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

A case of isolated vasculitis in the mammary gland of a 68-year-old female is described. Over a few months tender lumps occurred in both breasts. There were no signs of generalized disease. The sedimentation rate was normal and mammographic examination revealed no signs of malignant disease. The tumours were removed and histological examination showed inflammation, thrombosis and necrosis of medium and small arteries. We discuss the possibility of an isolated polyarteritis nodosa localized to the breast.
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PMID:Vasculitis of the breast. Case report and literature review. 257 47

Sixty-five patients with biopsy-proven Wegener's granulomatosis (WG), 54 with systemic vasculitis, 22 with relapsing polychondritis, 20 with sarcoidosis, 20 with malignant pulmonary lesions, and 15 with other conditions underwent determination of anticytoplasmic autoantibodies (ACPA) by the indirect immunofluorescence technique on neutrophil cytospin preparations to assess the specificity of ACPA for WG, their sensitivity in relationship to the extent and activity of the disease, and their value for follow-up of WG. Of these 65 patients with WG, 38 were ACPA positive. Two patients in the vasculitis group, best categorized as having microscopic polyarteritis, were ACPA positive. We obtained 125 serum samples from the 65 patients with WG and assigned them to one of two categories (limited or generalized), based on the extent of disease. Each of these categories was then subdivided into "active" or "in remission." Median ACPA titers were significantly different between active disease and remission in each category, as well as between active limited and active generalized disease. All patients whose disease changed from active to in remission had reductions in ACPA titer levels; those who experienced flares had titer increases. Patients with intercurrent illnesses or complications of treatment, mimicking WG flares, did not have titer increases. We conclude that ACPA determined by the indirect immunofluorescence technique is highly specific for WG. The sensitivity is dependent on the extent and activity of WG, and serial titer determinations are valuable in monitoring disease activity.
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PMID:Anticytoplasmic autoantibodies in the diagnosis and follow-up of Wegener's granulomatosis. 264 95

In normal serum complement prevents precipitation of antigen-antibody complexes (PIP). However rheumatoid arthritis (RA) serum contains an inhibitor of this complement-mediated function. We have undertaken two prospective studies in order to look for any relationship between the presence and levels of inhibitory activity in sera and synovial fluids (SF) of patients with RA and disease activity (study A), and the presence of systemic manifestations (nodules and vasculitis) of RA (study B). In study A, levels of inhibitory activity were highest in the sera and synovial fluids of patients with seropositive RA. However there was no correlation between the inhibitory levels and indices of generalised disease activity (articular index, erythrocyte sedimentation rate (ESR), haemoglobin, white cell and platelet counts). Local joint tenderness score correlated weakly with the inhibitory level in SF (P less than 0.05). There was no correlation, however, with either the SF protein concentration or white cell count. In study B, PIP was shown to be lower in patients with the systemic manifestations of RA than in those with purely articular manifestations. PIP was particularly low in those patients with vasculitis compared to those with subcutaneous nodules. Serum levels of inhibitory activity were highest in patients with vasculitis and lowest in those with articular disease only, whereas patients with nodules had intermediate levels. Our conclusion is that inhibition of immune precipitation is not associated with disease activity, but is associated with the extra-articular manifestations of RA. The inhibitory factor may play a role in the pathogenesis of RA.
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PMID:An inhibitor of complement-mediated prevention of immune precipitation in rheumatoid arthritis--relationship to disease activity and systemic manifestations. 379 56

We describe 25 cases of Wegener's granulomatosis presenting with solitary lung lesions to compare the clinical and pathologic findings in these cases with those of the more common multifocal disease and also to evaluate the clinical significance of solitary lung lesions occurring in the absence of extrapulmonary disease. The clinical findings in our patients with solitary Wegener's were similar to those in generalized disease. Men were affected slightly more often than women, and the average age of onset was 53 years. Likewise, no major pathologic differences were found between solitary and multifocal disease. Classic necrotizing granulomatous inflammation and necrotizing vasculitis were the most common findings, although other variants were occasionally encountered, including the eosinophilic variant (two cases), the bronchocentric variant (one case), and small-vessel vasculitis and capillaritis (one case). Three cases had prominent areas of bronchiolitis obliterans-organizing pneumonia. Progressive disease occurred in all seven patients who manifested solitary lung lesions without extrapulmonary involvement and were not treated because the diagnosis was initially unrecognized. Pathologists need to be alert to the possibility of Wegener's granulomatosis causing solitary lung lesions because treatment should be promptly instituted.
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PMID:Solitary lung lesions in Wegener's granulomatosis. Pathologic findings and clinical significance in 25 cases. 772 64

