Types Of Renal Involvement Research Articles

#1671 Prevalence and factors predicting non diabetic kidney disease in type 2 diabetic patients

Abstract Background and Aims Renal involvement in type 2 diabetes is mostly presumed to be due to diabetic nephropathy, however a significant majority of diabetic patients can have pure non diabetic kidney disease (NDKD) or NDKD superimposed on diabetic kidney disease (DKD). Kidney biopsy cannot be routinely performed for all diabetic patients and hence short of conclusive biomarkers we need to explore various factors that can predict the occurrence of NDKD. Method We conducted a retrospective review of all native kidney biopsies conducted in patients with type 2 diabetes at our institute to identify the prevalence and factors that predict NDKD. The demographic data, clinical data, laboratory parameters, and histological results of the patients were obtained from their medical records. Binary logistic regression analysis was performed to evaluate the predictive factors for NDKD. Patients were categorized into 3 groups-DKD alone (presence of Kimmelstiel-Wilson nodules, capsular drops, fibrin caps and absence of any other NDKD features), NDKD along (presence of vasculopathy, interstitial fibrosis and specific glomerular changes in absence of classical DKD changes) and lastly DKD+NDKD (presence of histological changes of both DKD+NDKD). Results We analyzed a total of 69 patients. The mean (SD) age of the cohort was 51.94 ± 12.7 years and males constituted the majority (68%). Around 2/3rd of the entire cohort had hypertension, with no significant differences among the groups. Mean 24-hour urinary proteinuria was 6.6 ± 4.9 g/TV and 15.9% patients had microscopic hematuria. The mean (SD) duration of diabetes was 6.9 ± 6.2 years. Patients with pure DKD, pure NDKD and NDKD superimposed on DKD constituted 46.3%, 33.3% and 20% respectively of the cohort. The most common indication for renal biopsy in our cohort was rapid GFR decline in 38 patients (54.2%), followed by massive proteinuria in 27 (38.5%) and lastly positive serological work up for multiple myeloma in 5 patients (7.1%). Around 2/3rd of pure DKD patients in our cohort had diabetic retinopathy (DR), whereas only around 1/5th (26%) of pure NDKD patients had DR (p=0.005). DR was absent in around 74% of our diabetic patients who had pure NDKD. Non diabetic kidney disease in our cohort was seen in 46.3% patients and combined with DKD in 20% of patients. Among patients with NDKD, the most common cause was membranous glomerulonephritis (MGN) (43.4%) followed by acute tubulointerstitial nephritis (ATIN) (17.3%). Among combined DKD and NDKD, most common histological diagnosis was MGN (32.4%) followed by ATIN (16.2%). Independent factors predicting NDKD were shorter duration of diabetes (OR 0.74, CI 0.59–0.94, P=0.01) and absence of diabetic retinopathy (OR 0.15, 95% CI 0.09–0.26, P = 0.01). Conclusion Judicious use of kidney biopsy revealed NDKD in nearly half of T2DM patients especially in those with short duration of diabetes and absence of diabetic retinopathy (DR). Kidney biopsy is strongly recommended for T2DM patients with atypical presentation and in absence of DR.

Prevalence and Factors Predicting Nondiabetic Kidney Disease in Type 2 Diabetic Patients

ABSTRACT Background: Renal involvement in type 2 diabetes is mostly presumed to be due to diabetic nephropathy; however, a significant majority of diabetic patients can have pure nondiabetic kidney disease (NDKD) or NDKD superimposed on diabetic kidney disease (DKD). Kidney biopsy cannot be routinely performed for all diabetic patients, and hence, short of conclusive biomarkers, we need to explore various factors that can predict the occurrence of NDKD. Methods: We conducted a retrospective review of all native kidney biopsies conducted in patients with type 2 diabetes at our institute to identify the prevalence and factors that predict NDKD. The demographic data, clinical data, laboratory parameters, and histological results of the patients were obtained from their medical records. Binary logistic regression analysis was performed to evaluate the predictive factors for NDKD. Results: We analyzed a total of 69 patients. The mean (standard deviation) age of the cohort was 51.94 ± 12.7 years and males constituted the majority (68%). Patients with pure DKD, pure NDKD, and NDKD superimposed on DKD constituted 46.3%, 33.3%, and 20%, respectively, of the cohort. Around two-third of pure DKD patients in our cohort had diabetic retinopathy (DR), whereas only around one-fifth (26%) of pure NDKD patients had DR (P = 0.005). Membranous glomerulonephritis (MGN) was the most common histological lesion in the NDKD group (43%), followed by acute tubulointerstitial nephritis (ATIN) (17.3%). Among combined DKD and NDKD, the most common histological diagnosis was pyelonephritis (28.6%), followed by MGN and ATIN (14.3%). Independent factors predicting NDKD were shorter duration of diabetes (odds ratio [OR] = 0.74, confidence interval [CI] =0.59–0.94, P = 0.01) and absence of DR (OR = 0.15, 95% CI = 0.09–0.26, P = 0.01). Conclusion: Kidney biopsy revealed NDKD in nearly half of type 2 diabetes mellitus (T2DM) patients, especially in those with short duration of diabetes and absence of DR. Kidney biopsy is strongly recommended for T2DM patients with atypical presentation and in the absence of DR.

