Gene G. Hunder's research while affiliated with Mayo Clinic - Rochester and other places

Publications (279)

Chapter
Primary CNS vasculitis is an uncommon disorder of unknown cause that is restricted to brain and spinal cord. The median age of onset is 50 years. The neurological manifestations are diverse, but generally consist of headache, altered cognition, focal weakness, or stroke. Serological markers of inflammation are usually normal. Cerebrospinal fluid is...
Article
Objectives: To evaluate long-term treatment and outcomes of patients with primary central nervous system vasculitis (PCNSV). Methods: In this cohort of 191 consecutive patients with PCNSV seen at Mayo Clinic, Rochester, MN, over 35 years with long-term follow-up we analyzed response to and duration of therapy, frequency of relapses, long-term re...
Chapter
Primary central nervous system vasculitis (PCNSV) is an infrequent and not well understood form of vasculitis that is limited to the brain and spinal cord. However, PCNSV is the most frequent vasculitis involving the central nervous system. Men and women are similarly affected, and the median age at diagnosis is approximately 50 years.
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Full-text available
In this article, the authors offer a new perspective on how the administration of Compound E (ie, cortisone) to a volunteer Mayo Clinic patient with rheumatoid arthritis and the patient's subsequent miraculous improvement led not only to a major, successful clinical trial but also a Nobel Prize. The early and late side effects as an undesirable out...
Article
Objectives To assess the efficacy and safety of Rituximab (RTX) in adult primary central nervous system vasculitis (PCNSV). Methods We retrospectively assessed the effect of RTX in 6 patients with PCNSV. Five of the 6 were refractory to high dose glucocorticoids (GCs) and/or conventional immunosuppressants (IS). The sixth was newly diagnosed and r...
Article
Objective: To describe the clinical, laboratory, and imaging features and course of patients with primary central nervous system vasculitis (PCNSV) presenting with an intracranial tumor-like mass (TLM). Methods: We retrospectively studied a cohort of 191 consecutive patients with PCNSV seen at the Mayo Clinic, Rochester, MN over a 35-year period...
Conference Paper
Background Primary central nervous system vasculitis (PCNSV) rarely presents as a tumor-like mass lesion (ML). Objectives The aim of this study was to determine the frequency of ML in a large cohort of patients with PCNSV and compare the presenting clinical, laboratory, and imaging features in those with ML to those without. Methods We retrospect...
Article
Objectives: To record the clinical findings, response to therapy, and course of patients with primary CNS vasculitis (PCNSV) associated with lymphoma. Patients and methods: We reviewed the histories of 936 patients with a diagnosis of any type of vasculitis and lymphoma who were seen at the Mayo Clinic over a 32-year period. Ten patients with bo...
Article
Full-text available
Although clinical signs and symptoms of giant cell arteritis improve promptly after starting glucocorticoid therapy, reports have suggested that the vascular inflammation may persist. To assess the duration and quality of histopathologic changes in treated patients, we prospectively obtained second temporal artery biopsies in patients treated for 3...
Chapter
Primary central nervous system vasculitis is an uncommon disorder of unknown cause that is limited to the brain and spinal cord. The median age of onset is 50 years. The neurological manifestations are diverse, but commonly include headache, altered cognition, focal weakness, or stroke. Serological markers of inflammation are usually normal. Cerebr...
Article
Background Vasculitis as a complication of lymphoproliferative diseases is relatively uncommon. Some cases of primary central nervous system vasculitis (PCNSV) have been described in association with lymphoma, particularly Hodgkin's lymphoma (HL). Objectives The aim of this study is to report the presenting clinical, laboratory, and imaging featur...
Article
Background: Factors associated with dissection from inflammatory aortic aneurysms may be different from those in the general population. Objective: The aim of this study was to evaluate the risk factors for aortic dissection/rupture in patients with giant cell arteritis (GCA) and aortic aneurysms. Methods: A population-based incident cohort of...
