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Abstract

Clinical Nephrology, Vol. 88 – Suppl. 1/2017 (S1-S6)

Tubular urinary indexes reliably distinguish between primary tubulointerstitial and primary glomerular diseases in patients referred for kidney biopsy

Andrej Škoberne¹, Špela Borštnar¹, Nuša Avguštin¹, Damjan Kovač¹, Andreja Aleš Rigler¹, Željka Večerić-Haler¹, Jernej Pajek¹, Mladen Krsnik², Nika Kojc³, Dušan Ferluga³, and Jelka Lindič¹

¹Department of Nephrology and ²Institute of Clinical Chemistry and Biochemistry, University Medical Center Ljubljana, and ³Institute of Pathology, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia
Aims: Kidney biopsy remains the gold standard for accurately diagnosing renal diseases. Urinalysis and assessment of renal function are the cornerstones for assessment of patients prior to biopsy. There is significant overlap in the results of routine urine parameters (proteinuria, erythrocyturia, leukocyturia) among different kidney diseases, which hinders the possibility of adequately estimating disease etiology prior to the biopsy. The aim of our study was to assess whether diverse markers of glomerular and tubular proteinuria – urinary albumin, IgG, α-1-microglobulin (α-1-m) and N-acetyl-β-D-glucosaminidase (NAG) – are capable of distinguishing between patients with primary tubulointerstitial (TID) and primary glomerular disease (GLOM). Methods: Our study is a retrospective, single-center, consecutive case series of patients referred for kidney biopsy. We analyzed routine urinalysis results performed on a second morning urine sample immediately prior to the biopsy. Results: Patients with TID had significantly higher values of α-1-m and NAG, with lower values of albumin and IgG in the urine compared to patients with GLOM. Three tubular urinary indexes had high sensitivity and specificity for distinguishing TID from GLOM: NAG/albumin, α-1-m/proteinuria, and α-1-m/albumin, with the highest values in the latter index (96.6% and 98.2%, respectively, cut-off point ≥ 0.33). Conclusions: Prior to kidney biopsy, tubular urinary indexes may present a valuable tool in distinguishing patients with TID from patients with GLOM. Correspondence to:
Andrej Škoberne, MD, PhD
Department of Nephrology
University Medical Centre Ljubljana
Zaloška 7, 1000 Ljubljana, Slovenia
Email: andrej.skoberne@ gmx.net

Abstract

Clinical Nephrology, Vol. 88 – Suppl. 1/2017 (S7-S9)

Determination of red blood cells in urinary sediment: Do pH and specific gravity of urine matter?

Nuša Avguštin¹, Alexander Jerman¹, Žiga Rotar², Špela Borštnar¹, Andrej Škoberne¹, and Jelka Lindič¹

¹Department of Nephrology and ²Department of Rheumatology,  University Medical Centre Ljubljana, Ljubljana, Slovenia
Introduction: It has been postulated that erythrocyte cell lysis in urine is common in the case of low urine specific gravity and high urine pH. We aimed to verify the influence of urine dipstick pH and specific gravity on erythrocyturia in the urine sediment in the case of positive dipstick hemoglobinuria. Methods: We retrospectively collected data on dipstick specific gravity, pH, and urine sediment analysis done by nephrologists in the clinical and research urine laboratory at the Department of Nephrology, University Medical Center Ljubljana. Results: During the 6-year observation period, we analyzed 843 second morning midstream urine samples with positive dipstick hemoglobinuria. Erythrocyturia in urinary sediment was detected significantly less often in urine samples with concomitant hemoglobinuria 1+ and pH < 6.0, (odds ratio (OR) 0.40; 95% confidence interval (CI) 0.21 – 0.76, p = 0.005). The difference was maintained in multivariate analysis including patient age, gender, and specific gravity (OR 0.32; 95% CI 0.15 – 0.65, p = 0.002). In samples with higher grade of hemoglobinuria (≥ 2+), the impact of cell lysis in the case of low pH was negligible. Specific gravity did not have any influence on erythrocyte detection in urinary sediment. Conclusions: Urinary pH < 6.0 impaired the detection of erythrocytes in urinary sediment, while the dipstick measured urine specific gravity did not have any impact on it. Urine samples with low-grade hemoglobinuria and low pH without erythrocyte detection in urinary sediment should be evaluated again to avoid false-negative results. Correspondence to:
Assist. Prof. Jelka Lindič, MD, PhD
Department of Nephrology
University Medical Centre Ljubljana
Zaloška cesta 7, 1000 Ljubljana, Slovenia
Email: [email protected]