Glomerulonephritis as a renal manifestation in a patient with systemic sclerosis overlapped with anti-neutrophil cytoplasmic antibody-associated vasculitis

Introduction and Importance: Systemic sclerosis (SSc) is a systemic immune disorder that may overlap with other rheumatologic disease; however, overlapping with antineutrophil cytoplasmic antibody-associated vasculitis is rare. Case Presentation: A 28-year-old Syrian male patient with SSc diagnosed according to the American College of Rheumatology/European League against Rheumatism 2013 criteria with a disease duration of 4 years, was admitted to the hospital complaining of palpable purpura in the lower limbs and hemoptysis and later, a rise in creatinine level. Laboratory tests showed high levels of perinuclear antineutrophil cytoplasmic antibodies (p-ANCA). The renal biopsy results were consistent with the diagnosis of glomerulonephritis. He was treated with methylprednisolone, cyclophosphamide, and rituximab, as he was diagnosed with SSc overlapping antineutrophil cytoplasmic antibody-associated vasculitis. Clinical Discussion: SSc most commonly renal manifestations are proliferative vasculopathy leading to scleroderma renal crisis. However, other types of renal involvement were also reported in SSc patients with comorbid autoimmune diseases such as glomerulonephritis and signs of concurrent vasculitis. SSc may overlap with rheumatoid arthritis, systemic lupus erythromatosus, polymyositis/dermatomyositis (PM/DM), and Sjogren Syndrome. Overlapping with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is mentioned in rare cases. The authors reported a rare case of overlapping SSc with antineutrophil cytoplasmic antibody-associated vasculitis with renal involvement. Conclusion: The authors revealed a rare case of overlapping SSc with antineutrophil cytoplasmic antibody-associated vasculitis with renal involvement. In SSc, renal involvement as glomerulonephritis is infrequent and should be detect in other rheumatologic disease such as systemic lupus erythematosus or antineutrophil cytoplasmic antibody-associated vasculitis.

Discrepancies between clinical and pathological findings seen at renal biopsy in rheumatological diseases.

Renal biopsy contributes to the diagnosis, follow-up, and treatment of many rheumatic conditions. This study assessed the diagnostic role and safety of renal biopsies in a tertiary rheumatology clinic. Renal biopsies performed between June 2020 and December 2022 were screened, and demographic, clinical, histopathological, and safety data were collected from patient records. In this study, 33 males and 38 females were included. Except for 1 patient who received acetylsalicylic acid, antiaggregant, and/or anticoagulant drugs were stopped before the biopsy. Complications included a decrease of hemoglobin in 8 patients (11.3%) and microscopic hematuria in 40 patients (56.3%). Control ultrasonography was performed in 16 patients (22.5%), and a self-limiting hematoma was found in 4 of them (5.6%) without additional complications. While less than 10 glomeruli were obtained in 9 patients (9.9%), diagnosis success was 94.4%. Histopathological data were consistent with one of the pre-biopsy diagnoses in 54 of 67 cases (80.6%) but showed discrepancies in 19.4% (n=13) of patients. A repeat biopsy was performed in 7 patients for re-staging or insufficient biopsy. Renal biopsy significantly contributes to rheumatology practice, especially in patients with complex clinical and laboratory findings or in whom different treatments can be given according to the presence, severity, and type of renal involvement. Although the possibility of obtaining insufficient tissue and the need for re-staging and repeat biopsy in the follow-up might be expected, complication risk does not seem to be a big concern. Renal biopsy often evidenced discrepancies between pre-biopsy diagnosis and histopathological findings.

