Mesangial Proliferative Glomerulonephritis Research Articles

Epidemiological characteristics and pathological changes of primary glomerular diseases.

ObjectiveBy analyzing the pathological characteristics and clinical data of renal biopsy in our hospital in the past 20 years, to further understand the epidemic characteristics and pathological changes of primary glomerular disease, and to provide regional data for the big data of kidney disease in my country.MethodsA retrospective analysis of 9448 patients with primary glomerular disease who were hospitalized in our hospital from January 1, 2000 to December 31, 2019, aged 18 years or older, and undergoing renal biopsy. Divided every 5 years into a group, a total of 4 groups (first group 2000.1.1–2004.12.31, second groups 2005.1.1–2009.12.31; third groups 2010.1.1–2014.12.31, fourth groups 2015.1.1–2019.12.31).Results① There were more males than females, and male: female vs 1.53:1. The proportion of men in the past five years has increased compared with the previous 15 years. ② Mostly middle-aged, with a median age of 41.39 years old. The age is increasing over time. There are differences between the four groups, P <0.001; ③ The most common clinical manifestations are nephrotic syndrome, followed by chronic glomerulonephritis. Occult glomerulonephritis, the proportion of patients with nephrotic syndrome increases over time, first to fourth group (40.08%< 42.64% < 47.08%< 53.69%); ④ The most common pathology type from 2000 to 2009 was mesangial proliferative glomerulonephritis. IgA nephropathy was the most common type from 2010 to 2014, but the proportion of membranous nephropathy increased year by year, and it became the most common pathological type from 2015 to 2019; ⑤ The clinical and pathological manifestations of different genders are different, but there is no statistical difference.ConclusionIn the past 20 years, the primary glomerular disease is mainly middle-aged. There are more men than women. The most common type of clinical manifestation is nephrotic syndrome. The pathological type is mesangial proliferative glomerulonephritis. Over time, the average age is increasing, and the proportion of patients with renal syndrome is increasing. IgA nephropathy is the most common pathological type from 2010 to 2014, and membranous nephropathy has become the main pathological type in the past 5 years.

COQ8B glomerular nephropathy: Outcomes after kidney transplantation and analysis of characteristics in Chinese population.

BackgroundMutation in the COQ8B gene can cause COQ8B glomerular nephropathy (COQ8B-GN), which is rare and associated with steroid-resistant nephrotic syndrome (SRNS) as well as rapid progression to end-stage renal disease (ESRD). The aim of this study was to analyze the prognosis and recurrence risk of COQ8B-GN in patients after kidney transplantation (KTx) and summarize the characteristics of the Chinese population.MethodsA retrospective study included four cases treated in our hospital with a diagnosis of COQ8B-GN. Chinese and foreign studies were searched from database inception to February 2022.ResultsA total of four cases were included, with the age of onset ranging from 4 to 9 years. The initial presentations were SRNS and asymptomatic proteinuria. Only one had an extrarenal manifestation (thyroid cyst). All patients progressed to ESRD at a mean time of 42 months after onset. With a total follow-up time ranging from 12 to 87 months, three of them had received transplantation. While one case needed a second KTx due to graft failure caused by chronic rejection, two recipients had excellent graft function. No recurrence in allograft was observed. There have been 18 cases of KTx recipients reported globally with follow-up information. Except for two cases of graft failure caused by hyperacute rejection and chronic rejection, respectively, the rest all had good graft function without recurrence. In addition, 44 cases of COQ8B-GN in the Chinese population were identified. At the onset, 75% of the patients were aged ≤10 years with initial symptoms of asymptomatic proteinuria, nephrotic syndrome (NS), or SRNS. By the time of literature publication, 59% of patients had progressed to ESRD (mean age of 10.3 ± 3.6 years). The median time from onset to ESRD was 21 months. Renal pathology mainly showed focal segmental glomerulosclerosis (FSGS), accounting for 61.8% of all biopsies, followed by mesangial proliferative glomerulonephritis (20.6%). The first three prevalent mutations in the COQ8B gene among the Chinese population were c. 748G>C, c. 737G>A, and c. 532C>T.ConclusionCOQ8B-GN in the Chinese population may present with asymptomatic proteinuria, NS, or SRNS initially, with most onsets before the age of 10 years. A lot of patients progress to ESRD in early adolescence. FSGS on biopsy and c. 748G>C in the genetic test are the most frequently seen in Chinese COQ8B-GN patients. KTx is feasible for patients with ESRD due to the low risk of recurrence, but we should pay attention to graft rejection.

