was 2.4% for women and 1.1% for men. The second most common inflammatory autoimmune rheumatic disease was PMR, with a lifetime risk of 2.4% for women and 1.7% for men. The overall lifetime risk of inflammatory autoimmune rheumatic disease was 8.4% for women and 5.1% for men. Conclusion One in 12 women and 1 in 20 men will develop an inflammatory autoimmune rheumatic disease during their lifetime. These results can serve as useful guides in counseling patients regarding their lifetime risk of these conditions and have important implications regarding disease awareness campaigns.
419 citations
TL;DR: Neither headache nor visual symptoms developed when the glucocorticoids were tapered, and the diplopia resolved after 6 days of treatment with 60 mg of prednisone daily.
Abstract: A 79-year-old woman presents with new-onset pain in her neck and both shoulders. She takes 7.5 mg of prednisone per day for giant-cell arteritis. Occipital tenderness and diplopia developed 11 months before presentation. At that time, her erythrocyte sedimentation rate was elevated, at 78 mm per hour, and a temporal-artery biopsy revealed granulomatous arteritis. The diplopia resolved after 6 days of treatment with 60 mg of prednisone daily. Neither headache nor visual symptoms developed when the glucocorticoids were tapered. How should this patient’s care be managed?
289 citations
TL;DR: Results of the current study show an increased risk of strokes, in the vertebrobasilar territory in particular, at the time of GCA diagnosis and suggest a potential protective role of anemia against the development of these cerebrovascular complications.
196 citations
Cites background from "Epidemiology of giant cell arteriti..."
...However, the incidence of this vasculitis increases with age and peaks in white individuals aged older than 70 years.(27) Due to the progressive aging of the population in Western countries along with well-characterized genetic components,(12) GCA has became the most common type of systemic vasculitis in North American and European people over the age of 50 years....
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TL;DR: Diagnosis of PMR and GCA is based on clinical features and elevated levels of inflammatory markers; Temporal artery biopsy remains the gold standard to support the diagnosis of GCA; imaging studies are useful to delineate large-vessel involvement in GCA.
Abstract: Polymyalgia rheumatica (PMR) and giant cell arteritis (GCA) are inflammatory diseases that typically affect white individuals >50 years. Women are affected ∼2-3 times more often than men. PMR and GCA occur together more frequently than expected by chance. The main symptoms of PMR are pain and stiffness in the shoulders, and often in the neck and pelvic girdle. Imaging studies reveal inflammation of joints and bursae of the affected areas. GCA is a large-vessel and medium-vessel arteritis predominantly involving the branches of the aortic arch. The typical clinical manifestations of GCA are new headache, jaw claudication and visual loss. PMR and GCA usually remit within 6 months to 2 years from disease onset. Some patients, however, have a relapsing course and might require long-standing treatment. Diagnosis of PMR and GCA is based on clinical features and elevated levels of inflammatory markers. Temporal artery biopsy remains the gold standard to support the diagnosis of GCA; imaging studies are useful to delineate large-vessel involvement in GCA. Glucocorticoids remain the cornerstone of treatment of both PMR and GCA, but patients with GCA require higher doses. Synthetic immunosuppressive drugs also have a role in disease management, whereas the role of biologic agents is currently unclear.
173 citations
University of Kansas1, University of California, San Francisco2, Mayo Clinic3, Cleveland Clinic4, Columbia University5, McMaster University6, Northwestern University7, Emory University8, National Institutes of Health9, Children's Mercy Hospital10, University of Pennsylvania11, Johns Hopkins University12, Harvard University13, Boston Children's Hospital14, Vanderbilt University15, St Mary's Hospital16, University of Utah17, University at Buffalo18, University of South Florida19, American College of Rheumatology20
TL;DR: In this paper, the authors provide evidence-based recommendations and expert guidance for the management of giant cell arteritis (GCA) and Takayasu arteritis as exemplars of large vessel vasculitis.
Abstract: Objective To provide evidence-based recommendations and expert guidance for the management of giant cell arteritis (GCA) and Takayasu arteritis (TAK) as exemplars of large vessel vasculitis. Methods Clinical questions regarding diagnostic testing, treatment, and management were developed in the population, intervention, comparator, and outcome (PICO) format for GCA and TAK (27 for GCA, 27 for TAK). Systematic literature reviews were conducted for each PICO question. The Grading of Recommendations Assessment, Development and Evaluation methodology was used to rate the quality of the evidence. Recommendations were developed by the Voting Panel, comprising adult and pediatric rheumatologists and patients. Each recommendation required ≥70% consensus among the Voting Panel. Results We present 22 recommendations and 2 ungraded position statements for GCA, and 20 recommendations and 1 ungraded position statement for TAK. These recommendations and statements address clinical questions relating to the use of diagnostic testing, including imaging, treatments, and surgical interventions in GCA and TAK. Recommendations for GCA include support for the use of glucocorticoid-sparing immunosuppressive agents and the use of imaging to identify large vessel involvement. Recommendations for TAK include the use of nonglucocorticoid immunosuppressive agents with glucocorticoids as initial therapy. There were only 2 strong recommendations; the remaining recommendations were conditional due to the low quality of evidence available for most PICO questions. Conclusion These recommendations provide guidance regarding the evaluation and management of patients with GCA and TAK, including diagnostic strategies, use of pharmacologic agents, and surgical interventions.
