Glomerular Disease Associated with Takayasu Arteritis:6 Cases Analysis and Review of the Literature

Objective To evaluate the clinical features,renal histopathology and therapeutic response to glucocorticoid and immunosuppressive agents in patients with glomerular disease associated with Takayasu arteritis(TA).Methods Patients with TA and renal biopsy-confirmed glomerular disease were investigated retrospectively.None of them had renal artery stenosis or occlusive changes.Results Six patients with glomerulopathy,accounting for 3.75% of the 160 TA patients admitted to our hospital at the same period,were analyzed.All of them were females with a mean age of 35.5 ± 10.0 years.Four cases presented with lower extremity edema.Laboratory tests showed that one was nephrotic syndrome,three were nephrotic range proteinuria,and two of them had mild renal dysfunction.The other two patients were asymptomatic microscopic hematuria and proteinuria.Renal pathology revealed mild immunoglobulin A nephropathy in two cases,mild mesangial proliferative glomerulonephritis(GN),membranoproliferative GN,minimal change disease,and fibrillary GN in one case respectively.Five cases received glucocorticoids and cyclophosphamide therapy.Proteinuria and microscopic hematuria disappeared in 2 to 4 weeks after the initiation of therapy in three cases.The patient with membranoproliferative GN also reached complete remission of proteinuria and recovered renal function 6 months after the treatment.Conclusions TA may induce glomerular disease as a part of its histological spectrum.Apart from ischemic glomerular disease,glomerular disease should be suspected when TA patients have microscopic hematuria or proteinuria,that may be therapeutically responsive to glucocorticoids and immunosuppressive agent in relative early phase.

Primary glomerular diseases in the elderly

Primary glomerular diseases in the elderly population are a frustrating topic due to difficulties in both the diagnosis and decision making about treatment. The most frequent type of primary glomerular disease in elderly is membranous nephropathy; while its counterpart in younger population is Ig A nephropathy. The most frequent cause of nephrotic syndrome in the elderly is also membranous nephropathy. Pauci-immune crescentic glomerulonephritis(GN) rate increases both in elderly and very elderly population. Pauci-immune crescentic GNs should be regarded as urgencies in elderly patients as in their younger counterparts due to potential for causing end-stage renal disease in case of delayed diagnosis and treatment, and also causing mortality due to alveolar hemorrhage in patients with pulmonary involvement. Renal biopsy is the inevitable diagnostic method in the elderly as in all other age groups. Renal biopsy prevents unnecessary treatments and provides prognostic data. So advanced age should not be the sole contraindication for renal biopsy. The course of primary glomerular diseases may differ in the elderly population. Acute kidney injury is more frequent in the course and renal functions may be worse at presentation. These patients are more prone to be hypertensive. The decision about adding immune suppressive therapies to conservative methods should be made considering many factors like co-morbidities, drug side effects and potential drug interactions, risk of infection, patient preference, life expectancy and renal functions at the time of diagnosis.

Spectrum of Primary Glomerular Diseases in Patients Presenting with Urinary Abnormalities

Introduction: Primary Glomerular Diseases are a spectrum of renal disorders of unknown aetiology with distinct characteristics, specific natural history and prognosis. A thorough evaluation is prerequisite to establish the diagnosis since many systemic diseases and secondary aetiology masquerade as primary diseases. Methods: This prospective observational study was conducted at a tertiary care centre and included 30 patients, with clinical features suggestive of primary glomerular diseases, of which 23 patients (76.6%) were males and 07 patients (23.33%) were females. The mean age at presentation was 37.23 ± 12.89 years. Among the observed spectrum of Primary Glomerular Diseases, IgA Nephropathy (IgA N) was seen in 26.67% patients, Focal Segmental Glomerulosclerosis (FSGS) in 20% patients and Membranous Glomerulopathy (MGN) in 13.33% patients, whereas the incidence of other abnormalities had less percentage contribution. Proteinuria was the commonest presentation seen in 60% patients, followed by Microscopic Haematuria in 20%. Mean Serum Creatinine was 0.99 ± 0.16 mg/dl. Mean Serum Albumin was 2.51 ± 0.76 gm/dl. Overall Nephrotic range proteinuria was observed in 15 (50%) patients. Results: IgA N, FSGS & MGN were the commonest observed Primary Glomerular Diseases. Proteinuria, Haematuria, Anasarca and Pedal Oedema were the commonest observed clinical presentations. Conclusions: In this studied series IgA Nephropathy, FSGS and MGN were the most prevelant diagnoses in the patients presenting with Urinary Abnormality. Nephrotic range Protenuria was the major indication for biopsy, there is a temporal variation in glomerulopathies wherein there is increase in incidence of IgA Nephropathy and decrease in incidence of FSGS.

