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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 (S83-S90)

Increase in spot urine protein excretion is associated with late kidney graft rejection and predicts rejection phenotype

Miha Arnol¹#², Manca Oblak¹, Gregor Mlinšek¹, Jelka Lindič¹, Aljoša Kandus¹, Dušan Ferluga³, Nika Kojc³, and Jadranka Buturović-Ponikvar¹#²

¹Department of Nephrology, University Medical Center Ljubljana, ²Faculty of Medicine, and ³Institute of Pathology, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia
Aims: A noninvasive test that foretells kidney graft rejection before loss of kidney function would be desirable. We hypothesized that an increase in estimated protein excretion rate (ePER) from spot urine samples is associated with graft rejection and predicts rejection phenotype. Methods: 151 patients who had undergone first-indication kidney biopsy due to graft dysfunction beyond 3 months after transplant were identified from a national cohort of 616 transplant recipients between 2000 and 2012 (25%). ePER were calculated from spot urine protein-to-creatinine ratios at baseline, 3 months before biopsy (ePER_–3m), and at the time of biopsy (ePER_biopsy) and were correlated with histologic biopsy findings. Results: Levels of ePER 3 months before biopsy and at the time of biopsy were greater in 32 patients with antibody-mediated rejection (ABMR) than in 77 patients with T-cell-mediated rejection (TCMR) and 42 patients with other findings (median ePER_–3m 912 vs. 320 vs. 232 mg/day/1.73m²; and median ePER_biopsy 1,672 vs. 356 vs. 268 mg/day/1.73m²; p < 0.001). Receiver operator characteristics (ROC) analyses demonstrated that ePER_–3m and ePER_biopsy had good diagnostic accuracy to discriminate between biopsy specimens showing ABMR vs. those showing TCMR or other histologic findings (area under the ROC curve 0.84, 95% CI 0.75 – 0.93 and 0.89, 95% CI 0.82 – 0.97, respectively; p < 0.001). Conclusions: An increase in ePER before kidney graft dysfunction appears to be associated with graft rejection and predicts ABMR phenotype. Correspondence to:
Miha Arnol, MD, PhD
Department of Nephrology
Center for Kidney Transplantation
University Medical Center Ljubljana
Zaloska 7, Ljubljana 1000, Slovenia
Email: [email protected]

Abstract

Clinical Nephrology, Vol. 88 – Suppl. 1/2017 (S97-S100)

Acute granulomatous interstitial nephritis and acute rejection in a kidney transplant recipient after zoledronic acid therapy – a case report and review of the literature

Nuša Avguštin¹, Damjan Kovač¹, Nika Kojc², Gregor Mlinšek², and Jelka Lindič¹

¹Department of Nephrology, University Medical Center Ljubljana, and ²Institute of Pathology, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia
Background: Acute granulomatous interstitial nephritis (AGIN) in native kidneys is most commonly linked to drugs. In allografts, it is a rare complication, and it occurs mostly with infections. Case presentation: Our case report presents AGIN with simultaneous acute cellular rejections and acute tubular necrosis in a kidney transplant patient 2 weeks after intravenous application of zoledronic acid. A kidney biopsy showed signs of destructive AGIN with acute cellular rejection. After treatment with methylprednisolone pulses and immunosuppressive therapy modification, rebiopsy confirmed complete regression of AGIN with less intense persistent acute cellular rejection and acute tubular necrosis. Kidney function improved after glucocorticoid and intravenous immunoglobulin G therapy. Conclusion: To our knowledge, this is the first case of AGIN related to bisphosphonate zoledronate in a kidney transplant patient with consequent acute cellular rejection. In using intravenous zoledronate infusion in a kidney transplant recipient, we should be aware that it could potentially induce acute granulomatous tubulointerstitial nephritis and acute rejection. Correspondence to:
Jelka Lindič, MD, PhD
Department of Nephrology
University Medical Center Ljubljana
Zaloška cesta 7, 1000 Ljubljana, Slovenia
Email: [email protected]

Abstract

Clinical Nephrology, Vol. 88 – Suppl. 1/2017 (S109-S114)

Outcome of polyomavirus nephropathy in renal transplant patients: a single-center experience

Nika Kojc¹#², Andreja Aleš Rigler³, Gregor Mlinšek³, Damjan Kovač³, Dušan Ferluga¹, and Miha Arnol²#³