Granulomatosis with polyangiitis presenting as a solitary renal mass: a case report

BackgroundGranulomatosis with polyangiitis (GPA) is characterized by necrotizing granulomatous vasculitis involving small-sized vessels in the upper airways, lower airways, and kidneys. Renal pathology is usually characterized by focal segmental necrotizing glomerulonephritis, which often leads to rapidly progressive renal failure. This type of renal involvement is usually unapparent on radiography. The presence of a renal mass in a patient with GPA, although extremely rare, is recognizable. Herein, we report a rare case of GPA presenting as a solitary renal mass and present a review of the literature.Case presentationA 75-year-old woman presented with a solitary right kidney mass measuring 4 × 3.5 cm detected by enhanced computed tomography. There was no history of sinusitis, rhinitis, cough, or pneumonia suggestive of systemic GPA. Nephrectomy was performed based on the suspicion of renal cell carcinoma or malignant lymphoma. Three months later, she was admitted because her serum creatinine levels increased from 54.81 μmol/L to 405.76 μmol/L accompanied by a high C-reactive protein level of 159 mg/L. Anti-neutrophil cytoplasmic antibodies against myeloperoxidase and anti-proteinase 3 were negative. Histological examinations of the solitary renal mass revealed necrotizing granulomatous arteritis in the cortex and medullary vasa recta, surrounded by interstitial fibrosis, and focal segmental necrotizing glomerulonephritis in the localized lesion; however, signs of vasculitis were not observed in areas other than the solitary mass. Therefore, the patient was diagnosed with granulomatosis with polyangiitis (GPA). Despite treatment with prednisolone (30 mg/day), the patient developed oliguria with an elevation of her serum creatinine level to 583.44 μmol/L, which required hemodialysis within one month after the initiation of steroid therapy. The patient could successfully discontinue hemodialysis 21 months later, following a decrease in her serum creatinine level to 251.06 μmol/L.ConclusionsGPA should be considered as one of the differential diagnoses of a solitary renal mass. Furthermore, patients with solitary renal masses associated with GPA may exhibit a favorable response to steroid or immunosuppressive treatment.

Sodium-Glucose Cotransporter 2 (SGLT-2) Inhibitors: Delving Into the Potential Benefits of Cardiorenal Protection Beyond the Treatment of Type-2 Diabetes Mellitus.

Diabetes mellitus is a leading cause of morbidity and mortality and a significant risk factor for the early onset of chronic kidney disease and heart disease. Hyperglycemia and insulin resistance are key factors that play a role in the pathogenesis of type 2 diabetes. Renal glucose reabsorption is a critical component of glycemic regulation. Sodium-glucose cotransporter 2 (SGLT2) inhibitors, commonly known as gliflozins, lower blood sugar levels by inhibiting glucose absorption in the proximal tubule of the kidney. SGLT2 inhibitors are currently used primarily as antidiabetic medications; however, their advantages go well beyond just glycemic control. This article has reviewed the mechanisms behind cardiac and renal involvement in type 2 diabetes and their inseparable interconnections. This article has also discussed the pharmacokinetic and pharmacodynamic profile of different SGLT2 inhibitors available in the market. Finally, this review has provided a perspective on the outcome trials, which provide evidence supporting a potential benefit of SGLT2 inhibitors in reducing cardiovascular and renal risks and possible mechanisms that mediate the renal and cardiovascular protection conferred.

Characterization of kidney complications in patients with sickle cell anemia.

Sickle cell nephropathy (SCN) is a poorly studied complication of pediatric patients. It appears in different forms, including glomerulopathy, and tubulopathies. To describe acute and chronic renal complications in patients with sickle cell anemia (SCA). Re trospective study. Pediatric patients with confirmed diagnosis of sickle cell disease were included who had a nephro-urology study. Hemoglobin electrophoresis pattern, presence and type of renal involvement, and presence of cardiac involvement were recorded. Bivariate analysis was perfor med to compare patients with and without SCN. 79 patients were included, 59.5% of them were men, and the most frequent electrophoresis pattern was Hb-SS (60.9%). The SCN oc curred in 70% of patients with an average age of 114 months (RIQ 65-157). The most frequently observed alterations were glomerular hyperfiltration, microalbuminuria, acute kidney injury, ar terial hypertension, and hyposthenuria. In the bivariate analysis, an abnormal echocardiogram result was presented more frequently in patients with SCN (84.8% vs. 54.3% p = 0.01), as well as more frequent use of nephrotoxic drugs (74.5% vs. 54.2% p = 0.07). Our findings suggest that sickle cell nephropathy may occur at an early age, where glomerular hyperfiltration is very common. Cardiopulmonary complications in patients with SCA may be related to the presence of SCN.

