Crohn's disease is a condition of chronic inflammation potentially involving any location of the alimentary tract from mouth to anus. Numerous extraintestinal manifestations can also be present. Urologic complications of inflammatory bowel disease are seen in up to 25% of patients, but renal parenchymal disease has been rarely reported. IgA nephropathy is recognized worldwide as a most common form of primary glomerulonephritis. Clinical manifestations vary, ranging from microscopic hematuria to nephrotic syndrome. Recently, IgA nephropathy associated with systemic diseases has been reported. We describe a case of a 22 year-old man with Crohn's disease associated with IgA nephropathy. At the age of 8 years, microscopic hematuria appeared. After fourteen years, he presented with melena, mild fever, recurrent oral ulcer, microscopic hematuria and proteinuria. Colonoscopic examination revealed characteristic features of Crohn's disease such as multiple ulcers. Microscopic findings showed superficial ulceration with small noncaseating granulomas. Renal biopsy revealed IgA nephropathy. The patient was treated with oral prednisolone, olsalazine, and metronidazole followed by maintenance therapy with sulfasalazine and azathioprine resulting in clinical improvement of Crohn's disease and IgA nephropathy.
Adult ; Crohn Disease/*complications/pathology ; Glomerulonephritis, IGA/*complications/pathology ; Humans ; Male

OBJECTIVESeveral studies have suggested that non-diabetic renal disease (NDRD) was common among non-insulin dependent diabetes mellitus (NIDDM) patients with renal involvement.

METHODSThe prevalence of NDRD among Chinese NIDDM population in PUMC hospital center was retrospectively studied. Renal biopsy specimens were evaluated with light-, immunofluorescence- and electron-microscopy. The cohort consisted of 33 NIDDM patients who received renal biopsy.

RESULTSPatients with both isolated diabetic nephropathy (DN, n = 7) and NDRD (n = 22) had comparable duration of DM, creatinine clearance, serum creatinine, albumin and 24 hours proteinuria, as well as incidences of retinopathy, neuropathy and hypertension. Significantly more patients with NDRD had haematuria (P = 0.030) or non-nephrotic proteinuria (P = 0.016). IgA nephropathy accounted for 40.9% of the NDRD identified.

CONCLUSIONSIn this study, haematuria and non-nephrotic proteinuria predicted the presence of NDRD among NIDDM patients complicated with renal disease. IgA nephropathy is the most frequent type of NDRD in Chinese.

Adult ; Aged ; Biopsy, Needle ; Cohort Studies ; Diabetes Mellitus, Type 2 ; complications ; pathology ; Female ; Glomerulonephritis ; complications ; pathology ; Glomerulonephritis, IGA ; complications ; pathology ; Humans ; Kidney ; pathology ; Male ; Middle Aged ; Retrospective Studies

A retrospective study of 223 patients with IgA nephropathy (IgAN) was performed to clarify the prognostic factors and the renal survival rates of the disease. One hundred twenty-two patients were followed-up for more than 6 months after their renal biopsy (mean follow-up duration: 43.0 months), and 20 of them (16.4%) had progressed to end-stage renal disease (ESRD). Using univariate analysis, 8 risk factors (2 clinical and 6 histopathological findings) for developing ESRD were identified: renal insufficiency at initial presentation (serum creatinine > or = 1.5 mg/dl); heavy proteinuria(> or = 3.5 gm/day); moderate to severe histopathologic findings such as class IV/V lesions by W.H.O. classification, mesangial hypercellularity, glomerular sclerosis, interstitial infiltration, interstitial fibrosis, and tubular atrophy. In multivariate regression analysis, class IV/V lesions and renal insufficiency at initial presentation were the independent prognostic factors of IgAN. The renal survival rates were 100% at 1 year, 97.0% at 3 years, and 78.9% at 5 years. In conclusion, it seems that about 20% of IgAN patients have a risk to progress to ESRD within 5 years, and a careful follow-up is recommended especially in patients who have either renal insufficiency at the time of presentation or severe renal pathology (class IV/V lesions).
Adolescent ; Adult ; Female ; Glomerulonephritis, IGA/*complications/pathology ; Human ; Kidney Failure, Chronic/*epidemiology/*etiology/pathology ; Male ; Prognosis ; Retrospective Studies ; Risk Factors

