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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We describe three patients with a well-established clinical diagnosis of systemic lupus erythematosus in whom the renal biopsy lesion unexpectedly is diagnostic of IgA nephropathy, not superimposed with any features of lupus nephritis. Whereas the clinical presentation and follow-up of renal disease in these patients indicate a relatively indolent course, the extrarenal manifestations of systemic lupus erythematosys have been relatively severe, and one patient died of systemic infection. IgA nephropathy hitherto has not been described in patients with systemic lupus erythematosus, and such an observation stresses that atypical glomerular lesions in these patients should raise the possibility of a nonlupus glomerulopathy.
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PMID:IgA nephropathy: a rare lesion in systemic lupus erythematosus. 773 41

A 9-year-old girl presented with mild proteinuria and microscopic hematuria. Renal biopsy disclosed highly organized fibrillary deposits in glomerular mesangial areas by ultrastructural examination. The microfibrils were 10-15 nm in diameter, up to 1,200 nm in length and in parallel array. They did not have a microtubular appearance. The diameter of the microfibrils was larger than that of amyloid fibrils. Congo red and crystal violet stains were negative. There was no clinical or serologic evidence of paraproteinemia, cryoglobulinemia, light chain disease or systemic lupus erythematosus. To date, most of the reported cases of fibrillary glomerulonephritis have occurred in adults; this disorder is extremely rare in children. We believe that this glomerulopathy can occur in the pediatric age group and should be considered in renal biopsy diagnoses.
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PMID:Fibrillary glomerulonephritis in a 9-year-old girl. 789 2

We report a case of hypocomplementemic urticarial vasculitis syndrome (HUVS) with membranous glomerulopathy in a 62-year-old man who had a 2-month history of secondary iritis. He was transferred to our hospital because of uncontrollable edema and respiratory dysfunction. Physical examination revealed anasarca, pulmonary edema, hypertension and urticaria-like eruption on his arms. Urinalysis, blood chemistry and serological studies showed massive proteinuria (10.5g/day) with numerous granular casts, hypoalbuminemia (1.5g/dl), renal dysfunction (creatinine; 1.6mg/dl, BUN; 86mg/dl), hypercholesterolemia (total cholesterol; 455mg/dl), positive results for antinuclear factor, microsome test, thyroid test, lupus anticoaglant, antithyroglobulin test and rheumatoid factor, but LE cell or double-strand anti DNA antibody was negative. Serum complement levels were persistently low as CH50 of 13 U/ml and Clq of 6.0 micrograms/dl. The patient serum precipitated with normal human Clq by immunodiffusion analysis, indicating the presence of anti-Clq antibody. Renal biopsy revealed membranous glomerulopathy with prominent fine granular deposition of Clq along the glomerular basement membrane by immunofluorescent study and subepithelial dense deposit by electron microscopy. Corticosteroid treatment was ineffective for hypocomplementemia and nephrotic syndrome. Acute subendocardial infarction occurred on the 25th hospital day and he died of acute respiratory distress syndrome on the 45th hospital day. Autopsy revealed leucocytoclastic vasculitis in the alveolar wall. HUVS was confirmed by clinical symptoms, such as iritis and urticaria-like eruption, serum anti-Clq antibody, the absence of any specific autoantibody for systemic lupus erythematosus (SLE) and leucocytoclastic vasculitis in the alveolar wall.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Nephrotic syndrome due to membranous glomerulopathy in hypocomplementemic urticarial vasculitis syndrome;--a case report]. 807 26

Fibrillary glomerulopathy is a category of glomerular disease that is defined by the ultrastructural feature of organized deposits of extracellular, nonbranching, microfibrils. The best-known disease in this category is amyloidosis, but cryoglobulinemia, light chain deposition disease, systemic lupus erythematosus, immunotactoid glomerulopathy, and diabetic fibrillosis may have similar ultrastructural findings and comprise the differential diagnosis of the fibrillary glomerulopathies. Because they have disease-specific therapeutic and prognostic implications, differentiating among these entities is important for nephrologists and nephropathologists. To aid the physician, we will review the fibrillary glomerulopathies using an algorithm based on morphology, clinical features, and serologic assessment. We believe this approach will prove to be practical and useful to the practicing nephrologist.
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PMID:The fibrillary glomerulopathies. 817 22

Immunotactoid glomerulopathy is characterized by the ultrastructural finding of fibrillary or microtubular deposits in patients without systemic diseases such as SLE, diabetes, paraproteinemias, cryoglobulinemia, or amyloidosis. These deposits correspond in most (but not all) cases to immunoglobulin and complement deposits as shown by immunohistochemical techniques. Different light microscopic patterns (mesangioproliferative, membranous, membranoproliferative, and crescentic) have been reported. Clinical presenting feature is characterized by proteinuria (often of nephrotic range), hematuria, and hypertension in most cases. Chronic renal failure requiring hemodialysis or transplantation is described in more than half the patients. Pathogenesis has not yet been elucidated and only some speculative hypotheses have so far been suggested. At present there is no clear evidence that we are dealing with a new pathologic entity, but larger series must be collected and studied in order to find a correct taxonomic collocation of this glomerulopathy.
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PMID:Immunotactoid glomerulopathy (ITGP): a not fully defined clinicopathologic entity. 851 98