Patterns of kidney diseases diagnosed by kidney biopsy and the impact of the COVID-19 pandemic in Yogyakarta,Indonesia:A single-center study

BACKGROUND Glomerular diseases rank third among the causes of chronic kidney disease worldwide and in Indonesia,and its burden continues to increase,especially regarding the sociodemographic index.Kidney biopsy remains the gold standard for the diagnosis and classification of glomerular diseases.It is crucial for developing treatment plans,determining the degree of histologic changes,and identifying disease relapse.AIM To describe the patterns of biopsy-proven kidney diseases in adult patients.METHODS We retrospectively reviewed the demographic,histopathologic,clinical,and laboratory data of 75 adult patients with biopsy-proven kidney diseases at our institution recorded from 2017 to 2022.RESULTS Among the patients,43(57.3%)were females,and the mean age was 31.52 years±11.70 years.The most common histopathologies were lupus nephritis(LN)(33.3%),minimal change disease(MCD)(26.7%),and focal segmental glomerulosclerosis(10.7%).LN(41.7%)was frequently diagnosed in women and MCD(28.1%)in men.The most common cause of nephritic syndrome was LN(36.7%)and of nephrotic syndrome was MCD(40%).CONCLUSION Different kidney disease patterns were observed in different sexes,age categories,clinical syndromes,and biopsy dates relative to the coronavirus disease 2019 pandemic.

Study on the Relationship between Blood Stasis Syndrome and Clinical Pathology in 227 Patients with Primary Glomerular Disease

To investigate the relationship between the severity of Chinese medicine(CM) bloodstasis syndrome(BSS) with clinical features and renal lesion indexes of the primary glomerular disease. Methods:An epidemiological survey was conducted to collect the data of 227 patients diagnosed as chronic primary glomerular diseases,and their severity of BSS were scored three days before renal biopsies were performed.The following clinical indexes were analyzed:age,course of glomerular diseases,24-h urine protein ration(Up...

Multifaceted relationship between diabetes and kidney diseases:Beyond diabete

Diabetes mellitus is one of the most common causes of chronic kidney disease.Kidney involvement in patients with diabetes has a wide spectrum of clinical presentations ranging from asymptomatic to overt proteinuria and kidney failure.The development of kidney disease in diabetes is associated with structural changes in multiple kidney compartments,such as the vascular system and glomeruli.Glomerular alterations include thickening of the glomerular basement membrane,loss of podocytes,and segmental mesangiolysis,which may lead to microaneurysms and the development of pathognomonic Kimmelstiel-Wilson nodules.Beyond lesions directly related to diabetes,awareness of the possible coexistence of nondiabetic kidney disease in patients with diabetes is increasing.These nondiabetic lesions include focal segmental glomerulosclerosis,IgA nephropathy,and other primary or secondary renal disorders.Differential diagnosis of these conditions is crucial in guiding clinical management and therapeutic approaches.However,the relationship between diabetes and the kidney is bidirectional;thus,new-onset diabetes may also occur as a complication of the treatment in patients with renal diseases.Here,we review the complex and multifaceted correlation between diabetes and kidney diseases and discuss clinical presentation and course,differential diagnosis,and therapeutic opportunities offered by novel drugs.

Epidemiology of biopsy-proven glomerular diseases in Chinese children: A scoping review

Background: Glomerular disease is the leading cause of chronic kidney disease globally. No scoping review reports have focused on China’’s spectrum of glomerular diseases in children. This study aimed to systematically identify and describe retrospective studies on pediatric glomerular disease based on available data on sex, age, study period, and region.Methods: Six databases were systematically searched for relevant studies from initiation to December 2021 in PubMed, Embase, Web of Science, Global Health Library, Wangfang Database, and CNKI.Results: Thirty-four studies were identified in the scoping review, including 40,430 patients with biopsy-proven diagnoses. The proportion of boys was significantly higher than that of girls. In this study, 28,280 (70%) cases were primary glomerular disease, 10,547 (26.1%) cases were diagnosed as secondary glomerular disease, and 1146 (2.8%) cases were hereditary glomerular disease. Minimal change disease is the most common glomerular disease among children in China, followed by mesangial proliferative glomerulonephritis, IgA nephropathy, and purpura nephritis. We observed increments in glomerular diseases in periods 2 (2001–2010) and 3 (2011–2021). The proportion of major glomerular diseases varies significantly in the different regions of China.Conclusion: The spectrum of pediatric glomerular diseases varied across sex, age groups, study periods, and regions, and has changed considerably over the past 30 years.

