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)

The solid phase Clq radioimmunoassay was used to detect immune complexes in sera from patients with systemic lupus erythematosus (14/25), rheumatoid arthritis (4/5), vasculitis (5/15), infective endocarditis (2/2), acute rheumatic fever (2/3), pre-eclamptic toxaemia (0/14), lung cancer (3/7), glomerulonephritis (26/98) and renal transplant patients (0/5). The best correlation with disease activity was seen in systemic lupus erythematosus and infective endocarditis where serial immune complex determinations were clearly of value in monitoring therapy. The findings in primary glomerulonephritis indicate only a limited usefulness of the assay in that serum immune complexes were detected in a minority (22/73) of patients with glomerular immune deposits. In particular the data do not support a role for Clq fixing immune complexes in the pathogenesis of membranous glomerulonephritis or in pre-eclamptic toxaemia.
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PMID:Serum immune complexes and disease. 15 40

Two cases with different and not previously described fatal renal complications during treatment with penicillamine are reported. A man with seronegative rheumatoid arthritis with features of systemic lupus erythematosus was treated with penicillamine for six months and developed a mild membranous glomerulonephritis and a severe renal vasculitis leading to uremia and death. A woman with primary biliary cirrhosis was treated with penicillamine for nine months and developed a nephrotic syndrome, the renal biopsy showing minimal change glomerulonephritis. The nephrotic syndrome responded to prednisone but the patient died, probably from septicemia. Penicillamine may thus cause glomerular damage without deposition of immune complexes. A restricted use of the drug is recommended.
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PMID:Fatal renal vasculitis and minimal change glomerulonephritis complicating treatment with penicillamine. Report on two cases. 76 Apr 1

The first recognized human kindred with hereditary deficiency of the fifth component of complement (C5) is described. The proband, a 20-year-old black female with systemic lupus erythematosus since age 11, lacked serum hemolytic complement activity, even during remission. C5 was undetectable in her serum by both immunodiffusion and hemolytic assays. Other complement components were normal during remission of lupus, but C1, C4, C2, and C3 levels fell during exacerbations. A younger half-sister, who had no underlying disease, was also found to lack immunochemically detectable C5. By hemolytic assay, she exhibited 1-2% of the normal serum C5 level and normal concentrations of other complement components. C5 levels of other family members were either normal or approximately half-normal, consistent with autosomal codominant inheritance of the gene determining C5 deficiency. Normal hemolytic titers were restored to both homozygous C5-deficient (C5D) sera by addition of highly purified human C5. In specific C5 titrations, however, it was noted that when limited amounts of C5 were assayed in the presence of low dilutions of either C5D serum, curving rather than linear dose-response plots were consistently obtained, suggesting some inhibitory effect. Further studies suggested that low dilutions of C5D serum contain a factor (or factors) interfering at some step in the hemolytic assay of C5, rather than a true C5 inhibitor or inactivator. Of clinical interest are (a) the documentation of membranous glomerulonephritis, vasculitis, and arthritis in an individual lacking C5 (and its biologic functions), and (b) a remarkable propensity to bacterial infections in the proband, even during periods of low-dose or alternate-day corticosteroid therapy. Other observations indicate that the C5D state is compatible with normal coagulation function and the capacity to mount a neutrophilic leukocytosis during pyogenic infection.
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PMID:Hereditary deficiency of the fifth component of complement in man. I. Clinical, immunochemical, and family studies. 93 97

Most recent information on the management of glomerular diseases is clustered in three areas. In nephrotic syndrome, interest has focused on the use of cyclosporine in steroid-resistant patients, treatment of progressive membranous nephropathy with alkylating agents, and symptomatic management of unresponsive cases with angiotensin-converting enzyme inhibitors. The most recent data on lupus nephritis establish the efficacy of intravenous cyclophosphamide in the long-term preservation of renal function and the lack of benefit of plasmapheresis in patients with severe disease. In rapidly progressive glomerulonephritis, the discovery of circulating antibodies to neutrophil cytoplasmic antigens has proved valuable in diagnosing certain forms of renal vasculitis. A rational approach to treating such patients is beginning to crystallize.
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PMID:Management of glomerular diseases of primary and secondary origin. 136 24