Abstract

Clinical Nephrology, Vol. 88 – Suppl. 1/2017 (S78-S82)

Role of dry lean body mass for estimation of glomerular filtration rate in kidney transplant recipients

Špela Borštnar¹,², Jelka Lindič¹, Andrej Škoberne¹, Luka Ležaič³, Aljaž Sočan³, Aljoša Kandus¹, and Damjan Kovač¹

¹Department of Nephrology, University Medical Centre Ljubljana, Ljubljana, ²Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia ³Department of Nuclear Medicine, University Medical Centre Ljubljana, Ljubljana, Slovenia
Background: The use of equations that predict glomerular filtration rate (GFR) in patients with a kidney graft is still a matter of debate. The purpose of this study was to determine the level of accuracy of GFR equations and the relevance of dry lean body mass in the assessment of GFR. Methods: In a prospective clinical study, 100 patients with a kidney graft were included. Estimated GFR with Modification of Diet in Renal Disease equation (MDRD), Chronic Kidney Disease Epidemiology Collaboration equation (CKD EPI) with serum creatinine concentration (CKD EPI Cr), serum cystatin C concentration (CKD EPI CysC) or both (CKD EPI Cr-CysC), and creatinine clearance calculated with Cockcroft-Gault equation (CG) was compared with GFR measured by ⁵¹Cr-EDTA clearance (mGFR ⁵¹Cr-EDTA). Dry lean body mass (body mass without fat mass and body water) was measured with bioimpedance analysis. Results: All of the estimating equations overestimated mGFR ⁵¹Cr-EDTA by a significant degree (bias ± SD in mL/min/1.73m², 30% accuracy in brackets): CG 16.8 ± 14.1 (44%), MDRD 12.5 ± 15.3 (54%), CKD EPI Cr 15.1 ± 15.3 (50%), CKD EPI CysC 8.0 ± 16.6 (56%), CKD EPI Cr-CysC 10.3 ± 13.4 (55%). Dry lean body mass significantly correlated with mGFR ⁵¹Cr-EDTA, but not with estimated GFRs. Conclusion: The estimating GFR equations are neither accurate nor precise in renal transplant recipients. Dry lean body mass is an important parameter that could potentially improve the GFR estimation in this population. Correspondence to:
Špela Borštnar
Department of Nephrology
University Medical Center Ljubljana
Zaloška 7, 1000 Ljubljana, Slovenia
Email: [email protected]

Abstract

Clinical Nephrology, Vol. 88 – Suppl. 1/2017 (S101-S108)

Prevalence and risk factors for nonvertebral bone fractures in kidney transplant recipients – a single-center retrospective analysis

Alexander Jerman¹, Jelka Lindič¹, Andrej Škoberne¹, Špela Borštnar¹, Maja Martinuč Bergoč², Uroš Godnov³, and Damjan Kovač¹

