Sonography in giant-cell arteritis.
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Giant Cell Arteritis↗
Biomedical subjects
G G Hunder
Publications and source records attributed to G G Hunder.
Adverse outcomes of antiinflammatory therapy among patients with polymyalgia rheumatica.
OBJECTIVE: To evaluate the incidence and risks of adverse events associated with therapy (both corticosteroids [CS] and nonsteroidal antiinflammatory drugs [NSAIDs]) among a previously identified, population-based cohort of patients first diagnosed with polymyalgia rheumatica (PMR) between 1970 and 1991 who were followed up over the long term. METHODS: Information on demographics, PMR diagnosis, disease course, and drug therapy, in addition to data on adverse events commonly associated with CS and NSAID treatment, was obtained from the Rochester Epidemiology Project database. Cox proportional hazards and regression analysis models were used to evaluate the relationship between the occurrence of these events and therapy. RESULTS: Of the 232 patients (69 male, 163 female) included in the study, the mean age at PMR diagnosis was 72.9 years, the average followup was 8.0 years, and 30 patients were also diagnosed with giant cell (temporal) arteritis. Among the 175 patients (49 male, 126 female) treated with CS, the mean duration of CS therapy was 2.4 years, the average daily dose was 9.6 mg, and the mean cumulative dose was 8.4 gm. In total, 65% of the 124 patients treated with CS alone experienced at least 1 adverse event, compared with 67% of the 57 patients treated with NSAIDs alone and 80% of the 51 patients treated with CS and NSAIDs. The average time from initiation of therapy to the first adverse event was 1.6 years (n = 160). Proportional hazards modeling identified 3 variables that independently increased the risk of adverse events: age at PMR diagnosis, a cumulative dose of prednisone > or = 1,800 mg, and female sex. Person-year analysis revealed that the risks of diabetes mellitus, vertebral fractures, femoral neck fractures, and hip fractures were 2-5 times greater among PMR patients compared with age- and sex-matched individuals from the same population. Medical care or consultation by a rheumatologist was a highly significant predictor of a lower initial CS dose. CONCLUSION: The use of CS and NSAIDs in the treatment of PMR is associated with important long-term morbidity.
Adrenal Cortex Hormones↗
Giant cell arteritis and polymyalgia rheumatica.
Giant cell arteritis and polymyalgia rheumatica are linked conditions that frequently occur in the same patient. They are more common in northern Europe and persons of European descent than in other populations. Recent investigations have begun to provide information about the pathogenesis of both syndromes. Both respond to corticosteroids but at different dose levels. Although a number of vascular complications may occur, the outlook is excellent.
Europe↗
Giant cell (temporal) arteritis.
Headache is the most frequent symptom for which a patient with giant cell arteritis (GCA) presents to a neurologist. Amaurosis fugax and ischemic optic neuropathy are well recognized complications. Less commonly recognized neurologic complications include transient ischemic attacks, cerebral infarctions, acute confusional states, multi-infarct dementia, ischemic cervical myelopathy, and ischemic mononeuropathies. Because patients with GCA generally respond well to corticosteroid therapy, prompt diagnosis can minimize neurologic damage.
Adrenal Cortex Hormones↗
The implications of recognizing large-vessel involvement in elderly patients with giant cell arteritis.
Giant cell arteritis is associated with a markedly increased risk of aortic aneurysm formation or aortic rupture, particularly in the thoracic aorta. Although this association is not new, it has gained renewed attention recently. In this paper, the nature of aortic aneurysm formation in association with giant cell arteritis is discussed, along with the implications of recognizing large-vessel involvement in elderly patients with giant cell arteritis.
Aged↗
Peripheral neuropathy associated with sicca complex.
Peripheral neuropathy occurs in Sjögren's syndrome, a disorder in which systemic immunologic phenomena, including vasculitis, are common. Neuropathy also occurs with isolated sicca complex (keratoconjunctivits sicca and xerostomia); whether this represents a distinct syndrome is unclear. We retrospectively studied 54 patients with sicca complex and peripheral neuropathy to determine mode of presentation, neuropathic patterns, frequency and pattern of serologic abnormalities, and frequency of systemic disease, including necrotizing vasculitis. Peripheral neuropathy was the presenting problem in 87%. Although sicca symptoms occurred in 93%, they were a presenting complaint in only 11% and were usually mild, reported only after specific inquiry. Minor salivary gland biopsy was positive in 73%. Sensory neuropathies strongly predominated; 61% of patients manifested either sensory polyneuropathy or polyganglionopathy. Less common patterns included sensorimotor polyneuropathy (17%) and polyradiculoneuropathy (11%). Vasculitic neuropathy was demonstrated in only two patients, but nonspecific epineurial inflammation was present in 70% of nerve biopsies. Clinical evidence of systemic disease was uncommon, particularly in the sensory polyganglionopathy group, in whom extraglandular features other than weight loss occurred in only 1 of 12 patients. Antibodies to extractable nuclear antigens, the most specific serologic marker of Sjögren's syndrome, were present in 10.4%. We conclude that peripheral neuropathy and isolated sicca complex form a distinctive syndrome in which neuropathy is the presenting feature and sicca is easily overlooked; sensory polyneuropathy and polyganglionopathy predominate; serology is confirmatory but very insensitive; and extraglandular disease, including vasculitis, is uncommon compared with typical Sjögren's syndrome. Tests of ocular or salivary involvement are needed for diagnosis, and demonstration of inflammation in biopsied nerve is supportive. Improved definition of this disorder should permit further studies of natural history and efficacy of immunotherapy.
