OBJECTIVETo evaluate the relation between the pathological features of lupus nephritis (LN) and the clinical activity and laboratory examination.

METHODSRenal biopsies were obtained from 49 cases of lupus nephritis and classified according to ISN/RPS(2003) classification. The clinical activity, laboratory results and the renal pathological features of the disease were analyzed.

RESULTSAll the cases showed pathologies in the kidney. Type IV and V LN cases had a high incidence of nephrotic syndrome, and type II and III cases frequently showed latent nephritis. The NIH index and biopsy index indicated the degree of pathological lesions and were significantly related to the clinical features. Nearly all the indexes including NIH index, biopsy index, the clinical activity and the laboratory examination suggested stronger activity of type IV LN than the other types.

CONCLUSIONThe pathological changes, clinical activity and laboratory examination results of LN are related with each other. The clinical activity and laboratory examination of LN can be used to estimate the pathological type and degree of renal lesion in LN.

Adolescent ; Adult ; Biopsy ; Child ; Female ; Humans ; Kidney ; pathology ; Lupus Nephritis ; classification ; diagnosis ; pathology ; Male ; Middle Aged ; Young Adult

PURPOSE: Systemic lupus erythematosus (SLE) is a chronic multisystemic autoimmune disease with complex clinical manifestations. It probably involves genetic, environmental and immunologic factors. In this study, we investigated the clinical manifestations, laboratory findings and prognosis of pediatric SLE to aid clinical care of pediatric SLE. METHODS: The data of 45 patients who were diagnosed as pediatric SLE in Severance Children's Hospital from Jan. 1996 to Dec. 2005 were analysed retrospectively. RESULTS: The mean age at diagnosis was 10.8 (0-15) years old. And the ratio of male to female patients was 1:4. The initial manifestations were facial edema (51.1 percent), malar rash (44.4 percent), and fever (28.9 percent). The ANA (97.8 percent), anti-ds DNA antibody (82.2 percent), lupus nephritis (71.1 percent), malar rash (71.1 percent), and cytopenia (66.7 percent) were the most common findings among the classification criteria by ACR (American College of Rhematology, 1997). CONCLUSION: Clinical manifestations and prognosis are various in pediatric SLE. Intensive studies of SLE in children should be continued for more effective treatment.
Adolescent ; Autoimmune Diseases ; Child* ; Classification ; Diagnosis ; DNA ; Edema ; Exanthema ; Female ; Fever ; Humans ; Immunologic Factors ; Lupus Erythematosus, Systemic* ; Lupus Nephritis ; Male ; Prognosis ; Retrospective Studies

We investigated the clinical features of nephrotic syndrome in Korea according to sex, age and pathologic diagnosis under the classification system supported by WHO. Adult nephrotic patients who underwent renal biopsy from 1979 to 1993 at the Department of Internal Medicine, Seoul National University Hospital, with the exception of diabetic nephropathy and multiple myeloma, were analyzed in terms of their clinical manifestations, laboratory data, and pathologic features. 1) The number of our total nephrotic patients was 956 and their mean age was 32.6. Male to female ratio was 1.96:1. 2) Among total nephrotic syndrome patients, there were 736 (77%) primary NS and 220 (23%) secondary NS patients. 3) The pathologic diagnoses of the primary NS were minimal change nephrotic syndrome (41.6%), membraneous nephropathy (20.9%), focal glomerulosclerosis (17.3%), IgA nephropathy (9%), membranoproliferative GN (4.5%), sclerosing GN (2.4%), and mesangial proliferative GN (2.2%). 4) Among primary NS patients with ages between 15 and 40, the percentage of minimal change nephrotic syndrome was highest(47.7%), while among those above 40 of age, membraneous nephropathy was most common(42.1%). 5) The pathologic diagnoses of the secondary NS were hepatitis B virus associated GN (46.8%), lupus nephritis (39.5%), amyloidosis (5.9%), post-infectious GN (2.7%), Henoch-Schoenlein nephritis (2.7%). We had one each case of thrombotic thrombocytopenic purpura, Fabry's disease, mixed essential cryoglobulinemia, light chain disease, and Alport's syndrome. In conclusion, minimal change nephrotic syndrome was the most common disease among the primary NS and hepatitis B virus associated GN was the most common among the secondary NS.
Adult* ; Amyloidosis ; Biopsy ; Classification ; Cryoglobulinemia ; Diabetic Nephropathies ; Diagnosis ; Fabry Disease ; Female ; Glomerulonephritis, IGA ; Glomerulosclerosis, Focal Segmental ; Hepatitis B virus ; Humans ; Internal Medicine ; Korea ; Lupus Nephritis ; Male ; Multiple Myeloma ; Nephritis ; Nephritis, Hereditary ; Nephrosis, Lipoid ; Nephrotic Syndrome* ; Purpura, Thrombotic Thrombocytopenic ; Seoul

