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)

Analysis of 80 cases of SLE with renal involvement revealed the following. Angiitis involving arterioles and interlobular arteries was observed in 17 out of 42 cases with diffuse proliferative lupus GN. This was not observed in other types of lupus GN. Out of 17 cases of angiitis, 7 were complicated by thrombosis. Irregular deposits of immunoglobulins and complement were demonstrated in the vessel walls and in luminal thrombi and suggested an immune-complex origin. Fibrinogen was also demonstrable frequently. The vascular lesions were associated with severe glomerular and tubulointerstitial injury and a poor prognosis. A relatively favorable outcome was observed in SLE without renal angiitis.
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PMID:Prognostic significance of renal angiitis in systemic lupus erythematosus (SLE). 635 37

Renal vasculitis frequently presents itself as rapidly progressive glomerulonephritis, but its diagnosis may be hampered by the difficulty in demonstrating classic vasculitic lesions in renal biopsy specimens. Early diagnosis of renal vasculitis has been greatly enhanced by the advent of antineutrophil cytoplasmic autoantibodies (ANCA). On indirect immunofluorescence microscopy, cytoplasmic ANCA (C-ANCA) show cytoplasmic staining of alcohol-fixed neutrophils and are directed against proteinase 3 in the primary granules of neutrophils. Perinuclear ANCA (P-ANCA) show perinuclear staining due to redistribution of granular antigens, and are specific for myeloperoxidase in the primary granules of the vasculitic patients. C-ANCA are most frequently associated with Wegener's granulomatosis and P-ANCA, with "idiopathic" necrotizing and crescentic glomerulonephritis (renal-limited disease). Patients with microscopic polyarteritis may be associated with either P-ANCA or C-ANCA and there is a considerable overlap between Wegener's granulomatosis and microscopic polyarteritis in both clinical features and serologic patterns. ANCA are not only the markers for vasculitis but may also play a role in the pathogenesis by activating the neutrophils to attack target blood vessels. There is also a crude correlation between ANCA titer and the activity of vasculitis. ANCA-associated vasculitis responds well to steroid and/or cyclophosphamide therapy. Renal failure in these patients is frequently reversible if treated early. Long-term patient and kidney survival rates are good with proper treatment and are far better than those of the other causes of rapidly progressive glomerulonephritis. Potential morbidity of steroid and immunosuppressive therapy should be reduced by the use of low effective doses and by close clinical observation and management.
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PMID:Antineutrophil cytoplasmic autoantibody--associated glomerulonephritis: potentially reversible disease. 786 52

A case of self-limited renal vasculitis during pregnancy in a patient with a history of recurrent fetal loss and with a positive cardiolipin immunoglobulin G antibody test is described. The renal disease manifested as an acute renal failure in the second trimester of the patient's third pregnancy, concurrent with severe pre-eclampsia. Vasculitis is a rare manifestation of antiphospholipid syndrome that has been described mostly in peripheral arteries. Renal vasculitis, however, has not yet been reported in association with this syndrome. The full spectrum of renal involvement in antiphospholipid syndrome is presently being determined, and we suggest that renal vasculitis be included in that spectrum.
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PMID:Primary antiphospholipid syndrome and self-limited renal vasculitis during pregnancy: case report and review of the literature. 807 77

Renal vasculitis syndromes include particular characteristic changes in concentrations of some cytokines in plasma or urine. Preliminary results suggest that the systemic lupus erythematodes with affliction of the kidneys is specifically concomitted by the increase in IL-8, both in plasma and urine. ANCA-positive renal vasculitis syndromes appear to coincide with a typical increase in the synthesis of interleukin-6 in the kidneys. We suggest that the monitoring of individual cytokine levels in plasma and urine will enable to study in greater detail the immunopathogenesis of renal vasculitis syndromes and the extent of local production of cytokines which may cause further progression of renal lesions. (Fig. 4, Tab. 1, Ref. 10.).
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PMID:[Adhesion molecules and cytokines in vasculitides]. 862 Mar 22

The term 'vasculitides' encompasses a group of inflammatory disorders which may affect the kidney by damaging its blood supply. Although the kidney may be affected by many types of systemic vasculitis, renal involvement is particularly frequent in some forms of systemic necrotizing vasculitis which are considered primary, such as Wegener's granulomatosis and microscopic polyarteritis, including its 'renal-limited' variant. Renal vasculitis can occur at any age, but is seen particularly frequently in middle-aged and elderly subjects, in whom clinical presentation and prognosis are significantly worse. In addition to the specific problems related to age, this review focuses on a few aspects that are still the subjects of debate: classification, the role of ANCA, renal pathology and the treatment of renal vasculitis.
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PMID:Renal vasculitis. 905 38

