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Clinicopathologic Features of Antibrush Border Antibody Disease.
INTRODUCTION: Antibrush border antibody disease (ABBA) is an autoimmune tubulointerstitial kidney disease that primarily affects older individuals and results in progressive kidney failure. It is rare with only 20 reported cases. Here, we describe a case series to further define the clinicopathologic spectrum and natural history, and to inform management. METHODS: We identified 67 patients with ABBA who underwent kidney biopsy, including 65 native and 2 transplants. Demographics, clinical findings, and laboratory data were obtained. Histopathologic data included light microscopy, immunofluorescence, electron microscopy and immunostaining for LRP2, CUBN, and AMN. Follow-up data, including treatment(s), laboratory values, and outcomes, were available from 51 patients. RESULTS: Patients with ABBA were predominantly male with a median age of 72 years. Median serum creatinine was 2.7 mg/dl, proteinuria was 2.8 g/day, and hematuria was present in two-thirds of the patients. Tubular injury with LRP2-positive tubular basement membrane (TBM) deposits were seen in 94.2% of patients. Thirty-eight patients (56.7%) had a second kidney disease, commonly glomerular diseases with high-grade proteinuria. These diseases included podocytopathies, membranous nephropathy (MN), IgA nephropathy, diabetic glomerulopathy, lupus nephritis (LN), crescentic glomerulonephritis (GN), tubulointerstitial nephritis, and involvement by lymphoma. The majority of patients were treated with immunosuppression. Of those patients with follow-up, 29.4% achieved remission, 70.6% had no response, and 52.8% required dialysis or were deceased. Untreated patients were at the highest risk. CONCLUSION: ABBA is a rare autoimmune kidney disease that often occurs with other kidney diseases. Although the overall prognosis of ABBA is poor, there is potential benefit from immunosuppression
The Simons Observatory: Design, integration, and testing of the small aperture telescopes
International audienceThe Simons Observatory (SO) is a cosmic microwave background (CMB) survey experiment that includes small-aperture telescopes (SATs) observing from an altitude of 5,200 m in the Atacama Desert in Chile. The SO SATs will cover six spectral bands between 27 and 280 GHz to search for primordial B-modes to a sensitivity of , with quantified systematic errors well below this value. Each SAT is a self-contained cryogenic telescope with a 35 field of view, 42 cm diameter optical aperture, 40 K half-wave plate, 1 K refractive optics, and TES detectors. We describe the nominal design of the SATs and present details about the integration and testing for one operating at 93 and 145 GHz
The Simons Observatory: Design, integration, and testing of the small aperture telescopes
International audienceThe Simons Observatory (SO) is a cosmic microwave background (CMB) survey experiment that includes small-aperture telescopes (SATs) observing from an altitude of 5,200 m in the Atacama Desert in Chile. The SO SATs will cover six spectral bands between 27 and 280 GHz to search for primordial B-modes to a sensitivity of , with quantified systematic errors well below this value. Each SAT is a self-contained cryogenic telescope with a 35 field of view, 42 cm diameter optical aperture, 40 K half-wave plate, 1 K refractive optics, and TES detectors. We describe the nominal design of the SATs and present details about the integration and testing for one operating at 93 and 145 GHz
The Simons Observatory: Design, integration, and testing of the small aperture telescopes
International audienceThe Simons Observatory (SO) is a cosmic microwave background (CMB) survey experiment that includes small-aperture telescopes (SATs) observing from an altitude of 5,200 m in the Atacama Desert in Chile. The SO SATs will cover six spectral bands between 27 and 280 GHz to search for primordial B-modes to a sensitivity of , with quantified systematic errors well below this value. Each SAT is a self-contained cryogenic telescope with a 35 field of view, 42 cm diameter optical aperture, 40 K half-wave plate, 1 K refractive optics, and TES detectors. We describe the nominal design of the SATs and present details about the integration and testing for one operating at 93 and 145 GHz