In systemic lupus erythematosus patients, prevalence of renal involvement is high, even in the absence of overt clinical manifestation. Lupus nephritis can usually be categorized according to the widely used WHO classification. however, clinically significant renal disease unrelated to lupus nephritis have rarely been described in patients with SLE. Especially, there has been few reported cases of IgA nephropathy with SLE since first reported in 1995, but their relationship is not apparent yet. We experienced a 72-year-old female who was admitted to our hospital due to generalized edema, arthralgia, proteinuria, microscopic hematuria. Her diagnosis of IgA nephropathy was estabilished on biopsy. At the same time, she was diagnosed as SLE. in that the serology for ANA was positive at 1:640, lupus anticoagulant and anti-cardiolipin antibody were positive, hemolytic anemia, arthritis and oral ulcer. and eventually she was diagnosed end-stage renal disease 2 months later. In conclusion, atypical glomerular lesion in SLE should raise the possibility of a non-lupus glomerulopathy, including IgA nephropathy.
Aged ; Anemia, Hemolytic ; Arthralgia ; Arthritis ; Biopsy ; Classification ; Diagnosis ; Edema ; Female ; Glomerulonephritis ; Glomerulonephritis, IGA* ; Hematuria ; Humans ; Immunoglobulin A* ; Kidney Failure, Chronic ; Lupus Coagulation Inhibitor ; Lupus Erythematosus, Systemic* ; Lupus Nephritis ; Oral Ulcer ; Prevalence ; Proteinuria

About 50-80% of patients with lupus suffer from lupus nephritis which is one of major causes of morbidity and mortality. Renal pathologists and nephrologists should evaluate the degree of histological damages to establish therapeutic plans for lupus nephritis. In order to standardize definitions, to emphasize clinically relevant lesions, and to improve interobserver reproducibility, the International Society of Nephrology/Renal Pathology Society (ISN/RPS) classification was proposed. Recently, several retrospective validation studies concerning the utility of the ISN/RPS classification, especially among class IV, were performed. In these reports, reproducibility is improved by the definition of diagnostic term, but the outcome related with classification, especially in class IV, is controversial. We performed retrospective analysis of 99 biopsy- proven subjects with lupus nephritis in our facility using the ISN/RPS classification. The class IV-G group tended to exhibit a worse renal outcome, but the difference compared with IV-S was not significant. In a Cox proportional hazards models, Independent histological predictors of poor renal outcome were extracapillary proliferation, glomerular sclerosis and fibrous crescents, while hyaline thrombi and fibrous adhesions were of favorable renal outcome. Both were similarly observed in IV-G and IV-S. The more qualitative categorization by the response to standard treatment may be needed to emphasize clinically relevant lesion related to renal outcome.
Cell Proliferation ; Humans ; Kidney/pathology ; Kidney Glomerulus/*pathology ; Lupus Nephritis/*classification/*diagnosis ; Nephrology/*methods/standards ; Proportional Hazards Models ; Sclerosis/pathology ; Societies, Medical ; Treatment Outcome

BACKGROUND: All types of membranoproliferative glomerulonephritis (MPGN) are progressive diseases with poor prognoses. Recently, a newly proposed classification of these diseases separated them into immune complex- and complement- mediated diseases. We investigated the frequency of C3 glomerulonephritis among previously diagnosed MPGN patients. METHODS: We conducted a retrospective study of patients diagnosed with MPGN at three tertiary care institutions between 2001 and 2010. We investigated the incidence of complement-mediated disease among patients diagnosed with MPGN. Progressive renal dysfunction was defined as a 50% reduction in the glomerular filtration rate or the need for renal replacement therapy. RESULTS: Among the 3,294 renal biopsy patients, 77 (2.3%) were diagnosed with MPGN; 31 cases were excluded, of which seven were diagnosed with systemic lupus nephritis, and the others were not followed for a minimum of 12 months after biopsy. Based on the new classification, complement-mediated MPGN was diagnosed in two patients (4.3%); only one patient developed progressive renal dysfunction. Among the immune complex-mediated MPGN patients, 17 patients developed progressive renal dysfunction. Serum albumin and creatinine levels at the time of MPGN diagnosis were risk factors of renal deterioration, after adjusting for low C3 levels and nephrotic syndrome. CONCLUSION: Complement-mediated glomerulonephritis was present in 4.3% of patients previously diagnosed with MPGN.
Biopsy ; Classification* ; Complement C3 ; Creatinine ; Diagnosis ; Glomerular Filtration Rate ; Glomerulonephritis ; Glomerulonephritis, Membranoproliferative* ; Glomerulonephritis, Membranous ; Humans ; Incidence ; Lupus Nephritis ; Nephrotic Syndrome ; Prognosis ; Renal Replacement Therapy ; Retrospective Studies ; Risk Factors ; Serum Albumin ; Tertiary Healthcare

