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ALL WGs (743)
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Criteria: NEPH Found: 43 cases
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Case 1 - ecp24, Nephropathology
Presented by: Lenka Bauerova, Prague, Czech Republic

What’s hiding behind IgA nephropathy?

A-31 year-old woman was admitted to the hospital for proteinuria, fatigue, edema of the hands, tinnitus and sudden hearing loss of the left ear. Physical and laboratory examination revealed arterial hypertension, nephrotic proteinuria (5g/day) and microscopic hematuria. Kidney function was normal with a serum creatinine level of 73μmol/l (0,79mg/dl). Immunology was negative (ELISA testing – IgA, IgM,IgG, C3 and C4 complement, extractable nuclear antigen (ENA), anti- nuclear antibodies (ANA), ds-DNA antibodies, ANCA, rheuma factor). Audiometry showed hearing abnormalities. In her family history, a father, uncle and sister suffer from IgA nephropathy (IgAN). She had no history of taking chronic medication or drug abuse. A kidney biopsy was performed with a clinical diagnosis of IgAN or Alport syndrome.
PASAFOGFig 1 IF - IgAFig 2 PASFig 3 PASMFig 4 Semithin sectionFig 5 Ultrastructure
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Case 2 - ecp24, Nephropathology
Presented by: Marlene Praet, Gent, Belgium

Man of 53 years old with known alcohol abuse smoking 10 to 12 cigars a day.
He developed a glottis carcinoma in 1994 with T3N0M0.
During the period from 2007 to 2010 he was known at the Respiratory Department because of recurrent hemoptoe presenting a cystic lesion at the Right Upper Lobe of the Lung.

A lobectomy was performed revealing pachypleuritis with underlying scar of the parenchyma and chronic inflammation. No malignancy was discovered.

The patient developed eventually a hydropneumothorax with infectious clinical picture: crp 15 mg/dl, WBC 19000, fever 39 °C. In the sputum H. Influenzae was cultivated.

3 weeks later the patient was admitted in emergency with acute renal failure presenting creat 4.21 mg/dl, proteinuria 4.3 g/L, macroscopic hematuria, oliguria, WBC 21700, crp 10.6 mg/dl. A renal biopsy was performed.
KR low 11-14399Ag meth11-14399Ag meth 2 11-14399KR 11-14399
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Case 3 - ecp24, Nephropathology
Presented by: Eduardo Vazquez Martul, La Coruna, Spain

Male 59 years old with previous history of pulmonary disease with diagnostic of sarcoidosis three year before, with mediastinic and interstitial pulmonary affectation. No history of renal dysfunction was detected. The Crp was 1.3 mg/dl at that moment. Corticoid treatment was supplied with improving of lung symptoms.

He was hospitalized because a deterioration of renal function with rise Crp to 3.4mg/dl, was detected in a routine analysis. The patient only report malaise without urinary symptoms since one month before. No fever, thoracic, lumbar pain or dermatologic alteration was detected. The BT was 120/80. He denied intake any antibiotic or anti-inflammatory treatment.

Hemogramme: erythrocytes: 4.56,leucocyte:6.130, lymphocyte 21%, monocyte 11%, neutrophil 3.780, glucose 90mg, urea 977, total bilirubin 0.6 mg., uric acid 8 mg, calcium 11, P 3.9, Na 138, K 4.6 urea 80 mg, cholesterol 182mg, triglyceride 180 mg.

Urine analysis: sediment normal, no hematuria., no proteinuria. Density 1.010.
Image 1Image 2Image 3
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Case 4 - ecp24, Nephropathology
Presented by: Marion Rabant, Paris, France

We report the history of a 50 year-old Caucasian woman. At the age of 28 years, she presented with a brutal nephrotic syndrome due to membranous nephropathy (MN) on a single functioning kidney. This MN led to end stage renal disease, and peritoneal dialysis was started at the age of 41 years. Of note, she had a spontaneous abortion when she was 30, without any other pregnancy. After one year of dialysis, she underwent a first deceased-donor kidney transplantation, complicated by arterial thrombosis at day 1 leading to transplantectomy. Simultaneously, the presence of lupus anticoagulant and anti-b2GPI antibodies was discovered. We concluded to an antiphospholipid (AP) syndrome and started long term anticoagulation.

