Renal amyloidosis secondary to anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is extremely rare. Here, we reported a 77-year-old woman with ANCA-associated vasculitis. Renal biopsy with Masson trichrome staining showed pauci-immune crescentic glomerulonephritis, and electron microscopy showed amyloid deposition in the mesangial area. Immunofluorescence revealed kappa light chain and lambda light chain negative. Bone marrow biopsy revealed no clonal plasma cell. Finally, she was diagnosed as ANCA-associated vasculitis with secondary renal amyloid A amyloidosis.
Female ; Humans ; Aged ; Glomerulonephritis/diagnosis* ; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/pathology* ; Antibodies, Antineutrophil Cytoplasmic ; Kidney/pathology* ; Amyloidosis/complications*

BACKGROUND: Immunoglobulin A nephropathy (IgAN) is the most common pathological type of glomerular disease. Kidney biopsy, the gold standard for IgAN diagnosis, has not been routinely applied in hospitals worldwide due to its invasion nature. Thus, we aim to establish a non-invasive diagnostic model and determine markers to evaluate disease severity by analyzing the serological parameters and pathological stages of patients with IgAN. METHODS: A total of 272 biopsy-diagnosed IgAN inpatients and 518 non-IgA nephropathy inpatients from the Department of Nephrology of Chinese People's Liberation Army General Hospital were recruited for this study. Routine blood examination, blood coagulation testing, immunoglobulin-complement testing, and clinical biochemistry testing were conducted and pathological stages were analyzed according to Lee grading system. The serological parameters and pathological stages were analyzed. The receiver operating characteristic (ROC) analysis was performed to estimate the diagnostic value of the clinical factors. Logistic regression was used to establish the diagnostic model. RESULTS: There were 15 significantly different serological parameters between the IgAN and non-IgAN groups (all P < 0.05). The ROC analysis was performed to measure the diagnostic value for IgAN of these parameters and the results showed that the area under the ROC curve (AUC) of total protein (TP), total cholesterol (TC), fibrinogen (FIB), D-dimer (D2), immunoglobulin A (IgA), and immunoglobulin G (IgG) were more than 0.70. The AUC of the "TC + FIB + D2 + IgA + age" combination was 0.86, with a sensitivity of 85.98% and a specificity of 73.85%. Pathological grades of I, II, III, IV, and V accounted for 2.21%, 17.65%, 62.50%, 11.76%, and 5.88%, respectively, with grade III being the most prevalent. The levels of urea nitrogen (UN) (13.57 ± 5.95 vs. 6.06 ± 3.63, 5.92 ± 2.97, 5.41 ± 1.73, and 8.41 ± 3.72 mmol/L, respectively) and creatinine (Cr) (292.19 ± 162.21 vs. 80.42 ± 24.75, 103.79 ± 72.72, 96.41 ± 33.79, and 163.04 ± 47.51 μmol/L, respectively) were significantly higher in grade V than in the other grades, and the levels of TP (64.45 ± 7.56, 67.16 ± 6.94, 63.22 ± 8.56, and 61.41 ± 10.86 vs. 37.47 ± 5.6 mg/d, respectively), direct bilirubin (DB) (2.34 ± 1.23, 2.58 ± 1.40, 1.91 ± 0.97, and 1.81 ± 1.44 vs. 0.74 ± 0.57 μmol/L, respectively), and IgA (310.35 ± 103.78, 318.48 ± 107.54, 292.58 ± 81.85, and 323.29 ± 181.67 vs. 227.17 ± 68.12 g/L, respectively) were significantly increased in grades II-V compared with grade I (all P < 0.05). CONCLUSIONS The established diagnostic model that combined multiple factors (TC, FIB, D2, IgA, and age) might be used for IgAN non-invasive diagnosis. TP, DB, IgA, Cr, and UN have the potential to be used to evaluate IgAN disease severity.
Adult ; Biomarkers ; blood ; Blood Urea Nitrogen ; Cholesterol ; blood ; Creatinine ; blood ; Female ; Fibrinogen ; metabolism ; Glomerulonephritis, IGA ; blood ; diagnosis ; pathology ; Humans ; Immunoglobulin A ; blood ; Logistic Models ; Male ; Middle Aged ; Multivariate Analysis ; ROC Curve