Renal disease in patients with celiac disease.

Celiac disease, an inflammatory disease of small bowel caused by sensitivity to dietary gluten and related protein, affects approximately 0.5-1% of the population in the Western world. Extra-intestinal symptoms and associated diseases are increasingly recognized including diabetes mellitus type 1, thyroid disease, dermatitis herpetiformis and ataxia. There have also been a number of reports of various types of renal involvement in patients with celiac disease including diabetes nephropathy, IgA nephropathy, membranous nephropathy, membranoproliferative glomerulonephritis, nephrotic syndrome related to malabsorption, oxalate nephropathy, and associations of celiac disease with chronic kidney disease and end-stage kidney disease. This review aims to present the current literature on possible pathologic mechanisms underlying renal disease in patients with celiac disease.

Clinical and pathological characteristics of non-diabetic renal disease in type 2 diabetes patients.

ABSTRACTBackgroundRenal involvement in type 2 diabetes is mainly due to diabetic nephropathy (DN). Nevertheless, a sizable proportion of diabetic patients could actually have nondiabetic renal diseases (NDRDs) or DN plus NDRDs. This study aimed to explore the pathological features of NDRD in diabetic patients and to assess the predictability of diagnosing NDRD (±DN) versus isolated DN on the basis of clinical parameters.MethodsMedical records of type 2 diabetes patients who underwent renal biopsy under suspicion of NDRD from January 2011 through November 2015 were analyzed retrospectively.ResultsA total of 101 patients were enrolled in this study. The most frequent indication for renal biopsy was recent onset of nephrotic syndrome (41%), followed by rapidly progressive renal failure (29%) and active urinary sediment (21%). On renal biopsy, 51% of patients had isolated DN, 20% had isolated NDRD and 29% had DN plus NDRD. IgA nephropathy was the most common cause of isolated NDRD, whereas acute tubular necrosis (39%) and acute interstitial nephritis (33%) were the main causes of NDRD superimposed on DN. Male gender, short-duration diabetes (<8 years), lower glycated hemoglobin and active urinary sediment (≥10 red and white blood cells per high-power field) were independent predictors of NDRD according to multiple logistic regression analysis.ConclusionsJudicious use of renal biopsy revealed NDRD (±DN) in nearly half of type 2 diabetes patients with atypical renal presentation, especially in male patients with well-controlled diabetes, those who have had diabetes for a short duration and those with active urinary sediment.

Idiopathic Retroperitoneal Fibrosis.

Idiopathic retroperitoneal fibrosis (RPF), reviewed herein, is a rare fibro-inflammatory disease that develops around the abdominal aorta and the iliac arteries, and spreads into the adjacent retroperitoneum, where it frequently causes ureteral obstruction and renal failure. The clinical phenotype of RPF is complex, because it can be associated with fibro-inflammatory disorders involving other organs, is considered part of the spectrum of IgG4-related disease, and often arises in patients with other autoimmune conditions. Obstructive uropathy is the most common complication, although other types of renal involvement may occur, including stenosis of the renal arteries and veins, renal atrophy, and different types of associated GN. Environmental and genetic factors contribute to disease susceptibility, whereas the immunopathogenesis of RPF is mediated by different immune cell types that eventually promote fibroblast activation. The diagnosis is made on the basis of computed tomography or magnetic resonance imaging, and positron emission tomography is a useful tool in disease staging and follow-up. Treatment of idiopathic RPF aims at relieving ureteral obstruction and inducing disease regression, and includes the use of glucocorticoids, combined or not with other traditional immunosuppressants. However, biologic therapies such as the Bcell-depleting agent rituximab are emerging as potentially efficacious agents in difficult-to-treat cases.