Adult↗
A case-control and nerve biopsy study of CREST multiple mononeuropathy.
From 536 patients with the CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasis), seven were identified as having peripheral neuropathy not attributable to another cause. Peripheral neuropathy developed 0 to 25 years after their first symptoms of scleroderma. Unexplained neuropathy in CREST patients (seven patients) was more frequent than in control subjects (two patients) matched for age, sex, time of evaluation, and geographic referral region. Multiple mononeuropathy occurred significantly more frequently in the CREST group (six patients) than in the control group (0 patients). Four sural nerve biopsy specimens from the CREST patients demonstrated multifocal fiber loss and perivascular inflammation; one was diagnostic for necrotizing vasculitis and two others were highly suggestive for necrotizing vasculitis. The density of myelinated fibers in three nerves from CREST patients was significantly decreased, whereas the index of dispersion (a measure of multifocal fiber loss) was increased, and the frequency of axonal degeneration was significantly increased. Based on these clinical and pathologic findings, we conclude that in the CREST syndrome multiple mononeuropathy, although occurring infrequently, occurs more frequently than by chance and necrotizing vasculitis is the cause of this multiple mononeuropathy.
Aged↗
Giant cell arteritis in polymyalgia rheumatica.
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Giant Cell Arteritis↗
Distal extremity swelling with pitting edema in polymyalgia rheumatica. Report on nineteen cases.
OBJECTIVE: To determine the frequency and clinical characteristics of diffuse distal extremity swelling with pitting edema occurring in polymyalgia rheumatica (PMR). METHODS: Clinical features and laboratory findings were recorded for all 245 residents of Olmsted County, Minnesota who developed PMR over a 22-year period (1970-1991). Those who exhibited > or = 1 episode of diffuse distal extremity edema with pitting were selected for this study, and were evaluated further. RESULTS: Thirteen women and 6 men in this incidence cohort of PMR had > or = 1 episode of distal extremity swelling with pitting edema. Giant cell arteritis was also identified in 5 patients. In 11 patients, the swelling and edema development concurrently with proximal PMR symptoms. In 2 patients, the distal swelling was the initial manifestation, and in 6 patients, the distal symptoms developed during relapses or recurrences of PMR. Both upper and lower extremities were affected, usually in a symmetric manner. Other peripheral manifestations were also common. The distal swelling and pitting edema responded promptly to corticosteroids, and slowly or incompletely to nonsteroidal anti-inflammatory drugs; a similar response was observed in the proximal symptoms. The distal swelling appeared to represent tenosynovitis and synovitis of regional structures. CONCLUSION: Distal extremity swelling with pitting edema represents a manifestation of PMR that has not been well described in previous studies. Awareness of this finding will help facilitate the proper diagnosis and institution of appropriate therapy for this disease.
Adrenal Cortex Hormones↗
Splinter hemorrhages following arterial puncture.
Splinter hemorrhages can be a feature of the antiphospholipid syndrome. We describe a patient in whom splinter hemorrhages developed following radial artery puncture. The implications of this findings in patients with the antiphospholipid syndrome are discussed.
Adult↗
Recognition of tissue residing antigen by T cells in vasculitic lesions of giant cell arteritis.