A STUDY OF THE RELATIONSHIP BETWEEN URINARY ALBUMIN EXCRETION QUANTITY AND SERUM CHOLESTEROL IN PRIMARY GLOMERULAR DISEASE

A study was made to analyse.the relationship between serum total cholesterol(STC) and the other three parameters,urinary albumin excretion quantity(UAEQ), serum β2-microglobulin(Sβ2-MG), serum albumin(SA)in a group of 38 patients with primary glomerular disease. The patients were classified into two groups according to proteinuria and serum albumin:Nephr it is group: proteinuria<3.5 g/d,serum albumin>30 g/L;Nephrosis group:proteinuria≥3.5 g/d,serum albumin≤30g/L. STC showed higher correlation with UAEQ(P< 0. 05￣0. 01),but did not have any significant correlation with Sβ2-MG berore and after the treatment or the two groups.STC had negative correlation with SA berore the treat ment of nephrosis group(P<0.01),but STC had no significant correlation with SA in nephritis group and after the treatment of nephrosis group. These data suggest that the decrease of SA is not the sole cause of hypercholestcrolemia. The presence of an association between STC and UAEQ indicates that the kidneys play a certain role in cholesterol metabolism.

Review of plasma exchange and rituximab for prevention of recurrent focal segmental glomerulosclerosis after a prior graft loss

BACKGROUND Focal segmental glomerulosclerosis(FSGS)often recurs after transplantation,leading to graft dysfunction and graft loss.Patients who have lost prior grafts due to recurrence are at particularly high risk of re-recurrence in subsequent grafts.Rituximab and plasma exchange have been used pre-emptively to prevent post-transplant recurrence.However,the efficacy of such preventative measures remains unclear.AIM To investigate the outcomes of preventative rituximab and plasma exchange for recurrent FSGS in transplant recipients after prior graft loss.METHODS We conducted a systematic review of 11 studies with 32 patients who had experienced prior graft loss due to post-transplant FSGS recurrence and were treated with either pre-emptive plasma exchange alone,rituximab alone,or a combination of both.RESULTS Overall,47%of the 32 patients experienced recurrence despite prophylactic treatment.Re-recurrence was seen in 25%(1/4)with pre-emptive rituximab alone,and 45%recurrence(9/20)with plasma exchange alone.Re-recurrence was noted in 63%with the use of combined plasma exchange and rituximab.CONCLUSION There is a paucity of available evidence in the literature to draw clear conclusions on the benefits of pre-emptive measures to prevent FSGS re-recurrence.The small sample sizes and variations in protocols call for larger and controlled studies to serve this patient population at high risk of recurrence and graft loss.

IgA nephropathy treatment with traditional Chinese medicine:A case report

BACKGROUND IgA nephropathy(IgAN)is a common primary glomerular disease that leads to end-stage renal disease with poor therapy efficacy.Traditional Chinese medicine(TCM)is effective in the treatment of IgAN and has the potential to become an alternative treatment for IgAN.Professor Yan-Qin Zou is a nephropathy expert,a National Chinese Medicine Master,and an heir to the Menghe School of Medicine.CASE SUMMARY A 28-year-old man had positive urinary protein and elevated serum creatinine(Scr)results and was diagnosed with IgAN 2-3 years prior to the outpatient department visit at our hospital in 2017.Professor Zou used the following methods to treat the patient:Invigorating the spleen and tonifying the kidney,removing dampness and clearing turbidity,quickening the blood and transforming stasis,and freeing vessels and regulating collaterals.She adjusted the prescription in accordance with the patient’s symptoms.After 6 mo of treatment,the symptoms had resolved and serological indexes were also decreased[Scr from 288.5 to 188.6μmol/L,blood urea nitrogen(BUN)from 10.9 to 9.5 mmol/L,serum uric acid(UA)from 612 to 503μmol/L].During follow-up,BUN,Scr,and UA levels remained stable.CONCLUSION Professor Zou’s therapeutic strategy to treat IgAN using TCM was efficacious and a good reference for application.

