The publication of the Kidney Disease: Improving Global Outcomes (KDIGO) guidelines on the treatment of glomerular diseases in 2012 marked a milestone in this field, asitisthe first time that comprehensive guidelines are provided for such disease entities. The current review focuses on major findings, both path ogenesis related and clinical, in the primary glomerulonephritis that have been made after the guidelines came into effect.
Glomerulonephritis* ; Glomerulonephritis, IGA ; Glomerulonephritis, Membranoproliferative ; Glomerulonephritis, Membranous ; Glomerulosclerosis, Focal Segmental ; Kidney Diseases ; Nephrosis, Lipoid ; Publications

BACKGROUND: Since the introduction of cyclosporine, the short-term renal allograft survival has significantly improved. However, the long-term success is still limited by the development of chronic rejection and recurrent disease. Post-transplant glomerulonephritis (post-Tx GN) including recurrent disease is becoming an important cause of graft dysfunction. METHODS: From November 1988 to June 2004, a total of 629 renal transplants involving 588 patients were performed at our medical center. RESULTS: The prevalence rate of post-Tx GN was 11.9% in 629 renal transplant. Among 75 transplants diagnosed as post-Tx GN, IgA nephropathy (62.7%) was the most common histologic diagnosis, followed by focal segmental glomerulosclerosis (26.7 %), membranous glomerulonephritis (8.0%), membranoproliferative glomerulonephritis (1.3%) and diabetic nephropathy (1.3%). Documented histologic recurrence occurred in only 24.2% of patients with prior biopsy-proven glomerulonephritis of their native kidneys. The actuarial allograft survival at 5 and 10 years posttransplantation with post-Tx GN was 80.5 % and 27.9%, respectively; and the corresponding graft survival for patients without post-Tx GN was 74.9% and 52.3%, respectively (p<0.05). However, there was no significant difference in the graft survival according to type of post-Tx GN. The 5 and 10 year graft survival for patients with proteinuria over than 3.5 g/24 hr were 62.5% and 0%, which is significantly lower compared with 85.3% and 28.7% for patients with proteinuria less than 3.5 g/24 hr (p<0.01). CONCLUSION: In conclusion, post-Tx GN is associated with decreased long-term graft survival and nephrotic range proteinuria is most important prognostic factor for graft survival. A prospective study with rigorous efforts to make pretransplant diagnosis and standardized criteria for allograft biopsy will more accurately characterize the natural history of post-Tx GN and may provide insight regarding treatment.
Allografts* ; Biopsy ; Cyclosporine ; Diabetic Nephropathies ; Diagnosis ; Glomerulonephritis* ; Glomerulonephritis, IGA ; Glomerulonephritis, Membranoproliferative ; Glomerulonephritis, Membranous ; Glomerulosclerosis, Focal Segmental ; Graft Survival ; Humans ; Kidney ; Natural History ; Prevalence ; Proteinuria ; Recurrence ; Transplants

PURPOSE: To evaluate the exact prevalence of primary glomerular diseases in Korea. METHODS: We analyzed a retrospective cohort of biopsy proven 1,100 patients with primary glomerular disease in OO Hospital from April 1990 to March 2010. RESULTS: Pathologic diagnosises of 1,100 cases were as follows: IgA nephropathy (IgAN), 557 cases (50.6%), was the most common followed by 200 cases (18.1%) of minor glomerular abnormalities (MGA), 168 cases (15.2%) of focal segmental glomerulosclerosis (FSGS), 93 cases (8.0%) of membranous nephropathy (MN), 31 cases (2.8%) of membranoproliferative glomerulonephritis type I (MPGN), 17 cases (1.5%) of focal glomerulonephritis and 7 cases (0.6%) of diffuse mesangial proliferative glomerulonephritis (DMGN) in order. In idiopathic nephrotic syndrome, the most common pathologic diagnosis was minimal change nephrotic syndrome (MCNS) (40.2%), followed by FSGS (27.5%), MN (24.2%), MPGN (8.1%) and DMGN (0.5%). When the incidence rates between 1990-1992 and 2008-2010 were compared, IgAN and FSGS increased from 34.7, 12.5 to 47.8%, 30.4%, but MCNS (from 33.3 to 6.5 %) decreased significantly. CONCLUSION: IgAN was the most common primary glomerulonephritis. During the past 20 years, the prevalence of IgAN and FSGS were increased, while MCNS and MN were decreased.
Biopsy ; Cohort Studies ; Glomerulonephritis ; Glomerulonephritis, IGA ; Glomerulonephritis, Membranoproliferative ; Glomerulonephritis, Membranous ; Glomerulosclerosis, Focal Segmental ; Humans ; Incidence ; Nephrosis, Lipoid ; Nephrotic Syndrome ; Prevalence ; Retrospective Studies