¹Institute of Pathology, ²Medical Faculty, University of Ljubljana, and ³Department of Nephrology, University Medical Center Ljubljana, Ljubljana, Slovenia
Background: Reduction of immunosuppression is a common therapeutic strategy in patients with polyomavirus nephropathy (PVN) but may be associated with acute rejection. This study aimed to evaluate the morphology of PVN in renal biopsies after reduction of immunosuppression. Methods: Eight of 241 patients who received a kidney transplant between January 2012 and December 2015 presented with BK viremia and biopsy-proven PVN. Morphological evaluation according to Banff criteria and correlation with viremia and kidney function after immunosuppression reduction was performed. Results: PVN grades A and B were diagnosed on average 4.7 months post-transplant in 1 and 7 patients, respectively. Indication biopsies after immunosuppression reduction showed an increase in tubulitis and interstitial inflammation score compared to those at the time of the PVN diagnosis. Surveillance biopsies 1 year after transplantation revealed resolution of interstitial inflammation and tubulitis accompanied by clearance of BK viremia in 4 patients (50%), including 1 patient with rejection. One patient showed residual interstitial inflammation after viral clearance. In these patients, renal function returned to baseline. One patient with persisting low BK virus (BKV) in serum and kidney showed a decrease of tubulointerstitial inflammation but scarring was seen. Rejection occurred in 3 patients (38%). Conclusion: PVN-associated interstitial inflammation and tubulitis cannot be differentiated morphologically from T-cell-mediated tubulointerstitial rejection. Significant interstitial inflammation and tubulitis in PVN under low-dose immunosuppression might represent immune reconstitution injury, which is reduced after successful BKV clearance from the serum and kidney. Concomitant rejection in PVN patients on low immunosuppression might be efficiently treated with transient pulse immunosuppressive therapy. Correspondence to:
Nika Kojc, MD, PhD
Institute of Pathology, Medical Faculty
University of Ljubljana
Korytkova 2, 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

Clinical Nephrology, Vol. 96 – Suppl 1/2021 (S6-S15)

Characteristics of renal sarcoidosis and tubulointerstitial nephritis and uveitis syndrome in Slovenia: An analysis of the national renal biopsy registry and patient datasets in the last decade

Maja Frelih¹, Nejc Piko², Željka Večerić-Haler³,⁴, Nika Kojc¹, and Špela Borštnar³,⁴

¹Institute of Pathology, Faculty of Medicine, University of Ljubljana, Ljubljana, ²Department of Dialysis, University Medical Center Maribor, Maribor, ³Department of Nephrology, University Medical Center Ljubljana, and ⁴Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia
Background: Sarcoidosis is characterized by granulomatous inflammation in multiple organs. Renal involvement is rare, and granulomatous tubulointerstitial nephritis (TIN) is the predominant histologic feature. TIN is also a hallmark of tubulointerstitial nephritis and uveitis (TINU) syndrome. Diagnoses of both sarcoidosis and TINU syndrome are usually made by exclusion and by combining clinical and histological findings, and often remain misdiagnosed. The aim of this retrospective study was to determine the characteristics of renal sarcoidosis and TINU syndrome in Slovenia in the last decade (2010 – 2020). Materials and methods: A thorough search of the national database of renal biopsies from January 2010 to December 2020 was performed. Inclusion criteria were TIN and a clinical history of either sarcoidosis or TINU syndrome. To compare the characteristics of our cohort with others, we also reviewed the global literature reported since 2010. Results: 13 patients (9 female, 4 male) were included in our study. Indications for kidney biopsy were acute kidney injury (n = 8), acute exacerbation of chronic kidney disease (n = 4), and proteinuria (n = 1). Seven patients had clinical and histological evidence of sarcoidosis, and 6 patients were classified as having TINU syndrome. All patients were treated with corticosteroids. Of the 13 patients, 11 had improved kidney function 6 months after treatment, and proteinuria decreased in 9 patients. One patient was on dialysis at the time of diagnosis and remained so thereafter. Conclusion: Renal sarcoidosis and TINU syndrome are rare but important causes of kidney injury, with a favorable long-term prognosis if properly diagnosed and treated in a timely manner.Correspondence to:
Špela Borštnar, MD, Msc, Department of Nephrology, University Medical Centre Ljubljana, Zaloška cesta 2, 1000 Ljubljana, Slovenia
Email: [email protected]

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 (S24-S30)

Histomorphological and clinical characteristics of patients with thin glomerular basement membrane nephropathy

Aleksandra Pečovnik¹, Željka Večerić Haler²,³, Karmen Wechtersbach⁴, Jerica Pleško⁴, Maja Frelih⁴, Jelka Lindič²,³, and Nika Kojc⁴