A 31 year old man first developed steroid-resistant idiopathic membranous glomerulonephritis in 1981. Stable normal renal function was maintained until August 1988 when he suffered a clinical relapse with heavy proteinuria and declining renal function. Immunosuppressive therapy with prednisolone and cyclophosphamide was instituted in an attempt to arrest this relapse. Despite this, he later developed acute renal failure with histological evidence of crescentic transformation of his nephritis. This unusual transformation was not associated with features of systemic vasculitis or positive anti-glomerular basement membrane and anti-neutrophil cytoplasmic antibodies.
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PMID:Crescentic transformation in primary membranous glomerulonephritis. 192 31

The relationships between autoantibodies to soluble cellular antigens and clinical features in systemic lupus erythematosus (SLE) were investigated in a large clinical-serological study. The absence of these precipitins in serum was associated with a low prevalence of vasculitis and membranous nephropathy (MGN). Other significant findings were the associations between nRNP antibody and Raynaud's phenomenon and MGN, SSB antibody and sicca complex, PCNA antibody and a young age at onset, and Bu antibody and an old age at onset. However, the most impressive findings were in DA1-positive patients which showed a unique prevalence of photosensitive skin lesions, lymphoadenopathy and hepatosplenomegaly. The present study confirms the usefulness of antibodies to soluble cellular antigens in the classification of patients with SLE.
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PMID:The clinical significance of autoantibodies to soluble cellular antigens in systemic lupus erythematosus. 196 95

Glomerular disease was assessed in persons who had renal biopsies and who were age 60 years and older in retrospective reviews of referral-based cohorts in Europe, the United States, and Japan. From a cumulative total of 514 patients reported from five centers, 337 (66%) patients had primary glomerular diseases and 177 (34%) had various secondary renal diseases. Amyloidosis was the largest histopathologic group among the secondary disorders. Idiopathic membranous nephropathy was the most common primary glomerulopathy (107/337 [32%] patients) and also comprised 46% of nephrotic patients. In our experience covering two consecutive 13-year periods from 1959 to 1984, there was an increase both in total number of patients, and in percentage more than 60 years old, with membranous nephropathy in the more recent study period compared with the earlier period reflecting a trend to more biopsy procedures that included older patients. Non-immune-mediated rapidly progressive glomerulonephritis (RPGN), thought by some to be more common in older adults, has been shown to represent a crescentic glomerulonephritis associated with systemic necrotizing vasculitis. Treatment with corticosteroids had a short-term favorable effect on renal function in this disorder over that achieved in antiglomerular basement membrane-associated or idiopathic RPGN. However, the 5-year patient survival was remarkably lower in older patients irrespective of the disease entity. On the other hand, age was not associated with progressive renal failure in patients with membranous nephropathy. Finally, minimal change glomerulopathy accounted for 35 of 204 (17%) nephrotic subjects, and complete remissions occurred after corticosteroid treatment at the same frequency (80% to 90%) observed in younger patients.
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PMID:Treatment of glomerulonephritis in the elderly. 222 Jul 75

