Rapidly Progressive Renal Failure Research Articles

Rapidly Progressive Renal Failure in Young Adult: An Atypical Presentation of Multiple Myeloma - A Case Report

Introduction: Patients frequently present with renal impairment that does not fit the criteria for acute kidney injury or chronic kidney disease. This condition, known as rapidly progressive renal failure (RPRF), encompasses a diverse range of clinical syndromes characterized by the progressive renal impairment over days to weeks. Case Report: We present a case of a 37-year-old male with a short history of uremic symptoms and decreased urine output with no significant history or examination findings except pallor. The patient had a creatinine of 1.6 mg/dL in April 2022, rising to 4.4 mg/dL, then 11.6 mg/dL during May, 17.6 mg/dL in June, presented to us with a creatinine of 24.6 mg/dL, with normal kidney size and corticomedullary differentiation (CMD). Beta-2-microglobulin was elevated, and renal biopsy showed features suggestive of multiple myeloma (MM). Atypical appearing tubular casts showing 3+ patternless staining for kappa light chains and negativity for lambda light chains were observed. The patient was started on chemotherapy for MM, and renal function was gradually improving. Conclusion: Renal failure as the sole presentation of MM is rare, especially in young adults. This case emphasizes an atypical RPRF, particularly given the patient’s young age.

Rapidly Progressive Renal Failure in Type 2 Diabetic versus Non-diabetic Patients: Changing Trends, Natural History, and Outcome in an Indian Tertiary Care Center.

Rapidly progressive renal failure (RPRF) is not typical of diabetic nephropathy and suggests non-diabetic kidney disease (NDKD). We conducted an analysis of the data of RPRF patients (28 diabetic and 88 non-diabetic patients) with doubled creatinine over 2 weeks to 3 months and/or presented with >4 mg serum creatinine without prior renal disease to ascertain the types of lesions and compare the patients' histopathology. The primary outcome was dependence on dialysis at 1 year. Anti-neutrophilic cytoplasmic antibody-associated pauci-immune glomerulonephritis was the most common cause of RPRF in both groups. No particular lesion was more frequent in either group. Dependence on dialysis at 1 year was similar in both groups and was associated with dependence on dialysis at presentation but not diabetes. Crescentic glomerulonephritis was the most common in non-diabetic patients (57.9 vs. 25%, P = 0.002), and acute tubular necrosis (ATN) was seen in diabetic patients (21.4 vs. 11.4%, P = 0.179). Both factors were associated with adverse renal outcomes. Diffuse global glomerulosclerosis at presentation suggested a poor outcome in both groups. Diabetic nephropathy was seen in 14.29%, and its presence did not affect the outcome. The etiology of RPRF in diabetic patients has changed and is similar to that in non-diabetic patients, with no specific lesions predominating. Diabetic nephropathy does not alter the outcome for those with RPRF. Diffuse global glomerulosclerosis, being on dialysis at presentation, and ATN in a diabetic patient indicate a poor outcome and need close follow-up. Diabetic retinopathy should not prevent us from investigating for NDKD.

