Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0042384 (vasculitis)
 20,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We describe a heterogeneous group of 4 elderly patients with atypical vasculitis of the temporal artery who presented clinically with the polymyalgia rheumatica-temporal arteritis syndrome. The first had apparently isolated eosinophilic necrotizing vasculitis without peripheral blood eosinophilia, history of asthma, or pulmonary vasculitis (so called limited form of Churg-Strauss syndrome). Temporal artery biopsy in the 2nd patient revealed small vessel necrotizing vasculitis in the fragment of skeletal muscle surrounding a normal temporal artery (i.e., negative for giant cell arteritis, GCA). This patient subsequently developed rheumatoid polysynovitis. The temporal artery biopsies in the remaining 2 patients showed vasa vasorum vasculitis, without GCA, associated with malignancy in Patient 3 and as an unexpected and apparently isolated finding in Patient 4. All 4 patients responded to the standard corticosteroid therapy for giant cell temporal arteritis. Unusual temporal artery biopsies such as these should be interpreted in relation to clinical findings.
 ...
PMID:Non-giant cell arteritis of the temporal artery presenting as the polymyalgia rheumatica-temporal arteritis syndrome. 859 67

Several disease mechanisms have been studied in the model of GCA. GCA patients exhibit a genetic susceptibility which has been mapped to the HLA-DRB1 gene. Polymorphic amino acid residues localized at the floor of the antigen binding site are highly selected in GCA patients suggesting a role for antigen binding and presentation in the disease. This and other genetic risk factors might actually be a surrogate for the distinct geographic distribution of the disease with a marked preference for Northern Europe. Studies on functional aspects of T cells accumulating in the vasculitic foci have demonstrated a strong bias for Th1 helper T cells which locally release IL-2 and IFN-gamma. IFN-gamma appears to be a key cytokine in this vasculitis. IFN-gamma producing T cells represent a minority of the tissue infiltrating cell population suggesting that very few cells have disease relevance and the majority of T cells is recruited as bystanders. IFN-gamma secreting CD4 T cells preferentially localize to the adventitial-medial junction and are thus placed distant from the center of pathology, the intima and internal media. TCRs expressed by T cells accumulated in the affected tissue are not randomly distributed but are biased toward selected specificities. These selected T cells undergo proliferation in the tissue and can be isolated from nonadjacent and independent sites of the vasculitis. This distribution pattern indicates a common driving factor, suspected to be a tissue residing antigen [8]. Further support for an antigen driving this pathological T cell response comes from the finding that temporal artery specimens engrafted into SCID mice continue to show the typical disease process indicating that all components relevant for the disease are contained in the temporal artery wall. So far no shared TCR utilized by different patients has been identified, raising the question whether distinct antigens can elicit GCA as a common pathway of reactivity. Besides its role in investigating pathomechanisms the SCID mouse model of GCA provides the unique opportunity to study the therapeutic effects of established and novel treatments. It can be expected that some of the pathogenic rules established for GCA can be applied to other vasculitic syndromes.
 ...
PMID:The pathogenic role of T lymphocytes in vasculitis. 894 85

Forty-six cases of vasculitis affecting the female genital tract are described; only 41 similar cases have been previously reported, either as case reports or small series. The age range of the patients was from 22 to 80 years, and most of them presented with abnormal bleeding or were being treated conditions unrelated to the vasculitis. There were 39 hysterectomy specimens (26 of which were derived from total abdominal hysterectomies) and seven specimens of the cervix only. The vasculitis was confined to the cervix in 30 of the 46 cases; in 24 of these, the entire uterus was available for examination. In 23 cases, only a single vessel was involved, and in the other 23 there was more extensive vessel involvement. In all cases, the involved vessels were arterioles and small arteries. In 42 cases, the arteritis was of the polyarteritis nodosa (PAN) type, and in four, of the giant cell type (GCA). Follow-up ranged from < 1 year to 23 (mean, 3) years. Systemic manifestations were previously diagnosed or subsequently developed in only four patients, three with PAN and one with GCA; in each of them, the genital tract vasculitis was found only in the cervix (in one of these, however, the specimen was a loop excision of the cervix and the rest of the uterus was not assessable). The three patients with PAN subsequently developed extragenital PAN (one case), PAN and rheumatoid arthritis (one case), or PAN and polymyalgia rheumatica (one case). The patient with GCA had previously documented temporal arteritis and temperomandibular arthritis. The findings in this series and in previously reported cases indicate that vasculitis of the female genital tract is only rarely associated with systemic vasculitis.
 ...
PMID:Vasculitis of the female genital tract with clinicopathologic correlation: a study of 46 cases with follow-up. 1090 75