Staphylococcus aureus Infection-Related Glomerulonephritis with Dominant IgA Deposition

Since 1995, when we reported the case of a patient with glomerulonephritis with IgA deposition that occurred after a methicillin-resistant Staphylococcus aureus (MRSA) infection, many reports of MRSA infection-associated glomerulonephritis have accumulated. This disease is being systematized as Staphylococcus infection-associated glomerulonephritis (SAGN) in light of the apparent cause of infection, and as immunoglobulin A-dominant deposition infection-related glomerulonephritis (IgA-IRGN) in light of its histopathology. This glomerulonephritis usually presents as rapidly progressive glomerulonephritis or acute kidney injury with various degrees of proteinuria and microscopic hematuria along with an ongoing infection. Its renal pathology has shown several types of mesangial and/or endocapillary proliferative glomerulonephritis with various degrees of crescent formation and tubulointerstitial nephritis. IgA, IgG, and C3 staining in the mesangium and along the glomerular capillary walls have been observed on immunofluorescence examinations. A marked activation of T cells, an increase in specific variable regions of the T-cell receptor β-chain-positive cells, hypercytokinemia, and increased polyclonal immune complexes have also been observed in this glomerulonephritis. In the development of this disease, staphylococcal enterotoxin may be involved as a superantigen, but further investigations are needed to clarify the mechanisms underlying this disease. Here, we review 336 cases of IgA-IRGN and 218 cases of SAGN.

EOS789, pan-phosphate transporter inhibitor, ameliorates the progression of kidney injury in anti-GBM-induced glomerulonephritis rats.

Hyperphosphatemia associated with chronic kidney disease (CKD) not only dysregulates mineral metabolism and bone diseases, but also strongly contributes to the progression of kidney disease itself. We have identified a novel drug for hyperphosphatemia, EOS789, that interacts with several sodium‐dependent phosphate transporters (NaPi‐IIb, PiT‐1, and PiT‐2) known to contribute to intestinal phosphate absorption. In this study, we investigated whether EOS789 could ameliorate kidney disease progression in glomerulonephritis rats. Anti‐glomerular basement membrane (GBM) nephritis was induced in rats by intravenously administering two types of anti‐rat GBM antibodies. We evaluated the effect of EOS789 administered in food admixture on hyperphosphatemia and kidney disease progression. In an anti‐GBM nephritis rats, which exhibit a significant increase in serum phosphate and a decline in renal function, EOS789 dose‐dependently improved hyperphosphatemia and EOS789 at 0.3% food admixture significantly ameliorated kidney dysfunction as shown in the decline of serum creatinine and BUN. Renal histopathology analysis showed that EOS789 significantly decreased crescent formation in glomeruli. To elucidate the mechanism underlying glomerular disease progression, human mesangial cells were used. High phosphate concentration in media significantly increased the expression of Collagen 1A1, 3A1, and αSMA mRNA in human mesangial cells and EOS789 dose‐dependently suppressed these fibrotic markers. These results indicate that EOS789 prevented glomerular crescent formation caused by mesangial fibrosis by ameliorating hyperphosphatemia. In conclusion, EOS789 would not only be useful against hyperphosphatemia but may also have the potential to relieve mesangial proliferative glomerulonephritis with crescent formation.