150 citations
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1,188 citations
TL;DR: Increased awareness of large-artery complication in GCA, particularly early-occurring aortic dissection, may decrease associated mortality.
Abstract: Objective
To determine the incidence and predictors of large-artery complication (aortic aneurysm, aortic dissection, and/or large-artery stenosis) in patients with giant cell arteritis (GCA).
Methods
The cohort of all residents of Olmsted County, Minnesota, in whom GCA was diagnosed between January 1, 1950, and December 31, 1999, was followed up. The incidence of aortic aneurysm, aortic dissection, and large-artery stenosis was determined. Possible predictors and correlates of large-artery complication were assessed.
Results
Forty-six incident cases of large-artery complication (representing 27% of the 168 patients in the cohort) were identified. These included 30 incident cases (18%) of aortic aneurysm and/or aortic dissection. Of these cases, 18 (11%) involved the thoracic aorta, with aortic dissection developing in 9 (5%). There were 21 incident cases (13%) of large-artery stenosis. Fifteen patients (9%) had incident cervical artery stenosis, and 6 (4%) had incident subclavian/axillary/brachial artery stenosis. One patient (0.6%) had incident iliac/femoral artery stenosis attributable to GCA. Hyperlipidemia and coronary artery disease were associated with aortic aneurysm and/or dissection (P < 0.05 for both). Cranial symptoms (headache, scalp tenderness, abnormal temporal arteries) were negatively associated with large-artery stenosis (hazard ratio [HR] 0.10 [95% confidence interval (95% CI) 0.03–0.35, P < 0.0005]), as was a higher erythrocyte sedimentation rate (HR 0.80 [95% CI 0.67–0.95, P < 0.05] per 10 mm/hour).
Conclusion
Large-artery complication is common in GCA. Increased awareness of large-artery complication in GCA, particularly early-occurring aortic dissection, may decrease associated mortality.
505 citations
TL;DR: In this article, the authors discuss the prevalence of rheumatic diseases in the elderly, and their spectre largely varies, and some of them are typical of younger patients, having certain different clinical features if present in elderly patients.
Abstract: Rheumatic diseases in the elderly are quite frequent, and their spectre largely varies. Some of them are typical of younger patients, having certain different clinical features if present in the elderly; others occur predominantly in the elderly patients. Typical diseases of the latter group are polymyalgia rheumatica (PMR) and giant cell arteritis (GCA).
431 citations
TL;DR: The present study supports the claim that ocular ischemic complications are still frequent in biopsy-proven GCA patients from southern Europe and suggests a higher inflammatory response may be a protective factor against the development of cranial isChemic events.
382 citations
TL;DR: Advances in the understanding of the immunologic events causing giant-cell arteritis have provided new opportunities for diagnostic testing and therapeutic interventions, and the goal is to develop reliable diagnostic procedures that detect vasculitis in distinct vascular beds.
Abstract: Giant-cell arteritis is an immune-mediated disease characterized by granulomatous infiltrates in the wall of medium-size and large arteries. The immunopathology consists of 2 components. Excessive cytokine production (for example, of interleukin-1 and interleukin-6) induces systemic inflammation with an exuberant acute-phase response. In parallel, interferon-gamma, which is released by T cells captured in the arterial wall, activates tissue-injurious macrophages. In response to the immune injury, the artery generates hyperplasia of the intima that leads to luminal occlusion and subsequent tissue ischemia. Despite the systemic character of the disease, distinct vascular territories are preferentially affected. On the basis of the predominant involvement, clinical subtypes can be distinguished: cranial giant-cell arteritis with ischemic complications in the eye, the face, and the central nervous system; large-vessel giant-cell arteritis with occlusions in the subclavian or axillary vessels; aortic giant-cell arteritis; giant-cell arteritis presenting as an intense systemic inflammatory syndrome with nonstenosing vasculitis; and "isolated" polymyalgia rheumatica with myalgias, systemic inflammation, and subclinical vasculitis. Temporal artery biopsy remains the diagnostic procedure of choice to detect arteritis in cranial vessels. In other vascular territories, giant-cell arteritis is most commonly diagnosed by vascular imaging. Laboratory studies characteristically document the marked elevations of nonspecific acute-phase reactants, such as C-reactive protein and erythrocyte sedimentation rate. Cytokines, such as interleukin-6, that induce the acute-phase reaction are currently being explored as more sensitive biological markers of disease activity. Corticosteroids are highly effective in suppressing systemic inflammation, but they do not eliminate the immune responses in the vessel wall. In general, the clinical outcome of giant-cell arteritis is excellent, and efforts must now concentrate on tailoring therapies to the needs of the individual patient.
361 citations