¹University Medical Center Ljubljana, Department of Nephrology, Ljubljana, ²General Hospital of Nova Gorica, Department of Nephrology, Šempeter, and ³Faculty of Management, University of Primorska, Koper, Slovenia
Background: Complex and longstanding bone disease superimposed by harmful influences of immunosuppression is the reason for increased risk of bone fracture in kidney transplant recipients. The aim of our study was to analyze the incidence and prevalence of nonvertebral bone fractures and early (in the first post-transplant year) clinical and laboratory risk factors for suffering bone fracture in the long-term post-transplant period. Methods: Clinical and laboratory data as well as bone mineral density (BMD) measurements of 507 first kidney transplant recipients who were transplanted in the period from 1976 to 2011 were analyzed. Results: The mean age of included patients was 54.3 ± 12.0 years, there were 45% females, and mean time on renal replacement treatment prior to transplantation was 63.4 ± 43.6 months. The average observation time post-transplant was 9.7 years (1.4 – 36.3 years). Post-transplant, 64 (12.6%) patients suffered 89 nonvertebral fractures (44 patients suffered 1 fracture, 15 patients 2 fractures, and 5 patients 3 fractures). Patients with fractures had significantly lower late BMD of femoral neck in the period of 1 – 10 years post-transplant, had osteopenia and osteoporosis more frequently in the same time period, and higher serum alkaline phosphatase in the first year post-transplant. 13 patients (13/64, 20.3%) had major fractures. Patients with major fractures were significantly older than patients with no major fractures and had lower serum albumin. Frequency of treatment with bisphosphonate, calcium, or phosphate did not differ between the groups. Vitamin D supplement (active form in 98% of cases) was prescribed more frequently in the group without fractures, but this was not statistically significant. Conclusion: Fracture rate in our transplant patient population was comparable to that reported in the literature. Except for a higher level of serum total alkaline phosphatase in the fracture group, we found no other early laboratory risk factors for bone fractures. BMD at the femoral region 1 – 10 years after kidney transplantation but not BMD at the time of transplantation was a risk factor for nonvertebral fractures. Osteopenia and osteoporosis in the post-transplant period were found to be a fracture risk factor. Correspondence to:
Assoc. Prof. Damjan Kovač, MD, PhD, University Medical Centre Ljubljana, Zaloška cesta 7, 1000 Ljubljana, Slovenia
Email: [email protected]

Abstract

Clinical Nephrology, Vol. 88 – Suppl. 1/2017 (S27-S31)

Rituximab for the treatment of membranous nephropathy: a single-center experience

Andreja Aleš Rigler¹, Alexander Jerman¹, Aleša Orsag¹, Nika Kojc², Damjan Kovač¹, Andrej Škoberne¹, Špela Borštnar¹, Željka Večerić Haler¹, Nuša Avguštin¹, Radoslav Kveder¹, Dušan Ferluga², Alenka Vizjak², and Jelka Lindič¹

¹Department of Nephrology, University Medical Center Ljubljana, and  ²Institute of Pathology, Medical Faculty, University of Ljubljana, Ljubljana, Slovenia
Background: Treatment of idiopathic membranous nephropathy with rituximab was introduced more than a decade ago following experimental data that suggested involvement of B-cell-mediated reactions in its pathogenesis. It was a logical step towards a more selective therapy with less severe side effects as compared to the recommended first-line immunosuppressive therapy with corticosteroids and different immunosuppressant drugs. Methods: We retrospectively analyzed the anonymous data of patients who were treated with rituximab for idiopathic membranous nephropathy at our institution from January 2006 to July 2016. Daily proteinuria and serum creatinine were analyzed 3, 6, 9, and 12 months after rituximab application. The patients were divided into 4 groups according to proteinuria. We separately analyzed remission rates in the whole group and in groups with different quantity of daily proteinuria. Other history data and laboratory parameters were also compared within different groups of patients. Results: The study involved 29 rituximab treatments in 26 patients: 7 (26.9%) female and 19 (73.1%) male patients. In 16 out of 29 treatment cases (55.1%), patients had been previously treated with cyclophosphamide and steroids, or cyclosporine with low dose of steroids, or both. In 72.4% of patients, antiphospholipase A₂ receptor antibodies were present. In 2 cases of treatment (6.9%), patients received rituximab 375 mg/m² of body surface area in 3 and 4 weekly doses, respectively. In all other cases, repeated rituximab applications were given as needed according to the levels of circulating CD-20 B-cells. The total remission rate in our cohort of patients was 37.9% (11 out of 29 cases). The average serum creatinine in the group of patients who achieved remission was significantly lower than in the group without remission (86.5 vs. 155.5 µmol/L, p = 0.003). There was no difference in the duration of the disease prior to treatment with rituximab between the groups (53.6 and 56.4 months, respectively). The remission rate was highest in the group with daily proteinuria less than 4 g per day (83.3%). There were no remissions in the group of patients with daily proteinuria more than 12 g per day. Conclusion: The remission rate after rituximab treatment in our cohort of patients with idiopathic membranous nephropathy was lower than in other studies. The reason for this is possibly the application of a single dose of rituximab in the majority of patients, which might have been insufficient in patients with higher proteinuria. Correspondence to:
Andreja Aleš Rigler, MD, PhD
Department of Nephrology
University Medical Center Ljubljana
Zaloška 7, 1000 Ljubljana, Slovenia
Email: [email protected]

