Anti-PLA2R Antibody Development During NELL1-Associated Membranous Glomerulonephritis Treatment: A Case Report.

A Japanese man in his early 70s was referred to our hospital because of massive proteinuria. Analysis of his kidney biopsy demonstrated glomerular subepithelial immune deposits containing immunoglobulin (Ig)G, which was dominant for the IgG1 subclass. Immunoperoxidase staining for neural epidermal growth factor-like 1 protein (NELL1) was positive on the glomerular capillary walls, whereas neither serum anti-phospholipase A2 receptor (PLA2R) antibodies nor immunofluorescence staining for PLA2R was positive. Detailed investigation revealed no associated conditions, including underlying malignancies, and thus he was diagnosed as having NELL1-associated idiopathic membranous nephropathy (MN). The patient was treated with steroids, which substantially improved his nephrotic syndrome. Interestingly, serum anti-NELL1 as well as anti-PLA2R antibodies became positive during his clinical course. Serology-based approaches are currently proposed for the treatment of patients suspected of having MN; however, an accurate diagnosis of the present patient would have been difficult if such an approach was performed only at a later phase of the disease. Several target antigens for the glomerular immune deposits observed in patients with MN have recently been identified, and dual positivity of antibodies to these antigens reportedly occurs in some patients. Further accumulation and analyses of such patients are needed to establish more appropriate diagnostic approaches for MN.

TAFRO Syndrome in a Kidney Transplant Recipient That Was Diagnosed on Autopsy: A Case Report.

A 57-year-old man who received a kidney transplant 4 years previously owing to unknown underlying disease presented with thrombocytopenia and fever. Hepatosplenomegaly and lymphadenopathy were observed, and development of prominent anasarca and worsening of renal function yielded the diagnosis of TAFRO syndrome. He was treated with high-dose steroids and plasmapheresis, and a thrombopoietin receptor agonist was administered for refractory thrombocytopenia. However, his general condition worsened, and he died on day 92. Histopathological analysis of a kidney autopsy specimen showed thrombotic microangiopathy characterized by glomerular endothelial swelling, mesangiolysis, and double contours of the glomerular capillary walls. His bone marrow showed megakaryocytic hyperplasia with mild reticulin fibrosis. Interestingly, these clinical and pathological features were remarkably similar to those the patient demonstrated before the kidney transplant, suggesting the recurrence of TAFRO syndrome. TAFRO syndrome is a rare systemic disorder whose concept has recently been established, but information on its long-term outcome is scarce. To our knowledge, this is the first case of TAFRO syndrome developing in a kidney transplant recipient, which suggests that disease recurrence occurs many years after the kidney transplant.

Complete remission of DnaJ homolog subfamily B member 9-positive fibrillary glomerulonephritis following steroid monotherapy in an elderly Japanese woman.

A 74-year-old Japanese woman was referred to our department because of anasarca and massive proteinuria. She was clinically diagnosed with nephrotic syndrome, and renal biopsy showed membranoproliferative glomerulonephritis accompanied by marked glomerular infiltration with macrophages and full-house immunofluorescence glomerular deposition. Furthermore, randomly arranged nonbranching fibrils, approximately 12 nm in diameter, were found by electron microscopy, and immunostaining for DnaJ homolog subfamily B member 9 (DNAJB9), a recently identified diagnostic biomarker of fibrillary glomerulonephritis (FGN), showed positive result, thereby confirming the diagnosis of FGN. Steroid treatment was initiated, and she obtained complete remission of nephrotic syndrome and has maintained it. FGN is an uncommon form of glomerular disease, and reported cases of DNAJB9-positive FGN among Asians, particularly among Japanese population, are rare. There have been no established therapeutic regimens and its renal prognosis is generally unfavorable. The present case suggests that some patients with FGN can achieve favorable clinical outcomes through steroid monotherapy.

Development of anti-glomerular basement membrane glomerulonephritis during the course of IgA nephropathy: a case report.