BACKGROUND: Chronic kidney disease has deleterious influences on pregnancy, both fetus and mother. To determine the pregnancy outcome and associated risk factors, we analyzed 36 pregnancies in 26 women with various chronic kidney diseases. METHODS: Retrospective analysis of 36 pregnancies was performed in women with chronic kidney disease who underwent antenatal care and delivery at Pusan National University Hospital from January 1993 to December 2002. RESULTS: The mean age of patients was 29.7 +/- 3.6 years. Underlying kidney disease was lupus nephritis in 10 patients (11 pregnancies), IgA nephropathy in 7 patients (8 pregnancies), focal segmental glomerulosclerosis in 4 patients (9 pregnancies), membranoproliferative glomerulonephritis in 4 patients (7 pregnancies), membranous glomerulonephritis in 1 patient (1 pregnancy). Of the 36 pregnancies, fetal loss occurred in 14 pregnancies (38.9%), premature delivery 7 pregnancies (19.4%) and normal delivery 15 pregnancies (41.7%). Deterioration of maternal renal function occurred in 10 pregnancies (27.8%), hypertension 18 pregnancies (50.0%) and aggravation of proteinuria 26 pregnancies (72.2%). Fetal loss and deterioration of maternal renal function were more frequent in patients with preconception serum creatinine value (SCr) >or=1.4 mg/dL than in those with SCr <1.4 mg/dL (85.7% vs. 27.6%, p<0.05; 100% vs. 10.3%, p<0.05, respectively). Neither fetal loss nor deterioration of maternal renal function was associated with hypertension and aggravation of proteinuria. In multivariate analysis, preconception SCr was associated with fetal loss (p=0.014, OR 32.7, 95% CI 2.0-526.0) and BP >or=140/90 mmHg during pregnancy was associated with low birth weight (p=0.027, OR 0.034, 95% CI 0.002-0.682). Deterioration of maternal renal function during pregnancy was recovered in 40.0%, hypertension was recovered in 50.0% and proteinuria was recovered in 77.8% within 1 year after delivery. CONCLUSION: Preconceptional impairment of maternal renal function and uncontrolled hypertension during pregnancy seem to be an important factors associated with fetal loss and low birth weight, respectively.
Busan ; Creatinine ; Female ; Fetus ; Glomerulonephritis, IGA ; Glomerulonephritis, Membranoproliferative ; Glomerulonephritis, Membranous ; Glomerulosclerosis, Focal Segmental ; Humans ; Hypertension ; Infant, Low Birth Weight ; Infant, Newborn ; Kidney Diseases ; Lupus Nephritis ; Mothers ; Multivariate Analysis ; Pregnancy ; Pregnancy Outcome* ; Pregnancy* ; Proteinuria ; Renal Insufficiency, Chronic* ; Retrospective Studies ; Risk Factors