Renal angiomyolipoma is a tumor composed of a mixture of thick-walled blood vessels, smooth muscle, and mature adipose tissue. It may present as a single unilateral lesion or as a multifocal uni- or bilateral neoplasm. The histologic spectrum of angiomyolipoma is wide but as far as we know intratumoral fibrinoid vasculitis has not been described in the tumor. This is the first report of isolated intratumoral fibrinoid vasculitis observed in two nodules of a unilateral multifocal renal angiomyolipoma. The vasculitis arose in a hypertensive 62-year-old woman who presented with a history of dull right flank and low back pain of 3 months' duration. There were no signs of generalized disease. Immunophenotyping of the vascular cellular infiltrate disclosed abundant T lymphocytes, significant numbers of histiocytes, and absence of B lymphocytes. The diagnosis of isolated intratumoral arteritis depends on the exclusion of systemic disease. It is important to distinguish cases like this one to avoid misdiagnosis and to prevent unnecessary treatment.
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PMID:Isolated fibrinoid vasculitis in renal angiomyolipoma. 964 92

Successful maintenance therapy with mycophenolate mofetil (MMF) 2 g/d and low-dose oral corticosteroids (OCS) over a period of 15 mo was given to patients with Wegener's granulomatosis (WG) (n = 9) and microscopic polyangiitis (MPA) (n = 2). All patients had severe generalized disease with pauci-immune necrotizing glomerulonephritis and received standard induction therapy with oral cyclophosphamide and OCS for a mean of 14 wk until remission was achieved. Of 11 patients, only one WG patient relapsed in the 14th month of maintenance therapy. Maintenance therapy with MMF was able to further reduce grumbling disease activity as measured by the Birmingham vasculitis activity score (BVAS2) and proteinuria that were still present by the end of induction therapy. OCS could be reduced to a median daily dose of 5 mg and discontinued in three patients. Possible drug-related adverse effects were transient and included abdominal pain, respiratory infection, diarrhea, leukopenia, and a cytomegalovirus-colitis in one patient that was successfully treated with ganciclovir. It is concluded that MMF in combination with low-dose OCS is well tolerated and effective for maintenance therapy of WG and MPA. Long-term treatment with MMF in these diseases is attractive because of its low toxicity. MMF will have to be studied further and compared with cyclophosphamide or azathioprine maintenance therapy in randomized trials.
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PMID:Mycophenolate mofetil for maintenance therapy of Wegener's granulomatosis and microscopic polyangiitis: a pilot study in 11 patients with renal involvement. 1047 49

The immune response in Wegener's granulomatosis (WG) has been characterized as a predominant, potentially pathogenic Th1-like reaction by blood T cells and T-cell clones from diseased tissues. To elucidate further the immunopathogenic mechanisms, this study analysed the phenotypes of inflammatory infiltrates in frozen nasal biopsies with involvement of the upper respiratory tract only (localized or 'initial phase' WG) and with multi-organ involvement, including systemic vasculitis (generalized WG). The expression and production of Th1 and Th2 cytokines were examined in tissue specimens and peripheral blood mononuclear cells (PBMCs) of localized and generalized WG. The number of CD3+ T cells in inflammatory infiltrates ranged from 50 to 70%, together with approximately 30% CD14+ monocytes/macrophages. An average of 40% of T cells expressed CD26 in nasal biopsies of localized WG, compared with about 16% in specimens of generalized WG. In parallel, a higher number of interferon-gamma (IFN-gamma)-positive cells were detected in nasal tissue of localized than in generalized WG. PBMCs from localized WG similarly exhibited higher spontaneous IFN-gamma production in contrast to generalized WG (207 vs. 3 pg/ml, p<0.05). Interleukin-4 (IL-4) mRNA was found in higher amounts in generalized than in localized WG. IL-4 production was negligible in both disease and controls. In addition, both IL-10 mRNA and IL-10 protein levels of activated PBMCs from localized WG were elevated when compared with generalized disease (574 vs. 154 pg/ml, p<0.05) or healthy controls (574 vs. 246 pg/ml, p<0.05). It is conluded that in nasal tissues, mainly CD4+/CD26+ T cells as well as IFN-gamma-positive cells may support a polarized Th1-like immune response. Furthermore, the data suggest that this in situ immune response is already initiated and established in localized WG, accompanied by increased peripheral IFN-gamma and IL-10 production.
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PMID:Localized Wegener's granulomatosis: predominance of CD26 and IFN-gamma expression. 1095 8

