Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

There is a spectrum of glomerular pathology in patients with severe lupus glomerulonephritis (GN) that includes (1) severe segmental GN (SEG) with greater than or equal to 50% of glomeruli involved by active segmental inflammation, (2) diffuse GN, and (3) membranous GN with associated severe SEG or diffuse GN (MGN + PGN). The clinical and laboratory characteristics at entry and at follow-up of 85 patients in a prospective therapeutic trial of plasmapheresis were examined to determine if these morphologic variants had prognostic implications. Addition of plasmapheresis to the therapeutic regimen did not affect outcome, and the two treatment groups were analyzed together. Patients with the three patterns of lupus GN were similar demographically and clinically, and they had similar serum creatinines at entry (SEG, 1.87 +/- 0.28 mg/dL [mean +/- SE], v diffuse GN, 2.11 +/- 0.21, v MGN + PGN, 2.12 +/- 0.26; P = 0.75). Although significant differences were found in the initial serum C3 (SEG, 46 +/- 5 mg/dL, v diffuse GN, 34 +/- 3, v MGN + PGN, 45 +/- 3; P = 0.02) and urinary protein excretion (SEG, 3.6 +/- 0.6 g/24 h, v diffuse GN, 6.0 +/- 0.7, v MGN + PGN, 6.7 +/- 0.9; p = 0.03), none of the clinical or laboratory data predicted the morphologic pattern of the glomerular lesion. Adverse outcomes included defined clinical stop points, nonfatal renal failure, and death. One half of the patients with MGN + PGN (13/26) had an adverse outcome, compared with 5/24 patients with SEG and 11/35 patients with diffuse GN. This trend was supported by actuarial analysis of outcomes showing that patients with MGN + PGN had the lowest cumulative proportion without adverse outcome after 175 weeks of follow-up (MGN + PGN, 0.40, v SEG, 0.77, v diffuse GN, 0.64; P = 0.04). We concluded that (1) at presentation, the specific glomerular lesion in severe lupus GN cannot be predicted on clinical or serological criteria alone; (2) on the basis of morbidity and mortality, cases with all three morphologic variants should be classified as severe lupus GN; and (3) patients with MGN + PGN appear to experience more adverse outcomes than patients with SEG or diffuse GN.
 ...
PMID:Clinical outcome of three discrete histologic patterns of injury in severe lupus glomerulonephritis. 265 May 38

After using a cellular digestion technique to extract cells from the basement membranes of frozen kidney tissue, we employed scanning electron microscopy to examine the acellular glomerular basement membranes (AGBM) from normal kidneys and from kidneys of patients with idiopathic membranous glomerulopathy (MGN). This method revealed, in the AGBM, previously unrecognized three-dimensional patterns of pathologic changes. These patterns correlated with increasing MGN stage as defined by Ehrenreich and Churg. On the epimembranous AGBM surface these patterns were composed of ridge-like trabeculae, irregular plaques, and reticulated crater-like deformities. The endothelial AGBM surfaces were smooth in stages I and II MGN, but in stage III MGN the endothelial surfaces were irregular and perforated. In contrast to lupus-related MGN, where some immune-complex-like material remained after cellular extraction, epimembranous immune-complex-like material in idiopathic MGN was extracted.
 ...
PMID:Scanning electron microscopy of the acellular glomerular basement membranes in idiopathic membranous glomerulopathy. 394 44

Frozen samples of minimal change glomerulopathy (MCG), and of membranous, segmental and diffuse lupus glomerulonephritis (MGN, SGN, DLGN) were studied to assess the distribution of tenascin (Ten), and the extradomains A and B (EDA- and EDB-) and oncofetal (Onc-) isoforms of cellular fibronectin (cFn). Cryosections were immunostained by the ABC method with specific monoclonal antibodies. In MCG, mesangial Ten and EDA-cFn reactions were increased. In MGN, mesangial Ten and EDA-cFn staining was enhanced except in segmental scars; convincing reactions were seen in cases with membranous transformation; spikes stained strongly. In SGN, variably intense staining for Ten and all cFn isoforms was seen in glomerular necrosis, proliferation and crescents; parietal epithelium EDA-cFn staining was noted. In DLGN, strong and extensive mesangial Ten and EDA-cFn staining was seen as were focal EDB- and Onc-cFn reactions. Parietal cells with and without crescents stained variably with all Mabs. Obsolete glomeruli were unreactive save for rare periglomerular Ten rims. Interstitial inflammation and fibrosis in MGN, SGN and DLGN had moderate to strong Ten and EDA-cFn staining with rare traces of EDB- and Onc-cFn. We conclude that enhanced Ten and EDA-cFn is a potentially reversible response to glomerular injury whereas the expression of EDB- and Onc-cFn apparently result from necrosis and/or cellular proliferation which lead to scarring. And, while mesangial cells are the major source of these molecules, epithelial cells might also partake in their synthesis.
 ...
PMID:Immunolocalization of tenascin and cellular fibronectins in diverse glomerulopathies. 768 61