Article
Full-text available
Vascular inflammation is present in a subset of patients with cerebral amyloid angiopathy (CAA) and has a major influence in determining the disease manifestations. Radiological characterization of this subset is particularly important to achieve early recognition and treatment. We conducted this study to investigate the role of imaging in differen...
Article
Purpose of review This article summarizes the current evidence on the management of primary and secondary central nervous system (CNS) vasculitis. Recent findings Two recent retrospective cohort studies have described the treatment and outcomes of patients with adult primary central nervous system vasculitis (PCNSV). Although the majority of the pa...
Article
Background In a subset of patients with cerebral amyloid angiopathy (CAA) vascular inflammation is also present. Two pathologic subtypes have been described: one with perivascular inflammation (CAA-related inflammation or CAA-RI), and the second with a vasculitic transmural inflammatory infiltrate (Aβ-related angiitis or ABRA). Objectives To invest...
Article
Full-text available
Primary central nervous system vasculitis (PCNSV) is an uncommon condition in which lesions are limited to vessels of the brain and spinal cord. Because the clinical manifestations are not specific, the diagnosis is often difficult, and permanent disability and death are frequent outcomes. This study is based on a cohort of 163 consecutive patients...
Article
Objective. To describe the treatment and outcomes in primary central nervous system vasculitis (PCNSV). Methods. We retrospectively studied a cohort of 163 consecutive patients with PCNSV seen at the Mayo Clinic over a 29-year period. We analyzed treatments and responses, and factors predictive of outcomes. Results. 85% of patients treated with pre...
Article
To evaluate the efficacy and safety of mycophenolate mofetil (MMF) in adult primary central nervous system vasculitis (PCNSV). We studied a cohort of 163 patients with PCNSV who were seen at the Mayo Clinic from 1983 to 2011. We compared patients treated with MMF and those receiving other therapies. We identified 16 patients treated with MMF. MMF i...
Chapter
Microscopic polyangiitis is a pauci-immune necrotizing small- and medium-vessel vasculitis mainly involving the lung and kidneys but also the peripheral nerves and skin. A variety of skin lesions are observed in about half of patients. The most common cutaneous manifestations are palpable purpura, livedo racemosa, and skin nodules. Histology of ski...
Chapter
Giant cell arteritis is a primary systemic vasculitis involving large- and medium-sized vessels which affects almost exclusively patients aged 50 years or older. Cutaneous manifestations are rare because giant cell arteritis spares the small vessels, that is, those vessels that are typically associated with skin lesions. The most common skin abnorm...
Article
Background Background. Because of the relatively small numbers of patients evaluated in the studies, primary central nervous system vasculitis (PCNSV) response to treatment is still uncertain. Objectives To determine the response to therapy and long-term outcome of PCNSV we reviewed all cases of PCNSV seen at Mayo Clinic (Rochester, MN) from 1983 t...
Article
Primary central nervous system vasculitis (PCNSV) is an uncommon condition that affects the brain and the spinal cord. It is heterogeneous in presenting characteristics and outcomes. We report a patient with a catastrophic rapidly progressive course refractory to intensive treatment with pulses of methylprednisolone and iv cyclophosphamide. The con...
Article
Primary CNS vasculitis (PCNSV) is an uncommon disorder of unknown cause that is restricted to brain and spinal cord. Glucocorticoids alone or in combination with cyclophosphamide achieve a favorable response in most patients.(1,2) However, some patients are intolerant or respond poorly to cyclophosphamide, therefore there is the need for new treatm...
Chapter
Primary central nervous system vasculitis (PCNSV) is a rare vasculitis that affects the brain and occasionally the spinal cord of adults and children. Extensive progress has been achieved in the recognition of clinicopathological subsets that has translated into useful nosology reflective of the natural history and response to immunosuppressant tre...
Article
Glucocorticoids are highly effective in treating polymyalgia rheumatica and giant cell arteritis, but their use is associated with numerous adverse events. Therefore, it is important to use them for the shortest period of time possible. The published evidence suggests that discontinuation of GCis feasible in a substantial number of patients with po...