#6045 TWO-YEAR INPATIENT ACTIVITY OF A DEDICATED ONCONEPHROLOGY DEPARTMENT

Abstract Background and Aims Onconephrology is a rapidly evolving nephrology subspecialty that focuses on the interaction between cancer and the kidney. Our aim was to describe the clinical activity of a Nephrology Department in a cancer care center, in a 2-year period. Method We retrospectively analyzed the patients observed by the Nephrology Department at our center between 2021 and 2022. Demographic, clinical and laboratory data were collected. The statistical analysis was performed with SPSS software. Results During the 2-year period, there were 26,015 admissions, 636 of which required a Nephrology observation. Sixty-two per cent were female; the mean age was 68 years (SD ±12.4) with a median Charlson Comorbidity Index of 7. Most cancers were from gastrointestinal (24.1%, n = 153) or urologic origins (22.3%, n = 142), 37% had metastatic disease. Hematologic malignancies accounted for 12.1% of patients (n = 77). Nearly 44.5% of patients (n = 283) had chronic kidney disease (CKD), 20.8% (n = 59) of which were on hemodialysis (HD). Urinary tract obstruction (23.3%), cardiovascular risk factors (17.3%) and nephron loss (10.6%) were the most common causes of CKD. The main reasons for admission were renal dysfunction (23.7%; n = 151), infectious complications (23.3%, n = 148) and elective surgery (20.3%; n = 129). Of the patients observed, 40.7% (n = 259) had acute kidney injury (AKI), 23.3% (n = 148) acute-on-chronic kidney disease and 11.8% (n = 75) hydro-electrolytic disorders. Ten patients (1.6%) were evaluated for nephrotic syndrome (NS) or rapidly progressive renal failure (RPRF). Among AKI patients, intrinsic causes were the most prevalent (48.7% of all cases), with sepsis and ischemic and toxic acute tubular necrosis being the most frequent. Pre-renal and post-renal etiologies occurred in 29.7% and 21.6% of cases, respectively. Gynaecological and prostate tumours were the main neoplastic cause of post-renal AKI. There were 5 TMA cases in allogenic stem cell transplanted patients or treated with gemcitabine and six cases of acute interstitial nephritis, the majority immune check-point inhibitors-related. Over 54% (n = 142) of patients presented with AKI stage 3. About 13% (n = 34) needed renal replacement therapy (RRT), mainly continuous or hybrid techniques (58.8%; n = 20), and only 38% (n = 13) recovered renal function. The group of acute-on-chronic CKD patients included mostly stage 3b and 4 CKD. Most of them (54.1%; n = 80) had an intrinsic cause for kidney dysfunction. There was an equal distribution between stages of AKI severity. About 5% (n = 8) needed RRT and only two of those recovered. Hyponatremia was the most common electrolyte disturbance (56%; n = 42), followed by hypercalcemia (17.3%; n = 13). Over 40% of all cases of hyponatremia occurred in patients with metastatic lung cancer, probably related with the high prevalence of SIADH in this population. All but two patients with NS or RPRF underwent kidney biopsy. Among the patients with NS, its etiologies were: focal and segmental glomerulosclerosis secondary to pamidronate (n = 2), podocytopathy related to pazopanib (n = 1), paraneoplastic membranous nephropathy (n = 1), AL amyloidosis (n = 1) and TMA secondary to gemcitabine (n = 1). The causes of RPRF were ANCA-negative small vessel vasculitis, crescentic glomerulonephritis (GN) related to pembrolizumab and TMA. We also followed two patients with acute kidney disease, one with ATN secondary to bisphosphonates therapy and other with paraneoplastic fibrillary glomerulopathy. The median duration of hospitalization was 10 days (IQR 7.0 – 20.8), with median Nephrology follow-up time of 5.5 days (IQR 3.0 – 9.0). The overall mortality rate was 21.5%, and it was even higher on the subgroup with AKI (27.4%; p = 0.03). About 44% (n = 219) of patients were referred to outpatient Nephrology follow-up, at discharge. Conclusion Our data provides a picture of the rich inward activity developed at our cancer center by the nephrology team. The complexity of these patients highlights the importance of a multidisciplinary approach in their management. No surprisingly, there was a high mortality rate, which was even higher in the AKI-patients subset.