This study reports the quantitative analysis of complement receptor (CR1) molecules on erythrocyte surface, the amount of immunoglobulin-containing material (IgG-IC and IgA1-IM) on the erythrocyte surface, and the concentrations of circulating immune complexes (IgG-CIC and IgA-CIC); also reported are the HLA phenotypes of 44 patients affected by various forms of glomerulonephritis (including 20 primary IgA nephropathy, 11 membranous glomerulonephritis, 9 lupus nephritis and 4 renal vasculitis). Erythrocyte CR1 molecules were found to be decreased (P less than 0.02) and erythrocyte IgG-IC were less than in controls (P less than 0.025) in lupus nephritis patients, whereas IgG-CIC were significantly greater (P less than 0.02). In patients affected by primary IgA nephropathy, mean erythrocyte CR1 concentrations were significantly decreased (P less than 0.02). Patients with impaired renal function had mean erythrocyte CR1 values significantly greater than those with normal renal function (P less than 0.002). Immunoglobulin-containing material on the erythrocyte surface was not significantly increased, whereas the serum concentrations of both IgA-CIC and IgG-CIC were significantly increased (P less than 0.02). In membranous nephropathy erythrocyte CR1 molecules were quantitatively similar to control data and no increase in CIC was observed. Conversely, erythrocyte IgG-IC were significantly increased (P less than 0.01). No significant relationship among erythrocyte CR1 molecules, erythrocyte surface-associated immunoglobulins, CIC, and HLA phenotype was observed in any patient group.
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PMID:Complement receptor (CR1) and IgG or IgA on erythrocytes and in circulating immune complexes in patients with glomerulonephritis. 212 61

Camostat mesilate, a developed derivative of gabexate mesilate for oral use, was administered in a daily dose of 600 mg for 4 weeks to 17 patients with heavy proteinuria due to various nephropathies. Five patients had glomerulonephritis (3 patients with IgA nephropathy, one each with membranoproliferative GN and membranous nephropathy) and 3 had systemic vasculitis. These patients had been treated with glucocorticoid, cyclophosphamide, anticoagulants, and dipyridamole. Five patients had diabetic nephropathy and had been treated with conventional therapy including angiotensin converting enzyme inhibitors. Two cases with benign nephrosclerosis, one with Alport syndrome, and the rest with end-stage renal failure of undetermined cause were also included in this study. Urinary protein decreased promptly within 2 weeks (from 5.2 +/- 0.7 to 3.5 +/- 0.5, mean +/- SE, p less than 0.005), and serum total protein and albumin levels increased significantly. Serum creatinine levels did not change. Decreases in urinary protein excretion of more than 50% were observed in five out of eight patients with glomerulonephritis or systemic vasculitis, two out of five with diabetic nephropathy, and one with chronic renal failure. However, urinary protein excretion values remained at the same level in two patients with benign nephrosclerosis and a patient with Alport syndrome. We suggest that camostat mesilate caused a change in glomerular capillary permeability for macromolecules through its inhibitory effects on the kallikrein-kinin system, complement system, coagulation system, and platelet function, which contributed to the treatment of the various nephropathies.
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PMID:Effect of camostat mesilate on heavy proteinuria in various nephropathies. 279 62

To evaluate the diagnostic usefulness of gallium 67 scintigraphy in glomerular disease, 45 patients with various glomerulopathies, excluding lupus nephritis and renal vasculitis, were studied. Persistent renal visualization 48 hours after the gallium injection, a positive scintigram, was graded as + (less than), ++ (equal to), and +++ (greater than) the hepatic uptake. Positive scintigrams were seen in ten of 16 cases of focal segmental glomerulosclerosis, six of 11 cases of proliferative glomerulonephritis, and one case of minimal change, and one of two cases of membranous nephropathy; also in three of six cases of sickle glomerulopathy, two cases of diabetic neuropathy, one of two cases of amyloidosis, and one case of mild chronic allograft rejection. The 25 patients with positive scans were younger than the 20 with negative scans (31 +/- 12 v 42 +/- 17 years; P less than 0.01), and exhibited greater proteinuria (8.19 +/- 7.96 v 2.9 +/- 2.3 S/d; P less than 0.01) and lower serum creatinine values (2 +/- 2 v 4.1 +/- 2.8 mg/dL; P less than 0.01). The amount of proteinuria correlated directly with the intensity grade of the gallium image (P less than 0.02), but there was no correlation between the biopsy diagnosis and the outcome of the gallium scan. It was concluded that gallium scintigraphy is not useful in the differential diagnosis of the glomerular diseases under discussion. Younger patients with good renal function and heavy proteinuria are likely to have a positive renal scintigram regardless of the underlying glomerulopathy.
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PMID:Gallium 67 scintigraphy in glomerular disease. 319 76


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