The occurrence of immunoglobulin A nephropathy (IgAN) in patients with noninsulin dependent diabetes mellitus (NIDDM) is a rare event and of pathogenetic interest. It is not clear whether this is merely coincidence. We report here five patients with IgAN in NIDDM associated with or without diabetic glomerulosclerosis. All of the patients were Korean males. In three patients, diabetes mellitus was diagnosed at the same time with diagnosis of IgAN, and the known duration of the diabetes in the other two patients were three and seven years, respectively. There was no evidence of diabetic retinopathy in four patients, but it was found in one patient. In all cases, the diagnosis of IgAN was made by immunohistology.
Adult ; Biopsy ; Case Report ; Complement 3/analysis ; Diabetes Mellitus, Non-Insulin-Dependent/complications* ; Diabetic Nephropathies/pathology* ; Glomerular Mesangium/pathology ; Glomerulonephritis, IGA/pathology* ; Glomerulonephritis, IGA/etiology ; Human ; IgG/analysis ; Kidney Glomerulus/pathology ; Male ; Microscopy, Fluorescence ; Middle Age

OBJECTIVETo explore the changes in the clinicopathological features of patients with IgA nephropathy with elevated uric acid level.

METHODSA total of 171 patients with IgA nephropathy diagnosed at biopsy were classified into 3 groups, namely normotensive group with normal level uric acid (group 1), normotensive group with elevated uric acid level (group 2), and hypertensive group with elevated uric acid level (group 3). The clinicopathological features were compared between the 3 groups.

RESULTSFrom group 1 to group 3, the disease duration became elongated, body weight increased, systolic and diastolic pressures elevated, blood urea nitrogen and serum creatinine increased, glomerular filtration rate decreased, and 24-h urine protein increased; the apolipoprotein A, high-density lipoprotein and albumin levels decreased, while apolipoprotein B100, triglyceride, cholesterol and low-density lipoprotein increased. The glomerular damage, tubulointerstitial lesions and arteriole hypertrophy worsened, and Lee's grade III changes were predominant in group 1, grade III or IV in group 2 and grades III-V in group 3. Mesangial proliferative glomerulonephritis was the major pathological type in groups 1 and 2, as compared with focal segmental glomerulonephritis or sclerosing glomerulonephritis in group 3.

CONCLUSIONPatients with IgA nephropathy and elevated uric acid level have greater clinicopathological damage than those with normal uric acid level, and hypertension further aggravates such damages.

Adolescent ; Adult ; Creatinine ; blood ; Female ; Glomerulonephritis, IGA ; complications ; pathology ; Humans ; Hypertension ; complications ; pathology ; Hyperuricemia ; etiology ; pathology ; Kidney ; pathology ; Male ; Middle Aged ; Young Adult

OBJECTIVETo study the clinicopathologic features of membranous nephropathy coexisting with IgA nephropathy.

METHODSThe renal biopsies performed in Peking University First Hospital during the period from January, 1998 to April, 2006 were retrospectively reviewed. The clinicopathologic features of 11 cases of membranous nephropathy coexisting with IgA nephropathy were studied. Electron microscopy with immunogold labeling for IgG and IgA were also performed.

RESULTSThe mean age of patients was 39.9 years. The male-to-female ratio was 1:2.9. The patients mainly presented with proteinuria. Proteinuria of nephrotic level was seen in 7 cases (63.6%). Seven cases also had associated microscopic hematuria. None of them showed evidence of renal insufficiency. Cases with secondary diseases, such as hepatitis virus infection and systemic lupus erythematosus, were excluded from the study. Histologically, vacuolation and thickening of glomerular basement membrane was seen. There was also mild mesangial hypercellularity and increase in mesangial matrix. Occasional glomeruli with crescent formation were identified in 2 cases. Immunofluorescence study showed granular staining for IgG and C3 along glomerular capillary walls, in addition to clumps of IgA deposits in mesangium. Electron microscopy revealed subepithelial and mesangial electron-dense deposits. Immunogold labeling showed IgG and IgA localized in the subepithelial and mesangial deposits respectively.

