Immune-complex deposits in "pauci-immune" glomerulonephritis: a case report and brief review of recent literature.

Anti-neutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis is considered a "pauci-immune" disease, characterized by absent or mild glomerular tuft staining for immunoglobulin and/or complement. We describe a 72-year-old man with progressive renal failure over five months who was found to have P-ANCA associated crescentic glomerulonephritis. Renal biopsy also revealed immunofluorescence staining for Immunoglobulin G and C3. Treatment comprised corticosteroids, cyclophosphamide, and plasmapheresis but unfortunately kidney function did not recover, likely due to substantial interstitial fibrosis at diagnosis. This case illustrates that serologic evaluation for ANCAs should not be discounted when immune deposits are present. Prompt diagnosis is warranted.

Formation of downhill esophageal varices as a rare but serious complication of hemodialysis access: a case report and comprehensive literature review.

BACKGROUND: Proximal or "downhill" esophageal varices are a rare complication of superior vena caval (SVC) obstruction. Few reports describe downhill varices in dialysis patients with catheter-related SVC occlusion. METHODS: We studied a case of downhill esophageal varices in a dialysis patient from our center and reviewed the published literature on presentation, evaluation and treatment in other dialysis patients (MEDLINE database search). RESULTS: Including our current case, we identified eight reports of dialysis patients with downhill varices. All cases were recognized after presentation with gastrointestinal bleeding, in contrast to low reported bleeding rates of downhill varices in non-dialysis patients. Localized edema and superficial venous engorgement (signs of SVC occlusion) were each observed in four of eight patients. The duration of hemodialysis dependence ranged from 2.5 to 23 years, and dialysis access history included multiple central venous catheters when described (seven cases). Central venous imaging by direct, magnetic resonance or computerized tomographic venography documented SVC stenosis in all cases. Management included percutaneous transluminal angioplasty of the SVC with or without stenting in five of eight patients, three of whom developed restenosis during observation. Successful surgical venous bypass was performed in one patient after failed percutaneous venoplasty. Varices were treated with band ligation in four of eight cases without reported complications. CONCLUSIONS: Although rare, downhill esophageal varices should be considered in the differential diagnosis of upper gastrointestinal hemorrhage in dialysis patients exposed to central venous catheters. Diagnosis should prompt radiographic evaluation of SVC patency. Treatment requires timely and coordinated care by specialists in endovascular interventions and gastrointestinal endoscopy.