Spectrum of Biopsy Proven Renal Disease in the Elderly – A Retrospective Observational Study from South India

Abstract Background: Despite the increasing proportion of the elderly population, renal biopsies are restricted to few centers. We studied the elderly renal biopsy data from a tertiary care center in South India. Materials and Methods: A retrospective observational study was done in patients more than 60 years of age who underwent native kidney biopsy for various clinical indications from January 2014 to June 2022 and was compared with the renal pathology data of adults 18–60 years of age who underwent native kidney biopsy for various indications during the same study. Results: Hundred and five (n = 105) elderly biopsies were included in the study. The mean age was 65.7 years (interquartile range: 60–85 years). Forty-two (40%) were type 2 diabetics. Infection-related glomerulonephritis (IRGN) (20, 19.04%), acute tubular necrosis (ATN) (15, 14.28%), membranous nephropathy (11, 10.47%), and acute interstitial nephritis (10, 9.52%) were the common pathologies observed in the elderly. The most common indication for renal biopsy was rapidly progressive renal failure (RPRF) (n = 28, 26.6%) and IRGN was the most common cause of RPRF. 76.2% of diabetics had nondiabetic renal disease, the most common being IRGN (8, 25%). Crescentic glomerulonephritis (GN), ATN, and cast nephropathy were observed significantly more frequent in the elderly than in those 18–60 years of age. IRGN and pauci-immune vasculitis were the causes of crescentic GN in the elderly. Conclusion: Our study highlights the importance of IRGN as the most frequent pathology in the South Indian elderly population both in diabetics and nondiabetics.

POS-036 A YOUNG FEMALE WITH RENAL CORTICAL NECROSIS TREATED WITH INTRAVENOUS IMMUNOGLOBULIN IN A RESOURCE POOR SETTING: CASE REPORT

Renal cortical necrosis is a rare cause of acute kidney injury (AKI) characterized by diffuse or patchy ischemic coagulation necrosis of the cortex. One of the important causes is acute Thrombotic Microangiopathy (TMA).Thrombotic microangiopathy (TMA) is one of the most important causes of Renal cortical necrosis and Rapidly progressive renal failure (RPRF). It can be classified into Primary (hereditary or acquired) & secondary TMA. It is very crucial to diagnose & treat Renal cortical necrosis & TMA early to avoid morbidity & mortality.

Nephrotic syndrome with rapidly progressive renal failure (RPRF) in light chain myeloma

No abstract available

Sometimes an Ultrasound Scan Before the Kidney Biopsy Is Enough: An Unexpected Case of Rapidly Progressive Renal Failure(RPRF)

Sometimes an Ultrasound Scan Before the Kidney Biopsy Is Enough: An Unexpected Case of Rapidly Progressive Renal Failure(RPRF)

Post –Operative Bilateral Renal Sub capsular Hematoma, Successfully Treated with Laparoscopic Drainage – A Case Presentation

Page kidney is a rare phenomenon which present as uncontrolled hypertension and hypertensive emergencies. Here we bring to notice a case presenting with anuria and rapid progressive renal failure. We have also tried to focus on the use of imaging for diagnosis and benefits of prompt and effective therapy causing complete recovery of the kidneys.