PURPOSE: We examined the clinical characteristics and incidence of adults idiopathic nephrotic syndrome (NS) according to pathologic diagnosis, age, sex. METHODS: We retrospectively reviewed the clinical and pathological characteristics of primary glomerular lesions in adults idiopathic NS taken a renal biopsy from 1978 to 2005 at the Dongsan Medical Center. We compared the prevalence of adults idiopathic NS according to the pathologic diagnosis between two time intervals 1978 to 1990 and 1991 to 2005. RESULTS: The patients had mean age of 36.7+/-16.3 years and male to female ratio was 1.7:1 with male predominance. The frequency of histopathologic diagnoses were minimal change nephrotic syndrome (MCNS) 51.6%, membranous glomerulonephritis (MGN) 21.3%, focal segmental glomerulosclerosis (FSGS) 12.1%, IgA nephropathy 9.1%, membranoproliferative glomerulonephritis (MPGN) 4.2% in decreasing order of frequency. The mean age was youngest in MCNS (32.9+/-15.1) and oldest in MGN (46.2+/-16.6). Between 1978 to 1990 period and 1991 to 2005 period, the prevalence of MGN was significantly increased, whereas the prevalence of MPGN was decreased significantly. The prevalence of MCNS had a tendency to decrease and that of IgA nephropathy had a tendency to increase, however, both didn't reach statistical significance. The incidence of FSGS didn't show a significant change during the both study periods. CONCLUSION: MCNS was the most common disease among adults idiopathic NS. MGN was the most frequent etiology in patients older than 45 years. The incidence of MGN was increased over the 28-year period, and that of MPGN decreased significantly. There was no change in the frequency of FSGS.
Adult* ; Biopsy ; Diagnosis ; Female ; Glomerulonephritis, IGA ; Glomerulonephritis, Membranoproliferative ; Glomerulonephritis, Membranous ; Glomerulosclerosis, Focal Segmental ; Humans ; Incidence ; Korea* ; Male ; Nephrosis, Lipoid ; Nephrotic Syndrome* ; Prevalence ; Retrospective Studies

PURPOSE: The incidence of glomerular diseases varies according to population characteristics and time period. METHODS: A total of 3,000 renal biopsies were performed over the 29 years' period from 1978 to 2007. We reviewed the patient records of all patients who underwent renal biopsies at our institution. The patients were grouped for analysis in three time intervals: before 1990, 1991 to 2000, and after 2001. RESULTS: There were 2,377 cases of native kidney biopsies and 623 cases of allograft kidneys. The principal long-term changes were an increase in the mean age of patients with undergoing biopsy and an increase in the percentage of asymptomatic urinary abnormalities as an indication for biopsy. In the primary glomerulonephritis (GN), the most common pathologic diagnosis was IgA nephropathy (IgAN, 26.6%), followed by minimal change disease (MCD, 21.4%), membranous nephropathy (8.9%), focal segmental glomerulosclerosis (7.7%). The major changes noted in primary GN were a marked increase in the frequency of IgAN and decrease in the frequency of MCD. Major causes of secondary GN were lupus nephritis (37.9%), and hepatitis associated GN (28.9%). In allograft biopsies, acute rejection (42.3%) and chronic rejection (19.4%) were the two most common diagnoses. Documented complications of renal biopsies included perirenal hematoma (25.1%), but the rate of serious complications that required surgical intervention or embolization was very low (1.0%). There was no death or nephrectomy case in our study. CONCLUSION: IgAN was the most common primary GN in this study. The multi-center studies are needed to evaluate the distribution and changing trends of renal disease in Korea.
Biopsy ; Corneal Dystrophies, Hereditary ; Glomerulonephritis ; Glomerulonephritis, IGA ; Glomerulonephritis, Membranous ; Glomerulosclerosis, Focal Segmental ; Hematoma ; Hepatitis ; Humans ; Incidence ; Kidney ; Lupus Nephritis ; Nephrectomy ; Nephrosis, Lipoid ; Population Characteristics ; Rejection (Psychology) ; Transplantation, Homologous