Nephrotic syndrome in syngeneic hematopoietic stem cell transplantation recipients: A case report

BACKGROUND Hematopoietic stem cell transplantation(HSCT)is widely used in the treatment of hematological diseases.However,complications after transplantation,such as acute and chronic graft-vs-host disease(GVHD),still seriously affect the quality of life and even threaten the lives of patients.There is evidence that glomerular diseases can manifest as GVHD.However,GVHD should not occur as a result of syngeneic HSCT.CASE SUMMARY A 20-year-old male diagnosed with T lymphoblastic lymphoma(stage IIIA,aaIPI 1)in September 2013 was treated with six cycles of hyper-CVAD and achieved complete remission.He underwent syngeneic HSCT in June 2014,and had no kidney disease history before the transplant.However,nephrotic syndrome occurred 24 mo later in the patient after syngeneic HSCT.Renal biopsy was performed,which led to a diagnosis of atypical membranous nephropathy.After treatment with glucocorticoids combined with cyclophosphamide and cyclosporine,the nephrotic syndrome was completely relieved.CONCLUSION We report a case of delayed nephrotic syndrome after syngeneic HSCT.Antibodymediated autoimmune glomerular disease may be the underlying mechanism.After treatment with immunosuppressive agents,the nephrotic syndrome was completely relieved but further long-term follow-up is still needed.

Establishment and functional characterization of the reversibly immortalized mouse glomerular podocytes(imPODs)

Glomerular podocytes are highly specialized epithelial cells and play an essential role in establishing the selective permeability of the glomerular filtration barrier of kidney.Maintaining the viability and structural integrity of podocytes is critical to the clinical management of glomerular diseases,which requires a thorough understanding of podocyte cell biology.As mature podocytes lose proliferative capacity,a conditionally SV40 mutant tsA58-immortalized mouse podocyte line(designated as tsPC)was established from the Immortomouse over 20 years ago.However,the utility of the tsPC cells is hampered by the practical inconvenience of culturing these cells.In this study,we establish a user-friendly and reversibly-immortalized mouse podocyte line(designated as imPOD),on the basis of the tsPC cells by stably expressing the wildtype SV40 T-antigen,which is flanked with FRT sites.We show the imPOD cells exhibit long-term high proliferative activity,which can be effectively reversed by FLP recombinase.The imPOD cells express most podocyte-related markers,including WT-1,Nephrin,Tubulin and Vinculin,but not differentiation marker Synaptopodin.The imPOD cells do not form tumor-like masses in vivo.We further demonstrate that TGFb1 induces a podocyte injury-like response in the FLP-reverted imPOD cells by suppressing the expression of slit diaphragm-associated proteins P-Cadherin and ZO-1 and upregulating the expression of mesenchymal markers,a-SMA,Vimentin and Nestin,as well as fibrogenic factors CTGF and Col1a1.Collectively,our results strongly demonstrate that the newly engineered im-POD cells should be a valuable tool to study podocyte biology both under normal and under pathological conditions.

Indications and results of renal biopsy in children:a 36-year experience

Background This study was conducted to investigate retrospectively the indications for renal biopsy(RB)in native kidneys and to analyze pathological findings in a single tertiary pediatric hospital in Southern Italy for the last 36 years.Methods All patients who underwent RB at our hospital from 1979 to 2014 were included.All renal tissue specimens were studied under light and immunofluorescent microscopy,while electron microscopy was performed only for specific clinical indications.Results The study group included 213 patients(female 43.2%)who underwent 225 percutaneous native kidney biopsies.Median age was 10.4 years(range 0.6-24 years).The most frequent indication for RB was nephrotic syndrome(44.4%),fol-lowed by proteinuria(27.6%),asymptomatic hematuria(17.3%)and acute kidney injury(9.8%).Gross hematuria appeared after biopsy in less than 5%of the patients,but none of them needed blood transfusion.Adequate renal tissue sample was obtained in 95.5%of the renal biopsies.Primary glomerulonephritis(GN)was the most common finding(61.4%),followed by secondary GN(21.4%),tubulointerstitial diseases(3.7%)and hereditary nephropathy(2.8%),while in 10.7%of the cases,normal renal tissues were found.According to histopathological diagnosis,the most common causes of primary GN were IgA nephropathy(20.9%),followed by minimal change disease(18.1%)and focal segmental glomerulosclerosis(11.6%).Conclusions The epidemiology of glomerular disease in our single-center cohort is similar to that shown in other national and international reports.Moreover,our study shows that percutaneous ultrasound-guided RB is a safe,reliable and effec-tive technique in children.