SUN-436 CLINICOPATHOLOGICAL PROFILE OF RENAL DISEASE IN PARAPROTEINEMIA AND MONOCLONAL GAMMOPATHY OF RENAL SIGNIFICANCE

Presence of kidney injury in Plasma cell dyscrasias confers increased mortality. Several pathogenic mechanisms can contribute to renal failure in patient with myeloma, some of which are the result of nephrotoxic monoclonal Ig and some of which are independent of paraprotein deposition. Often multiple pathologies co-exist and contributes to renal failure. AIM:To study the clinical and pathological profile of renal diseases in patients with paraproteinemia and monoclonal gammopathy of renal significance. To study renal and patient outcome in such patients. This was a retrospective cum prospective observational study between March 2013 to July 2019 on the clinical profile, hematological characteristics, renal biopsy findings and outcome among 54 patients with either multiple myeloma or monoclonal gammopathy of renal significance (MGRS) as defined by international myeloma working group (IMWG) The males to female ratio was 1.8: 1(n=54). The mean age was 59 ± 8.03 years. Multiple myeloma criteria were meet in 65%(n=35) and MGRS in 35%(n=19). Rapidly progressing renal failure (RPRF) was the most common presentation 48% (n=26) followed by nephrotic syndrome 26%(n=14). The most common renal pathology was cast nephropathy 24 %(n=13). In MGRS the most common presentation was nephrotic syndrome 36.8% (n=7 out of 19) and light chain deposition disease (LCDD) 36.8%(n=7) the most common pathology. Out of the 28 (52%) patients requiring dialysis only 2 became dialysis independent at 1 month, and 17 (32%) patients died. Bortezomib based therapy was given in 17 (31.4%) patients. Infection rate were significantly higher in bortezomib group 70.5% (n=12) compared to non-bortezomib group 40.5%(n=15) (p-value is .04). Statistically significant factors associated with mortality were male sex, age more than 60 years, RPRF presentation, dialysis requiring renal failure, overt myeloma, cast nephropathy, and underlying diabetes mellitus.Table 1spectrum of plasma cell dyscrasia associated renal pathologyRenal pathology #Number of patients (n=54)MGRS (n=19)Cast nephropathy (CN)13 (24%)4 (21%)Amyloidosis (AL)9 (6.6%)6 (31.5%)CN + light chain deposition disease (LCDD)10 (18.5%)2 (10.5%)LCDD10 (18.5%)7 (36.8%)PGNMID (proliferative glomerulonephritis with monoclonal Ig deposition)1 (1.8%)0LCPT (light chain proximal tubulopathy)1(1.8%)0Cryoglobulinemic glomerulonephritis2(3.6%)0Biopsy not done8 (14.8%)0#46 patients out of 54 underwent renal biopsy. Open table in a new tab #46 patients out of 54 underwent renal biopsy. Most common presentation was rapidly progressive renal failure. Most common pathology seen was cast nephropathy. Patients with cast nephropathy had poor outcome.

Gerontolizing Nephrology: Spectrum of Histopathological Findings of Kidney Biopsy in the Elderly.

Introduction:The spectrum of renal disorder in the elderly differs from the younger population. There is a paucity of literature regarding kidney biopsy in elderly. This study aims to highlight the clinical profile and histopathological spectrum of the elderly patient undergoing a renal biopsy.Materials and Methods:This retrospective study included all patients (age ≥60 years) undergoing native renal biopsies from January 2012 to December 2017. The clinical profile, laboratory parameters, and renal biopsy findings of these patients were recorded from the case files.Results:Out of 1656 renal biopsies performed during the study period, 230 (13.9%%) performed on the elderly were included. Mean age was 64.02 ± 7.87 years (Range: 60-87 years), and males were predominant (70.4%). The commonest indication for biopsy was nephrotic syndrome (NS) (49.6%) followed by Rapidly progressive renal failure (RPRF) (20.9%) and Acute Kidney Injury (AKI) (15.7%). The most frequent histological diagnosis was membranous nephropathy (15.2%) followed by amyloidosis (13.9%) and Focal Segmental Glomerulosclerosis (FSGS) (13.0%). The commonest cause of NS was MGN (29.8%) followed by FSGS (24.6%) and amyloidosis (22.8%). The commonest cause of nephritic syndrome was Diffuse Proliferative Glomerulonephritis (29.4%) and Membranoproliferative Glomerulonephritis (29.4%). Hypertensive nephrosclerosis (40.0%) and diabetic nephropathy (26.7%)) were the commonest histological diagnosis in the patients who underwent renal biopsy for clinical Chronic kidney disease. Crescentic GN (35.4%) and Myeloma cast nephropathy 14.6%) were the commonest cause of RPRF while Acute Tubular Necrosis (41.7%) was the commonest cause of AKI. None of the patients had major complications.Conclusion:Renal biopsy is safe in the elderly and provides a wealth of information with regards to the diagnosis and prognosis of renal disorder.

