3rd Tübinger glomerulonephritis Workshop

Fokal-segmental Glomerulosclerosis

W. Schoeppe: Epidemiology of focal sclerosing glomerulonephritis.

(Nieren Hochdruckkrh 1992 (21) 270 - 272).
Frequency of focal sclerosing glomenilonephritis is not exactly known. The disease can emerge from various separatly defined histopathological entities. Prognosis is poor. Literatur still does not provide sufficiently discriminating elements to improve our ktnowledge on the epidemiology of the disease. Key words: Focal sclerosing glomerulonephritis - incidence demographic conditions.

G. Stein, R. Fünfstück: Diagnosis of focal segmental glomerulosclerosis.

(Nieren Hochdrukkrh 1992 (21) 273 - 276).
Diagnosis of focal segmental glomerulosclerosis regardless of the uncertainties surrounding the pathogenesis of the condition and the unresolved controversy as to its correct classification within the primary glomerulopathies the term focal and segnnental glomerulosclerosis (FSGS) has been widely accepted to denote a particular disease entity that effects both adults and cfrildren. It manifests itself clinically by nephrotic syndrome, nonselective proteinuria, microscopic hematuria with cell dysmorphia, hypertension, not invariably corticosteroide resistance and progressive deterioration of renal function. Whereas humoral immunparameters are not changed, an alteration in suppressor cell activity was demonstrated and may in part reflect a more fundamental defect of the immunological abnormaliåes of FSGS. T1ie diagnosis has to be defined liistologically from renal biopsy specimens. Factors affecting the prognosis include mesangial hypercellularity, percentage occurrence of focal segmental and global sclerosis, segmental hyalinosis, tubulo-interstitial disease, and vascular disease. Immunohistological studies of FSGS show segmental positive fluorescence staining with IgM and IgG corresponding to the nodular hyalin deposits. The tubulointerstitial findings and the presence of nephrotic syndrome at the time of biopsy are the most important paranneters in identifying cases of FSGN with a potenrially pure prognosis. Key words: Focal segniental glomenilosclerosis - laboratory findings - immunological parameters - histological picture.

N. Braun, M. H. Weber: Proteinuria in focal segmental glomerulosclerosis.

(Nieren Hochdruckkrh 1992 (21) 281 - 291).
Focal segmental glomerulosclerosis (FSGS) has been recognized clinically as a cause of therapy-resistant, high molecular weight (glomerular), proteinuria, often leading co the nephrotic syndrome. Progression to end-stage renal disease does not reduce the proteinuria. Although the exact mechanism of proteinuria is yet not understood, a reduction of charge permselectivity as well as size-selective permselectivity has been discussed. Charge pernnselectivity is reduced by accumulation of neutralizing lipoproteins and cationic proteins in the glomeroilar basement membrane and the mesangium. The diminished size-selective permselectivity is due to an increase in glomeroilar volume representing a maladaption to reduced renal mass in order to maintain glomerular filtration. The intense tubular protein load associated with tvnbular obstrtnction and dilatation is followed by a rupture of the tubular basement membrane and extravasation of protein into the interstitium which may cause an interstitial cellular reaction. This might explain the rapid deterioration of renal function and the tubular component of proteinuria often seen from the onset of disease. Key wonds: Focal segmental glomertilosclerosis - FSGS proteinuria - hyperfiltration - lipids - tubular impairment - SDS-PAGE.

