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

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

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

Recent change in major indication for renal biopsy from nephrotic syndrome to asymptomatic urinary abnormality (AU) in Korea makes IgA nephropathy the most common biopsy-confirmed glomerulonephritis (GN). Beside from the most common manifestation of AU, a significant portion (22%) of IgA nephropathy (IgAN) patient revealed the nephrotic-range proteinuria. About 1/3 of patients with minimal change disease (MCD), membranous nephropathy (MN) and focal segmental glomerulosclerosis (FSGS) presented with AU. In elderly patient (> 60 years), the proportion of cresentic glomerulonephritis, MN, and FSGS increased, while the prevalence of IgAN decreased compared to younger patients. MCD showed no change based on old age. GN was the third most common cause of ESRD constituting 11-15% of cases. ESRD patients from GN start dialysis younger and live longer on dialysis compared to diabetic and hypertensive patients in Korea.
Aged ; Biopsy ; Corneal Dystrophies, Hereditary ; Dialysis ; Glomerulonephritis ; Glomerulonephritis, IGA ; Glomerulonephritis, Membranous ; Glomerulosclerosis, Focal Segmental ; Humans ; Kidney Failure, Chronic ; Korea ; Nephrosis, Lipoid ; Nephrotic Syndrome ; Prevalence ; Proteinuria