Mesangial proliferative glomerulonephritis with monoclonal immunoglobulin deposits: why nephrobiopsy is crucial

Proliferative glomerulonephritis (GN) with monoclonal immunoglobulin deposits (PGNMIDs) is a recently described entity among the spectrum of monoclonal gammopathy of renal significance (MGRS). The disease is renal limited and manifests in chronic glomerular disease, altered renal function and albuminuria, sometimes in the nephrotic range. Acute nephritic syndrome is rare. Until recently, in the absence of criteria for symptomatic haematological disease, patients with monoclonal Ig-related nephropathies often experienced difficulties in getting appropriate chemotherapy. Noticeable progress in management was achieved with the introduction of the concept of monoclonal gammopathy of renal significance, which distinguishes the situation of nephropathies induced by dangerous small B-cell clones from that of monoclonal gammopathy of undetermined significance (MGUS) that does not feature any end-organ damage. Monoclonal gammapathy of renal significance is not an independent kidney disease, not “chronic glomerulonephritis”, but a condition in which kidney damage is secondary to clonal B-cell proliferation. In other words, MGRS is a precancerous disease combined with chronic kidney disease that requires immediate treatment. Renal prognosis is poor, with progression to end stage renal disease in 25 % of patients within 30 months and frequent early recurrence on the renal allograft. However, recent studies indicate that clone-targeted chemotherapy may significantly improve renal outcomes, opening future perspectives for the management of this rare disease.

MO248: A Renal Biopsy Might be a Mandatory Procedure in Patients with Hypertension and Decreased Renal Function Despite Normal Urinalysis Findings

Abstract BACKGROUND AND AIMS The most common cause of the end-stage renal disease is diabetes and hypertension. However, most centers do not try to find the cause of hypertension in patients with early-stage CKD but give antihypertensive medicine because most centers regard the cause as essential hypertension. Since blood pressure is regulated by the kidneys, we tried to find the cause of hypertension related to kidney disease. METHOD We carried out a single-center retrospective analysis of 1440 kidney biopsies performed for 8 years at a single center in Korea. All cases were done at the OPD level without admission, of which 367 cases (25.5%) showed hypertension/early-stage CKD with normal urinary findings at the time of renal biopsy. RESULTS The results are as follows in order of frequencies: IgA nephropathy (32.7%), mild focal nonspecific glomerulonephritis (GN) (17.7%), focal segmental glomerulosclerosis (FSGS) (15.3%), diffuse mesangial proliferative GN (12.8%), interstitial nephritis with tubular atrophy (7.6%), podocyte disease (3.3%) and membranous nephropathy (1.9%), and others include lupus nephritis, membranoproliferative GN, C1q nephropathy, C3GN, malignant hypertension (1.4%), etc. Most patients (95%) with hypertension/early-stage CKD were associated with chronic glomerulonephritis. Most patients with serious glomerular disease that may progress to ESRD, such as IgAN, FSGS and diffuse mesangial proliferative glomerulonephritis, have improved by aggressive immunotherapy. CONCLUSION In conclusion, before considering antihypertensive medicine in patients with early-stage CKD, a renal biopsy might be a mandatory procedure to save the kidney because more than 90% of cases are associated with glomerular disease.

Aqueous Extract and Polysaccharide of Aconiti Lateralis Radix Induce Apoptosis and G0/G1 Phase Cell Cycle Arrest by PI3K/AKT/mTOR Signaling Pathway in Mesangial Cells.

Mesangial proliferative glomerulonephritis (MesPGN) is a common renal disease that lacks effective drug intervention. Aconiti Lateralis Radix (Fuzi), a natural Chinese medical herb, is found with significant therapeutic effects on various diseases in the clinic. However, its effects on MesPGN have not been reported. This study is aimed to discuss the therapeutic effects of the aqueous extract of Aconiti Lateralis Radix (ALR) and the polysaccharides of Aconiti Lateralis Radix (PALR) on MesPGN as well as the underlying mechanism. In this study, we, firstly, studied the anti-MesPGN mechanism of ALR and PALR. ALR and PALR inhibit the proliferation of the mesangial cells through the PI3K/AKT/mTOR pathway, induce the G0/G1 phase of block and apoptosis, inhibit the activity of Cyclin E and CDK2, increase the expression of Bax, cleaved caspase-8/caspase-8, and cleaved caspase-3/caspase-3 proteins, and effectively inhibit the growth of the mesangial cells. Overall, our data suggest that ALR and PALR may be potential candidates for MesPGN and that PALR is more effective than ALR.