A 25-year-old woman complained of anasarca and was admitted to Sakura National hospital on the presumptive diagnosis of nephrotic syndrome with 10.7 g of 24-hour urinary protein. At first, lupus nephritis with antiphospholipid antibody syndrome was suspected because of prolongation of APTT, existence of lupus anticoagulant and elevation of serum anticardiolipin antibody titer (IgM) in addition to positive ANA, lymphocytopenia and the biologically false positive test for syphilis (BFPTS). On day 28 of hospitalization, renal biopsy findings revealed severe endocapillary cell damage, such as swelling and proliferation of endothelial cells, fragmentation and double contour of the basement membrane walls, which were located only in the capillary lumens with a few thrombi. Immunofluorescent micrography revealed the absence of specific immunoglobulin or complement deposit. Therefore, the diagnosis of lupus nephritis was negated as these findings were suggestive of characteristic glomerulopathy due to primary antiphospholipid antibody syndrome. She was treated initially with oral prednisolone 60 mg and intravenous infusion of heparin 20,000 units daily. Moreover, cyclophosphamide 750 mg was administered intravenously as pulse therapy on day 13 as her serum level of CH50 had fallen suddenly, and hemodialysis was necessary because her renal function had deteriorated and she was suffering from cough and orthopnea with overhydratin. After the combined therapy, BFPTS disappeared and APTT returned to the normal range: dialysis treatment was not required further after the 4th hemodialysis. Thereafter, renal function improved and complete remission of nephrotic syndrome was obtained. This patient was a case of primary antiphospholipid antibody syndrome in which endothelial cell damage was located exclusively in the capillary lumens and pulse cyclophosphamide therapy in addition to prednisolone and anticoagulant was effective. We present this instructive case to promote understanding of the pathogenesis of primary antiphospholipid antibody syndrome.
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PMID:[A case of primary antiphospholipid antibody syndrome with severe nephrotic syndrome showing remarkable endothelial cell damage in the capillary lumen]. 919 67

We report a 51-year-old man diagnosed as having immunotactoid glomerulopathy (IT) who achieved partial remission after approximately 1 year of a low-dose prednisolone regimen. On admission, he was noted to show proteinuria, hypoproteinemia, and hypocomplementemia. On electron microscopy of the renal biopsy specimen, the mesangial and subendothelial areas were expanded because of the electron-dense deposits, which were represented by mostly straight and nonbranching hollow microtubule structures. The microtubular width was on average 22.0 nm. Clinical and histological findings did not support the diagnosis of amyloidosis, cryoglobulinemic glomerulonephritis, systemic lupus erythematosus, or paraproteinemias. Under treatment with oral prednisolone, 4 months later, the patient's serum albumin level increased from its lowest level of 2.3 to 3.6 g/dL, and CH50 from the lowest level of less than 6.3 to 32.4 U/mL. A 24-hour collection of urine showed that the protein had decreased from its highest level of 3.9 g to 2.0 g. This case suggests the effectiveness of long-term, low-dose prednisolone therapy for IT.
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PMID:Immunotactoid glomerulopathy: report of a case. 921 17

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.
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PMID:Lupus and nonlupus membranous glomerulopathy. Quantitative comparison of the subepithelial deposits and glomerular basement membrane including clinicomorphologic correlations. 922 53

We report a patient with unusual glomerulonephritis. A 24-year-old Japanese female was hospitalized in October 1995 because of nephrotic syndrome. Lobular form glomerulonephritis with mesangial proliferation associated with massive wide-spread accumulation of slightly eosinophilic, periodic acid Schiff-positive amorphous materials in the luminal side of the capillary walls and paramesangial area was observed in the renal biopsy specimen. Immunofluorescent study revealed massive strong staining for IgM and C4 along the capillary walls and in the mesangium. Deposits of IgA, IgG, C3 and fibrinogen were also observed. Electron microscopy showed normal thickness of the capillary basement membrane and a large amount of subendothelial and paramesangial electron dense, finely granular deposits without fibrils or tubular structures. There were no clinical or laboratory findings of systemic diseases, such as systemic lupus erythematosus and cryoglobulinemia. Therefore, we believed that this case involved an unusual idiopathic glomerular disease with massive subendothelial and paramesangial immune deposits. Glomerulonephritis in this patient appeared to be resistant to treatment with corticosteroids and that this glomerulopathy may be a progressive disease as shown during the 3-year observation. Furthermore, our patient had idiopathic hyperprolactinemia and subclinical hypothyroidism. However, the relationship between glomerulonephritis and endocrinopathy in our patient is unknown.
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PMID:Lobular form idiopathic glomerulonephritis with massive subendothelial and paramesangial immune deposits, a three-year follow-up case. 940 18

The authors present an account on contemporary knowledge of the diagnosis and differential diagnosis of fibrillar glomerulopathies. The latter are characterized by extracellular localized microfibrils and microtubules resp. in the glomeruli of the kidneys, their diameter being 8-60 nm. They are divided into amyloid and non-amyloid types. The others are classified according to the immunofluorescent finding into immunoglobulin positive and negative ones. The differential diagnosis is important in particular in immunoglobulin positive ones as they are present in serious diseases such as cryoglobulinaemia, monoclonal gammapathy, systemic lupus erythematosus and immunotactoid glomerulopathy.
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PMID:[Fibrillary glomerulopathy]. 960 86


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