Giant cell (temporal) arteritis (GCA) is the most common systemic vasculitis in Western countries. It involves large and medium-sized vessels with predisposition to the cranial arteries in the elderly. Cranial ischemic complications, in particular permanent visual loss, constitute the most feared aspects of this vasculitis. Although the use of corticosteroids and a higher physician awareness may have contributed to a decrease in the frequency of severe ischemic complications, permanent visual loss is still present in 7%-14% of patients. To investigate further the incidence, trends, and clinical spectrum of visual manifestations in patients with GCA, we examined the features of patients with biopsy-proven GCA diagnosed at the single reference hospital for a defined population in northwestern Spain during an 18-year period. Predictive factors for the development of any visual manifestation, not only permanent visual loss, were also examined. Between 1981 and 1998, 161 patients were diagnosed with biopsy-proven GCA. Visual ischemic complications were observed in 42 (26.1%), and irreversible blindness, mainly due to anterior ischemic optic neuropathy and frequently preceded by amaurosis fugax, was found in 24 (14.9%). Despite a progressive increase in the number of new cases diagnosed, there was not a significant change in the proportion of patients with visual manifestations during the study period (p = 0.37). Patients with visual ischemic complications had lower clinical and laboratory biologic markers of inflammation. Indeed, during the last years of the study, anemia was associated with a very low risk of visual complications. Also, HLA-DRB1*04-positive patients had visual manifestations more commonly. Patients with other ischemic complications developed irreversible blindness more frequently. The best predictors of any visual complication were HLA-DRB1*04 phenotype (odds ratio [OR] 7.47) and the absence of anemia at the time of admission (OR for patients with anemia = 0.07). The best predictors of irreversible blindness (permanent visual loss) were amaurosis fugax (OR 12.63) and cerebrovascular accidents (OR 26.51). The present study supports the claim that ocular ischemic complications are still frequent in biopsy-proven GCA patients from southern Europe. The presence of other ischemic complications constitutes an alarm for the development of irreversible blindness. In contrast, a higher inflammatory response may be a protective factor against the development of cranial ischemic events.
 ...
PMID:Visual manifestations of giant cell arteritis. Trends and clinical spectrum in 161 patients. 1103 76

Systemic vasculitis might present as a tumorlike lesion that initially could misdirect the correct diagnosis and the appropriate medical treatment. The aim of the present study is to summarize all reported cases of tumorlike presentation in systemic vasculitides, in order to have comprehensive data on the characteristics of this unusual phenomenon. We report 4 cases of systemic vasculitis presenting as a tumorlike lesion. In addition, we performed a MEDLINE search of all English-language papers published from 1966 to 1999, looking for vasculitis presenting as tumorlike lesion. Details were included concerning vasculitis classification, specific characteristics, location of the "tumor," the presence or absence of systemic involvement, and whether surgery was performed before diagnosis. Seventy-nine cases of vasculitis presenting as a tumorlike lesion were found in the literature, in addition to the 4 new cases described. The average age of the reported cases was 50.5 +/- 15.8 years, and 51% were female. In 82% of the cases the "tumor" was associated with constitutional symptoms and elevated erythrocyte sedimentation rate (ESR). The most common vasculitis categories with tumorlike presentation were Wegener granulomatosis (WG; 28 cases) and giant cell arteritis (GCA; 17 cases). In almost half the patients, surgery was performed before diagnosis. All patients with GCA presented with either a breast or an ovarian tumor. The most common location of a tumorlike lesion was the breast (22%), followed by central nervous system lesions (16%). Other frequent locations were the ovary (10%), caused exclusively by GCA and polyarteritis nodosa (PAN), and the male genitourinary system, almost all caused by PAN. Including vasculitis in the differential diagnosis of a tumorlike lesion might lead to an earlier diagnosis and consequently to prompt and appropriate treatment, avoiding needless operations. Constitutional symptoms and elevated ESR should alert clinicians to the possible diagnosis of vasculitis rather than a tumor. The association of GCA with ovarian pseudotumor is distinct and has not been emphasized before. We therefore suggest that GCA should be included in the list of differential diagnosis of an ovarian or breast tumor in an elderly woman, particularly when systemic symptoms and parameters of inflammation are present.
 ...
PMID:Systemic vasculitis presenting as a tumorlike lesion. Four case reports and an analysis of 79 reported cases. 1114 33

In summary, systemic vasculitides constitute a heterogeneous group of overlapping diseases that are somewhat more common than previously considered. Although the causes of vasculitis are largely unknown, epidemiologic studies have implicated geographic, genetic, and environmental factors. Ethnicity, various genes such as those of the MHC, gender, and environmental factors seem to account for the different incidence rates of these syndromes. GCA is the most common vasculitis in elderly people from Western countries. Small-sized cutaneous vasculitides, particularly HSP in children and HV in adults, are also common diseases. Increased physician awareness and the routine use of ANCA tests may contribute to an increase in the recognition of conditions such as WG and MPA.
 ...
PMID:Epidemiology of the vasculitides. 1172 61