Th17 Cells Participate in Thy1.1 Glomerulonephritis Which Is Ameliorated by Tacrolimus

Introduction: Thy1.1 glomerulonephritis (Thy1.1 GN) in rats is widely used as an experimental model of mesangial proliferative glomerulonephritis (GN). We previously reported that T-helper (Th) cells were accumulated in glomeruli from the early phase of this model and that not Th2 cells but Th1 cells play an important role in the development of glomerular alterations. Although Th17 is reported to be involved in the pathogenesis of several autoimmune diseases, the role of Th17 cells in the pathogenesis of mesangial alterations in Thy1.1 GN remains unclear. Methods: The kinetics of the infiltration of subsets of Th cells and the expression of IL-17 in Thy1.1 GN were analyzed. Next, the localization and the cell types of IL-17 receptor (IL-17R)-positive cells and IL-6-positive cells were analyzed. Then, the effect of tacrolimus on the expressions of Th17-related cytokines in Thy1.1 GN was analyzed. Results: Not only Th1 cells but also Th17 cells were recruited into glomeruli from the early phase of the disease. mRNA expression of IL-17 in glomeruli was elevated. The increased positive expression of IL-17R was detected in the mesangial area, and some of IL-17R-positive cells were co-stained with IL-6. Tacrolimus treatment ameliorated mesangial alterations by suppressing the expressions of Th17-related cytokines such as IL-17 and IL-6. Conclusion: Th17 cells participate in the development of Thy1.1 GN, a mimic of mesangial proliferative GN, and Th17 cells and their related cytokines are pertinent therapeutic targets.

Ameliorating role of microRNA-378 carried by umbilical cord mesenchymal stem cells-released extracellular vesicles in mesangial proliferative glomerulonephritis

BackgroundMesenchymal stem cells (MSCs) and their released extracellular vesicles (Evs) have shown protective effects against kidney diseases. This study aims to study the functions of umbilical cord MSCs-released Evs (ucMSC-Evs) and their implicated molecules in mesangial proliferative glomerulonephritis (MsPGN).MethodsA rat model of MsPGN was induced by anti-Thy-1.1, and rat mesangial cells (rMCs) HBZY-1 were treated with PDGF-BB/DD to mimic MsPGN condition in vitro. Rats and cells were treated with different doses of ucMSC-Evs, and then the pathological changes in renal tissues and proliferation of rMCs were determined. Differentially expressed microRNAs (miRNAs) after Evs treatment were screened by microarray analysis. The interactions among miR-378, PSMD14, and TGFBR1 were analyzed. Gain- and loss-of function studies of miR-378 and PSMD14 were performed to explore their effects on tissue hyperplasia and rMC proliferation and their interactions with the TGF-β1/Smad2/3 signaling pathway.ResultsThe ucMSC-Evs treatment ameliorated mesangial hyperplasia and fibrosis in rat renal tissues and suppressed the aberrant proliferation of rMCs in a dose-dependent manner. miR-378 was the most upregulated miRNA in tissues and cells after ucMSC-Evs treatment. miR-378 directly targeted PSMD14, and PSMD14 maintained the stability of TGFBR1 through deubiquitination modification, which led to TGF-β1/Smad2/3 activation. Either miR-378 knockdown or PSMD14 overexpression diminished the protective functions of ucMSC-Evs by activating the TGF-β1/Smad2/3 signaling pathway.ConclusionUcMSC-Evs ameliorate pathological process in MsPGN through the delivery of miR-378, which suppresses PSMD14-mediated TGFBR1 stability and inactivates the TGF-β1/Smad2/3 signaling pathway to reduce tissue hyperplasia and rMC proliferation.Graphical 6tcW_AV6VyAYJya28kLbKyVideo abstract