We report a 54-year-old woman with systemic sclerosis who presented alveolar hemorrhage. She noticed shortness of breath in May 1997. She was diagnosed having interstitial pneumonitis and systemic sclerosis with sclerodactylia of bilateral fingers in December. She developed bloody sputum and dyspnea suddenly on March 12, 1999. Bloody lavage fluid with hemosiderin-laden macropharge was observed by bronchial fiber scopic examination. The elevated level of MPO-ANCA was detected without anti-DNA antibody. Administration of intravenous methylprednisolone(1g per day, 3 days) followed by 40 mg per day of prednisolone achieved complete response, and MPO-ANCA level was decreased. Mild proteinuria and microhematuria was detected on admission, but renal biopsy revealed no findings of crescent formation or angiitis. With coadministration of oral cyclophosphamide she doesn't have any renal involvement or recurrence of alveolar hemorrhage during these four years. MPO-ANCA level remains negative. Alveolar hemorrhage, which is uncommon pulmonary event in systemic sclerosis, is often coexist with renal angiitis in the context of "pulmo-renal syndrome". MPO-ANCA is said to be related to pulmo-renal syndrome and is sometimes induced by D-penicillamine. This patient is really important in considering the pathogenesis of alveolar hemorrhage because she had no renal vasculitic lesions or D-penicillamine intake.
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PMID:[Case of systemic sclerosis presenting with alveolar hemorrhage and positive anti-neutrophil cytoplasmic myeloperoxidase antibody(MPO-ANCA) without pathological renal involvement]. 1459 64

Alveolar hemorrhage (AH) is an important pulmonary manifestation of small vessel vasculitis because severe presentations are the most common vasculitic cause of early death. Renal vasculitis is usually present with AH; the combination is known as pulmonary-renal syndrome. Early diagnosis and intensive therapy are of particular importance to reduce early mortality and improve longer-term outcomes. The commonest immune-mediated cause of AH is anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) (80%), with other vasculitides, including systemic lupus erythematosus and anti-glomerular basement membrane disease accounting for 20%. One quarter of AAV patients develop AH, which when mild is associated with a good outcome, but mortality rises to 50% for cases with respiratory failure requiring ventilator support. The prognosis of AH in the other vasculitides is generally favorable, but cases are rare and experience is limited. Treatment follows similar regimens to those for other AAV presentations, although when severe there is widespread use of parenteral glucocorticoids together with plasma exchange. These interventions have developed empirically supported by a theoretical rationale but have not been validated by randomized clinical trials. Sepsis and cardiovascular and thromboembolic events are important early complications. and long-term follow-up is required to monitor for and prevent relapse and manage disease-related damage. A minority of cases develop on a background of pulmonary fibrosis, or progressive pulmonary fibrosis develops after vasculitis has gone into remission.
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PMID:Management of alveolar hemorrhage in lung vasculitides. 2167 19

Glomerulonephritis (GN) is a common manifestation of the antineutrophil cytoplasmic antibody-associated systemic vasculitides (AASV), which include granulomatosis with polyangiitis and microscopic polyangiitis. The level of renal involvement at presentation is highly predictive of survival and should be assessed early so that kidney function can be preserved. AASV patients with urinary sediment but normal function have a twofold greater risk of death than those with no renal involvement. Those with impaired renal function at diagnosis have a fivefold greater risk of death. Renal vasculitis is most prevalent in older patients, who have more severe disease and poorer prognoses. Renal biopsy not only establishes diagnosis but provides information on severity of renal-function impairment and prognosis. Induction of remission with cyclophosphamide is standard treatment. For patients with crescentic, rapidly progressive GN, adjunctive plasma exchange can promote renal recovery. Renal failure occurs in one-fourth of AASV patients after 3 to 4 years; 60% of patients receiving dialysis for acute GN can recover independent renal function. Renal transplant patients with vasculitis fare as well as renal transplant patients without vasculitis. Lastly, renal vasculitis is an independent risk factor for cardiovascular events.
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PMID:Renal disease in small-vessel vasculitis. 2320 40

A significant amount of cocaine used in the United States today is adulterated with levamisole. In some instances, prolonged use of contaminated cocaine is associated with the development of levamisole-induced vasculitis (LIV) with features of cutaneous vasculitis and agranulocytosis along with other constitutional symptoms and arthritis. We describe a case of a crack cocaine user with LIV, who developed significant renal disease secondary to crescentic glomerulonephritis, confirmed on renal biopsy. Renal vasculitis is an uncommon feature of LIV and significantly affects clinical course and management.
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PMID:Levamisole-Induced Vasculitis with Renal Involvement. 2626 14