Eight years later, she underwent a second kidney transplantation from a 64-year-old deceased donor. Due to the presence of anti-HLA class I donor specific antibody (DSA), she had an immunosuppressive regimen based on thymoglobulin, steroids, MMF, tacrolimus, 5 plasmapheresis and IVIG (x4).

After transplantation, the serum creatinine slowly decreased to 120 umol/L at month-3 with a measured glomerular filtration rate (mGFR) of 40 ml/min. A first biopsy, performed at day 10 because of delayed graft function, showed only acute tubular necrosis. The 3-month protocol biopsy revealed chronic scarring (IF/TA grade II) with severe vascular lesions (cv2, ah2). At one-year, serum creatinine and mGFR remained stable and protocol biopsy showed severe interstitial fibrosis (IF/TA grade III), chronic vascular changes (cv2, ah2), isolated glomerulitis with negative C4d and one anti-HLA class I DSA (score 4/8). She was treated for humoral rejection with steroids, plasmapheresis, rituximab and IVIG (x4). However, she rapidly presented with nephrotic range proteinuria and rapid decline of renal function. A fourth biopsy showed typical features of AP-associated nephropathy including severe fibrous intimal hyperplasia, thrombotic microangiopathy (TMA) and ischemic parenchyma. We did not observe any recurrence of MN and proteinuria was attributed to TMA. Concomitantly, she presented with myocardial infarction leading to suspect an atypical form of catastrophic AP syndrome. In this setting, high rate plasmapheresis and high dose steroids were started. The serum creatinine is now 230umol/l and proteinuria is still nephrotic.
Diapositive1Diapositive2Diapositive3Diapositive4Diapositive5Diapositive6Diapositive7
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Case 5 - ecp24, Nephropathology
Presented by: Helmut Hopfer, Basel, Switzerland

A 37 year old woman with end-stage renal failure of unknown origin received a kidney tansplant from a 7 year old deceased donor. Because of 2 donor-specific antibodies an induction therapy with ATG-Fresenius was performed. Maintenance immunosuppression consisted of tacrolimus, mycophenolate mofetil and steroids. The transplant had an immediate good function. An acute humoral rejecetion was diagnosed and treated 3 weeks after transplantation and a stable transplant function was achieved. BK-viruria was noted by week 8 and BK-viremia persisted above 10.000 c/ml. Immunosuppression was reduced. At 3 months the BK-viral load was 5.100.000 c/ml and a protocol biopsy confirmed the diagnosis of polyomavirus nephropathy. Immunosuppression was further reduced. BK-viremia remained high for the next 8 weeks, but then started to decline to 40.000 c/ml. At the same time serum creatinine had slowly risen from 140 to 180 umol/l. A second protocol biopsy was performed (6 months after transplantation).
Slide 1
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Case 6 - ecp24, Nephropathology
Presented by: Isabelle Brochériou, Paris, France

A 58 year-old male patient presented with hematuria, acute renal failure, weight loss and fatigue, soon followed by oliguria and anuria. Plasma creatinine level was 1600µmol/L, and was normal 3 months earlier. Urinalysis could not be performed because of anuria. Abdominal computerized tomography showed a spontaneous hyperdensity of the right renal pelvis and a bilateral ureteral thickening suggesting recent bleeding. Serum electrophoresis and immunofixation revealed a monoclonal IgDl (4.7g/L) and a free l LC.

A kidney biopsy showed acute tubular necrosis with intra-luminal obstruction by crystals, looking either like single parallelepipeds or flower-like clusters.
Masson TrichromeMasson Trichrome 3Masson Trichrome 2May Grunwald Giemsa Gentian purple Fluorescence anti-lambdaElectron micrograph
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Case 7 - ecp24, Nephropathology
Presented by: Bela Ivanyi, Szeged, Hungary

“Tumorous nodules” in the native kidney in a woman transplanted 3 years earlier because of “chronic pyelonephritis”-induced renal failure.