Abstract

Clinical Nephrology, Vol. 88 – Suppl. 1/2017 (S91-S96)

Treatment of antibody-mediated rejection of kidney grafts with bortezomib and/or rituximab compared to standard regimen: experience of Slovene National Center

Teja Oblak¹, Jelka Lindič¹, Jakob Gubenšek¹#², Radoslav Kveder¹, Andreja Aleš Rigler¹, Andrej Škoberne¹, Željka Večerić Haler¹, Špela Borštnar¹, Nuša Avguštin¹, Rafael Ponikvar¹#², Gregor Mlinšek¹, Dušan Ferluga²#³, Nika Kojc²#³, Uroš Godnov⁴, and Damjan Kovač¹

¹Department of Nephrology, University Medical Center Ljubljana, ²Faculty of Medicine, ³Insititute of Pathology, Faculty of Medicine, University of Ljubljana, Ljubljana, and ⁴Faculty of Management, University of Primorska, Koper, Slovenia
Background: The aim of our study was to determine outcomes of standard treatment of antibody-mediated rejection (ABMR) of kidney grafts as compared to the addition of bortezomib or rituximab. Methods: The cohort of this retrospective study included patients treated for ABMR of kidney grafts at our national center in the period of 2005 – 2017, divided into two groups: standard (ST) group treated standardly with plasmapheresis or immunoadsorption, intravenous immunoglobulins, and corticosteroids, and BR group treated with the addition of bortezomib and/or rituximab. Patient and graft survival at 2 years was analyzed by Kaplan-Meier method, and predictors of graft survival were analyzed by Cox regression. Results: There were 78 patients with ABMR (48 in the ST group, 30 in the BR group), 41 (53%) were men, mean age 49.5 ± 13.8 years. In ST and BR, respectively, mean serum creatinine was 267 ± 164 and 208 ± 112 µmol/L (p = 0.088), donor-specific antibodies (DSA) were positive in 75% and 97% (p = 0.022), and ABMR was acute in 50% and 33% (p = 0.149). Patient survival at 2 years was 89% in the ST and 100% in the BR group (p = 0.125). Cumulative proportion of kidney graft survival at 1 and 2 years was 67% and 53% in the ST group and 73% and 48% in the BR group, respectively, (p = 0.641). Chronic ABMR (HR 5.22, p = 0.004) was significant, while dialysis dependency at biopsy (HR 3.28, p = 0.072), serum creatinine at kidney biopsy (HR 1.003, p = 0.082), and presence of DQ-DSA (HR 3.37, p = 0.062) were borderline significant predictors of worse graft outcome. Infections were relatively common in both groups, with a trend towards more rehospitalizations due to infections in the first 6 months after treatment in the BR group (p = 0.066). In 5 patients (17%), treatment with bortezomib was discontinued prematurely due to cytopenia. Conclusions: Bortezomib or rituximab, added to standard treatment, did not significantly improve kidney graft survival and was also not associated with significant side effects, except cytopenia in some cases. Treatment of acute ABMR resulted in better graft survival than chronic ABMR. Correspondence to:
Assoc. Prof. Damjan, Kovač, MD, PhD
Department of Nephrology
University Medica Centre Ljubljana
Zaloška cesta 7, 1525 Ljubljana, Slovenia
Email: [email protected]

Abstract

The whole issue as pdf-file

Editors of the Proceedings: Damjan Kovač, Radoslav Kveder, and Andrej Škoberne

Abstract

Clinical Nephrology, Vol. 96 – Suppl. 1/2021 (S16-S23)

Complement activation and cryoglobulinemia are common in monoclonal gammopathy of renal significance: Data from a case series