Article
To analyze the clinical findings, response to therapy, and outcomes of patients with cerebral vascular amyloid-β (Aβ) deposition with and without inflammatory vascular infiltration. We report 78 consecutive patients with cerebral vascular Aβ deposition examined at Mayo Clinic Rochester over 25 years (1987 through 2011). Specimens reviewed by a neur...
Article
In large-vessel vasculitis, imaging studies are useful to document temporal artery involvement and crucial to show large-vessel involvement. Color Doppler sonography, magnetic resonance, and computed tomography show early vasculitic lesions. Angiography delineates later vascular complications well. Color Doppler sonography, magnetic resonance angio...
Article
Objectives To evaluate the inflammatory involvement of lumbar interspinous bursitis in patients with polymyalgia rheumatica (PMR) using magnetic resonance imaging (MRI). Methods Ten consecutive, untreated new patients with PMR and pain in the shoulder and pelvic girdles were investigated. Seven patients with spondyloarthritis (4 with psoriatic spon...
Article
Objectives: To evaluate incidence-trends and timing of large-vessel (LV) manifestations in patients with giant cell arteritis (GCA), and to examine the influence of LV manifestations on survival. Methods: A population-based incident cohort of patients diagnosed with GCA between 1950 and 2004 was used. LV involvement was defined as large-artery s...
Article
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 pe...
Article
Primary CNS vasculitis is an uncommon disorder of unknown cause that is restricted to brain and spinal cord. The median age of onset is 50 years. The neurological manifestations are diverse, but generally consist of headache, altered cognition, focal weakness, or stroke. Serological markers of inflammation are usually normal. Cerebrospinal fluid is...
Article
Primary vasculitis of the central nervous system (PCNSV) is a rare and diagnostically challenging form of vasculitis limited to the brain and/or spinal cord. It is a complex and often severe disease with multifaceted clinical and pathological appearances, suggesting multiple disease subtypes and the potential existence of multiple etiologic pathway...
Article
The aim of this review was to cover the recent major advances made in the fields of clinical assessment, diagnosis and treatment of adult primary central nervous system vasculitis (PCNSV). To prevent misdiagnosis, particularly with the reversible cerebral vasoconstriction syndromes, new criteria on the basis of the levels of certainty of the diagno...
Article
Philip S. Hench, MD, the first Mayo Clinic rheumatologist, came to Mayo Clinic in 1921. Because of his efforts in patient care, education, and research, and those of his colleagues, Mayo Clinic has been considered the first academic rheumatology center established in the United States. An early, popular lecture he gave to the internal medicine resi...
Article
To evaluate the utility of erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) for the diagnosis of giant cell arteritis (GCA) and to determine the frequency of normal ESR and CRP at diagnosis of GCA. All patients undergoing temporal artery biopsy (TAB) between 2000 and 2008 were identified. Only subjects with both ESR and CRP at the...
Article
To describe a subset of cases in a large retrospectively identified cohort of patients with primary central nervous system vasculitis (PCNSV) who present with intracranial hemorrhage. The study consisted of a cohort of 131 consecutive patients with PCNSV who were seen at the Mayo Clinic over a 25-year period from 1983 to 2007. The diagnosis of PCNS...
Article
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 lifet...
Article
The term "chronic periaortitis" (CP), proposed by Mitchinson in 1984, comprises 3 main entities: idiopathic retroperitoneal fibrosis (IRF), inflammatory abdominal aortic aneurysms (IAAAs), and perianeurysmal retroperitoneal fibrosis (PRF).The presence of constitutional symptoms, high acute-phase reactants, positive autoantibodies, and associated au...
Article
Primary central nervous system vasculitis (PCNSV) is a rare primary vasculitis limited to the brain and spinal cord. It can affect any age group, but has a predilection for subjects aged 40 to 60 years without clear gender predominance. Clinical manifestations are nonspecific, including headache, non-focal neurological features and, less frequently...