PURPOSE: Systemic lupus erythematosus(SLE) is an autoimmune disease with multi-system involvement and renal damage is a major cause of morbidity and mortality in children. Renal involvement is more common and severe in children than in adults. Therefore, renal biopsy plays a crucial role in planning effective therapy. In this study, we investigated the clinical and pathological findings of lupus nephritis in children to aid clinical care of the disease. METHODS: The clinical and pathological data of 40 patients who were diagnosed as SLE with renal involvement in Shinchon Severance Hospital from Jan. 1990 to Sep. 2002 were analyzed retrospectively. RESULTS: The ratio of male to female patients was 1:3 and the median age at diagnosis was 12.1 (2-18) years old. FANA(95.0%), anti-ds DNA antibody (87.5%), malar rash (80.0%) were the most common findings among the classification criteria by ARA. Microscopic hematuria with proteinuria (75.0%), nephrotic syndrome (55.0%), and microscopic hematuria alone (15.0%) were the most common renal presentations in the respective order at diagnosis. There were 27 cases with WHO class IV lupus nephritis confirmed by renal biopsy and 3 cases with pathological changes of WHO class type. Different treatment modalities were carried out : prednisolone only in 5 cases, prednisol-one+azat-hioprine in 9 cases, prednisolone+azathioprine+intravenous cyclophosphamide in 14 cases, prednisolone+cyclosporine A+intravenous cyclophosphamide in 12 cases, plasma exchange in 9 cases and intravenous gamma-globulin in 2 cases. The average follow-up period was 51.8 40.5 months. During 51.8+/-40.5 months. During follow-up, 4 patients expired. The risk factors associated with mortality were male, WHO class IV and acute renal failure at diagnosis. CONCLUSION: Renal involvement was noted in 63.5% of childhood SLE, and 67.5% of renal lesion was WHO class IV lupus nephritis which is known to be associated with a poor prognosis. Therefore aggressive treatment employing immunosuppressant during the early stages of disease could be helpful in improving long-term prognosis. But careful attention should be given to optimize the treatment due to unique problems associated with growth, psychosocial development and gonadal toxicity, especially in children.
Acute Kidney Injury ; Adult ; Autoimmune Diseases ; Biopsy ; Child* ; Classification ; Cyclophosphamide ; Diagnosis ; DNA ; Exanthema ; Female ; Follow-Up Studies ; gamma-Globulins ; Gonads ; Hematuria ; Humans ; Lupus Nephritis* ; Male ; Mortality ; Nephrotic Syndrome ; Plasma Exchange ; Prednisolone ; Prognosis ; Proteinuria ; Retrospective Studies ; Risk Factors ; Treatment Outcome*

PURPOSE:Rapidly progressive glomerulonephritis (RPGN) is characterized by the rapid increase in serum creatitnin and crescents formation involving more than 50% of glomeruli. 10 patients who had been treated for RPGN were studied retrospectively for thier underlying diseases and clinical features. METHOD: Cilinical review was performed on 10 children who were diagnosed with RPGN by clinical features and renal biopsy and followed up at department of pediatrics during the last 10 years, from May 1990 to May 2000. RESULT: There were 6 males and 4 females between the ages of 2.1 and 14.3 years (mean 10.9+/-.8). 3 had Henoch-Sch nlein purpura nephritis; 2, idiopathic rapidly progressive glomerulonephritis; 2, lupus nephritis; 1, hemolytic uremic syndrome; 1, membranous glomerulonephritis and 1, microscopic polyangiitis. The most common chief complaints were gross hematuria and oliguria. Initial clinical features included proteinuria, edema, hypertension, nausea and arthralgia. Mean serum BUN was 74.2+/-39.1 mg/dL; mean serum creatinin, 3.2+/-1.8 mg/dL and mean creatinin clearance, 26.5+/-13.2 mL/min/1.73m2. Antineutrophil cytoplasmic antibody was positive only in microscopic polyangiitis. ANA and Anti-DNA antibody were positive in two lupus nephritis patients. Serum complements were decreased in 4 patients. All patients except Hemolytic uremic syndrome received steroid pulse therapy and immunosupressive agents. 3 patients were performed acute peritoneal dialysis and 2 patients were given plasmapheresis. At the last follow up, 1 patient was dead, 4 patients had elevated serum creatinin, 2 of these 4 patients were on chronic ambulatory peritoneal dialysis and 6 patients had normal renal function. CONCLUSION: Rapidly progressive glomerulonephritis is a medical emergency that requires very rapid diagnosis, classification, and therapy. Appropriate therapy selected on the basis of underlying disease mechanism can substantially improve renal survival.
Antibodies, Antineutrophil Cytoplasmic ; Arthralgia ; Biopsy ; Child ; Classification ; Complement System Proteins ; Creatinine ; Diagnosis ; Edema ; Emergencies ; Female ; Follow-Up Studies ; Glomerulonephritis* ; Glomerulonephritis, Membranous ; Hematuria ; Hemolytic-Uremic Syndrome ; Humans ; Hypertension ; Lupus Nephritis ; Male ; Microscopic Polyangiitis ; Nausea ; Nephritis ; Oliguria ; Pediatrics ; Peritoneal Dialysis ; Plasmapheresis ; Proteinuria ; Purpura ; Renal Insufficiency ; Retrospective Studies