BACKGROUND: The glomerulonephritis appears in various clinical presentations. Renal biopsy is important diagnostic tool for treatment decision and prognosis prediction of glomerulonephritis. We tried to study on the clinical presentations of glomerulonephritis through histopathological findings and treatment responses in elderly patients. METHODS: We made an retrospective analysis in elderly patients aged over 60 years performed renal biopsy in our medical center from January 1991 to February 2005. RESULTS: Of all the 42 patients, the mean age of the patients was 64.6+/-3.5 years, 24 male and 18 female patients were included(1.3:1). Patients aged 60-64 years (n=26, 62%) were majority. On clinical indications of renal biopsies, 17 patients had nephrotic syndrome, 10 had asymptomatic urinary abnormality, 5 had gross hematuria, and 5 had acute azotemia. The results of renal biopsies presented that 30 patients (71%) had primary glomerulonephritis, 9 patients (22%) had secondary glomerulonephritis. Among primary glomerulonephritis, membranous nephropathy (n=10) was most common, followed by IgA nephropathy (n=7),focal segmental glomerulos-clerosis (n=3), membranoproliferative glomerulonephritis (n=2), and mesangioproliferative glomerulonephritis (n=2). In secondary glomer-ulonephritis, there were diabetic nephropathy (n=2), lupus nephritis (n=2), cancer-related nephritis (n=2), poststreptococcal glomer-ulonephritis (n=1), Henoch-Schonlein nephritis (n=1), amyloidosis (n=1). In the cases of nephrotic syndrome, primary nephrotic syndrome (n=12, 71%) is more prevalent than secondary nephrotic syndrome (n=3, 18%). The most common cause of primary nephrotic syndrome was membranous nephropathy (n=8). The causes of asymptomatic urinary abnormality were IgA nephropathy (n=4), lupus nephritis (n=2), membranous nephropathy (n=1), mesangioproliferative glomerulonephritis (n=1). By the complications of renal biopsy, only a few patients presented new-onset hematuria, hematoma, aggravation of hypertension, but, severe complication including an infection or a death was none. 8 of 12 patients with primary nephrotic syndrome were treated, 5 patients of those were shown complete response (n=3) or partial response (n=2). CONCLUSION: Various clinical presentations of glomerulonephritis were shown to elderly patients. Number of complications after renal biopsies were relatively small, and good responsiveness to treatment could be expected in the elderly patients. Therefore it is reasonable to perform a renal biopsy if indicated.
Aged* ; Amyloidosis ; Azotemia ; Biopsy ; Diabetic Nephropathies ; Female ; Glomerulonephritis* ; Glomerulonephritis, IGA ; Glomerulonephritis, Membranoproliferative ; Glomerulonephritis, Membranous ; Hematoma ; Hematuria ; Humans ; Hypertension ; Lupus Nephritis ; Male ; Nephritis ; Nephrotic Syndrome ; Prognosis ; Retrospective Studies

The cells of glomerular mesangium is composed mostly of intrinsic contractile mesangial cells and a few macrophages. Injury to the mesangium is central to many glomerular diseases. This study was aimed to evaluate and compare the expressions of alpha-smooth muscle actin (ASMA) and lysozyme in the mesangium of various human glomerular diseases and also of according to the severity of their progressions. We performed immunohistochemical and transmission electromicroscopic examinations in 51 cases of renal biopsy including 5 normal kidneys. The results were as follows; (1) ASMA staining was negligible in normal glomeruli. (2) Increased ASMA staining was observed in the mesangium of glomeruli from all specimens of primary glomerular disease, regardless of their diagnosis. (3) The staining intensity of ASMA in mesangium was mild in minimal change disease and membranous glomerulonephritis, and strong in focal segmental glomerulosclerosis (FSGS), diffuse mesangial hypercellularity, membranoproliferative glomerulonephritis (MPGN), and IgA nephropathy (IgAN). (4) The staining intensity of ASMA have no correlation with mesangial immune deposits. (5) The staining intensity of ASMA in mesangium was inversely correlated with the disease progression in FSGS and IgAN. (6) Glomeruli showing global or segmental sclerosis invariably lacked ASMA. (7) Compared with ASMA, the mesangial cells with lysozyme expression were very rare, even though it was in proportion to ASMA staining. Interstitial ASMA expression was confined to fibrotic area in various glomerular diseases. In conclusion, the expression of ASMA and lysozyme in mesangium are increased in a variety of glomerular diseases, regardless of disease entity. Their intensity was in proportion to the mesangial cell proliferation. In progressive glomerulonephritis, such as IgAN and FSGS, the increased expression of ASMA was prominent in the early lesion, and decreased with the progression of the glomerular sclerosis.
Actin Cytoskeleton ; Actins* ; Biopsy ; Diagnosis ; Disease Progression ; Glomerular Mesangium ; Glomerulonephritis ; Glomerulonephritis, IGA ; Glomerulonephritis, Membranoproliferative ; Glomerulonephritis, Membranous ; Glomerulosclerosis, Focal Segmental ; Humans ; Kidney ; Macrophages ; Mesangial Cells ; Muramidase* ; Muscle, Smooth* ; Nephrosis, Lipoid ; Sclerosis

