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A Vizjak

Publications and source records attributed to A Vizjak.

35 records · Page 2Linked to original sources

Immunotactoid glomerulopathy with unusually thick extracellular microtubules and nodular glomerulosclerosis in a diabetic patient.

It has recently been suggested that immunotactoid glomerulopathy be separated from much more common fibrillary glomerulonephritis by ultrastructural features of highly organized immune deposits containing tubules of more than 30 nm in diameter. We report and discuss the results of a light, immunofluorescence and electron microscopic study of a needle renal biopsy from a 75-year-old, non-insulin dependant diabetic female presented with nephrotic syndrome, hypertension and a progressive renal failure. A unique coexistence of nodular glomerulosclerosis, as traditionally ascribed to diabetes with a peculiar type of immunotactoid glomerulopathy was confirmed by the exclusion of amyloidosis, monoclonal gammopathies, systemic autoimmune diseases and cryoglobulinemia. Mesangial, scattered subepithelial and segmentally prominent subendothelial immune deposits were found highly organized in mostly parallel arrays of 40 to 91 nm thick tubules. The average thickness of 67 nm exceeds the average diameter of tubules in all other 11 published cases of immunotactoid glomerulopathy to date. By immunofluorescence, predominantly capillary wall, thick, ribbon-like glomerular deposits contained IgG, IgM, kappa and lambda light chains of equal intensity, C3, C4 and fibrin related antigens. Mild to moderate glomerular cell proliferation associated with nodular sclerosis has been assumed to be causally related to immunotactoid deposits.

Aged↗

Arteritis of both carotid arteries in a patient with focal, crescentic glomerulonephritis and anti-neutrophil cytoplasmic autoantibodies.

We report a 32-yr-old woman who suffered a stroke as a consequence of arteritis of both internal carotid arteries confirmed by selective carotid arteriography. Laryngeal inflammation and kidney biopsy proven focal crescentic glomerulonephritis were also present in this patient. Anti-neutrophil cytoplasmic autoantibodies with specificity for proteinase 3 were detected in high titre during the active phase of the disease. This overlap syndrome with features indicating both Takayasu's arteritis and Wegener's granulomatosis suggests a common pathogenesis for these diseases.

Adult↗

The dissociation of arterial hypertension and lupus glomerulonephritis in systemic lupus erythematosus.

In spite of several articles questioning the general opinion that arterial hypertension in patients with systemic lupus erythematosus (SLE) is only the consequence of lupus glomerulonephritis (LGN), this still remains the usual pathophysiologic explanation. The purpose of this study was to explore the correlations between hypertension and LGN and to assess the importance of hypertension control for the prognosis of patients. A retrospective analysis of 173 patients with SLE over a period of 14 years was performed. For most of the patients, data were available from regular follow-up visits over an average of 6 years. Our results show a dissociation of hypertension and LGN and an association of hypertension and renal dysfunction. Severe hypertensive renal vascular lesions correlated well with a decrease of renal function. Successful treatment of hypertension is therefore essential in order to prevent deterioration of renal function in patients with LGN.

Adolescent↗

Membranous nephropathy with severe tubulointerstitial and vascular changes in a patient with psoriatic arthritis treated with non-steroidal anti-inflammatory drugs.

We report on a patient who suffered from psoriasis vulgaris and psoriatic arthritis and developed nephrotic syndrome after long-term therapy with non-steroidal anti-inflammatory drugs. Membranous nephropathy with tubulointerstitial and vascular (intimal hyperplasia) changes were found on renal biopsy using optical, immunofluorescent and electron microscopy. Treatment with methylprednisolone "pulse" therapy (1 g/24 h during 3 days) and continuing with prednisone 0.5 mg/kg/24 h was ineffective and end-stage renal failure developed 6 months after the diagnosis had been established.

Adult↗

Renal extraglomerular vascular immune deposits in IgA glomerulonephritis.

Kidney biopsies of 425 patients with IgA glomerulonephritis were studied to reveal the incidence, composition and possible clinical significance of extraglomerular vascular immune deposits. IgA deposits were detected in 20 cases, IgM in 28 (in 5 together with IgA), C3 in 317 and no vascular deposits in 60 cases. C3 and IgA deposits were granular, resembling mesangial deposits, while IgM deposits were lumpy, similar to IgM deposits in sclerotic and hyalinized glomeruli. The incidence of vascular lesions in patients with IgA (30%) and C3 deposits (24%) was not significantly higher as compared to those without vascular deposits (20%), but was significantly higher in patients with IgM deposits (68%, P < 0.00004). Only the presence of vascular IgM deposits correlated significantly with severe glomerulosclerosis, arterial hypertension and elevated serum creatinine levels (all P < 0.001). We conclude that neither C3 nor IgA deposits, in spite of their suggested immune complex nature, contribute significantly to the development of vascular lesions. Lumpy IgM deposits, probably the result of insudation of plasma proteins into the blood vessel walls, were associated with advanced vascular lesions and glomerulosclerosis and are probably a part of non-immune mediated progression of IgA glomerulonephritis.

Adult↗

Renal function, protein excretion and pathology of Balkan endemic nephropathy. I. Renal function.