Non-diabetic renal disease with or without diabetic nephropathy in type 2 diabetes: clinical predictors and outcome

Background: Renal involvement in type 2 diabetes is mostly due to diabetic nephropathy (DN), but a subset of diabetic patients could present with pure non-diabetic renal disease (NDRD) or NDRD superimposed on DN. We conducted a prospective cohort study to identify the underline renal pathology in type 2 diabetic patients with defined clinical criteria for renal biopsy. Methods: A total of 46 patients (27 female, mean age of 48.9 ± 11.9 years) with type 2 diabetes mellitus (DM) and atypical features of DN with unexpected proteinuria, hematuria, and/or renal impairment were enrolled in this study. Results: Of 46 patients with type 2 diabetes, 16 (34.8%) had DN, 20 (43.5%) had NDRD, and 10 (21.7%) had NDRD superimposed on DN. Membranous nephropathy (34%) was the most common NDRD. Patients with NDRD had a lower frequency of diabetic retinopathy (5%), shorter duration of diabetes, higher range of proteinuria, and better kidney survival. In multiple logistic regression analysis, only lack of diabetic retinopathy was independent predictor of NDRD. Positive and negative predictive value of diabetic retinopathy (DR) for diabetic nephropathy was 94 and 68%, respectively. Conclusion: Kidney biopsy is strongly recommended for patients with type 2 diabetes and atypical renal presentation for DN, particularly in the absence of DR. This approach could lead to diagnosis of NDRD with better renal survival.

Analyzing renal involvement in 100 cases of hematological malignancy

Background: Hematological malignancies are the most common nonrenal neoplasms affecting the kidneys. The incidence and type of renal involvement in these malignancies are variable and differ according to malignancy. Objective: To study the prevalence of renal involvement in hematological malignancies and to study the clinical profile of patients in various malignancies. Materials and Methods: A total of 100 consecutive patients of hematological malignancies (leukemia, lymphoma, and multiple myeloma) were prospectively studied for renal involvement and clinical profile. Results: There were 72 men and 28 women with mean age of 46.85 ± 19.23 years. Among them, 43% patients had leukemia, 33% patients had lymphoma, and 24% patients had multiple myeloma. Renal failure was present in 37% of the total patients and one-third patients were having azotemic symptoms at presentation. Mean blood urea and serum creatinine levels were 73.75 ± 77.49 and 2.42 ± 3.22 mg/dl, respectively. Mean glomerular filtration rate (GFR) was 71.29 ± 54.92 ml/min and 17% patients had GFR < 15 ml/min; 62.5% patients of multiple myeloma had renal failure. Four patients had acute urate nephropathy (three patients had acute leukemia and one patient had multiple myeloma). Of the total, 18% patients had hypercalcemia and 41.7% patients had myeloma. Hyperuricemia was observed in 55.5% patients. Tumor lysis syndrome was seen in three patients and all these patients were having multiple myeloma. Male sex, multiple myeloma, and hyperuricemia were significant factors contributing to renal failure (p < 0.05). Conclusion: All patients with hematological malignancies should be evaluated for renal dysfunction and all preventive measures should be used in these patients, especially when initiated on chemotherapy.

Study of Renal Profile in Babies with Perinatal Asphyxia in a Tertiary Care Hospital: A Prospective Case Control Study

Introduction: Perinatal asphyxia is one of the important causes of preventable cerebral injury occurring in the neonatal period. Kidney is one of the most commonly affected organs leading to renal functional abnormality and blood electrolyte imbalance. This was a prospective case control study done in the NICU and neonatal unit at a tertiary care hospital. The objective of this study were to detect renal functional abnormality and electrolyte imbalance (sodium and potassium) among babies with perinatal asphyxia and to correlate severity and type of renal involvement with degree of asphyxia. Materials and Methods: Thirty two neonates for perinatal asphyxia and 32 babies selected randomly from non asphyxiated babies for the control group. Blood samples were taken for measurement of serum urea, creatinine, sodium and potassium levels on 1st and 3rd day of life. If any abnormality detected, values were repeated every alternate days till it become normal. Results: There were total 32 cases (asphyxiated). Among 32 cases 14 (43.75 %) had elevated levels of urea and creatinine on day 1 [Mean urea (43.21± 23.08), creatinine (1.14 ± 0.57)], 18 (56.25%) had elevated levels of urea and creatinine on day 3.Mean urea (58.06 ± 28.52) and creatinine (1.24±0.5) were significantly higher on day 3 (p value&lt;0.05) in study group as compared to control. Mean urea and creatinine levels showed increasing trend with degree of severity of hypoxic ischaemic encephalopathy. Eighteen babies with perinatal asphyxia developed renal failure (56.25%). 18 had Hyponatremia on day 1 (56.25%), 3 of them had value &lt; 125 meq /l. Conclusion: Among study cases significantly higher values of urea and creatinine were found than controls. The values were positively correlated to the degree of asphyxia. Though, mean sodium and potassium level was within the normal limit, the value of potassium was higher among cases than controls. DOI: http://dx.doi.org/10.3126/jnps.v33i3.8385 J. Nepal Paediatr. Soc. 2013;33(3):206-212