Warfarin-Related Nephropathy Manifested as Diffuse Mesangial Proliferative Glomerulonephritis

Warfarin is associated with anticoagulant-related nephropathy (ARN), one of the potential side effects. This is evidenced by a progressively increasing number of detected cases of deterioration in the kidney function even in patients with normal baseline function (GFR {glomerular filtration rate}) in addition to the chronic kidney disease (CKD) patients who are already vulnerable to ARN. There has been a clinical correlation in a rapid decline of kidney function and international normalized ratio (INR) levels of greater than three. ARN is a significant but underdiagnosed complication of anticoagulation that is associated with increased renal morbidity and all-cause mortality. We want to emphasize the importance of monitoring kidney function regularly and adjusting the appropriate doses of warfarin. We present a case of a 57-year-old female who was on warfarin for the mechanical aortic valve, presented with acute kidney injury and supratherapeutic INR. Her renal biopsy showed diffuse mesangial proliferative glomerulonephritis.

Clinico-Pathological Correlations of Poststreptococcal Glomerulonephritis

Abstract Postinfectious glomerulonephritis is associated with bacterial, viral, fungal, and parasitic infectious agents and histologically appears most often as acute diffuse endocapillary or proliferative glomerulonephritis secondary infection with: group A streptococcus, streptococcus viridans, staphilococus aureus, diploccocus pneumoniae, Brucella melitensis, Salmonella typhi, Yershinia enterocolitica, Plasmodium falciparum, meningococcus, Mycoplasma, Klebsiella, varicella, variola, mumps. Less commonly, it appears as diffuse crescentic glomerulonephritis and a lot of infectious causes are incriminated like: streptococcus, legionella, varicella, Treponema pallidum or as focal crescentic glomerulonephritis: streptococcus A. It rarely appears as mesangiocapillary glomerulonephritis secondary infection with: streptococcus viridans, hepatitis C virus; diffuse or focal mesangial proliferative glomerulonephritis: hepatitis B virus, salmonella, adenovirus, influenza virus, salmonella; focal segmental, necrotizing and sclerosing glomerulonephritis: bacterial endocarditis; membranous glomerulonephritis: hepatitis B virus, syphilis, filarial, Mycobacterium, plasmodium falciparum; focal proliferative: Mycoplasma; mesangiolytic glomerulonephritis :Echo virus. Poststreptococcal glomerulonephritis (PSGN) is caused by prior infection with specific nephritogenic strains of group A beta-hemolytic streptococcus. The clinical presentation of PSGN varies from asymptomatic, microscopic hematuria to the full-blown acute nephritic syndrome, characterized by red to brown urine, proteinuria (which can reach the nephrotic range), edema, hypertension, and acute kidney injury. The prognosis is generally favorable, especially in children, but in some cases, the long-term prognosis is not benign. Managing a case of PSGN requires cooperation between internists, nephrologists, infectious disease consultants, pharmacists, and nursing staff, functioning as an interprofessional team, to provide excellent care for their patients.

Asymptomatic, reddish-brown papules and plaques in an elderly Japanese woman

Asymptomatic, reddish-brown papules and plaques in an elderly Japanese woman

Modulation of IL-6 Expression by KLF4-Mediated Transactivation and PCAF-Mediated Acetylation in Sublytic C5b-9-Induced Rat Glomerular Mesangial Cells.