¹Department of Pathology and Cytology, General Hospital Celje, Celje, ²Clinical Department of Nephrology, University Clinical Center Ljubljana, ³Faculty of Medicine, and ⁴Institute of Pathology, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia
Background: Thin basement membrane nephropathy (TBMN) is a disorder characterized by ultrastructural abnormalities of the glomerular basement membrane (GBM), representing a spectrum of genetic and clinical phenotypes ranging from benign hematuria to proteinuria and chronic kidney disease. Recent studies have shown that a significant percentage of patients who initially present with hematuria later develop proteinuria and worsening renal function. Materials and methods: We retrospectively analyzed records of patients diagnosed with TBMN, including their clinical, laboratory, and histological features, in Slovenia from 2015 to 2020. Results: TBMN was the main diagnosis at kidney biopsy in 34 (65%) of 52 included patients, while in 18 patients (35%) TBMN was diagnosed in addition to other renal diseases. In the isolated TBMN group, 29 of 34 patients had glomerulosclerosis (global, global and segmental, segmental only) accompanied by interstitial fibrosis/tubular atrophy of varying degrees. 13 patients with isolated TBMN had signs of advanced chronic kidney disease at the time of diagnosis, with estimated glomerular filtration rate < 60 mL/min/1.73m2. 29 patients had proteinuria, which exceeded 3 g/day in 4 patients. TBMN represents a proportion of patients with focal segmental glomerulosclerosis (FSGS) that have often been classified in the past as etiologically indeterminate FSGS. Conclusion: Ultrastructural examination showing diffuse thinning of the GBM is crucial for the TBMN diagnosis. TBMN was the main diagnosis of kidney biopsy in 2/3 of our patients, while it was accompanied by other renal diseases in 1/3. Up to 1/3 of patients with isolated TBMN had evidence of advanced chronic kidney disease at the time of diagnosis.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. 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 (S85-S91)

Anti-nephrin antibodies in minimal change disease: Case report series 

Maša Knehtl¹, Nejc Piko², Tadej Petreski¹, Tadej Zorman², Tina Stropnik-Galuf², Nika Kojc³, Karmen Wechtersbach³, Robert Ekart²,⁴, and Sebastjan Bevc¹,⁴

¹Department of Nephrology, ²Department of Dialysis, Clinic for Internal Medicine, University Medical Center Maribor, Maribor, ³Institute of Pathology, Faculty of Medicine, University of Ljubljana, Ljubljana, and ⁴Faculty of Medicine, University of Maribor, Maribor, Slovenia
Introduction: Minimal change disease (MCD) is one of the causes of nephrotic syndrome (NS) in adults. Recently, anti-nephrin antibodies have been detected in a certain subset of patients with MCD, supporting the proposed autoimmune etiology and appearing to be markers of disease activity. Despite their diagnostic and prognostic potential, the use of anti-nephrin autoantibodies in routine clinical practice is not yet widespread. Studies have shown that patients with anti-nephrin-associated MCD have a more fulminant NS and a better response to antibody-depleting therapy than those who are anti-nephrin-negative. Materials and methods: We report cases of a 79- and a 42-year-old male patient presenting with new-onset NS and acute kidney injury. Both patients had negative immunologic tests, including antibodies against phospholipase A2-receptor (anti-PLA2R) and thrombospondin-7A (anti-THSD7A). Renal biopsy was performed in both patients. Results: In both cases, light microscopy of kidney samples from thick-needle biopsy showed acute tubular injury attributed to severe proteinuria. Electron microscopy revealed diffuse (90%) effacement of the podocyte foot processes without electron-dense deposits. Immunofluorescence presented discrete intracytoplasmic IgG podocyte deposits with a high probability of MCD due to anti-nephrin autoantibodies. In addition to therapy with angiotensin convertase inhibitors, calcium channel blockers, and furosemide, we started treatment with low-dose oral glucocorticoids and mycophenolic acid (case 1) or oral glucocorticoids alone (case 2) depending on patients’ comorbidities. We administered rituximab, resulting in a fast and complete resolution of proteinuria and improvement of kidney function. Conclusion: Anti-nephrin autoantibodies have been detected in a subgroup of patients with MCD supporting the autoimmune etiology of the disease. Targeted anti-B-cell therapy with rituximab is an additional therapeutic option in patients with relapsing or treatment-resistant disease, or drug-related adverse effects to standard therapy. Correspondence to:
Assistant Professor Maša Knehtl, MD, PhD, Department of Nephrology, Clinic for Internal Medicine, University Medical Center Maribor, Ljubljanska ulica 5, Maribor, 2000, Slovenia
Email: [email protected]

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