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G G Hunder

Publications and source records attributed to G G Hunder.

At least 19 recordsLinked to original sources

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

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

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

Epidemiology of polymyalgia rheumatica in Olmsted County, Minnesota, 1970-1991.

OBJECTIVE: To determine the incidence, prevalence, and survival of polymyalgia rheumatica (PMR) over a 22-year period in Olmsted County, Minnesota. METHODS: Using the unified record system at the Mayo Clinic for the Olmsted County population, we reviewed all medical records with a diagnosis of PMR from 1970 through 1991. RESULTS: There were 245 (173 female; 72 male) incidence cases of PMR between 1970 and 1991. The average annual age- and sex-adjusted incidence of PMR per 100,000 population > or = 50 years was 52.5 (95% confidence interval [CI] 45.9-59.2), with a significantly higher incidence in females (61.7; 95% CI 52.3-71.2) than in males (39.9; 95% CI 30.7-49.2). The incidence varied over the period of observation, but no significant trends were found. The prevalence of PMR among persons > or = 50 years on January 1, 1992 was 6/1,000. There was a small but significantly increased survival rate among male PMR patients compared with the general population. CONCLUSION: Our data demonstrate that PMR is a common nonfatal disease in the elderly, the incidence and clinical manifestations of which have varied but remained relatively stable over the last 2 decades.

Age Distribution

Common rheumatologic diseases in elderly patients.

OBJECTIVE: To review common rheumatologic disorders that affect elderly patients and emphasize the unique diagnostic and therapeutic challenges inherent in the management of rheumatologic diseases in this age-group. DESIGN: We summarize our approach to treatment and management of specific rheumatologic problems in geriatric patients and discuss pertinent studies from the literature. RESULTS: Among the spectrum of rheumatologic disorders frequently encountered in the elderly population are polymyalgia rheumatica, fibromyalgia, giant cell arteritis, crystalline arthropathies (gout and pseudogout), and degenerative joint disease. The initial manifestations of these rheumatologic diseases in elderly patients may differ from the typical findings in younger patients. Geriatric patients may have nonspecific complaints, a decline in physical function, or even confusion. Because of physiologic changes associated with aging and a decrease in functional reserves, elderly patients are susceptible to adverse effects of pharmacologic therapy (including nonsteroidal anti-inflammatory medications, corticosteroids, narcotic analgesics, allopurinol, and colchicine). Clinicians should be alert for such problems as hepatotoxicity and occult gastrointestinal blood loss. Comorbid conditions such as cardiovascular disease and cognitive impairment may complicate management strategies and may limit the goals of both surgical intervention and rehabilitation programs in elderly patients. CONCLUSION: Rheumatologic disorders in geriatric patients pose special challenges to primary-care physicians. In the selection of optimal pharmacologic and nonpharmacologic therapeutic modalities, clinicians should focus on maintaining or improving the patient's quality of life and level of independent function.

Activities of Daily Living

Steroid sparing activity of tenidap in patients with polymyalgia rheumatica: a multicenter double blind randomized placebo controlled study.

OBJECTIVE: To determine whether tenidap treatment would allow reduction or replacement of systemic corticosteroid treatment in patients with polymyalgia rheumatica (PMR). METHODS: A 15-week double blind, randomized, multicenter, placebo-controlled study of tenidap sodium (120 mg/day) in patients with symptomatically controlled PMR receiving 10 mg/day prednisone was conducted. After receiving study drug for 3 weeks, prednisone dose was reduced by 2.5 mg/day every 3 weeks. The lowest clinically effective dose of prednisone was recorded as 10, 7.5, 5, 2.5 or 0 mg/day. RESULTS: Thirty-two patients were randomized to tenidap or placebo. As prednisone was reduced more placebo patients experienced an exacerbation of PMR symptoms, elevation of erythrocyte sedimentation rate and increased serum C-reactive protein. Twice as many placebo patients (10 of 16) as tenidap patients (5 of 16) discontinued due to lack of efficacy. The lowest effective dose of prednisone could be determined in 27 of the 32 patients, 11 receiving tenidap and 16 placebo. A significantly (p = 0.027) greater proportion of patients receiving tenidap (5 of 11) than placebo (1 of 16) were able to discontinue prednisone without experiencing a symptomatic flare. CONCLUSION: As prednisone was reduced, symptoms of PMR were controlled better by tenidap than by placebo. Forty-five percent of evaluable patients receiving tenidap were able to discontinue prednisone without a disease flare compared to 6% for placebo.

Aged

The use of clinical characteristics to predict the results of temporal artery biopsy among patients with suspected giant cell arteritis.

OBJECTIVE: To develop a mathematical model which predicts temporal artery biopsy results. METHODS: We collected clinical and laboratory data as well as biopsy results among a consecutive cohort of all individuals who underwent temporal artery biopsy at Mayo Medical Center between January 1, 1988 and December 31, 1991. All biopsies were independently reviewed by one pathologist. Logistic regression was used to identify a set of variables which best predicted the biopsy results. This model was then used to identify patients who were highly likely (> or = 95% predictive value) to have either a negative or a positive biopsy. A receiver operating characteristic (ROC) curve was generated using the best fit model. RESULTS: Of the 525 people in the study, there were 187 men and 338 women. The logistic regression model and the ROC curve generated from this model were of modest value in predicting biopsy results from prebiopsy clinical characteristics. However, this model identified 60 (11%) individuals who had a > or = 95% probability of having a negative biopsy. None of these individuals had any symptoms of claudication, only 5 of 60 (8%) had temporal artery abnormalities on examination, 45 (75%) had synovitis (suggesting an alternate diagnosis), and their median erythrocyte sedimentation rate was only 31 mm/h (Westergren). CONCLUSIONS: In individuals with these findings, we recommend a careful search for other diagnoses before temporal artery biopsy.

Aged