Interleukin-6 (IL-6) overproduction has been considered to contribute to inflammatory damage of glomerular mesangial cells (GMCs) in human mesangial proliferative glomerulonephritis (MsPGN) and its rat model called Thy-1 nephritis (Thy-1N). However, the regulatory mechanisms of IL-6 expression in GMCs upon sublytic C5b-9 timulation remain poorly understood. We found that Krüppel-like factor 4 (KLF4) bound to the IL-6 promoter (−618 to −126 nt) and activated IL-6 gene transcription. Furthermore, lysine residue 224 of KLF4 was acetylated by p300/CBP-associated factor (PCAF), which was important for KLF4-mediated transactivation. Moreover, lysine residue 5 on histone H2B and lysine residue 9 on histone H3 at the IL-6 promoter were also acetylated by PCAF, which resulted in an increase in IL-6 transcription. Besides, NF-κB activation promoted IL-6 expression by elevating the expression of PCAF. Overall, these findings suggest that sublytic C5b-9-induced the expression of IL-6 involves KLF4-mediated transactivation, PCAF-mediated acetylation of KLF4 and histones, and NF-κB activation in GMCs.

Expression and Significance of Toll-Like Receptor 1 and Toll-Like Receptor 3 in Renal Tissues of Children with Primary Nephrotic Syndrome

To explore the expression and clinical significance of toll-like receptor 1 and toll-like receptor 3 in renal tissues of children with primary nephrotic syndrome. A total of 120 children with primary nephrotic syndrome kidney tissues who underwent renal biopsy were selected as the observation group, which can be divided into minimal change disease type (40 cases), membranous nephropathy type (20 cases), mesangial proliferative glomerulonephritis type (30 cases) and focal segmental glomerulosclerosis type (30 cases). In addition, the normal kidney tissues of 20 children with Wilms tumor admitted to the same period were selected as the control group and immunohistochemistry was used. The method was used to detect the expression levels of toll-like receptor 1 and toll-like receptor 3 in the kidney tissues of the two groups. Compared with the control group, the different pathological types in the observation group had higher expression levels of toll-like receptor 1 in renal tubular tissues. The expression levels of toll-like receptor 1 in minimal change disease type renal tubular tissues in different pathological types of tissues were significantly higher than those in other groups, followed by membranous nephropathy. In mesangial proliferative glomerulonephritis type and focal segmental glomerulosclerosis type, the difference was statistically significant (all p<0.05). Compared with the control group, the different pathological type groups in the observation group had higher expression levels of toll-like receptor 3 in renal tubular tissues. The expression levels of toll-like receptor 3 in mesangial proliferative glomerulonephritis type renal tubular tissues in different pathological types of tissues were significantly higher than those in other groups, followed by membranous nephropathy type, minimal change disease type and focal segmental glomerulosclerosis type, the difference was statistically significant (all p<0.05). Toll-like receptor 1 and toll-like receptor 3 have high expression levels in the renal tissues of children with primary nephrotic syndrome and their expression levels have certain differences in different pathological types.

An Unusual Complication of Mesenteric-Portal Axis Thrombosis and Septic Shock in a Patient with Mesangial Proliferative Glomerulonephritis

Thromboembolic events contribute to the most serious outcomes in patients with nephrotic syndrome. We report a case of a young male patient with mesangial proliferative glomerulonephritis presenting with nephrotic syndrome, which rapidly progressed to mesenteric and portal veins thrombosis and septic shock, ultimately leading to death.

The adverse effect of long term intake of Monosodium Glutamate on kidney performance

Monosodium glutamate (MSG) is a food additive that is considered as a water and environmental pollutant and affects the tissues of the living being. This study was aimed to find the effect of long-term administration of MSG on the mass of mesangial cells of the kidneys. Forty adult male rats were divided into four groups (10 each). Control groups 1&2 were supplied orally with distilled water for 30 and 60 days, respectively. Treatment groups 1&2 were supplied orally with 15 mg/kg Bwt of MSG for 30 & 60 days, respectively. Control and treatment groups were sacrificed, specimens of kidneys were obtained, fixed with 10% neutral buffered formalin, processed by Routine histological techniques, stained by Hematoxylin and eosin, and PAS (Periodic Acid-Schiff) stains then examined under the light microscope. The result found enlargement in a mesangial mass represented by hypertrophy and hyperplasia of mesangial cells leading to mesangial proliferative glomerulonephritis. Accordingly, the study showed an increase in creatinine values, indicating a disturbance in renal function. This will lead to a decrease in the sizes of the glomeruli of renal corpuscles and a relative increase of Bowman’s space. With the time of the experiment, the glomerular capillaries and gates of basement membranes will be closed, resulting in renal filtration disorders. It was concluded that the long-term intake of MSG leads to indirect narrowing of the glomerular capillary lumen, causing kidney failure.