The objective of this study was to explore the nature of the antigen-specific T cell response in giant cell arteritis by analyzing clonally expanded T cells in temporal artery specimens. In temporal artery tissue from eight patients, 10% of the T cell receptor beta chain repertoire was systematically screened for clonal T cells by reverse-transcriptase polymerase chain reaction with selected BV, BJ, and BC specific primers and by direct sequencing of the amplified product. In five additional patients tissue-derived T cell clones were characterized. All expanded clonotypes were analyzed for their presence at different sites of the inflamed artery. T cell lines were tested for their proliferation to autologous monocytes pulsed with temporal artery extracts from patients with giant cell arteritis, polymyalgia rheumatica, and unrelated diseases. Clonally expanded T cells were identified in 30% of the BV-J combinations of the sampled repertoire. A subset of these clones were encountered at different sites of the inflammation, but not in the peripheral blood. The T cell receptor beta chain sequences were diverse. The patients had between none and five such clonotypes in the sampled repertoire, suggesting that only few T cell specificities in each patient are involved in antigen recognition. One of these T cell clonotypes was shown to proliferate in response to an antigen selectively expressed in temporal artery specimens from giant cell arteritis and from polymyalgia rheumatica patients. Clonotypes with identical T cell receptor beta chain sequences can be found at distinct sites of the inflammation in giant cell arteritis, suggesting recognition of the same antigen at different locations. At least for some of these T cell clones the antigen is shared between different giant cell arteritis and polymyalgia rheumatica patients but not expressed in temporal arteries of patients with unrelated diseases. While different HLA-DR4+ patients utilize distinct T cell specificities, the actual number of responding T cells in individual patients is small and may be disease limiting.
Antigens, CD↗
Long-term survival of patients with Wegener's granulomatosis from the American College of Rheumatology Wegener's Granulomatosis Classification Criteria Cohort.
OBJECTIVE: To determine survivorship in Wegener's granulomatosis (WG) in a well-defined multicenter cohort. METHODS: Follow-up was obtained for 77 of the 85 patients enrolled in the 1990 American College of Rheumatology vasculitis classification study. RESULTS: There were 28 deaths (10 females and 18 males) among the 77 patients available for follow-up. Standardized mortality ratios (SMR) were calculated with mortality data from the general population and from this group of patients with WG (an SMR of 1 indicates that expected and observed survival are identical). Overall survivorship among patients with WG was substantially reduced in this cohort (SMR = 4.685 +/- 0.65; for females SMR = 6.814 +/- 1.571; for males SMR = 3.998 +/- 0.69). CONCLUSION: The life expectancy of patients with WG is reduced compared with the general population.
Cause of Death↗
Long-term survival of patients with giant cell arteritis in the American College of Rheumatology giant cell arteritis classification criteria cohort.
OBJECTIVE: To characterize survivorship among patients with giant cell arteritis in a well-defined, multicenter cohort. PATIENTS AND METHODS: Follow-up was obtained for 205 (95.8%) of the 214 patients enrolled in the 1990 American College of Rheumatology vasculitis classification study. Standardized mortality ratios (SMR) were calculated comparing mortality data from this group of patients with giant cell arteritis versus the general population. RESULTS: There were 49 deaths (33 women and 16 men among the 205 patients available for follow-up. Survivorship was virtually identical to that of the general population (SMR = 1.034 +/- 0.121), and was similar for women (SMR = 1.022 +/- 0.149) and men (SMR = 1.078 +/- 0.206) (SMR = 1 indicates that expected and observed survival are identical). CONCLUSION: The life expectancy of patients with giant cell arteritis is the same as that of the general population.
Female↗
The inflammatory infiltrate in giant cell arteritis selects against B lymphocytes.
OBJECTIVE: To understand mechanisms regulating the cellular composition of the inflammatory infiltrate in giant cell arteritis (GCA) and to explore whether B lymphocytes, such as neoplastic B cells in chronic lymphocytic leukemia (CLL), can infiltrate the inflamed arterial wall of GCA. METHODS: A Mayo Clinic database search was conducted to identify patients that developed GCA after the onset of CLL between 1950 and 1994. Inflammatory infiltrates were analyzed immunohistochemically for T cells, B cells, and plasma cells. RESULTS: Five patients with CLL were identified who subsequently developed clinical findings suggestive of GCA. Three of the 5 patients had biopsy proven disease, presented with typical clinical and histomorphological features of GCA, and responded promptly to corticosteroid treatment. Leukemic B cells known for their ability to diffusely seed organs were absent in the inflammatory lesions in the temporal artery. Plasma cells that contribute to the vasculitic infiltrate in patients without CLL were markedly reduced in the GCA lesions of patients with CLL. Two other patients had symptoms suggestive of GCA. Their temporal artery specimens were remarkable for small adventitial lymphocytic infiltrates but lacked typical panarteritis. CONCLUSION: The inflammatory infiltrate of GCA is tightly regulated, and cell accumulation in the granulomas is an active, not a passive, mechanism. The inflammatory pathway in GCA is focused on T cells and macrophages and excludes B cells.
Antibodies, Monoclonal↗
Clinical differentiation between giant cell (temporal) arteritis and Takayasu's arteritis.
