Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound   Target Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound

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Query: EC:1.11.1.7 (peroxidase)
65,474 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 74-year-old man was admitted to our hospital because of edema of the lower legs, fever, and increasing fatigue. Laboratory evaluation revealed proteinuria, microhematuria, leukocytosis, thrombocytosis, anemia, a high level of C-reactive protein. A test for myeloperoxidase-antineutrophil cytoplasmic antibodies was highly positive. Microscopic polyarteritis nodosa was diagnosed and therapy with prednisolone was begun. Examination of a renal biopsy sample showed necrotizing crescentic glomerulonephritis. A chest roentgenogram and CT scan disclosed bilateral basilar interstitial changes. Six months later, the patient was admitted again because of disturbance of consciousness, malnutrition, and hyponatremia. After admission, alveolar infiltrates developed in the right lung and the patient died on the 5th hospital day as a result of respiratory failure. An autopsy revealed Candida pneumonia of the right lung and massive intra-alveolar hemorrhage, which was believed to have caused the respiratory failure. Other findings were usual interstitial pneumonia, cellular small-vessel angiitis in the lungs, and healed angiitis in the kidneys and liver. In this case of microscopic polyangiitis and chronic interstitial pneumonia, steroid therapy was effective against the angiitis, but the patient died of an opportunistic infection and alveolar hemorrhage.
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PMID:[Microscopic polyangiitis and pulmonary fibrosis in a patient who died of Candida pneumonia and intra-alveolar hemorrhage]. 936 70

Microscopic polyarteritis nodosa (mPAN) is a rare disorder in pediatric field of systemic small vessel vasculitis, and affects skin and musculoskeletal system accompanied by progressive necrotizing glomerulonephritis. We described here a 15-year-old boy with positive anti-neutrophil cytoplasmic antibody (MPO-ANCA) and severe mPAN, who was effectively treated with intravenous methylprednisolone pulse therapy followed by monthly cyclophosphamide pulses for 1 year. The histologic examination of renal biopsy specimen showed a severely desolated disease characterized by fibrinoid necrosis, crescent formation of most glomeruli and interstitial infiltration of inflammatory cells. The elevated titers of MPO-ANCA were useful markers for diagnosis, and the serial determinations of the antibody titers were indicative of disease activity. Moreover, dramatic clinical improvement after the induction of the combinatorial therapy and the disappearance of MPO-ANCA was correlated in the disease course. In this report, the serial determination of MPO-ANCA constituted a useful diagnostic tool and a sensitive marker of disease activity.
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PMID:[A child of microscopic polyarteritis nodosa effectively treated with intravenous methylprednisolone pulses and serial cyclophosphamide pulse therapy]. 1053 84

Microscopic polyangiitis, Wegener's granulomatosis, Churg-Strauss syndrome, and pauci-immune necrotizing glomerulonephritis share pathogenic, pathological, and clinical features. They all involve capillaries, venules, arterioles, and small arteries. Approximately 90% of patients have autoantibodies either to myeloperoxidase (MPO-ANCA) or to proteinase 3 (PR3-ANCA). The clinical manifestations of ANCA-small vessel vasculitis are protean. These can be limited to the kidney alone, or may involve the upper respiratory tract, the lungs, the skin, or a number of other organs in various combinations. The characteristic feature of the glomerular lesion is a focal necrotizing glomerulonephritis associated with crescent formation and little or no glomerular staining for immunoglobulin by immunofluorescence microscopy. The renal manifestations can present as a rapidly progressive glomerulonephritis or that of a more indolent, remitting, and relapsing course that leads to substantial glomerulosclerosis. The two main prognostic markers of the long-term outcome are the presence of pulmonary hemorrhage (which accounts for at least half of all deaths) and the entry serum creatinine. The higher the entry serum creatinine, the higher the risk of developing end-stage renal disease. The treatment of ANCA-small vessel vasculitis and glomerulonephritis rests primarily on the use of induction high-dose corticosteroids and cyclophosphamide. Patients with pulmonary hemorrhage also benefit from plasmapheresis. With the use of an alkylating agent, the rate of remission is of the order of 75%, but relapses occur in about 30% of patients who achieve a remission, and in about 17% of patients after renal transplantation. Despite the improved outcome of patients with ANCA vasculitis in the recent decade, their long-term prognosis continues to be primarily determined by a rapid diagnosis, and the prompt institution of therapy.
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PMID:ANCA glomerulonephritis and vasculitis: a Chapel Hill perspective. 1085 33