Ангиопатия при ревматоидном артрите

Актуальность. Среди всех болезней ревматологического профиля на долю ревматоидного артрита (РА) приходится 3 % случаев, причем численность таких больных растет. Для РА характерно системное поражение сосудов (ангиопатия), характеризующееся по международной Чэпел-Хиллской классификации как «васкулит, связанный с системным заболеванием». Цель работы: улучшить качество диагностики, установить новые звенья патогенеза и выделить прогностические критерии течения поражения сосудов при РА. Материалы и методы. Под наблюдением находился 131 больной РА. Соотношение мужчин и женщин составило 1 : 2, минимальная, умеренная и высокая степени активности заболевания — соответственно 1 : 2 : 1, средний возраст обследованных пациентов составил 45,70 ± 1,02 года, длительность клинической манифестации — 9,40 ± 0,68 года, I, II, III и IV стадии диагностированы у 8, 40, 34 и 19 % от числа больных соответственно. Выполняли эхокардиографию, сонографию и ультразвуковую допплерографию сосудов, биомикроскопию конъюнктивы, морфологическое исследование нефробиоптатов, определяли интегральные индексы клинической и инструментальной сосудистой патологии. Результаты. Системная ангиопатия наблюдалась у 61 % от числа больных РА, чаще в случаях высокой степени активности при наличии остеопороза, при этом развитие васкулита кожи и периферической вазонейропатии было тесно связано с уровнем в сыворотке крови антител к циклическому цитруллиновому пептиду, который, наряду с концентрацией С-реактивного протеина, обладает негативной прогностической значимостью в отношении сосудистой патологии. Появление дигитального артериита определяется активностью суставного синдрома, гломерулонефрита — высоким содержанием в крови циркулирующих иммунных комплексов, а наличие ангиопатии отражает повышение давления в малом круге кровообращения. Выводы. У больных РА развивается мезангиопролиферативный или мезангиокапиллярный гломерулонефрит в соотношении 2 : 1 с закономерным тубулоинтерстициальным компонентом и депозицией иммуноглобулинов и компонентов комплемента (в строме → клубочках → канальцах → сосудах), при этом структурные изменения сосудов почек тесно связаны с клинико-инструментальными проявлениями системной ревматоидной ангиопатии. Показатели в крови C-реактивного протеина более 25 мг/л и противоцитруллиновых антител более 40 Е/мл являются прогнознегативными критериями в отношении системной сосудистой патологии и повреждений стромы почек, а тяжелые изменения почечных канальцев относятся к факторам риска высоких темпов прогрессирования суставного синдрома.

Long-term renal outcomes of mesangial proliferative lupus nephritis in Chinese patients.

This study aimed to explore the long-term outcomes of mesangial proliferative lupus nephritis (LN class II) and the factors associated with its relapse and histological transformation in Chinese patients. 104 SLE patients with biopsy-proven LN class II were included and divided into proteinuria group (proteinuria ≥ 0.4g/24h, with or without microscopic hematuria) and hematuria group (microscopic hematuria with proteinuria < 0.4g/24h).Patients were treated with glucocorticoid alone (GC monotherapy) or GC in combination with other immunosuppressant (combination therapy). The rates of remission, relapse, histological transformation, end-stage renal disease (ESRD), adverse events, and risk factors related to the outcomes were analyzed. During the median follow-up of 77.5 (IQR 58-116.5) months, all the 104 patients achieved remission. Relapse occurred in 69 cases (66.3%), of which 37 were of renal relapse (35.6%). Histological transformation was found in 14 of the 16 (87.5%) cases who received repeated renal biopsy after renal relapse. At the end of follow-up, 3 (2.9%) patients developed ESRD. There were no significant differences in the rates of relapse, histological transformation, adverse events and in the time from remission to relapse between the proteinuria group and the hematuria group. In contrast, the cumulative relapse rate in the GC monotherapy group was much higher than that in the combination group (P < 0.01). Adverse events occurred in 55 (57.3%) patients during follow-up. Patients with LN class II have high rates of relapse and renal histological transformation and need optimal maintenance therapy. • The rates of relapse and histological transformation are high in patients with LN class II. • Patients with LN class II are suggested to receive combination therapy and consider repeat renal biopsy after renal relapse.