CONCLUSIONMembranous nephropathy coexisting with IgA nephropathy possesses the clinicopathologic features of both components. It might be caused by independent occurrence of the two entities.

Adult ; Female ; Glomerular Basement Membrane ; immunology ; pathology ; ultrastructure ; Glomerular Mesangium ; immunology ; pathology ; ultrastructure ; Glomerulonephritis, IGA ; complications ; immunology ; pathology ; Glomerulonephritis, Membranous ; complications ; immunology ; pathology ; Humans ; Immunoglobulin A ; metabolism ; Immunoglobulin G ; metabolism ; Kidney Glomerulus ; immunology ; pathology ; ultrastructure ; Male ; Middle Aged ; Retrospective Studies

OBJECTIVETo understand the clinical and pathological characteristics of IgA nephropathy (IgAN) with crescentic formation in children.

METHODSClinicopathological data of 29 children with IgAN accompanied by crescents were analyzed. These patients were divided into two groups according to the percentage of glomeruli affected by crescents more or less than 50%, and their data were compared.

RESULTS(1) CLINICAL FEATURES: all the patients had hematuria and proteinuria, and macrohematuria (86%) and proteinuria were also common, protein excreted in urine was more than 1 g per day in 76% of the patients. The patients with edema, hypertension, and renal insufficiency were less than fifty percent. Nine patients in Group A (glomeruli affected by crescents > or = 50%) were crescentic IgAN. Significantly more cases in Group A had persistent macrohematuria, hypertension and renal failure than in Group B (glomeruli affected by crescents < 50%) (P < 0.05), with especially severe proteinuria (P < 0.01). It was easy to find nephritic syndrome in Group A, and asymptomatic hematuria combined with proteinuria in Group B. (2) Renal pathology: the glomeruli were affected by crescents from 5% to 85%. There were 52% to 85% in Group A, and 5% to 40% in Group B. Most crescents were cellular. All the cases had a diffuse mesangial proliferation and tubular-interstitial injury to different degree. Three cases had crescentic IgAN. Glomerulosclerosis was significantly more often seen in Group A (P < 0.05) and tuft adhesion was more frequently seen in Group B (P < 0.05). (3) Immunofluorescence: All the patients presented deposition of IgA, IgM and C3. There were 45% specimens combined with the deposition of IgG. Five cases showed 'full house' (17%), four of them were in Group A. None had IgA deposition alone.

CONCLUSIONThe main clinical feature of IgAN with crescentic formation were hematuria combined with proteinuria, especially persistent gross hematuria and severe proteinuria. All of them showed diffuse mesangial proliferation and tubular-interstitial injury in morphology of kidney. Most of them had tuft adhesion. The main type of immunofluorescence were IgA + IgM and IgA + IgM + IgG deposition. Some showed 'full house' phenomenon. The clinical manifestation and renal lesions of IgAN with diffuse crescentic formation were worse than IgAN with glomeruli affected by crescents < 50%.

Adolescent ; Biopsy ; Child ; Child, Preschool ; Female ; Glomerulonephritis, IGA ; complications ; pathology ; Hematuria ; etiology ; Humans ; Hypertension ; etiology ; Kidney ; pathology ; Kidney Function Tests ; Male ; Prognosis ; Proteinuria ; etiology ; Renal Insufficiency ; etiology