C. Wanner, I. Zäuner, P. Schollmeyer: Hyperlipoproteinemia and focal segmental glomerulosclerosis.

(Nieren Hochdruckkrh 1992(21) 292 - 295).
Progressive focal segmental glomerulosclerosis is often accompanied by the nephrotic syndrome and hyperlipidemia is a consistent feature. Characterisrically, total and LDL cholesterol are elevated as well as lipoprotein(a), while those with heavier proteinuria or marked hypoalbuminemia often demonstrate significant increases in triglyceride and VLDL cholesterol. The mechanism leading to the abnornnalities of lipids have also been vigorously debated. It appears that increased synthesis of lipoproteins is a factor, with a more predominant contribution perhaps due to decreased lipid catabolism. There are only limited studies of the incidence of atherosclerotic complications and ischemic heart disease. A number of indirect evidence, however, underlines a probably underestimated problem in the long-ternn follow-up. Experimental studies have suggested an important role of abnormal lipid metabolism as an integral factor in modulating progressive glomerular damage. The mechanism whereby lipids may amplify glomerular injury may include an interaction with macrophages, alteration in vascular and mesangial functions, changes in production of mediator substances and local modification of lipoproteins. Clinical studies on the value of long-ternn lipid lowering therapy in patients with proteinuria must provide insights into the potential role of renal-related lipid abnormalities in the progression of human renal disease. Key words: Nephrotic syndrome - glomerulosclerosis lipoproteins - LDL.

C. T. Grupp, J. Steffgen, H. E. Franz: Complications in focal glomerular sclerosis.

(Nieren Hochdruckkrh 1992 (21) 296 - 298).
In focal glomerular sclerosis with the exception of hypertension further complications like progressive renal insuffiiciency are associated with the presence of a nephrotic syndrome. Because of indications for similar pathomechanisms in focal glomerular sclerosis and systemic artherosclerosis some of these complications occurring in all glomerulopathies like hyperlipedemia, hypenension and enhanced thromboembolic tendency might have a special significanice in this disease. Key words: Focal glomenilar sclerosis - complications thrombosis - hypertension - nephrotic syndrome - infections.

H. Köhler: Secondary focal and segmental glomerulosclerosis.

(Nieren Hochdruckkrh 1992 (21) 299 - 302).
Focal and segmental glomerulosclerosis (FSGS) may arise secondary to a variety of disorders such as heroin abuse, HIV-disease, reflux nephropathy and in association with the loss of renal mass. Different mechanisms seem to be operative. In some diseases hyperfiltration is likely to be a relevant pathogenetic factor and in HIV-disease nephrotic syndrome and progressive deterioration of renal function is seen essentially in blacks or heroin-addicts. In addition, FSGS may be superimposed on other renal lesions, such as minimal change disease or mesangial-proliferative glomerulonephritis. Key words: Focal and segmental glomerulosclerosis - heroinabuse - HIV-disease - reflux-nephropathy - hyperfiltration.

D. Bach, J. Vogt, P. Heering, B. Grabensee: Therapy of focal sclerosing glomerulonephritis with cyclosporine A.

(Nieren Hochdruckkrh 1992 (21) 303 - 306).
A part from its use in organ transplantation, cyclosporine A has found its role in the therapy of autoimmune diseases. It may lead to a drastic decrease of proteinuria in nephrotic syndrome due to glomerular diseases. We report on the clinical courses of 7 patients with histologically proven focal sclerosing glomerulonephritis, who had a therapy either with cyclosporine alone or in combination with prednison. Six out of seven patients had a decrease in proteinuria, accompanied by a correspondent increase in serum albumine. Three patients developed a decrease of renal function under therapy. Control biopsies did not show any cyclosporine-associated damage, therefore the decrease of function is very likely due to the natural course of the disease. Key words: Focal sclerosing glomerulonephritis - cyclosporine A - nephrotic syndrome.

T. Risler, M. Przechera, N. Braun, G. A. Müller: Therapy-resistant nephrotic syndrome.

(Nieren Hochdruckkrh 1992 (21) 307 - 310.
Generally the therapy of a nephrotic syndrome should try to improve the underlying disease. If this attempt is unsuccessful, symptomatic teatment of the complications will be imperative. In the case that renal function is preserved over a longer period and thus results in resistant life-threatening protein loss, even an intervention such as a medical nephrectomy is indicated. Although the patient may finally end up on dialysis and may be transplanted later on with the chance ofarecurrenceofhis disease, he has a better chance to survive. Key words: Nephrotic syndrome - focal segnnental glomerulosclerosis - therapeutic failure - pharmacological nephrectomy.