Article
To determine the incidence of herpes zoster (HZ) in GCA. Utilizing the resources of the Rochester Epidemiology Project, all incident cases of GCA diagnosed between 1 January 1950 and 31 December 2004 were identified. For each GCA patient, two subjects without GCA of the same gender and similar age and length of medical history were randomly selecte...
Article
To describe a subset of cases in a large cohort of patients with primary CNS vasculitis (PCNSV) who appear to have a rapidly progressive clinical course. In the present study, we use our updated cohort of 131 consecutive patients with PCNSV seen over the 25-year period of 1983-2007 at Mayo Clinic, Rochester, MN, USA. The diagnosis of PCNSV was base...
Article
The development of a new treatment modality necessarily means first in human use and brings with it the responsibility to both protect the patient and provide knowledge or benefit that is generalizable to others. Although their motivations and backgrounds vary, researchers and patients both share the excitement of discovery and the consequences of...
Article
Criteria for the classification of giant cell (temporal) arteritis were developed by comparing 214 patients who had this disease with 593 patients with other forms of vasculitis. For the traditional format classification, 5 criteria were selected: age greater than or equal to 50 years at disease onset, new onset of localized headache, temporal arte...
Article
Criteria for the classification of Wegener's granulomatosis (WG) were developed by comparing 85 patients who had this disease with 722 control patients with other forms of vasculitis. For the traditional format classification, 4 criteria were selected: abnormal urinary sediment (red cell casts or greater than 5 red blood cells per high power field)...
Article
Criteria for the classification of hypersensitivity vasculitis were developed by comparing 93 patients who had this disease with 714 control patients with other forms of vasculitis. For the traditional format classification, 5 criteria were selected: age greater than 16 at disease onset, history of taking a medication at onset that may have been a...
Article
The Americal College of Rheumatology Subcommittee on Classification of Vasculitis of the Diagnostic and Therapeutic Criteria Committee developed classification criteria for 7 forms of vasculitis: polyarteritis nodosa, Churg-Strauss syndrome, Wegener's granulomatosis, hypersensitivity vasculitis, Henoch-Schonlein purpura, giant cell (temporal) arter...
Article
Criteria for the classification of polyarteritis nodosa were developed by comparing 118 patients who had this disease with 689 control patients who had other forms of vasculitis. For the traditional format classification, 10 criteria were selected: weight loss greater than or equal to 4 kg, livedo reticularis, testicular pain or tenderness, myalgia...
Article
Criteria for the classification of Takayasu arteritis were developed by comparing 63 patients who had this disease with 744 control patients with other forms of vasculitis. Six criteria were selected for the traditional format classification: onset at age less than or equal to 40 years, claudication of an extremity, decreased brachial artery pulse,...
Article
To study the association between previous cancer and giant cell arteritis (GCA). Using the resources of the Rochester Epidemiology Project, we identified incident cases of GCA diagnosed between January 1, 1950 and December 31, 2004. Each GCA patient was matched for age, sex, and length of medical history to 2 subjects without GCA from the same popu...
Article
To describe the clinical features and outcomes of patients with localized vasculitis of the gastrointestinal tract (LVGT). Medical records of 608 patients diagnosed with vasculitis involving the intra-abdominal vasculature and/or abdominal viscera between January 1996 and December 2007 were reviewed. Only patients with histopathological confirmatio...
Article
In its early years, Mayo Clinic had primarily a surgical practice. Patients with musculoskeletal complaints were cared for by one of the Mayo brothers (Dr William J. Mayo or Dr Charles H. Mayo) or their initial partners. That such patients were seen in Rochester, MN, is evidenced by a report the brothers wrote in 1895 that described surgical treatm...
Article
To determine the incidence of cancer in a population-based cohort of patients with giant cell arteritis (GCA) compared with age- and sex-matched referent subjects. Using the resources of the Rochester Epidemiology Project, all incident cases of GCA diagnosed between January 1, 1950 and December 31, 2004 were identified. For each GCA patient, 2 subj...