SUN-038 RECOVERY OF KIDNEY FUNCTION IN GLOMERULONEPHRITIS WITH RAPIDLY PROGRESSIVE RENAL FAILURE AT CHO RAY HOSPITAL

It is uncommon for patients with glomerulonephritis (GN) who require hemodialysis (HD) to recover renal function. Lupus nephritis (LN) is one of the common GN in Vietnam. Our aim was to describe the renal outcome of 64 GN patients with rapidly progressive renal failure (RPRF) on HD at Cho Ray Hospital, Ho Chi Minh City, Vietnam. A prospective study was conducted in 64 GN patients with RPRF requiring HD from 2014 to 2018. RPRF was defined as a progression to renal failure within a period of 1 week to three months. A definitive diagnosis of the kidney disease was established by history, clinical examination, lab tests, and renal biopsy. The patients had received standard therapy for their primary medical condition. We followed the patients after initiation of HD (3 months min, 18 months max) to evaluate recovery sufficient to discontinue HD Patient median age 25 (Inter-quartile range 21,32); 49 females; 59/64 pts with lupus nephritis (LN) (54 class III or IV, 1 class V, and 1 unclassified). 3 patients had IgA nephropathy; 1 Anti-glomerular basement membrane GN; 1 idiopathic membranoproliferative GN. Thrombotic microangiopathy (TMA) was clinically diagnosed based on Coombs negative hemolytic anemia, thrombocytopenia, D-Dimer >600 ng/mL, LDH >600 U/L, schistocytes on blood smear, with or without kidney biopsy. TMA was found in 56 pts, of which 19 were biopsy proven. Six LN patients with TMA were tested for ADAMTS13, all of whom were negative. Patients with TMA were treated with therapeutic plasma exchange (26 cases) or plasma infusion (30 cases) and heparin anticoagulation. All 64 pts were on oral methylprednisolone (median 32mg/day, IQR 16, 48mg) for the first month then tapered. 41 pts (39 LN, 2 non Lupus GN) received median of 6 doses of monthly intravenous cyclophosphamide 0.5g/m2 over median 12 months follow up. 36/59 (61%) of LN pts recovered kidney function, and discontinued HD after median 39 days (IQR 24;72 days). They remained dialysis independent for a median of 10 months (IQR 6 ,11 months) follow up. Median of the last serum creatinine in patients who recovered function was 1.4 mg/dL (IQR 1.2; 1.7 mg/dL). In the 28 patients who failed to recover kidney function, 4 died and 24 continued HD after median 4 months. Recovery of renal function was more common in LN pts with TMA or patients with length on HD of less than 3. Recovery of function was not associated with age, gender, body mass index, hypertension, 24h proteinuria, hematuria, isolated TMA diagnosis, anemia, or serum albumin on admission). TableComparison between patients who recovered and failed to recover functionTotal (N=64)Recovered kidney function (N=36)Non recovered kidney function (N=28)pLupus nephritis5936 (61%)230.03Nonlupus GN50 (0%)5TMAneg/LNpos85 (62.5%)30.01TMApos/LNpos5131(60.7%)20TMApos/LNneg50(0%)5Length on HD ≤ 3 months4632(69.5%)140.005Length on HD > 3 months184(22.2%)14 Open table in a new tab RPRF in lupus nephritis patients with or without TMA had a good likelihood of function recovery after requiring hemodialysis.