Microscopic polyangiitis is a non-granulomatous necrotizing vasculitis involving small vessels. Clinical manifestations are highly polymorphic, but rapidly progressive glomerulonephritis is one of the most frequent and most severe manifestations of the disease. Biopsy of an affected organ and detection of circulating anti-neutrophil cytoplasmic antibodies (ANCA) are key elements for the positive diagnosis of microscopic polyangiitis. Biopsies can disclose necrotizing vasculitis affecting small vessels, without granulomas and without immune deposits. ANCA are very specific for microscopic polyangiitis, Wegener's granulomatosis and Churg-Strauss syndrome when they are positive by indirect immunofluorescence and are directed against myeloperoxidase or proteinase 3. Such ANCA are found in about 70% of patients with microscopic polyangiitis. Treatment of severe forms of microscopic polyangiitis is based on the administration of pulse methylprednisolone, oral corticosteroids and cyclophosphamide. In the mildest forms of the disease, one can probably try either to competely avoid using immunosuppressive drugs, or to replace cyclophosphamide with azathioprine. Treatment induces a complete remission of the disease in more than 90% of cases, but about 30% of the patients will experience a relapse, and progressive worsening of renal function can occur in patients with severe chronic renal failure.
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PMID:[Microscopic polyangiitis]. 1089 71

Microscopic polyangiitis is a very rare disease characterized by the lesions of arteriolae, venulae and capillaries--mainly of the kidneys and lungs, but also of other systems and organs. The elevated titer of anti-myeloperoxidase ANCA is very important immunological indicator. The main changes in our patient were related to the lung bleeding and rapidly progressive glomerulonephritis. The treatment has started according to the standard Fauci scheme adjusted to the level of disease severity and the age of patient (prednisone 60 mg/24 h, along with the gradual dosage decrease, cyclophosphamide 150 mg/24 h) and has lead to the clinical-laboratory remission. The patient had the leukocyte values irregularly controlled during the immunosuppressive therapy and agranulocytosis thus caused was not spotted in time, leading to the inadequate treatment of pneumonia that brought on the lethal outcome.
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PMID:[Microscopic polyangiitis]. 1154 60

Microscopic polyangiitis (MPA), or microscopic polyarteritis, is an idiopathic small vessel vasculitis that frequently causes glomerular damage and renal failure and skin and lung damage in many cases. The renal lesions include focal necrotizing glomerulonephritis, extracapillary proliferative (crescentic) glomerulonephritis, and tubulointerstitial infiltration with polymorphonuclear leukocytes and lymphocytes. MPA often is associated with the presence of antineutrophil cytoplasmic autoantibody (ANCA) (myeloperoxidase positive) as a diagnostic marker. MPA commonly is regarded as a serious condition that places the survival of the kidneys and the patient at risk. Typically, there is a prodrome of some weeks to months, with rapid decline in renal function and dialysis as a potential outcome if intensive immunosuppressive treatment is not given or is delayed. We describe an otherwise typical case of MPA occurring in a 52-year-old woman presenting with multisystem disease, antimyeloperoxidase ANCA antibodies, renal impairment, and necrotizing crescentic glomerulonephritis in whom this usual sequence of events was not followed because the patient refused steadfastly to have any treatment for nearly a decade. Renal function remained stable for nearly 10 years, although there were persistent proteinuria, microscopic hematuria, and antimyeloperoxidase ANCA antibodies. A late renal-pulmonary relapse occurred, and immunosuppression was permitted only briefly. Prolonged renal and patient survival in the absence of immunosuppressive treatment has been reported rarely in this context.
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PMID:Spontaneous and protracted partial remission of microscopic polyangiitis. 1197 57

Crescentic glomerulonephritis (CGN) is a clinicopathologic entity which is characterized by severe renal dysfunction of rapid onset with glomerular crescents. Type III CGN is associated with the absence of glomerular immune complex deposition (pauci-immune) and is associated with antineutrophil cytoplasmic antibody (ANCA). Microscopic polyangiitis and idiopathic pauci-immune necrotizing glomerulonephritis (NCGN) are strongly associated with ANCA directed against myeloperoxidase (anti-MPO). We describe here an unusual pediatric patient with MPO-ANCA-associated rapidly progressive glomerulonephritis (RPGN), emphasizing the management and outcome of the disease.
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PMID:Successful renal transplantation in a child with ANCA-associated microscopic polyangiitis. 1274 92