OBJECTIVE: To evaluate the clinical and pathological features of 94 children suffering from IgA nephropathy (IgAN) while estimating the prevalent situation in Hunan province. METHODS: To summarize the annual number of hospitalized children, those with kidney diseases, those accepted biopsy, and those confirmed as IgAN in both Xiangya Hospital and Second Xiangya Hospital undertaking kidney biopsy in Hunan province during 1995 and 2004. RESULTS: In the past 10 years, as the hospitalized population in both hospitals accrued to 9.98% each year. The rate of 7.5% was seen in those with kidney diseases. Among whom 56.3% accepted kidney biopsy and 94 of them were confirmed as IgAN. Hematuria was the main clinical presentation, seen in 71 cases, accounting to 76%, and even to 98% after excluding those with nephrotic syndrome and isolating proteinuria type of IgAN. Inflammation infiltration (91%), renal tubule degeneration (81%), and renal interstitial fibrosis (31%) were the major pathological features of 94 children, especially in nephrotic syndrome IgAN. CONCLUSION The number of children with IgAN synchronously accrues as hospitalized population, those with kidney diseases, and those by kidney biopsy. Hematuria is the major symptom. To routinely perform urine analysis and kidney biopsy in asymptomatic hematuria may improve the diagnosis. Inflammation infiltration, renal tubule degeneration, and renal interstitial fibrosis are the major pathological features in IgAN children, especially in nephrotic syndrome IgAN, probably relating to continuous proteinuria. Early control of proteinuria may delay or decrease renal tubule fibrosis.
Adolescent ; Biopsy, Needle ; Child ; Child, Preschool ; China ; epidemiology ; Female ; Glomerulonephritis, IGA ; complications ; epidemiology ; pathology ; Hematuria ; diagnosis ; etiology ; Hospitalization ; statistics & numerical data ; Humans ; Kidney ; pathology ; Male

OBJECTIVETo analyze the characteristics of proteinuria and glomerular lesions in Henoch-Schönlein purpura nephritis (HSPN) of adults.

METHODSThe pathologic results and clinical data of 103 HSPN patients were analyzed.

RESULTSPatients with mild or moderate proteinuria accounted for 40.8% and 37.9%, respectively. Patients with nephropathic proteinuria accounted for about 21.4%. Severer proteinuria resulted in higher blood pressure and increased cholesterol, and albumin decreased gradually (P < 0.01); meanwhile, total glomerular lesion, tubulointerstitial lesion, and vascular lesion also became severer (P < 0.01).

CONCLUSIONProteinuria may indirectly reflect the severity of glomerular lesions in HSPN of adults.

Adolescent ; Adult ; Aged ; Female ; Glomerulonephritis, IGA ; diagnosis ; etiology ; Humans ; Immunoglobulin A ; blood ; Immunoglobulin G ; blood ; Immunoglobulin M ; blood ; Kidney Glomerulus ; pathology ; Kidney Tubules ; pathology ; Male ; Middle Aged ; Nephritis ; diagnosis ; etiology ; Proteinuria ; etiology ; urine ; Purpura, Schoenlein-Henoch ; complications ; pathology ; Retrospective Studies

OBJECTIVETo study the long-term outcome of idiopathic IgA nephropathy and analyze its clinical and histological prognostic factors.

METHODSWe conducted a retrospective analysis of 495 cases of IgA nephropathy patients in our hospital from 1986 to 2002. Renal survival was analyzed by Kaplan-Meier method, univariate analysis and multivariate Cox regression analysis.

RESULTSThe cumulative renal survival rate was 85% at 10 years and 70.9% at 15 years. Univariate analysis found the following parameters at the time of biopsy to be significantly correlated with poor renal survival: serum creatinine > or = 133 micromol/L (P < 0.001), hypertension (P < 0.05), 24-hour urine protein excretion > or = 3 g (P < 0.001), histological subclass V (P < 0.001), and without the history of gross hematuria (P < 0.05). The Cox regression analysis showed that only the presence of hypertension (HR=7.75, 95%CI 1.02 to approximately 2.19) and serum creatinine level (HR=1.49, 95%CI 1.54 to approximately 38.90) were independent prognostic indicators.

CONCLUSIONSThe findings in Chinese IgA nephropathy patients are in agreement with the results of many other studies worldwide.

Adolescent ; Adult ; Child ; Creatinine ; blood ; Female ; Glomerulonephritis, IGA ; complications ; pathology ; physiopathology ; Humans ; Kidney ; pathology ; physiopathology ; Male ; Middle Aged ; Prognosis ; Regression Analysis ; Renal Insufficiency ; etiology ; pathology ; physiopathology ; Retrospective Studies ; Treatment Outcome