MON-224 DIAGNOSING RENAL THROMBOTIC MICROANGIOPATHY BASED ON HISTOPATHOLOGY IN RAPIDLY PROGRESSIVE RENAL FAILURE

Appart from glomerular and tubulointerstitial lesion of rapidly progressive renal failure (RPRF), renal vascular lesion is a critical prognostic finding in which renal thrombotic microangiopathy (TMA) present with severe clinical manifestation and have a high morality. The aim of this study were to describe (1) the histopatological features of TMA, (2) its classification and (3) the associated lesions in RPRF. We observed 133 kidney biopsies performed in patients presented with RPRF from 2013 to March 2018 by by using light microscopy. Renal TMA was classified as acute TMA, defined by luminal thrombi including fibrin and fragments of red blood cells and associated with ischemic capillaries, mesangiolysis and edematous and mucinous intimal thickening, often infiltrated with foam cells. Chronic TMA was characterized by onion-skin fibrointimal sclerosis. Chronic active TMA showed both acute and chronic changes. There were 38 cases of TMA diagnosed histopathologically including 31/38 (81.6%) lupus nephritis (LN) with 26/31 classIV, 1/31 class V, 1/31 class III-V, 2/31class IV-V; 6/38 (15.8%) IgA nephropathy (IgAN) and 1/38 (2.6%) focal segmental sclerosis (FSGS). All 45% acute TMA were LN. 23% chronic TMA included 13% LN, 8% IgAN and 2% FSGS. 32% Chronic active TMA were 24% LN and 8% IgAN. Only one LN (2.6%) showed thrombus in artery. 34.2% cases with capillary occlusion were LN. The associated arteriolar lesions including thrombi (55.3%), edematous and mucinous intimal thickening (73.7%), fibrointimal sclerosis (34.2%) and immunofluorescence staining of thrombi with fibrin (44.7%) presented both in LN and IgAN. 55% had ischemic glomeruli. The FSGS solely showed arteriolar fibrointimal sclerosis and ischemic loops suggested for chronic TMA. The mesangiolysis, endothelial swelling and subendothelial expansion were not recognised in our observation probably due to the limitation of the quality of the microscopic slides as well as the lack of electromicroscopic equipment. The duplication of the basement membrane features was not applied for defining chronic TMA in capillaries especially in LN because of its frequent presence in many chronic conditions. Pathological features of renal thrombotic microangiopathy can varies considerably and can be seen frequently in many different severe kidney conditions.

Importance of renal biopsy in patients aged 60 years and older: Experience from a tertiary care hospital.

As the life expectancy is increasing, there is a rise in elderly population and consequent increase in the patients with renal disease. There is an inconsistency between clinical and histopathological diagnosis in elderly, and so renal biopsy is important in these patients to decide appropriate clinical management and prognosis. This study outlines the importance of renal biopsy in elderly and describes the clinical and pathologic spectrum of renal diseases in patient ≥60 years. All patients (age ≥60 years) undergoing renal biopsies from January 2011 to December 2014 were included in this retrospective study. The clinical presentation and biochemical findings were recorded, and the patients were grouped based on their clinical presentation. Renal biopsies were also evaluated. The mean age of patients was 67.7 ± 6.4 years with a male:female ratio of 3:1. The most common clinical manifestation was nephrotic syndrome (37.4%) followed by rapidly progressive renal failure (RPRF) (20.6%). Amyloidosis and membranous nephropathy were two most common diagnoses in patients with nephrotic presentation whereas pauci-immune crescentic glomerulonephritis and cast nephropathy were common in patients presenting with RPRF. Clinical diagnosis differed from the histopathological diagnosis in 32% cases of nephrotic syndrome. There was good agreement between clinical diagnosis and histology in cases with RPRF. In 73% cases of elderly with (Type II) diabetes suspected of having nondiabetic renal disease clinically, renal biopsy showed evidence of diabetic nephropathy. Renal biopsy is essential in the diagnosis of renal diseases even in elderly. Amyloidosis and membranous nephropathy are common causes of nephrotic syndrome in elderly. Renal biopsy is very useful in diagnosing cast nephropathy and amyloidosis as they are not suspected clinically. It is also helpful in elderly diabetics without retinopathy to differentiate between diabetic and nondiabetic kidney diseases.