Kimura's disease is an angiolymphoid-proliferative disorder that manifests with benign subcutaneous swelling predominantly in the head and the neck. Kidney involvement, including proteinuria, occurs in 12-16% of patients with the disease, and 60-78% of such cases is nephrotic syndrome. Reported etiologies of nephrotic syndrome in Kimura's disease include membranous glomerulonephritis, mesangial proliferative glomerulonephritis, minimal-change disease, focal segmental glomerulosclerosis, diffuse proliferative glomerulonephritis and immunoglobulin A (IgA) nephropathy. There have been only two case reports of IgA nephropathy in Kimura's disease, in 1998. In this report, we present a third case of IgA nephropathy associated with Kimura's disease.
Angiolymphoid Hyperplasia with Eosinophilia ; Glomerulonephritis ; Glomerulonephritis, IGA* ; Glomerulonephritis, Membranous ; Glomerulosclerosis, Focal Segmental ; Head ; Humans ; Immunoglobulin A* ; Immunoglobulins* ; Kidney ; Neck ; Nephrotic Syndrome ; Proteinuria

Percutaneous renal biopsy is an essential component for diagnosis and management of glomerular diseases. In order to elucidate clinical and pathological features, 494 renal patients who had undergone renal biopsies at Gil Medical Center from January 1989 to June 1999 were studied retrospectively. The male to female ratio was 1.3 : 1 and average age was 33.2 years. There were 370(83.5%) cases of primary glomerular disease and 58(13.1%) cases of secondary glomerular disease. Among primary glomerular disease, IgA nephropathy was the most common(175 cases), followed by minimal change disease(84 cases), membranous glomerulonephritis(34 cases), and focal segmental glomerulosclerosis(33 cases). In secondary glomerular disease, lupus nephritis was the most common(21 cases), followed by 11 cases of hepatitis B associated glomerulonephritis, 9 cases of Henoch-Sch nlein purpura, and 4 cases of diabetic nephropathy. Among 99 cases of asymptomatic urinary abnormalities, IgA nephropathy was most common(69 cases) followed by 12 cases of thin basement membrane disease, 4 cases of minimal change disease, and 3 cases of focal segmental glomerulosclerosis, membranous glomerulonephritis, nonspecific glomerulonephritis. Among 159 cases of nephrotic syndrome, minimal change disease was most common(60 cases) followed by 25 cases of IgA nephropathy, 23 cases of focal segmental glomerulosclerosis, 21 cases of membranous glomerulonephritis, and 13 cases of lupus nephritis. Documented complication of renal biopsies included 23 cases of gross hematuria, 6 cases of perirenal hematoma, and 4 cases of infection. Death, AV fistula, aneurysm or serious compications that required surgical intervention were not reported. In conclusion, the percutaneous renal biopsy is relatively safe, and useful for diagnosis and management of glomerular diseases. The most common type of primary glomerular disease was IgA nephropathy.
Aneurysm ; Basement Membrane ; Biopsy* ; Diabetic Nephropathies ; Diagnosis ; Female ; Fistula ; Glomerulonephritis ; Glomerulonephritis, IGA ; Glomerulonephritis, Membranous ; Glomerulosclerosis, Focal Segmental ; Hematoma ; Hematuria ; Hepatitis B ; Humans ; Lupus Nephritis ; Male ; Nephrosis, Lipoid ; Nephrotic Syndrome ; Purpura ; Retrospective Studies