Andrej Škoberne¹,³, Matevž Škerget²,³, Nika Kojc⁴, Maja Frelih⁴, Andreja Aleš Rigler¹,³, Željka Večerić Haler¹,³, Špela Borštnar¹,³, Nuša Avguštin Rotar¹,³, Damjan Kovač¹,³, Jelka Lindič¹,³, and Samo Zver²,³

¹Department of Nephrology, ²Department of Hematology, University Medical Center Ljubljana, ³Faculty of Medicine, and ⁴Institute of Pathology, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia
Introduction: Monoclonal gammopathy of renal significance (MGRS) denotes kidney diseases caused by monoclonal immunoglobulins in patients who do not have an overt hematological malignancy. Treatment is primarily directed against the underlying clone. Complement activation and cryoglobulinemia are known factors that can contribute to tissue damage, however, the full extent of their involvement is not clear. Materials and methods: This was a retrospective study including all patients with MGRS referred for consultation to our hospital over a 3-year period. Results: We identified 17 patients, of which 12 had proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID). Treatment with anti-clonal or immunosuppressive therapy was successful in 60% of patients with PGNMID, and treatment success was more common in patients with λ chain (100%) compared to κ chain deposits (20%). Serum markers of complement involvement were identified in 41% of all patients (88% of tested samples), most commonly high serum C5b-9 values or anti-factor H autoantibodies (both 24%). Patients with complement involvement did not respond well to treatment, which was unsuccessful in all treated patients with anti-factor H autoantibodies and 75% of patients with high serum C5b-9 values. Cryoglobulinemia was identified in 29% of all patients (71% tested samples) and was monoclonal in 40% of positive cases and mixed in 60%. None of the patients with cryoglobulinemia had organized deposits, however, there was a trend toward more common intramembranous deposits. In patients with monoclonal cryoglobulinemia both anti-clonal and immunosuppressive treatment were unsuccessful. All patients with mixed cryoglobulinemia were treated successfully with immunosuppressive therapy. Conclusion: Treatment of patients with PGNMID was successful in most cases. Complement involvement as well as monoclonal and mixed cryoglobulinemia were relatively common in our cohort, with the first two generally associated with unsuccessful treatment and the latter with successful treatment.Correspondence to:
Andrej Škoberne, MD, PhD, Department of Nephrology, University Medical Centre Ljubljana, Zaloška cesta 7, 1000 Ljubljana, Slovenia
Email: [email protected]

Abstract

Clinical Nephrology, Vol. 96 – Suppl. 1/2021 (S49-S55)

The predictive value of urinary erythrocyte morphology for proliferative glomerular kidney disease

Nuša Avguštin Rotar¹,³, Alexander Jerman¹,³, Andrej Škoberne¹,³, Špela Borštnar¹,³, Nika Kojc¹,²,³, and Jelka Lindič¹,³

¹Department of Nephrology, University Medical Center Ljubljana, ²Institute of Pathology, Faculty of Medicine, and ³Medical Faculty, University of Ljubljana, Ljubljana, Slovenia
Background and aim: Glomerular erythrocyturia (GlomEry) is usually associated with proliferative kidney diseases. In our retrospective cohort, we aimed to validate the predictive value of GlomEry criteria ≥ 40% dysmorphic erythrocytes (DysEry) or ≥ 5% acanthocytes (AcaEry) or at least 1 erythrocytic cast (CastEry) and of two new indices – the count of DysEry per high power field (HPF) and per microliter of urine (Stansfeld-Webb (SW)) method, for proliferative disease. Materials and methods: We included patients with erythrocyturia from 2015 to 2016. Based on renal histology, we divided them into a proliferative and a non-proliferative disease group. Urine erythrocyte count was done using SW and urinary sediment examination was carried out by skilled nephrologists. Sensitivity, specificity, and cutoff values were determined using ROC curves. Results: We included 90 patients (33% women), median age of 63 (IQR 51, 71) years. In the proliferative group, proteinuria was lower (2.4 vs. 6.6 g/day), and SW erythrocyturia was higher (174 (IQR 60, 353) vs. 44 (IQR 20, 67) × 106/L) than in the non-proliferative group. The threshold to differentiate between the proliferative and non-proliferative group was determined at ˃ 43% of DysEry (sensitivity 73%, specificity 79%, AUC 0.808) and at ˃ 2% AcaEry (sensitivity 71%, specificity 56%, AUC 0.647). No significant difference in CastEry was found between groups. Among tested parameters, the calculated number of DysEry/HPF > 6.7 (sensitivity 77%, specificity 92%, AUC 0.878), followed by DysEry/SW > 28 × 106/L (sensitivity 76%, specificity 86%, AUC 0.879), discriminated those two groups best. Conclusion: In concordance with known GlomEry criteria, > 43% of DysEry predicted proliferative kidney disease, whereas CastEry did not, and AcaEry predicted poorly. The best predictor of proliferative glomerular disease was DysEry/HPF, closely followed by DysEry/SW.Correspondence to:
Nuša Avguštin Rotar MD, Department of Nephrology, University Medical Center Ljubljana, Zaloška cesta 7, 1000 Ljubljana, Slovenia
Email: [email protected]