Cytokines are small proteins that serve as chemical messengers between cells, regulating cell growth and differentiation, tissue repair and remodeling, and many aspects of the immune response. Cytokines are instrumental in determining the nature, magnitude, and duration of inflammatory reactions and, as such, represent ideal targets for interfering with pathogenic processes. In OCA and PMR, cytokines are encountered in two locations, the inflammatory infiltrates accumulating in the arterial wall and in the circulation. IL-6, a cytokine involved in stimulating acute-phase responses, is located upstream of many of the laboratory abnormalities considered helpful in diagnosing and managing GCA/PMR, including elevated ESR and CRP. IL-6 has the potential to be helpful in predicting disease severity and may allow for a tailoring of immunosuppressive therapy. There is evidence suggesting that IL-6 outperforms other chemical markers in detecting disease activity and could, therefore, have a role in monitoring treatment. Interesting pathogenic clues have been derived from studies of cytokines produced in the vascular lesions. IFN-gamma has emerged as a key regulator in determining the nature and direction of the inflammatory response. IFN-gamma appears to be critically involved in modulating the process of intimal hyperplasia, the most destructive consequence of vasculitis, and, as such, emerges as a prime target for novel therapeutic approaches.
 ...
PMID:Cytokines in giant-cell arteritis. 1208 74

Macrophage migration inhibitory factor (MIF) is a central proinflammatory cytokine that regulates innate and adaptive immune responses. To evaluate its role in primary vasculitides, we determined MIF by enzyme-linked immunoassay in the sera of patients with Wegener's granulomatosis (WG; n=26), microscopic polyangiitis (MPA; n=10), polyarteritis nodosa (PAN; n=9) and giant cell arteritis (GCA; n=11). Healthy controls (n=26) and patients with sarcoidosis (n=14) were studied in parallel. Serum levels of MIF were significantly higher in patients with WG (median 41.1, range 3.2-120 ng/ml) than those in healthy controls (6.0, 0.015-36.5 ng/ml; P<0.001) and in patients with sarcoidosis (13.8, 0.015-67.1 ng/ml; P<0.05). MIF values were higher in MPA patients (29.5, 9.9-69.4 ng/ml; P<0.01) in comparison with those in healthy controls. In particular, increased levels of MIF were associated with active disease as assessed by the Birmingham Vasculitis Activity Score. Sequential studies showed decreased levels of MIF after initiation of immunosuppressive therapy, with clinical improvement in WG and MPA patients. In contrast, serum levels of MIF were not significantly elevated in patients with PAN and GCA. The results suggest that MIF contributes to the inflammatory process and correlates with disease activity in antineutrophil cytoplasmic antibody-associated vasculitides.
 ...
PMID:Relationship between serum levels of macrophage migration inhibitory factor and the activity of antineutrophil cytoplasmic antibody-associated vasculitides. 1639 84

To provide a clinicopathologic review of vasculitis confined to the female genital tract, we describe three cases, and we searched PubMed from 1965 to 2006 with analysis of all relevant articles. We identified 118 additional cases in the literature of whom 108 had isolated necrotizing vasculitis similar to classical polyarteritis nodosa (PAN-type), and 10 presented isolated giant cell arteritis (GCA-type) of the female genital tract. In most cases, arteritis was discovered surprisingly. The mean age of these patients was 48.6 years for the PAN-type and 64.1 for the GCA-type. Vasculitis affected a single organ in 88 (81.5%) cases of which 71 (65.7%) involved the cervix in the PAN-type, whereas it was limited in the myometrium in five (50%) cases in the GCA-type. There was no progression to a systemic vasculitis in 99.1% of the cases. A comparable favorable outcome was reported after surgery in all cases with a mean follow-up of 40.8 months. We conclude that isolated vasculitis of the female genital tract is a distinct condition, with two different patterns but a similar favorable outcome after resection. Its knowledge is needed to avoid aggressive evaluation and therapy.
 ...
PMID:Isolated vasculitis of the female genital tract: a case series and review of literature. 1791 48

Large vessel vasculitis is characterized by local inflammation of the vessel wall of the aorta and its first and second degree branches. Giant cells are found regularly. The process leads to stenoses and occlusions as well as to aneurysmatic dilatation. Recent progress in vascular imaging (colour Doppler ultrasound, MRI and PET) allows to detect Takayasu arteritis at an earlier stage and to reveal the involvement of larger arteries in temporal arteritis (giant cell arteritis, GCA). With increasing frequency, a subtype of predominantly female and less aged GCA patients without cranial symptoms is noticed in whom obstructions are prevailing in the proximal arm vessels or in the aorta. The latter may be affected exclusively. Initial therapy is based on corticosteroids. In GCA, methotrexate hitherto is the only other immunosuppressive drug that has proved to be helpful. Disease activity has to be controlled both by laboratory parameters and vascular imaging.
 ...
PMID:[Large vessel vasculitis]. 1956 64


1 2 3 4 Next >>