PURPOSE: Smoking reportedly exerts deleterious effects on renal function; however, its effects on histology have not been clarified in patients with IgA nephropathy (IgAN). MATERIALS AND METHODS: Renal histology was evaluated in a cohort of 397 patients diagnosed with IgAN according to smoking status and dose in relation to renal function. RESULTS: Among the study cohort, which was predominantly male (88.5%), 52 patients (13%) were current smokers. These current smokers demonstrated more frequent hypertension and higher serum creatinine levels than non/ex-smokers at the time of diagnosis, which was apparent with increased smoking dose. The percentages of global glomerulosclerosis and arteriolar hyalinosis increased with increased smoking dose, whereas tubulointerstitial fibrosis or arterial intimal thickening did not. Glomerular mesangial alpha-smooth muscle actin expression were similar between current and non/ex-smokers matched for age, gender, hypertension, and histologic severity, although the number of glomerular CD68+ cells was significantly fewer in smokers. Initial serum creatinine level, estimated glomerular filtration rate (eGFR), and global glomerulosclerosis were found to be risk factors of serum creatinine doubling in both smokers and non/ex-smokers by univariate analysis during a mean follow-up of 3.8 years. CONCLUSION: In addition to dose dependent renal functional decline and hypertension, smoking contributes to renal disease progression by eliciting microvascular injury in IgAN patients.
Adult ; Aged ; Case-Control Studies ; Cohort Studies ; Creatinine/blood ; Disease Progression ; Female ; Glomerulonephritis, IGA/blood/*diagnosis/epidemiology ; Humans ; Immunohistochemistry ; Kidney/*pathology ; Kidney Function Tests ; Kidney Glomerulus/*pathology ; Male ; Middle Aged ; Risk Factors ; Smoking/*adverse effects/epidemiology

Diabetic nephropathy is a chronic microvascular complication of type 2 diabetes and the leading cause of end-stage renal disease. We report the case of a 34-year-old male, newly diagnosed with type 2 diabetes mellitus, who had advanced-stage nephropathy with glomerular crescents. A moderately-to-severely decreased glomerular filtration rate with nephrotic syndrome was seen at the time of diagnosis of diabetes. Proliferative diabetic retinopathy was detected, but there was no positive finding in serology tests for glomerulonephritis. Non-necrotizing cellular crescents and nodular glomerulosclerosis were observed in a kidney biopsy, and renal function declined rapidly to the end stage. We review data on diabetic glomerulosclerosis with cellular crescents and the rapid progression of nephropathy.
Adult ; Biopsy ; Diabetes Mellitus, Type 2 ; Diabetic Nephropathies* ; Diabetic Retinopathy ; Diagnosis ; Disease Progression ; Glomerular Filtration Rate ; Glomerulonephritis ; Humans ; Kidney ; Kidney Failure, Chronic* ; Male ; Nephrotic Syndrome ; Pathology

The increasing interest in healthcare and health screening events is revealing additional cases of asymptomatic isolated microscopic hematuria (IMH). However, a consensus of the evaluation and explanation of the IMH prognosis is controversial among physicians. Here, we present the natural course of IMH together with the pathological diagnosis and features to provide supportive data when approaching patients with IMH. We retrospectively evaluated 350 patients with IMH who underwent a renal biopsy between 2002 and 2011, and the pathological diagnosis and chronic histopathological features (glomerulosclerosis, interstitial fibrosis, and tubular atrophy) were reviewed. Deterioration of renal function was examined during follow up. The patients with IMH were evaluated for a mean of 86 months. IgA nephropathy was the most common diagnosis in 164 patients (46.9%). Chronic histopathological changes were observed in 166 (47.4%) but was not correlated with proteinuria or a decline in renal function. Ten patients developed proteinuria, and all of them had IgA nephropathy. Three patients progressed to chronic kidney disease with an estimated glomerular filtration rate < 60 mL/min/1.73 m2 but none progressed to end stage renal disease. In conclusion, IMH had a generally benign course during 7-years of observation, although IgA nephropathy should be monitored if it progresses to proteinuria. Future prospective randomized studies may help conclude the long-term prognosis and lead to a consensus for managing IMH.
Adolescent ; Adult ; Biopsy ; Female ; Glomerular Filtration Rate ; Glomerulonephritis, IGA/diagnosis ; Hematuria/*diagnosis/pathology ; Humans ; Kidney/*pathology/physiology ; Kidney Failure, Chronic/diagnosis ; Male ; Middle Aged ; Prognosis ; Proteinuria/diagnosis ; Retrospective Studies ; Young Adult

OBJECTIVETo investigate the value of serum IgA/C3 ratio in the diagnosis of IgA nephropathy and explore its relationship with the clinicopathological features of the patients.