Abstract

Clinical Nephrology, Vol. 96 – Suppl. 1/2021 (S62-S67)

Secondary oxalate nephropathy in a patient with exocrine pancreatic insufficiency successfully treated, with complete kidney recovery

Rok Tilia¹,², Nika Kojc³, and Andrej Škoberne¹,²

¹Department of Nephrology, University Medical Center Ljubljana, ²Faculty of Medicine, and ³Institute of Pathology, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia
Introduction: Oxalate nephropathy is a relatively rare and under-recognized condition that commonly presents as acute kidney injury (AKI) and often leads to end-stage renal disease. Complete recovery of kidney function is extremely rare even when treatment is instituted early on. Case presentation: We present the case of a 68-year-old man with known type 2 diabetes mellitus and an asymptomatic unrecognized exocrine pancreatic insufficiency, who was admitted due to dialysis-dependent AKI. Kidney biopsy revealed oxalate nephropathy. A wide diagnostic assessment and a multi-factorial treatment plan that included a change of diet, therapy for exocrine pancreatic insufficiency and fat malabsorption, sodium bicarbonate and potassium citrate, calcium supplements with meals, and methylprednisolone, resulted in complete recovery of kidney function. Conclusion: It is important for physicians to be aware of oxalate nephropathy in cases of prolonged AKI. After confirmation of diagnosis, a wide diagnostic approach is imperative to identify all the causes that have led to oxalosis. A multi-factorial therapeutic approach can lead to complete kidney recovery.Correspondence to:
Andrej Škoberne, MD, PhD, Department of Nephrology, University Medical Centre Ljubljana, Zaloška cesta 7, 1000 Ljubljana, Slovenia
Email: [email protected]

Abstract

Clinical Nephrology, Vol. 96 – Suppl. 1/2021 (S127-S132)

Glomerular and tubulointerstitial foscarnet nephropathy of kidney allograft with emphasis on ultrastructural findings: A case report

Jerica Pleško¹, Damjan Kovač²,³, Zoran Arsov⁴, Jelka Lindič²,³, Andrej Škoberne²,³, Dušan Ferluga¹, and Nika Kojc¹