Microscopic polyangiitis (MPA) is a systemic vasculitis histologically characterized by small-vessel involvement. Antineutrophil cytoplasmic antibodies (especially anti-myeloperoxidase antibodies) (MPO-ANCA) are often positive in serum. Although the skin is affected in 20-70% of patients, the precise description has been limited. This retrospective study analyzed clinical manifestations in patients of MPA with skin eruptions. Ten patients with skin eruptions diagnosed as MPA according to Chapel Hill consensus criteria consisted of 6 men and 4 women aged from 38 to 80 years (62.1 +/- 13.3). Clinical manifestations, laboratory data, and histological findings were examined. Purpura and petechiae in 6 patients, livedo in 2 patients, and erythema in 7 patients, especially erythema on the hands and\or fingers in 4 patients, were observed. Histological findings from the eruptions in 7 patients showed perivascular lymphocyte infiltration in the upper dermis in 4 patients, and infiltration of lymphocytes and a few neutrophils around small arteries in the middle to deep dermis in 2 patients and diffuse infiltration of histiocytes and lymphocytes in the middle dermis in 1 patient. Cutaneous involvements in MPA showed a wide spectrum of clinical and histological findings.
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PMID:Skin eruptions associated with microscopic polyangiitis. 1531 59

Microscopic polyangiitis is a systemic vasculitis affecting smal-l and medium-sized vessels and is characteristically associated with a focal and segmental necrotizing glomerulonephritis. It may present as a pulmonary-renal syndrome with rapidly progressive glomerulonephritis and alveolar hemorrhage, but the pattern of disease will vary according to the organ systems involved. Granulomatous disease of the upper or lower respiratory tract is not a feature, and its presence suggests the diagnosis of Wegener's granulomatosis. The etiology of the condition is unclear, but most patients have antineutrophil cytoplasm antibodies (ANCA) with specificity for either myeloperoxidase (MPO) or proteinase 3 (PR3), and there is increasing evidence that these may be pathogenic. Current treatment includes an induction phase using cyclophosphamide and steroids to attain remission, followed by a maintenance phase in which the levels of immunosuppression are gradually reduced. Azathioprine may be substituted for cyclophosphamide at 3 months. Adjunctive plasma exchange or intravenous methylprednisolone is used in the management of either or both severe renal disease and alveolar hemorrhage, and new evidence suggests that plasma exchange is more effective in recovery of renal function. Overall, 1-year survival in systemic vasculitis is around 85%, and up to 50% of patients relapse, although relapse is less common in those with MPO-ANCA. Newer therapies are being explored in an attempt to increase the efficacy and reduce the toxicity of treatment.
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PMID:Microscopic polyangiitis. 1608 96

Microscopic polyangiitis (MPA) is a rare and severe form of systemic necrotizing vasculitis associated with myeloperoxidase (MPO)-specific antineutrophil cytoplasmic antibody (ANCA). We previously reported significant association of HLA-DRB1*0901 with MPA. To define the susceptibility loci within the HLA region, we determined the genotypes of HLA-DQB1, DPB1, B and C in 50 patients with MPA and 77 unrelated Japanese controls. In addition to HLA-DRB1*0901, significant association of DQB1*0303 (allele carrier frequencies 50% in MPA, 29.9% in controls, odds ratio 2.35, P = 0.017) was detected. These alleles were in strong linkage disequilibrium (D' = 0.95, r2 = 0.82). Increased frequency was also observed for DPB1*0201, B*15111 and Cw*0303, which was at least partly accounted for by linkage disequilibrium with DRB1*0901 and DQB1*0303. These results indicate that DRB1*0901-DQB1*0303 haplotype represents the primary genetic risk for MPA within the HLA region in Japanese, and provides the basis that future functional studies on the role of HLA in MPA should target DR9, DQ9 and DR53 proteins encoded by this haplotype.
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PMID:Association of HLA-DRB1*0901-DQB1*0303 haplotype with microscopic polyangiitis in Japanese. 1620 5


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