In May 2007, a 54-year-old woman with chronic renal failure due to “bilateral chronic pyelonephritis” received an allograft from a deceased donor. Between 2007 and 2010, we examined biopsy samples of the allograft on 3 occasions, with the following results: September 2007 (protocol biopsy): no alteration; May 2008 (protocol biopsy): coinciding tacrolimus toxicity and acute T-cell-mediated tubulointerstitial rejection; and July 2010 (biopsy for cause; serum creatinine 221 µmol/L): C4d-positive chronic antibody-mediated rejection.

In October 2010, with the clinical diagnosis of tumour at the pyeloureteral border, the right native kidney of the patient was removed and submitted to our department for evaluation. Grossly, the right kidney was markedly shrunken, and several tumorous nodules were observed in the pelvis, calyces and parenchyma. The largest nodule, measuring 70x70x30 mm and situated at the pyeloureteral border, bulged into the pelvis and infiltrated the renal sinus. The pelvis and calyces were dilated; the papillae, medulla and cortex were atrophied. The tips of the papillae were pale and displayed calcified foci. The changes indicated end-stage kidney, multifocal tumourous involvement of the pelvis and the right kidney, and tumour-induced pyelectasis.

A representative right kidney slide, stained with haematoxylin and eosin, is submitted for examination by the participants of the slide seminar. What is your diagnosis?
Slide 1
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Case 8 - ecp24, Nephropathology
Presented by: Agnieszka Perkowska, Warsaw, Poland

After one year of dialysis treatment a 55 year old male with end-stage native kidneys insufficiency of unknown reason, received a renal transplant from 3 HLA mismatched, cadaveric, 57 years old female donor, who died due to intracranial hemorrhage. The donor and recipient were negative for HIV, HCV, and HBV infection. Apart from mild anemia, no changes in the recipient´s peripheral blood morphology were found at the time of transplantation. The initial immunosuppression consisted of CsA, MMF and Pred in typical doses. Three weeks after Tx patient was still oliguric, and dialysis-dependant. There was no erythrocyturia, the urine protein content was 25 mg/dl. On the day 21 a graft biopsy was performed.
slide 1 (HE)slide 2 (HE)slide 3 (HE)
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Case 1 - ecp23, Nephropathology
Presented by: Niels Marcussen, Odense, Denmark

77-year-old male was in January 2010 operated for colon adenocarcinoma. For many years he has been treated for hypertension with Beta-blockers, diuretics and alfa-blockers. In April 2010 he underwent colonoscopy which did not show any signs of recurrent disease. Prior to the colonoscopy, he was orally given sodium phasphate (OSP) bowel purgative. Two weeks later he is again admitted to hospital due to intestinal bleeding. At the time of admission his s-creatinine is 700 mmol/l and after rehydration 450 mmol/l. Proteinuria 1,1 g/d. No hyperphosphatemia og hypercalcaemia. A renal biopsy was done in May 2010.
Slide 1von Kossa 2MT
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Case 10 - ecp23, Nephropathology
Presented by: Lorraine Racusen, Baltimore, MD, United States

The patient is a 52 y/o WF with H/O obesity, HTN, and hypothyroidism (Hashimoto's thyroiditis), S/P appendectomy, tubal ligation, and laporoscopic cholecystectomy.  She was diagnosed with CNS sarcoid in 2004 with pulmonary (restictive lung disease) and renal involvement, resulting in stage IV CKD.  She underwent a pre-emptive compatible live donor transplant (husband donated in a paired donation program) in June 2009.  Post-transplant, creatinine improved to 1.2 mg/dl.  She had early urinary retention and UTI's, but creatinine remained stable.  In August 2010 she presented with a large incisional hernia and a left adnexal cyst c/w simple ovarian cyst.  She was admitted in January 2011 for hernia repair with mesh placement.  At the time, she was on Tacrolimus, MMF, prednisone, Ezetimibe, Clonazepam, Metoprolol, levothyroxine and Pepcid.  An allograft biopsy was performed intra-operatively, though creatinine was stable at 1.2 mg/dl.
Slide 1
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