¹Institute of Pathology, Faculty of Medicine, University of Ljubljana, ²Department of Nephrology, University Medical Center Ljubljana, ³Faculty of Medicine, University of Ljubljana, and ⁴Department of Condensed Matter Physics, Jozef Stefan Institute, Ljubljana, Slovenia
Introduction: Foscarnet (trisodium phosphonoformate hexahydrate) is standard treatment for ganciclovir-resistant cytomegalovirus (CMV) infections. In the kidney, foscarnet-induced injury may be attributed to reversible tubulointerstitial lesions, but foscarnet crystals have also been observed within glomerular capillaries, suggesting that foscarnet can lead to glomerular lesions such as crescentic glomerulonephritis. We present biopsy and autopsy findings of foscarnet induced nephropathy in a transplanted kidney, with a particular emphasis on the histopathology and electron micrographic peculiarities of drug crystal deposits. Case presentation: A 72-year-old Caucasian male patient with a deceased donor kidney was treated with several foscarnet applications due to ganciclovir-resistant CMV infection. Transplant kidney biopsy revealed massive glomerular crystalline precipitates, resulting in crescentic glomerulonephritis and tubular damage. The last foscarnet application was complicated with several infections and kidney graft failure. Autopsy revealed multi-organ damage due to foscarnet crystal precipitations associated with systemic CMV and fungal infection. On autopsy of kidney specimens, we succeeded in preserving the rectangular flat plate-like foscarnet crystals in stacks detected by transmission electron microscopy (TEM) after 100% alcohol fixation. The chemical composition of the crystals was confirmed by attenuated total reflection Fourier transform infrared (ATR-FTIR) spectroscopy. Conclusion: Transplant kidney biopsy remains the gold standard in distinguishing between foscarnet crystalline glomerular and/or tubulointerstitial lesions, and various forms of rejection and other causes of impaired renal function in transplant kidney.Correspondence to:
Nika Kojc, MD, PhD, Institute of Pathology, Faculty of Medicine, University of Ljubljana, Korytkova 2, 1000 Ljubljana, Slovenia
Email: [email protected]

Abstract

Clinical Nephrology, Vol. 104 – Suppl. 1/2025 (S66-S72)

Fibrillary glomerulonephritis: A retrospective analysis of a case series from a tertiary center

Ana Dovč¹,², Željka Večerić-Haler¹,², Nika Kojc³, Špela Borštnar¹,², Andrej Škoberne¹,², and Andreja Aleš Rigler¹,²

¹Department of Nephrology, Division of Internal Medicine, University Medical Center Ljubljana, ²Faculty of Medicine, and ³Institute of Pathology, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia
Introduction: Fibrillary glomerulonephritis is a rare diagnosis, and guidance on diagnosis and management is scant. We present a case series of patients with fibrillary glomerulonephritis from University Medical Center Ljubljana. Materials and methods: We conducted a retrospective analysis of patients with the diagnosis of fibrillary glomerulonephritis since 2006. We analyzed data on clinical presentation, treatment modalities, and kidney survival. For a subset of patients, data on DNAJB9 staining and markers of complement activation were also available. Results: We included 17 patients with fibrillary glomerulonephritis, 59% female, with a median age of 61 years (range 33 – 71). The most common clinical presentation was asymptomatic proteinuria with preserved kidney function (41%). Eight patients had complement activity testing performed, which revealed elevated serum C5b-9 at least at 1 time point in 5 patients (63%). One patient had positive anti-factor H antibodies, and 2 patients had positive anti-C1q antibodies. Nine patients had available staining results for DNAJB9, which was positive in all 9 cases. The median time of follow-up was 12 months. The most common form of treatment was with corticosteroids in 8 patients, followed by rituximab in 5 patients. Seven patients (41%) had at least partial remission with stabilization of kidney function, 8 patients (47%) reached end-stage kidney disease during follow-up. Due to the small number of patients, analysis of impact of treatment on kidney survival was not performed. Conclusion: Fibrillary glomerulonephritis is a heterogenous disease, and a significant number of patients present with a slowly progressive disease, warranting long-term treatment. New treatment strategies are necessary. Correspondence to:
Ana Dovč, MD, Department of Nephrology, Division of Internal Medicine, University Medical Centre Ljubljana, Zaloška 7, 1000 Ljubljana, Slovenia
Email: [email protected]

Abstract

Clinical Nephrology, Vol. 104 – Suppl. 1/2025 (S73-S84)

Clinical profile, treatment response, and outcomes in adult primary focal segmental glomerulosclerosis: A single-center experience 

Željka Večerić-Haler¹,², Andreja Aleš Rigler¹,², Andrej Škoberne¹,², Špela Borštnar¹,², Nuša Avguštin Rotar¹,², Damjan Kovač¹,², Ana Dovč¹,², Nika Kojc³, and Jelka Lindič¹,²