In the pathogenesis of anti-neutrophil cytoplasm antibodies (ANCA)-associated vasculitis, T cell contribution is indicated by T cell-dependent ANCA production combined with the presence of T cells in inflammatory infiltrates. However, the exact pathogenic role of T cells in ANCA-associated vasculitis remains to be determined. The Th1/Th2 concept is useful for understanding T cell involvement in pathological processes. This review focuses on T cells and particularly the Th1/Th2 paradigm in ANCA-associated vasculitis. Most research has been done in Wegener's granulomatosis, where a shift in T cell response, from a Th1 pattern in localized disease towards a Th0/Th2 pattern in generalized disease, appears to occur. Although less thoroughly studied, data in Churg-Strauss syndrome and microscopic polyangiitis indicate that these diseases are predominantly associated with Th2 patterns. Further studies elucidating the true nature of the polarization towards Th1 or Th2 in ANCA-associated vasculitis are clearly needed.
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PMID:The Th1 and Th2 paradigm in ANCA-associated vasculitis. 1450 20

The primary systemic vasculitides are a group of diseases characterized by an inflammatory process of the vessel walls and classified according to the smallest vessels involved. Small vessel vasculitides comprise the largest subgroup divided into diseases with a pauci-immune vasculitis and ANCA and diseases with deposition of immunoglobulin without ANCA. ANCA-associated systemic vasculitides include Wegener's granulomatosis, microscopic polyangiitis comprising renal-limited vasculitis and Churg-Strauss syndrome. Diagnosis is based on clinical manifestation, ANCA-testing and histology. Beside the role of ANCA as a diagnostic marker many studies and animal models have focused on the pathogenic role. The treatment of ANCA-associated vasculitis has changed from a standardized "Fauci-protocol" to an individualized less toxic strategy taking into consideration disease severity) organ manifestation, age of the patient and individual risk factors (e.g. increased bone marrow toxicity in patients with renal insufficiency). For remission induction patients are sub-grouped according to limited or generalized disease with moderate or severe renal involvement. Thus cyclophosphamide is only used in patients with generalized disease or - regarding Churg-Strauss-syndrome - patients with risk factors. For maintenance of remission azathioprine should be used in most of the patients.
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PMID:ANCA-associated vasculitis: diagnosis, clinical characteristics and treatment. 1770 98

Wegener's granulomatosis (WG) is a multisystem granulomatous, necrotizing vasculitis of presumed autoimmune origin that affects small- to medium-sized blood vessels. The respiratory tract and kidneys are typically involved (Gross and Reinhold-Keller, "Clinical features of primary ANCA-associated vasculitis" in Oxford textbook of rheumatology, third edition, 2004). The limited form usually involves the head and neck, lacks renal involvement, and may not progress to generalized disease (Cassan et al., Am. J. Med. 49:366-379, 1970). Ocular involvement, which may be the initial manifestation, is often encountered and can result in significant morbidity and possibly blindness (Pakrou et al., Semin. Arthritis Rheum. 35:284-292, 2006). We report an unusual case of WG presenting as an orbital mass. The diagnostic triad of granulomatous inflammation with multinucleated giant cells, vasculitis, and necrosis was discovered on histopathology (McDonald and Edwards, JAMA 173:1205-1209, 1960).
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PMID:Orbital Wegener's granulomatosis: a case report and review of the literature. 1860 79


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