Article
GCA (giant cell arteritis) is a granulomatous vasculitis of large and medium-sized arteries that occurs in individuals 50 years of age or older. [1] A recent study suggested that the age at incidence of GCA may be increasing. [2] To address this issue, we studied the trends in mean age at onset of GCA over a 55-year period.
Article
To describe the clinical features and outcomes of 19 patients with giant cell arteritis (GCA) and symptomatic lower extremity (LE) vasculitis. We reviewed medical records of all patients diagnosed with GCA and symptomatic LE involvement between January 1, 1983, and June 30, 2007, for clinical features, laboratory and radiographic findings, and outc...
Article
Full-text available
The present study was conducted to determine whether patients with polymyalgia rheumatica (PMR) are at an increased risk of peripheral arterial disease (PAD). An inception cohort of all Olmsted County, Minnesota residents diagnosed with PMR between 1 January 1970 and 31 December 1999 was compared with non-PMR subjects (two for each PMR subject) fro...
Article
Primary angiitis of the central nervous system (PACNS) is a form of vasculitis restricted to the brain and spinal cord, with protean clinical manifestations and often slowly progressive course. Outcomes vary, ranging from spontaneous resolution to rapid decline and death. Diagnosis of PACNS is based on angiography and/or biopsy. We reviewed surgica...
Article
Primary central nervous system vasculitis (PCNSV) is a rare and poorly understood syndrome. We describe the clinical findings in 8 patients who appear to have a distinct subset of PCNSV. We identified 101 consecutive patients with PCNSV who were seen between January 1, 1983, and December 31, 2003. The diagnosis was based on conventional angiography...
Article
Full-text available
To describe the clinical features and outcomes of patients with primary central nervous system vasculitis (PCNSV) and cerebral amyloid angiopathy (CAA) from a large cohort of consecutive patients with PCNSV treated at a single institution. We identified 101 consecutive patients with PCNSV admitted between January 1983 and December 2003. PCNSV diagn...
Article
Polymyalgia rheumatica and giant-cell arteritis are closely related disorders that affect people of middle age and older. They frequently occur together. Both are syndromes of unknown cause, but genetic and environmental factors might have a role in their pathogenesis. The symptoms of polymyalgia rheumatica seem to be related to synovitis of proxim...
Article
Full-text available
To evaluate the inflammatory involvement of cervical interspinous bursae in patients with polymyalgia rheumatica (PMR) using MRI. In all, 12 consecutive, untreated new patients with PMR were investigated. Five patients with fibromyalgia, two patients with cervical osteoarthritis and six patients with spondyloarthritis with neck pain served as contr...
Article
Primary CNS vasculitis (PCNSV) is an uncommon disease in which lesions are limited to the brain and spinal cord. Our objective was to evaluate the frequency, clinical features, and outcome of spinal cord involvement in PCNSV. We retrospectively identified 101 consecutive patients with PCNSV. Spinal cord involvement was documented for five. Clinical...
Article
Full-text available
The benefit con- ferred by GCs needs to be balanced against the common and well recognised complica- tions related to long-term GC use. In a population-based study of 120 patients with GCA, 86% of patients had adverse events including bone fractures, avascular necrosis of the hip, diabetes mellitus, infections, gastrointestinal bleeding, and catara...
Article
Primary central nervous system vasculitis (PCNSV) is an uncommon condition that affects the brain and spinal cord. This study was undertaken to evaluate the clinical features and outcomes among patients with PCNSV who presented with prominent gadolinium meningeal enhancement on magnetic resonance imaging (MRI). Through retrospective review using th...
Article
To provide a single source for the best available estimates of the US prevalence of and number of individuals affected by osteoarthritis, polymyalgia rheumatica and giant cell arteritis, gout, fibromyalgia, and carpal tunnel syndrome, as well as the symptoms of neck and back pain. A companion article (part I) addresses additional conditions. The Na...