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

BACKGROUND/AIMS: Although renal manifestations are often involved in patients with rheumatoid arthritis (RA), the causal relationship between RA and renal manifestations has not been clearly defined. The prevalence and causes of renal manifestations in patients with RA were investigated in this study. METHODS: The clinical data from 457 patients with RA and who were admitted to Hanyang University Hospital between 2001 and 2005 were retrospectively analyzed. Renal manifestations were defined as proteinuria (> or =300 mg/day) or azotemia (serum creatinine > or =1.7 mg/dL), with or without hematuria. RESULTS: Renal manifestation was present in 82 (17.9%) out of 457 RA patients. Among them, proteinuria was observed in 81 (17.7%), azotemia in 37 (8.1%) and hematuria with either proteinuria or azotemia in 35 (7.7%). For the cases with proteinuria, the amount of preteinuria was 1353+/-207 (mean+/-SD) mg/day. There was no significant correlation between the degree of proteinuria and the duration of RA. For the cases with azotemia, the serum creatinine was 3.98+/-0.35 mg/dL. The presence of azotemia had no significant association with the duration of RA (14.4+/-1.5 vs. 11.6+/-1.2 years, respectively). When the etiology of the renal manifestation was classified into primary and secondary renal disease, the latter included diabetic nephropathy in 13 (15.9%), hypertensive nephrosclerosis in 8 (9.8%), drug induced chronic tubulointerstitial disease in 11 (13.4%) and AA amyloidosis in 2. Renal biopsy revealed 10 cases of primary glomerulopathy, including IgA nephropathy in 3, membranous nephropathy in 2, mesangial proliferative glomerulonephritis in 1, focal segmental glomerulosclerosis in 1 and chronic sclerosing glomerulonephritis in 3. CONCLUSIONS: The prevalence of chronic kidney disease in patients with RA is high, although direct renal invasion by RA is very rarely encountered. Renal biopsy would be of great help to identify the various causes of renal manifestations in patients with RA.
Amyloidosis ; Arthritis, Rheumatoid ; Azotemia ; Biopsy ; Creatinine ; Diabetic Nephropathies ; Glomerulonephritis ; Glomerulonephritis, IGA ; Glomerulonephritis, Membranous ; Glomerulosclerosis, Focal Segmental ; Hematuria ; Humans ; Nephrosclerosis ; Prevalence ; Proteinuria ; Renal Insufficiency, Chronic ; Retrospective Studies

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

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

The association between malignancy and glomerular disease has been reported in older patients. Although the relationship between membranous glomerulonephritis or minimal change disease and solid tumors or hematologic malignancies, respectively, are known widely, focal segmental glomerulosclerosis have been described rarely in patients with solid tumors. We describe a patient with renal cell carcinoma who presented to nephrotic syndrome, volume overload and renal failure. On renal biopsy at contra-lateral kidney of renal cell carcinoma, the patient was diagnosed at focal segmental glomerulosclerosis. The proteinuria and renal function were ameliorated after resection renal cell carcinoma. We suggest that the focal segmental glomerulosclerosis is associated with renal cell carcinoma. This is the first case of the clinical cause of focal segmental glomerulosclerosis associated with renal cell carcinoma of papillary type.
Biopsy ; Carcinoma, Renal Cell ; Glomerulonephritis, Membranous ; Glomerulosclerosis, Focal Segmental ; Hematologic Neoplasms ; Humans ; Kidney ; Nephrosis, Lipoid ; Nephrotic Syndrome ; Proteinuria ; Renal Insufficiency

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

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

A 17-year-old man with psoriasis developed albuminuria and microscopic hematuria. Renal biopsy revealed a glomerulonephritis with features of both membranous glomerulonephritis and IgA nephropathy. Histologically the glomeruli exhibited variable degree of mesangial expansion and hypercellularity, three of which showed segmental hyalinosis and/or sclerosis. Direct immunofluorescence demonstrated granular IgG-bearing deposits along the peripheral glomerular capillaries and IgA deposits in the mesangium. His urinary abnormalities persisted after the remission of skin lesion induced by PUVA treatment. It suggests that although the psoriasis may induce the renal lesion, it is insufficient to treat only the skin lesion for clinical improvement of glomerulonephritis after the renal lesion is already established.
Adolescent ; Albuminuria ; Biopsy ; Capillaries ; Fluorescent Antibody Technique, Direct ; Glomerulonephritis ; Glomerulonephritis, IGA* ; Glomerulonephritis, Membranous* ; Glomerulosclerosis, Focal Segmental ; Hematuria ; Humans ; Immunoglobulin A* ; Psoriasis* ; Sclerosis ; Skin

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