METHODSSixty-six patients with IgA nephropathy, 111 with other glomerular diseases, and 40 healthy control subjects without kidney disease were tested for serum IgA and C3 levels using CRM470 adjusted standardized immune turbidimetric method, and the IgA/C3 ratio was calculated. According to Oxford and Lee's classification criteria, we analyzed the pathological grades of the renal biopsy samples from patients with IgA nephropathy. The ROC curve was used to assess the value of serum IgA and IgA/C3 ratio in predicting IgA nephropathy.

RESULTSPatients with IgA nephropathy had an elevated serum IgA/C3 ratio than those with other glomerular diseases and the control subjects, with an area under the ROC curve of 0.776. An elevated serum IgA/C3 ratio was not found to significantly correlate with the pathological grade of renal biopsy samples in patients with IgA nephropathy.

CONCLUSIONIn the absence of renal biopsy findings, serum IgA/C3 ratio can help in the diagnosis of IgA nephropathy.

Biopsy ; Case-Control Studies ; Complement C3 ; analysis ; Glomerulonephritis, IGA ; blood ; diagnosis ; Humans ; Immunoglobulin A ; blood ; Kidney ; pathology

BACKGROUND/AIMS: Immunoglobulin A nephropathy (IgAN) is the most common type of primary glomerulonephritis worldwide. Although several studies have identified IgAN prognostic factors in Korea, the follow-up period was insufficient to evaluate the natural history of IgAN. METHODS: A total of 471 patients were diagnosed with IgAN after percutaneous renal biopsy between April 1985 and March 2003. Patients with secondary IgAN and patients with a follow-up < 10 years since their diagnosis were excluded. Thus, 184 patients were enrolled. RESULTS: Among the 184 patients, 97 were males (52.7%) and 87 were females (47.3%). The mean age was 33.7 +/- 11.5 years, and the mean follow-up period was 181.3 +/- 46.3 months. During the follow up, 73 patients (36.9%) had progressed to end-stage renal disease (ESRD). The mean duration to ESRD was 98.1 +/- 55.9 months. The overall renal survival rate was 60.3%, the 10-years renal survival rate was 74.3%, and the 20-years renal survival rate was 49.3%. Univariate analyses indicated that hypertension, serum creatinine > 1.3 mg/dL, estimated glomerular filtration rate < 60 mL/min/1.73 m2, serum albumin < 3.5 g/dL, proteinuria > or = 1 g/day, and severe renal pathology by the Haas sub-classification were significantly associated with ESRD. When these factors were included in multivariate Cox regression analyses, only severe renal pathology by the Haas sub-classification was an independent prognostic factor for IgAN. CONCLUSIONS: Careful follow-up and treatment is recommended, particularly in patients with IgAN and severe renal pathology by the Haas sub-classification.
Biopsy ; Creatinine ; Diagnosis ; Female ; Follow-Up Studies* ; Glomerular Filtration Rate ; Glomerulonephritis ; Glomerulonephritis, IGA* ; Humans ; Hypertension ; Kidney Failure, Chronic ; Korea ; Male ; Natural History ; Pathology ; Prognosis ; Proteinuria ; Serum Albumin ; Survival Rate

Poststreptococcal glomerulonephritis (PSGN) is one of the most well-known and important infectious renal diseases resulting from a prior infection with group A beta-hemolytic streptococcus. The typical clinical characteristics of the disease reflect acute onset with gross hematuria, edema, hypertension and moderate proteinuria after the antecedent streptococcal infection. In children, usually PSGN is healed spontaneously but if it combines with fast progressing glomerulonephritis, it would be developed to chronic renal failure. Therefore, it is important to make a fast diagnosis and treatment by simple tools to predict the course and the prognosis of disease. Sonography is a simple tool for diagnosis but there is no typical renal sonographic finding in PSGN, so it is difficult to predict the course and the prognosis of disease by sonographic findings. In comparison between two cases of renal sonographic findings in PSGN, a patient who showed more increased echogenicity in more extended area of renal sonography had the severe results of renal pathology, prolonged treatment period and low serum C3 level. Here, we report the different findings of renal sonography and pathology depending on the degree of severity between two patients. Thus, it is necessary to gather more information from further studies to make a consensus about the relationship between the renal sonography and the prognosis of disease in PSGN.
Child ; Consensus ; Diagnosis ; Edema ; Glomerulonephritis* ; Hematuria ; Humans ; Hypertension ; Kidney Failure, Chronic ; Pathology ; Prognosis ; Proteinuria ; Streptococcal Infections ; Streptococcus ; Ultrasonography