OBJECTIVE: To determine whether giant cell (temporal) arteritis (GCA) can be differentiated from Takayasu's arteritis on the basis of clinical findings. METHODS: A comparative study contrasting 217 patients with GCA to 63 patients with Takayasu's arteritis was conducted, using the prospectively gathered large multicenter data set of the American College of Rheumatology Vasculitis Criteria Databank. Logistic regression and recursive partitioning were used to assess the capability of a variety of variables to separate these 2 vasculitic disorders. RESULTS: Age of 40 years at onset of disease was the most discriminatory single variable. Excluding age at onset, ethnic background and a combination of clinical signs indicating upper limb vascular impairment, shoulder stiffness, and tender scalp led to correct classifications of 95% in both GCA and Takayasu's arteritis. Vascular insufficiency of the upper extremities, a key feature of Takayasu's arteritis, was noted in 15% of patients with GCA, although to a lesser degree. CONCLUSION: Although similar in many respects, GCA and Takayasu's arteritis were easily definable and separable disorders, even when the typical age barrier of 40 years at onset of disease was excluded. These findings strengthen the concept that they are distinct disease entities.
Age of Onset↗
SLE nephropathy in a patient with HIV infection: case report and review of the literature.
We describe a 39-year-old bisexual man with clinical category B2 human immunodeficiency virus (HIV) infection who subsequently developed systemic lupus erythematosus (SLE). SLE was diagnosed on the basis of a clinical presentation of malar rash, polyarthritis, membranous glomerulonephritis, and characteristic serology. To our knowledge, this is the fourth reported case of a patient with HIV infection to develop SLE and the second adult patient with HIV and coexistent SLE nephropathy.
Adult↗
The incidence of giant cell arteritis in Olmsted County, Minnesota: apparent fluctuations in a cyclic pattern.
OBJECTIVE: To investigate trends in the incidence of giant cell arteritis over a 42-year period in Olmsted County, Minnesota. DESIGN: Population-based incidence study. SETTING: Olmsted County, Minnesota. METHODS: All incidence cases of giant cell arteritis first diagnosed between 1950 and 1991 were identified using the unified record system at Mayo Clinic. Age- and sex-specific incidence rates were calculated using the number of incidence cases as the numerator and population estimates as the denominator. Overall rates were age- and sex-adjusted to the 1980 United States white population. The annual incidence rates were graphically illustrated using a 3-year centered moving average. RESULTS: Between 1950 and 1991, 125 Olmsted County residents (103 women and 22 men) were diagnosed with giant cell arteritis. The age- and sex-adjusted incidence per 100,000 persons 50 years of age or older was 17.8 (95% CI, 14.7 to 21.0); incidence was significantly higher in women (24.2 [CI, 19.5 to 28.9]) than in men (8.2 [CI, 4.8 to 11.6]). Age-specific incidence rates increased with age (P < 0.0001). The annual incidence rates increased significantly over the study period (P = 0.002) and appear to have clustered in five peak periods, which occurred about every 7 years. A significant calendar-time effect was identified; it predicted an increase in incidence of 2.6% (CI, 0.9% to 4.3%) every 5 years. CONCLUSIONS: Our observation of a regular cyclic pattern in incidence rates over time supports the hypothesis of an infectious cause for giant cell arteritis. Similar studies in other populations are needed to confirm our findings.
Age Distribution↗
Increased incidence of aortic aneurysm and dissection in giant cell (temporal) arteritis. A population-based study.
OBJECTIVE: To determine the frequency of aneurysm and dissection of the aorta in patients with giant cell arteritis and to assess the effects of these events on these patients. DESIGN: Population-based cohort study. SETTING: A multispecialty and a primary care clinic in southern Minnesota. PATIENTS: 96 residents of Olmsted County, Minnesota, who developed giant cell arteritis between 1950 and 1985. The presence of aortic aneurysm, dissection, or both was confirmed using computed tomography, ultrasonography, angiography, or autopsy. RESULTS: 11 of the 96 patients were found to have thoracic aortic aneurysms. In 2 of these patients, the aneurysms were detected when giant cell arteritis was diagnosed. In the remaining 9 patients, the aneurysms occurred a median of 5.8 years after giant cell arteritis was diagnosed. Six of the 11 died suddenly of acute thoracic aortic dissection. Five patients who did not have thoracic aortic aneurysms developed isolated abdominal aortic aneurysms a median of 2.5 years after giant cell arteritis was diagnosed. The incidence of thoracic aortic aneurysm in patients with giant cell arteritis was 999 per 100,000 person-years; the incidence of abdominal aortic aneurysm in these patients was 555 per 100,000 person-years. Compared with all persons of the same age and sex living in Olmsted County, patients with giant cell arteritis were 17.3 times (95% Cl, 7.9 to 33.0) more likely to develop thoracic aortic aneurysm and 2.4 times (Cl, 0.8 to 5.5) more likely to develop isolated abdominal aortic aneurysm. CONCLUSIONS: Giant cell arteritis is associated with a markedly increased risk for the development of aortic aneurysm, which is often a late complication and may cause death.
Aged↗