Reproduction in Russian prepared at the initiative of RDS by courtesy of the EULAR Task Force on SLE, made on behalf and approved by EULAR/ERA-EDTA Working Group*. Reproduction rights of Russian version belong to RDS and EILAR/ERA-EDTA Working Group. Publication of original version: Ann Rheum Dis 2012; 71: 1771–1782. George Bertsias, Maria Tektonidou, Zahir Amoura, Martin Aringer, Ingeborg Bajema, Jo Berden, John Boletis, Ricard Cervera, Thomas Dörner, Andrea Doria, Franco Ferrario, Jürgen Floege, Frederic Houssiau, John P.A. Ioannidis, David Isenberg, Cees G.M. Kallenberg, Liz Lightstone, Stephen D. Marks, Alberto Martini, Gabriela Moroni, Irmgard Neumann, Manuel Praga, Matthias Schneider, Argyre Starra, Vladimir Tesar, Carlos Vasconcelos, Ronald van Vollenhoven, Elena Zakharova, Marion Haubitz, Caroline Gordon, David Jayne, Dimitrios T. Boumpas. Objectives. To develop recommendations for the management of adult and paediatric lupus nephritis (LN). Methods. The available evidence was systematically reviewed using PubMed. A modified Delphi method was used to compile questions, elicit expert opinions and reach consensus. Results. Immunosuppressive treatment should be guided by renal biopsy, and aiming for complete renal response (proteinuria <0,5 g/24-hr with normal or near-normal renal function). Hydroxychloroquine is recommended for all LN patients. Because of a more favourable efficacy/toxicity ratio, as initial treatment for patients with class III–IVA or A/C (±V) LN according to the International Society of Nephrology/Renal Pathology Society 2003 classification, we recommend mycophenolic acid (MPA) or low-dose intravenous cyclophosphamide in combination with glucocorticoids. In patients with adverse clinical or histological features, cyclophosphamide can be prescribed at higher doses, while azathioprine is an alternative for milder cases. For pure class V LN with nephrotic-range proteinuria, we recommend MPA in combination with oral glucocorticoids as initial treatment. In patients improving after initial treatment, subsequent immunosuppression with MPA or azathioprine is recommended for at least 3 years; in such cases, initial treatment with MPA should be followed by MPA. For MPA or cyclophosphamide failures, we recommend switching to the other agent, or to rituximab. In anticipation of pregnancy, patients should be switched to appropriate medications without reducing the intensity of treatment. There is no evidence to suggest that management of LN should differ in children versus adults. Conclusion. Recommendations for the management of LN were developed using an evidence-based approach followed by expert consensus.

The fibroblast growth factor 23 (FGF23) is an important regulator of phosphorus balance in the body. FGF23 influences transport of phosphorus and inhibits calcitriol by initiating the development of MBD in patients with CKD. So it seems logical to presribe phosphate binders to patients with renal dysfunction, who have normal blood phosphate level. However, attempts to use different phosphate binders in patients with moderate hyperphosphatemia some unexpected increase in vascular calcification have been observed. It is not recommended the use phosphate binders in patients with predialysis chronic renal failure before controlled studies will be performed.

The purpose of the study was to determine kidney function and blood supply in children with inherited thrombophilia. Methods: Laboratory tests were performed in 66 children with identified inherited thrombophilia, 22 of them have arterial or venous thrombosis, 44 did not had symptoms, however had strong family history of thromboembolism. The laboratory tests were performed by clinical analysis of blood and urine, biochemistry blood analysis with detection serum level of lipoprotein (a) and homocysteine, blood coagulation tests with activity levels of antithrombin, proteins C and S and antiphospholipid antibody syndrome. 24 h-urine test was also performed. The ultrasound examination with Doppler test was taken in all patients. None of patients had reduced renal function. In 7 (10,6%) children with inherited thrombophilia renal infarction was detected using ultrasound examination. The sonographic sign was hyperechogenic avascular wedge shape zone. All these patients have statistically significant difference in the presence of erythrocyturia, microalbuminuria, nicturia, malfunction of acidoammoniogenesis, enzymuria in comparison with patients with inherited thrombophilia, but without signs of renal infarction. The increased blood level of lipoprotein (a) by more than 2 times is related to kidneys damage in patients with inherited trombophilia that corresponds to increased risk by a factor of 300 or higher. We developed an algorithm to estimate the risk of renal vessel thrombosis in children with inherited thrombophilia depending on blood level of lipoprotein (a) and activity of natural inhibitors of coagulation.

The observational 12-months single center study have shown prevalence and changes of parameters of mineral and bone disturbances in CKD in the context of Russian National health insurance system. The current practice maintains the calciemia in target range in a majority of patients; in the significant part of patients the hyperparathyroidism has moderate degree and requires drug correction much more often than surgical intervention; the wide prevalence of low turnover bone disease may be explained by demographical factors. Phosphatemia demonstrates the most significant deviation from target values despite dialysis intensification and workup with diet. The main reason is lack of modern and safe phosphate-binder availability. Similar observational studies could reveal the shortcomings of the actual standards of medical care.

Rejection has always been one of the most important cause of late renal graft dysfunction. The aim of the study was to analyze the prevalence of different clinical and pathological variants of rejection that cause late graft dysfunction and to evaluate their impact on long-term outcome. A retrospective study included 294 needle core biopsy specimens from 265 renal transplant recipients with late (48,8 ± 46,1 months after transplantation) allograft dysfunction caused by late acute rejection (LAR n = 194) or chronic rejection (CR n = 78) or both (n = 22). C4d staining was performed by immunofluorescence (IF) on frozen sections using a standard protocol. Peritubular capillary C4d deposition was identified in 34% samples with acute rejection and in 59% cases of chronic rejection (65% cases of transplant glomerulopathy, and 50% of isolated chronic vasculopathy). 5-year graft survival for LAR vs CR vs combination were 48%, 34% и 17%, respectively. The outcome of C4d+ LAR was better (p < 0,01) than of C4d+ acute rejection: at 60 months, graft survival for diffuse C4d+ vs focal C4d+ vs C4d– were 29% vs 51% vs 43%, respectively. In cases of chronic rejection C4d+ vs C4d– was not statistically significant (34% vs 36%). By multivariate Cox analysis the risk of graft loss was associated with transplant glomerulopathy, intimal arteriitis, widespread interstitial fibrosis and plasma cell-rich infiltrates. Among clinical factors serum creatinine had a strongest impact on graft outcome: using Cox regression, the hazard ratio of graft loss was 1,6 (1,42–1,79) for each 0,1 mmol/l (p = 0,017). 5-year graft survival according to the initial serum creatinine were 97,5; 78,5; 67,8 and 20,7% for <0,2; 0,2–0,3; 0,3–0,4; >0,4 mmol/l respectively for LAR and 75; 33,9; 20,8 and 0% for CR. Proteinuria >3 g/d was a poor prognostic factor for LAR. No statistically significant difference was found for proteinuria vs mild to moderate urine protein level. In contrast for CR recipients only the absence of proteinuria had a significant positive impact on graft outcome compared with mild moderate or heavy proteinuria.

Clinical observation of successful treatment of benign prostatic hyperplasia by transurethral laser vaporization рhotoselective using 80-watt potassium titanyl phosphate laser in a patient with chronic renal failure on peritoneal dialysis.