AB0411 Renal involvement in patients with rheumatoid arthritis

BackgroundThere is no doubt that renal lesion is one of the most unfavorable forms of visceral involvement in patients with rheumatoid arthritis (RA). There are several types of renal damage...

Renal involvement in multiple myeloma

In this paper, the spectrum of renal involvement in the course of multiple myeloma (MM) is discussed. We describe the most important pathophysiological mechanisms underlying the development of renal complications observed in MM. In particular, we focused on the correlations between morphological changes in the kidneys and clinical signs and symptoms. Physicochemical characteristics of light chains that are synthesized in excess are critically important in the development of different types of renal involvement. It seems that patients with MM should be actively treated regardless of the type of lesions because the current methods allow to reverse renal lesions and reduce the negative effect of renal damage on prognosis in these patients.

Renal complications in children with malignancies: single-centre experience

Background and aim of the work: Renal complications in cancer patients are serious and may cause more morbidity and mortality. Acute renal dysfunction may affect optimal cancer treatment by requiring a decrease in chemotherapy dosage or by contraindicating potentially curative treatment. Our study aimed to evaluate different types of renal involvement before and after treatment of childhood malignancies. Patients and Methods: All oncology patients at Mansoura University Children's Hospital diagnosed with different types of malignancies in seven years period (January 2003 to December 2009) were retrospectively reviewed for renal involvement before or after treatment. Results: We evaluated 954 pediatric patients with different types of malignancies. Patients encountered renal involvement were 152 (15.9%). A total of 105 patients diagnosed with acute renal failure (ARF), 18 had renal masses, 3 had nephrotic syndrome and 26 had hemorrhagic cystitis. As regard outcome of acute renal failure, 70 patients (66.7%) showed complete recovery of renal function, while 27 patients died (25.7%). The remaining 8 patients (7.6%) were maintained on chronic replacement therapy. Conclusions: Renal involvement in children with cancer is a common problem. ARF is the commonest renal complication and most severe. It results from various causes. Early prediction and anticipation of renal insult and prompt management may reduce the risk of renal injury. All oncologists should keep in mind that they are not treating cancer only, but they are treating patient with cancer and should save the kidney as much as possible.

ANCA-associated crescentic glomerulonephritis with immune complex deposits

Several authors have reported cases of anti-neutrophil cytoplasmic antibody (ANCA)-associated crescentic glomerulonephritis (CGN) with definite immune complex (IC) deposits, however, the clinical and pathological significance of IC deposits in patients with ANCA-associated CGN remains unclear. Renal biopsy specimens from 28 patients with a diagnosis of CGN and positivity for anti-myeloperoxidase (MPO)-ANCA were retrospectively evaluated. Clinical data were compared between patients with IC deposits (Group A) and patients without IC deposits (Group B). In 12 patients (43%; Group A), IC deposits were detected in the mesangium and/or along the glomerular capillary walls, while typical pauci-immune CGN without IC deposits was found in 16 patients (57%; Group B). Compared with Group B, Group A had lower levels of MPO-ANCA (171 ± 156 vs. 352 ± 299 EU) and C-reactive protein (CRP) (0.63 ± 1.04 vs. 4.45 ± 4.00 mg/dl), as well as less pulmonary involvement (8.3% vs. 56.3%) at diagnosis. However, Group A had significantly heavier proteinuria (2.46 ± 1.67 vs. 0.76 ± 0.52 g/d). Group A was classified into three subgroups: Group A1 with mesangial IgA and C3 deposits, A2 with mesangial IgG and C3 deposits, and A3 with IgG and C3 deposits mainly in the capillary walls. ANCA-associated CGN causes two types of renal involvement, which are the pauci-immune type without IC deposits and the IC deposition type that involves three patterns of IC deposition in the glomeruli. The reason why IC deposits are associated with renal-limited vasculitis and not systemic vasculitis remains unclear.