Renal function studies were performed on 59 patients who had the clinical criteria for Balkan endemic nephropathy (BEN). They were divided into three groups according to DTPA clearance (DTPA). Group 1, 11 individuals, had a mean age of 41.6 years and DTPA greater than 100 ml/min. Group 2, 20 persons, had a mean age of 49 years and DTPA of 60 to 100 ml/min. Group 3 was made up of 28 people with a DTPA less than 60 ml/min and an average age of 50.4 years. No distinguishing specific or characteristic symptoms of BEN were found in any of the three groups. Anemia was not found to be an early indicator when compared to other forms of progressive renal disease. Proteinuria was minimal and intermittent in all three groups. Maximum concentrating ability was significantly reduced only in the third group. These features do not allow the clinical differentiation of BEN from other chronic progress tubulointerstitial nephropathies. The geographic isolation and familial nature of the disease, associated with minimal proteinuria make BEN a unique entity. Kidney size by ultrasound was decreased in all three groups, suggesting that this may be another early and characteristic feature to BEN.

Adult↗

Renal function, protein excretion, and pathology of Balkan endemic nephropathy. III. Light and electron microscopic studies.

This study was performed to evaluate histomorphological features of BEN in 50 kidney biopsies from patients who met the epidemiologic, clinical and laboratory criteria for BEN. This is the first such study reported in detail. The patients were divided into three groups based on the DTPA clearance values: group 1, greater than 99 ml/min, group 2, 51 to 99 ml/min, and group 3, 29 to 50 ml/min. All patients in all groups had an increase in proteinuria consisting of proteins less than 25,000 daltons. Multifocal interstitial sclerosis spreading from the superficial into the deep cortex was found in 49 (98%), tubular atrophy in 48 (96%), and global glomerular sclerosis with microvascular hyalinosis/sclerosis of sclerotic and atrophic changes were significantly increased when compared to age-related standards. An accelerated aging process may be assumed to occur in BEN. More peculiar additional findings with much lower incidence and extent included multifocal vascular and glomerular capillary changes resembling the chronic form of thrombotic microangiopathy group of diseases. These findings, together with the presence of arteriolar hyalinosis and tubulointerstitial sclerosis seen in patients with cyclosporine nephrotoxicity suggest that the mechanism of toxicity may be similar to BEN. We conclude that the histopathology is predominantly tubulointerstitial sclerosis without infiltrates. The combination of the histology, tubular proteinuria, geographic distribution, familial occurrence, and the remarkable association with papillary transitional cell carcinoma of the renal pelvis and ureters, qualifies BEN as a unique disease.

Adult↗

Renal function, protein excretion, and pathology of Balkan endemic nephropathy. IV. Immunohistology.

Few controversial observations on the deposition of immunoglobulins (Igs) and complement components in kidney biopsies of Balkan endemic nephropathy (BEN) patients have been reported. In the present study, direct or indirect immunofluorescence testing of the deposition of IgA, IgG, IgM, C3, C1q, C4 fibrin/fibrinogen, albumin, B2-microglobulin (beta 2m) and Tamm-Horsfall glycoprotein (THG) on frozen renal tissue sections was performed in 52 BEN patients. Glomerular findings were negative or mostly insignificant, with mild or moderate mesangial deposition of IgM in 16, IgA in 11, IgG in three, C3 in 15, C1q in two, C4 in one and fibrin/fibrinogen in two cases, respectively. The predominance of mesangial IgA deposits in five cases suggested IgA glomerulonephritis (GN) concomitant with BEN. Homogeneous lumpy or granular deposits in small extraglomerular vessels contained IgM in nine, C3 in 45, C1q in three, and C4 in one case. Focal linear or granular C3 was noted along the tubular basement membrane in eight cases. Resorptive droplets in tubular epithelial cells contained Igs and albumin, while complement, fibrin/fibrinogen and THG were found in tubular casts. There was no positive reaction with anti-beta 2m and anti-THG antibodies. According to these results, humoral immune mechanisms would not appear to play a pathogenetic role in BEN. However, immunohistologic examinations are important in recognition of possible concomitant immune complex-mediated GN.

Adult↗

Clinical presentations and vascular histopathology in autopsied patients with systemic lupus erythematosus and anticardiolipin antibodies.

OBJECTIVE: To examine histomorphological and immunohistological changes in an autopsy series of systemic lupus erythematosus (SLE) patients with or without anticardiolipin antibodies (aCL). METHODS: Fourteen SLE patients who died at our department from 1988 to 1996 were included. The patients' medical files were reviewed for the clinical history and the presence of IgG and IgM aCL. Autopsy samples of various organs, including regularly the kidneys, heart, brain and skin, were studied by standard histological methods and the direct immunofluorescence technique. RESULTS: Thirteen of 14 (93%) autopsied SLE patients were persistently positive for IgG aCL and had common overt thrombotic complications and/or other clinical features related to the antiphospholipid syndrome. Their autopsy tissue samples showed frequent occlusive vascular changes such as bland thromboses, thrombotic microangiopathy (TMA) related changes and arterial intimalfibrous hyperplasia. The immune complex related vascular changes were mostly unremarkable and present mainly in low aCL positive patients, who also had more aggressive types of lupus glomerulonephritis (GN). CONCLUSION: Increased IgG aCL were found in 13 out of 14 autopsied SLE patients who had predominant occlusive vascular histopathologic changes. The coincidence of bland thromboses with a characteristic TMA histopathology suggested two pathogenetic mechanisms associated with the presence of aCL, one related to abnormal coagulation and the other to endothelial cell injury. The extent of granular vascular immune deposits, typical of SLE, and the severity of lupus GN were inversely related to the aCL level.

Adult↗