BACKGROUND/AIMS: The purpose of this study was to investigate the expression of urokinase-type plasminogen activator (uPA), uPA receptor (uPAR), and plasminogen activator inhibitor (PAI)-1 on podocytes in immunoglobulin A (IgA) glomerulonephritis (GN). METHODS: Renal biopsy specimens from 52 IgA GN patients were deparaffinized and subjected to immunohistochemical staining for uPA, PAI-1, and uPAR. The biopsies were classified into three groups according to the expression of uPA and uPAR on podocytes: uPA, uPAR, and a negative group. The prevalences of the variables of the Oxford classification for IgA GN were compared among the groups. RESULTS: On podocytes, uPA was positive in 11 cases and uPAR was positive in 38 cases; by contrast, PAI-1 was negative in all cases. Expression of both uPA and uPAR on podocytes was less frequently accompanied by tubulointerstitial fibrosis. CONCLUSIONS: Our results suggest a possible protective effect of podocyte uPA/uPAR expression against interstitial fibrosis.
Adolescent ; Adult ; Aged ; Atrophy ; Biological Markers/analysis ; Biopsy ; Female ; Fibrosis ; Glomerulonephritis, IGA/diagnosis/*enzymology/immunology ; Humans ; Immunohistochemistry ; Male ; Middle Aged ; Plasminogen Activator Inhibitor 1/*analysis ; Podocytes/*enzymology/immunology/pathology ; Receptors, Urokinase Plasminogen Activator/*analysis ; Urokinase-Type Plasminogen Activator/*analysis ; Young Adult

BACKGROUNDIn China, the prevalence of chronic kidney disease has increased significantly. Many studies shows that the spectrum of kidney disease had changed in recent years. We retrospectively analyzed the pathological types of renal biopsy and its spectrum change at the General Hospital of the Chinese People's Liberation Army from December 1987 to December 2012, in order to offer new supporting evidences for further specifying the distribution of renal pathological types in China.

METHODSAccording to the "Revised Protocol for the Histological Typing of Glomerulopathy" (WHO, 1995), pathological diagnosis of renal biopsy was classified, detection rate of each pathological type was summarized (i.e., percentage of total renal biopsy cases), study period was divided at an interval of 5 years, and age-stratified distribution change of main pathological types was analyzed.

RESULTSThe proportion of pathological types in 11 618 cases of renal biopsy was as follows: primary glomerulonephritis (PGN, 70.7%), secondary glomerulonephritis (SGN, 20.7%), tubular-interstitial nephropathy (4.0%), hereditary/rare nephropathy (0.3%), end-stage renal disease (0.9%), and unclassified renal disease (3.3%). Among PGN, there was IgA nephropathy (IgAN, 37.0%), membranous nephropathy (MN, 11.8%), mesangial proliferative glomerulonephritis (MsPGN, 8.9%), minimal change disease (MCD, 6.6%), and focal segmental glomerulosclerosis (3.9%). Among SGN there was lupus nephritis (LN, 5.5%), Henoch-Schönlein purpura glomerulonephritis (5.3%), hepatitis B virus-associated nephritis (HBVAN, 3.03%), diabetic nephropathy (2.2%), and hypertension/malignant hypertension-associated renal damage (1.9%). Pathological data were analyzed from 1987-1992 to 2008-2012 (after age adjustment). Detection rate of IgAN tended to rise (P < 0.001). Detection rates of MN and MCD rose significantly (P < 0.001), but detection rate of MsPGN dropped significantly (P < 0.001). Among SGN, detection rate of HBVAN tended to drop (P < 0.001).

CONCLUSIONIn China, PGN was the most common glomerulopathy (mostly IgAN), LN was the most common SGN, and detection rate of MN and MCD rose significantly.

Adolescent ; Adult ; Aged ; Aged, 80 and over ; Biopsy ; methods ; Child ; Child, Preschool ; China ; Female ; Glomerulonephritis, Membranous ; diagnosis ; Humans ; Kidney ; pathology ; Kidney Diseases ; diagnosis ; Male ; Middle Aged ; Young Adult