Renal lesions associated with autoimmune pancreatitis: CT findings

Autoimmune pancreatitis (AIP) is a chronic inflammatory condition characterized by IgG4-positive plasma cells. Recent evidence suggests that it is a systemic disease affecting various organs. Tubulointerstitial nephritis has been reported in association with AIP. To investigate the incidence and types of renal involvement in patients with AIP. Eighteen patients with no history of renal disease and a diagnosis of AIP (on the basis of histopathologic findings or a combination of characteristic imaging features, increased serum IgG4 levels, and response to steroid treatment) were included. All patients underwent computed tomography (CT) imaging and follow-up ranged from 6 months to 2 years. CT images were reviewed for the presence of renal lesions. Seven patients had renal involvement (38.8%). None of the lesions was visible on non-contrast-enhanced CT scan. Parenchymal lesions appeared as multiple nodules showing decreased enhancement (four cases). Pyelonephritis, lymphoma, and metastases were considered in the differential diagnosis. An ill-defined low-attenuation mass-like lesion was found in one patient, while diffuse thickening of the renal pelvis wall was evident in the last two cases. Renal lesions regressed in all patients after steroid treatment, the larger one leaving a fibrous cortical scar. Different types of renal lesions in patients with AIP are relatively common, appearing as multiple nodules with decreased enhancement. These findings support the proposed concept of an IgG4-related systemic disease. Autoimmune disease should be suspected in cases of renal involvement in association with pancreatic focal or diffuse enlargement.

Stone Forming Risk Factors in Patients With Type Ia Glycogen Storage Disease

Patients with type Ia glycogen storage disease have an increased recurrent nephrolithiasis rate. We identified stone forming risk factors in patients with type Ia glycogen storage disease vs those in stone formers without the disease. Patients with type Ia glycogen storage disease were prospectively enrolled from our metabolic clinic. Patient 24-hour urine parameters were compared to those in age and gender matched stone forming controls. We collected 24-hour urine samples from 13 patients with type Ia glycogen storage disease. Average +/- SD age was 27.0 +/- 13.0 years and 6 patients (46%) were male. Compared to age and gender matched hypocitraturic, stone forming controls patients had profound hypocitraturia (urinary citrate 70 vs 344 mg daily, p = 0.009). When comparing creatinine adjusted urinary values, patients had profound hypocitraturia (0.119 vs 0.291 mg/mg creatinine, p = 0.005) and higher oxalate (0.026 vs 0.021 mg/mg creatinine, p = 0.038) vs other stone formers. Patients with type Ia glycogen storage disease have profound hypocitraturia, as evidenced by 24-hour urine collections, even compared to other stone formers. This may be related to a recurrent nephrolithiasis rate greater than in the overall population. These findings may be used to support different treatment modalities, timing and/or doses to prevent urinary lithiasis in patients with type Ia glycogen storage disease.

DNase1 activity in systemic lupus erythematosus patients with and without nephropathy

The objective of the study is to determine whether the activity of DNase1 is associated to the presence of nephropathy in patients with SLE. Forty-five patients affected with SLE and renal involvement were analyzed. The type of renal involvement was type III or IV glomerulonephritis. At least two serum samples were withdrawn from each patient, one obtained in a renal flare and the other obtained in a period of clinical stability. C3 and C4 complement levels and anti-DNA antibodies were determined. DNase1 activity was measured using a radial enzyme-diffusion method. Results suggest that when comparison of DNase1 activity was established between samples obtained during a phase of active renal involvement and those obtained in the clinically stable phase, we did not find statistically significant differences. When the comparison was performed with matched samples of the same patient, DNase1 activity was lower when patients had active renal involvement than when samples were taken in clinically stable phase (21.21 μg/ml ± 16.47 vs. 25.62 μg/ml ± 18.81, p < 0.05). No difference in DNase1 activity was observed between samples positive or negative for anti-DNA antibodies. No difference in DNase1 activity was found in patients with normal or decreased levels of C3 (25.09 μg/ml ± 17.78 vs. 20.01 μg/ml ± 16.15, p = 0.073) or C4 (23.52 μm/ml ± 16.60 vs. 19.62 μg/ml ± 17.54, p = 0.060). We conclude that low DNase1 activity is associated to the active phase of type III or IV nephropathy. Therefore, it is possible that this enzyme plays an important role in the development of SLE nephropathy.