¹Department of Nephrology, University Medical Center Ljubljana, ²Department of Internal Medicine, Faculty of Medicine, and ³Institute of Pathology, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia
Objective: Primary focal segmental glomerulosclerosis (FSGS) is a major cause of nephrotic syndrome in adults. This study evaluates the clinical profile, treatment response, and outcomes of patients with primary FSGS at the University Medical Centre (UMC) Ljubljana, Slovenia. Materials and methods: Patients diagnosed and treated at UMC Ljubljana from 2008 to 2024 were included. Clinical, laboratory, histological, and treatment data were analyzed. Results: 56 patients were followed for a median of 71 months (range: 2 – 186). At diagnosis, 91.1% had nephrotic syndrome, with proteinuria of 10.2 ± 9.8 g/day, serum albumin of 23.7 ± 6.5 g/L, and estimated glomerular filtration (eGFR) of 66.7 ± 27.7 mL/min/1.73m². Histological subtypes included tip (37.5%), cellular (25%), collapsing (14.3%), not-otherwise specified (16.1%), while in 7.1% the variant was not defined. First-line therapy, mainly corticosteroids, led to remission in 67.3%, but 71% experienced relapses or steroid resistance. Calcineurin inhibitors (CNIs) and corticosteroids were used in 20 patients, achieving 60% remission, however the majority of patients experienced relaps following their discontinuation. Rituximab was given to 23 patients with refractory disease, with 78.2% achieving remission (56.5% complete, 21.7% partial) in 5.1 ± 4.9 months. Among initial rituximab responders, 30.4% experienced relapse. At the last follow-up, mean eGFR was 59.6 ± 33.2 mL/min/1.73m², 21.4% progressed to end-stage renal disease (ESRD), and 5.3% died. Four patients underwent kidney transplantation, with early recurrence in 3. Conclusion: Despite high steroid resistance, use of CNIs and rituximab improved remission rates. However, ESRD remains a significant concern, highlighting the need for early intervention and optimized treatment strategies. Correspondence to:
Željka Večerić-Haler, MD, PhD, Department of Nephrology, University Medical Center Ljubljana, Zaloška 7, 1000 Ljubljana, Slovenia
Email: [email protected]

Abstract

Clinical Nephrology, Vol. 104 – Suppl. 1/2025 (S99-S104)

Real-world evidence for using urine osmolality as a practical tool in tolvaptan dose optimization in patients with ADPKD

Andreja Marn Pernat¹,², Kaja Hrovatin¹, Valentina Černetič Korelec¹, and Andrej Škoberne¹,²

¹Department of Nephrology, University Medical Center Ljubljana, and ²Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia
Introduction: The aim of this prospective single-center study was to evaluate the role of morning urine and 24-hour urine osmolality as biomarkers for guiding individualized dosing of tolvaptan in adults with rapidly progressive autosomal dominant polycystic kidney disease (ADPKD), with the aim of minimizing treatment discontinuations and adverse events. Materials and methods: 24 patients (13 men, 11 women; aged 21 – 54 years) received tolvaptan for 7 – 50 months. Dosing was titrated based on tolerability and achievement of urine osmolality ≤ 250 mOsm/kg, measured by freezing point osmometry in both morning spot and 24-hour urine samples. Results: 16 patients (66.7%) remained on 45/15 mg, 7 (29%) required 60/30 mg, and 1 patient (4%) escalated to 90/30 mg of tolvaptan. Morning urine osmolality decreased significantly from 404 ± 231 mOsm/kg to 153 ± 61 mOsm/kg (p < 0.001), and 24-hour urine osmolality was maintained at ≤ 250 mOsm/kg in all patients. No significant differences in osmolality were observed between the patients on 45/15 mg and 60/30 mg. Mild liver enzyme elevations occurred in 9% of 504 total measurements, with 1 patient discontinuing treatment due to a 3-fold rise in transaminases. Another patient discontinued treatment due to aquaretic side effects. Conclusion: Urine osmolality is a practical and effective biomarker for guiding individualized tolvaptan titration in patients with ADPKD. This approach enabled adequate vasopressin suppression and was associated with a low discontinuation rate, supporting its use in real-world clinical practice. Correspondence to:
Andreja Marn Pernat, MD, PhD, Department of Nephrology, University Medical Center Ljubljana, Zaloška 7, 1000 Ljubljana, Slovenia
Email: [email protected]

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