Renal Outcome of IgM Nephropathy: A Comparative Prospective Cohort Study.

Immunoglobulin M nephropathy (IgMN) is an idiopathic glomerulonephritis characterized by diffuse deposits of IgM in the glomerular mesangium. However, its renal prognosis remains unknown. We compared renal outcomes of IgMN patients with those of patients with minimal change disease (MCD), focal segmental glomerulosclerosis (FSGS), or mesangial proliferative glomerulonephritis (MsPGN) from a prospective observational cohort, with 1791 patients undergoing native kidney biopsy in eight hospitals affiliated with The Catholic University of Korea between December 2014 and October 2020. IgMN had more mesangial proliferation and matrix expansion than MsPGN and more tubular atrophy and interstitial fibrosis than MCD. IgMN patients had decreased eGFR than MCD patients in the earlier follow-up. However, there was no significant difference in urine protein or eGFR among all patients at the last follow-up. When IgMN was divided into three subtypes, patients with FSGS-like IgMN tended to have lower eGFR than those with MCD-like or MsPGN-like IgMN but higher proteinuria than MsPGN-like IgMN without showing a significant difference. The presence of hypertension at the time of kidney biopsy predicted ≥20% decline of eGFR over two years in IgMN patients. Our data indicate that IgMN would have a clinical course and renal prognosis similar to MCD, FSGS, and MsPGN.

Гломерулярные поражения почек у детей с ювенильным ревматоидным артритом (обзор литературы)

Обзор литературы посвящен описанию различных форм гломерулонефрита (ГН) у детей с полиартикулярной и системной формами ювенильного ревматоидного артрита (ЮРА). В доступной литературе представлен 21 клинический случай развития ГН, а именно: ANCA-ассоциированный ГН, мезангиопролиферативный ГН, в том числе IgA- и IgМ-нефропатия, мембранозная нефропатия, фокально-сегментарный гломерулосклероз, болезнь минимальных изменений, экстракапиллярный ГН. Механизм гломерулярных поражений при ЮРА объясняется гиперпродукцией провоспалительных цитокинов, а также нефротоксическим действием базисных противовоспалительных лекарственных средств. Подробно проанализированы клинические проявления и эффективность проводимой терапии каждого варианта ГН у детей с ЮРА. Больше всего публикаций посвящено ANCA-ассоциированному ГН, который развился у пациентов с торпидным течением и высокой степенью активности полиартикулярной и системной форм ЮРА. Особенностью ANCA-ассоциированного ГН явилось наличие гиперкреатининемии и практически в половине случаев— развитие терминальной почечной недостаточности, несмотря на проводимую иммунодепрессивную терапию. Единичные случаи других вариантов ГН описаны более 10 лет назад. Клинически отмечались протеинурия и реже— нефротический синдром, что стало основанием для прижизненного морфологического исследования почек. Иммунодепрессивная терапия была эффективной при мезангиопролиферативном ГН и болезни минимальных изменений. Во всех случаях при фокально-сегментарном гломерулосклерозе, экстракапиллярном ГН отмечено формирование терминальной почечной недостаточности. Благоприятный прогноз оказался у детей с мембранозной нефропатией, индуцированной препаратами, после отмены последних. Представлены публикации с положительным терапевтическим эффектом применения генно-инженерных биологических препаратов при ANCA-ассоциированном ГН, IgМ-нефропатии, гормонорезистентном варианте болезни минимальных изменений у детей с ЮРА.