We examined quantitatively 11 renal biopsy specimens from patients with class Va WHO lupus membranous glomerulopathy (LMGN) and 16 from patients with primary (nonlupus) membranous glomerulopathy (NLMGN) for whom both light and electron microscopy as well as immunofluorescence microscopy and full clinical data were available and compared these specimens with six cases of normal controls. In LMGN, subepithelial deposits resembled those seen in stage III of membranous glomerulopathy (MGN) according to the scheme proposed by Churg's group, i.e., for the present study only advanced cases of NLMGN (stage III according to this scheme) were selected. The electron micrographs were scanned in Primax flatbed A4 scanner and morphometric investigations were then performed by means of a computer image analysis system to compare glomerular basement membrane (GBM) thickness and the electron-microscopic density of the deposits in LMGN and NLMGN as well as to study whether these parameters could correlate with the clinical data. The study revealed that in LMGN the GBM thickness and the electron-microscopic density of the deposits were significantly increased in comparison with NLMGN. It should also be noted that both in LMGN and NLMGN groups the degree of proteinuria was closely correlated with the density of the deposits, but not with the GBM thickness. Moreover, no correlations were found between serum creatinine and the GBM thickness as well as between serum creatinine and the density of the deposits in these groups. In conclusion, the present data confirm that in LMGN and NLMGN proteinuria mainly depends on density of the subepithelial deposits. Furthermore, in cases with especially high density of these deposits systemic lupus erythematosus (SLE) should be taken into consideration, even if this etiology was not clinically suggested at the time of biopsy.
 ...
PMID:Lupus and nonlupus membranous glomerulopathy. Quantitative comparison of the subepithelial deposits and glomerular basement membrane including clinicomorphologic correlations. 922 53

Bowman's capsular and tubular basement membrane (TBM) deposits are an extremely unusual finding in non-lupus membranous glomerulopathy (MGN). We report three atypical cases of MGN with abundant Bowman's capsular and TBM deposits. In two cases, MGN was idiopathic; in the third case, MGN occurred in the renal allograft in the setting of HCV seropositivity. In addition to the usual glomerular capillary wall deposits, immunofluorescence and electron microscopy revealed extensive immune deposits within Bowman's capsule and TBMs, predominantly at the base of parietal and tubular epithelial cells. These cases suggest a potential pathomechanism of autoantibody to secreted epithelial antigens shared by visceral, parietal, and tubular epithelial cells. In all three cases, indirect immunofluorescence was unable to detect autoantibody to normal renal epithelial or matrix constituents. Furthermore, ELISA was unable to demonstrate circulating antibody to major extracellular matrix components. The implications of these findings for the pathogenesis of MGN are explored.
 ...
PMID:Membranous glomerulopathy with Bowman's capsular and tubular basement membrane deposits. 1114 Aug 9

We retrospectively reviewed the cases of 13 lupus nephritis children with pure membranous glomerulonephritis (MGN; Group A) and ten children with mixed proliferative and membranous nephritis (Group B). The children were identified through a territory-wide survey of patients between 1990 and 2003. All were ethnic Chinese. Age at diagnosis ranged from 3.7 to 18.6 years (Group A) and from 9.6 to 22.1 years (Group B). Female-to-male ratios were 12:1 (Group A) and 9:1 (Group B). Group A patients were more often nephrotic than Group B patients (11/13 vs. 5/10, p = 0.17). The glomerular filtration rate (GFR) at presentation was normal in all but two patients (one from each group). For induction, Group B patients consistently received prednisolone and cyclophosphamide; in contrast, the cytotoxic regimens in Group A patients varied from cyclophosphamide (five patients), mycophenolate mofetil (two patients), azathiorpine plus cyclosporine (one patient), and azathioprine alone (one patient). After a median follow-up of 7.6-7.8 years, one Group A patient had died of fulminant lupus. One survivor in Group B had a GFR < 90 ml/min per 1.73 m(2). Proteinuria persisted in five Group A patients and two Group B patients. In conclusion, Group B patients had good prognosis in terms of survival and proteinuria control. The only death occurred in Group A, and five of the 12 survivors in this group had persistent proteinuria. Further studies are needed to define the best treatment for pure lupus MGN.
 ...
PMID:Membranous lupus nephritis in Chinese children--a case series and review of the literature. 1962 43