Association of five-factor score with the mortality in Japanese patients with polyarteritis nodosa

Aim: To determine mortality and its predictive factors in Japanese patients with polyarteritis nodosa (PAN).Methods: This retrospective single-center study determined the mortality of 18 patients with PAN who were admitted to Juntendo University Hospital from 1994 to 2016. The variables at baseline, including patient demographics, clinical characteristics, and treatment, were analyzed for their association with mortality.Results: The median age of onset was 57.0 years. The 1-year survival rate was 100% (16/16) and the 5-year survival rate was 80.0% (8/10). The relationship between mortality, as defined by the survival rate and each variable was evaluated by Cox univariate analysis. A higher 2009 five-factor score (FFS) was associated with increased mortality, with a hazard ratio of 2.34 (p = .04). Analysis of the secondary outcome of relapse-free survival time revealed an association with rapid progressive renal failure, Birmingham Vasculitis Activity Score (BVAS), the 1996 FFS, and the 2009 FFS, with hazard ratios of 7.28 (p = .048), 1.26 (p = .02), 2.32 (p = .03), and 1.82 (p = .04), respectively.Conclusion: We investigated mortality, relapse-free survival, and their predictive factors in Japanese patients with PAN. The BVAS and the 1996 FFS at diagnosis may be prognostic factors for relapse-free survival, and the 2009 FFS at diagnosis may be a prognostic factor for both mortality and relapse-free survival.

Autosomal polycystic kidney disease

Autosomal dominant polycystic kidney disease (ADPKD) is the most common monogenetic kidney disease. The clinical course is highly variable, association to certain genetic mutations only weak. Great progress has been made in recent years in determining the pathophysiology of the disease. Diagnosis of ADPKD is almost always possible by ultrasound, genetic examination is confined to selected cases. In addition, imaging is important for prognosis where MRI and computer tomography are superior for determination of total kidney volume. Until recently, supportive therapy has been the only available therapeutic option. This includes optimal antihypertensive therapy with a goal blood pressure below 110/75 mm mercury, at least with normal renal function and without any other contraindication. In addition, fluid intake should be increased to 2.5-4 l per day. Finally, tolvaptan is available as a specific therapy in selected countries. This therapy should be restricted to cases with rapid progressive and early renal failure due to costs and side effects. The value of this agent in later stages of chronic renal failure is currently being evaluated in clinical studies. Additional specific therapies are in early clinical evaluations.

Histological Pattern of Renal Disease in Those with Type 2 Diabetes Mellitus (T2DM)

The prevalence of nondiabetic renal disease in those with T2DM is very common in multiple series across the world, however, data from India is limited. This study was done by including all subjects of T2DM; who underwent renal biopsies at EMS Memorial Cooperative Hospital, Perinthalmanna, Kerala, India, from September 2009 to August 2016. Seventy-one (Males:47, Females:24, Mean age: 52.93; SD 12.56 years) subjects with T2DM; who underwent renal biopsy; with a suspicion of non-diabetic renal disease were included. The indications for renal biopsy were: acute on chronic renal failure (ACRF) (35.2%), nephrotic syndrome (NS) (31%), acute renal failure (ARF) (14.1%), nephritic syndrome (14.1%), rapidly progressive renal failure (RPRF) (4.2%) and subnephrotic protienuria (1.4%). The prevalence rates of nondiabetic renal disease (NDRD), diabetic nephropathy (DN) and DN with NDRD were 50.71, 28.16 and 21.13 % respectively. The mean durations of T2DM were 12.45, 12.13 and 5.33 years in patients with DN, DN & NDRD and NDRD, respectively. Wide spectrum of glomerular diseases was observed among those with isolated NDRD; commonest being IgA nephropathy (IgAN) (9.86 %), followed by infection related glomerulonephritis (IRGN) (7.04 %), membranous nephropathy (MN) (5.63%), focal segmental glomerulosclerosis (FSGS) (4.22 %) and miscellaneous (14.1%) lesions. The acute interstitial nephritis (AIN) was the commonest of the tubulointerstitial diseases (TIDs) in those with isolated NDRD. Among the patients with DN with associated NDRD, acute tubular necrosis (ATN) (7.04%) and IRGN (5.63%) were the commonest associated lesions. This study underlines the importance of renal biopsy in patients of T2DM with atypical features.