BACKGROUND: Anti-glomerular basement membrane (GBM) glomerulonephritis does not usually coexist with another glomerulonephritis such as IgA nephropathy. We present a rare case having a combination of these two diseases, and furthermore, histological evaluation could be performed before and after the development of anti-GBM glomerulonephritis over a period of only10 months. CASE PRESENTATION: A 66-year-old woman was admitted with complaints of microscopic hematuria and mild proteinuria for the past 3 years. Serum creatinine level was normal at that time. The first renal biopsy was performed. Light microscopy revealed mesangial proliferative glomerulonephritis with fibro-cellular crescents in one out of 18 glomeruli, excluding one global sclerotic glomerulus. Immunofluorescence (IF) showed IgA and C3 deposition in the mesangium. Therefore, the diagnosis was IgA nephropathy. Eight months later, the patient's serum creatinine suddenly rose to 4.53 mg/dL and urinalysis showed 100 red blood cells per high power field with nephrotic range proteinuria (12.3 g/gCr). The serological tests revealed the presence of anti-GBM antibody at the titer of 116 IU/mL. Treatments were begun after admission, consisting of hemodialysis, plasma exchange, and intravenous methylprednisolone pulse therapy. At 4 weeks after admission, the second renal biopsy was performed. Light microscopy revealed crescents in 18 of 25 glomeruli, excluding six global sclerotic glomeruli. IF showed linear IgG deposition along the GBM in addition to granular IgA and C3 deposition. Based on these findings, the diagnosis of anti-GBM glomerulonephritis and IgA nephropathy was confirmed. Renal function was not restored despite treatment, but alveolar hemorrhage was prevented. CONCLUSIONS: We report a patient with a diagnosis of anti-GBM disease during the course of IgA nephropathy. This case strongly suggests that the presence of autoantibodies should be checked to rule out overlapping autoimmune conditions even in patient who have previously been diagnosed with chronic glomerulonephritis, such as IgA nephropathy, who present an unusually rapid clinical course.

[A case report of cholangiectasis with cholangiocarcinoma].

This is a case report of cholangiectasis with cholangiocarcinoma in a 37-year-old female. Both computed tomography (CT) and endoscopic retrograde cholangio-pancreatography (ERCP) demonstrated gallstone, and intrahepatic bile duct dilatation with the stone. The diagnosis was intrahepatic cholangiectasis without common bile duct-dilatation. Hepatectomy of segment 3 with resection of the extrahepatic bile duct and reconstruction of the biliary tract was performed. Upon pathological examination, an early stage of cholangiocarcinoma was pointed out within the small range of common bile duct without dilatation or stone. Cholangiocarcinoma with cholangiectasis has been reported to be induced by counter flow of pancreas and intestinal juice because of the pancreaticobiliary maljunction. So we usually choose a diversion operation as our tactics to get rid of the counter flow of those digestive juices. Despite the above-mentioned tendency, this is a very rare case of carcinogenesis in common bile duct without dilatation.

Adaptive statistical iterative reconstruction for volume-rendered computed tomography portovenography: improvement of image quality.

PURPOSE: Adaptive statistical iterative reconstruction (ASIR) is a reconstruction technique for computed tomography (CT) that reduces image noise. The purpose of our study was to investigate whether ASIR improves the quality of volume-rendered (VR) CT portovenography. MATERIALS AND METHODS: Institutional review board approval, with waived consent, was obtained. A total of 19 patients (12 men, 7 women; mean age 69.0 years; range 25-82 years) suspected of having liver lesions underwent three-phase enhanced CT. VR image sets were prepared with both the conventional method and ASIR. The required time to make VR images was recorded. Two radiologists performed independent qualitative evaluations of the image sets. The Wilcoxon signed-rank test was used for statistical analysis. Contrast-noise ratios (CNRs) of the portal and hepatic vein were also evaluated. RESULTS: Overall image quality was significantly improved by ASIR (P < 0.0001 and P = 0.0155 for each radiologist). ASIR enhanced CNRs of the portal and hepatic vein significantly (P < 0.0001). The time required to create VR images was significantly shorter with ASIR (84.7 vs. 117.1 s; P = 0.014). CONCLUSION: ASIR enhances CNRs and improves image quality in VR CT portovenography. It also shortens the time required to create liver VR CT portovenographs.