Forms of presentation of ANCA associated vasculitis – The 10 year experience of a medical intermediate care unit

Aims: To characterize the population admitted for antineutrophil cytoplasmic antibodies (ANCA) associated vasculitis to our medical intermediate care unit and its form of presentation. Material and Methods: Review of clinical files from 22 patients with positive ANCA antibodies or clinical suspicion of autoimmune vasculitis, admitted between January/03 to December/12 to our intermediate care unit. Results: Thirteen patients were female (59%). The mean age was 58years (32–83). Fifty-nine percent presented with rapidly progressive renal failure (RPRF), 27% with pulmonary–renal syndrome, 9% with mononeuritis multiplex and 5% with isolated respiratory failure.

A case of renal γ2 heavy-chain deposition disease accompanied by rapid progressive renal failure

The recently described heavy-chain deposition disease (HCDD) is a comparatively rare monoclonal immunoglobulin disorder characterized histopathologically by glomerular and tubular basement membrane deposition of nonamyloidotic monoclonal heavy chains without associated light chains. We have described a case of γ2-HCDD presenting with proteinuria, microhematuria, severe hypertension, and rapidly progressive renal failure, which serum creatinine level was only 1.52 mg/dL in early stage HCDD, and the clinicopathologic features of this case have been compared with other reported cases of γ2-HCDD. Renal biopsy disclosed nodular sclerosing glomerulopathy. Immunofluorescence analysis revealed IgG2 (2+) heavy chain and C3 (+) in the mesangium and along the capillary walls and tubular basement membranes without IgA, IgM, κ and λ light chains. Electron microscopy revealed electron-dense deposits along the glomerular and tubular basement membranes as well as in the mesangium. Moreover, regardless of therapy, the condition of the patient progressively deteriorated, with less than 3 months of renal survival. Rapid progressive renal failure was a common feature in both cases of γ2-HCDD. We propose that a possible link exists between prognosis of renal HCDD and the subclass of heavy chain deposited in the kidney.

Rapidly progressive renal failure--a rare presentation of granulomatous interstitial nephritis due to tuberculosis--case report and review of literature

Granulomatous interstitial nephritis (GIN) is a rare manifestation of renal tuberculosis (TB). We report a case of rapidly progressive renal failure (RPRF), granulomatous inflammation of cervical lymph node and GIN as presenting manifestations of TB. Aspiration cytology of cervical lymph node showed granulomatous necrotizing inflammation with acid-fast bacilli (AFB). The renal biopsy and urine specimen did not show AFB. Urine polymerase chain reaction (PCR) for Mycobacterium tuberculosis was positive. We observe that GIN due to TB can present as RPRF and emphasize the value of PCR-based techniques in making a correct diagnosis.

Erste in der Schweiz erworbene Nephropathia epidemica

Summary First case of nephropathia epidemica contracted in Switzerland The patient, a 43-year-old male living in western Switzerland, presented with unprecedented prostration, fever, vomiting, diarrhoea, gastric pain, dysuria and severe thrombocytopenia. After wide-ranging differential diagnosis comprising urosepsis among others, the patient was diagnosed as having rapid progressive renal failure due to acute tubulointerstitial nephritis. Extensive laboratory investigations finally revealed hantavirus-specific positive IgM and IgG by immunoblot technique. The diagnosis of nephropathia epidemica was confirmed by a hantavirus serotype-specific focus reduction neutralisation test performed on the convalescent serum obtained 4½ months after the onset of symptoms, revealing serotype Puumala virus as the causative agent. The patient was treated symptomatically and could be discharged in good health 10 days after disease onset. Hantaviruses are rodent-borne. This patient must have been infected in Switzerland since his last stay abroad had been prior to the upper limit of incubation time. Hantavirus infection should therefore be considered in Swiss patients with acute nephritis of unknown origin.