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LeitthemaANCA (antineutrophile zytoplasmatische Antikörper)-assoziierte Vaskulitiden sind die häufigsten primären systemischen Vaskulitiden der kleinen Gefäße des Erwachsenen. Aufgrund ihrer teilweise gravierenden Folgen mit der Gefahr von irreversiblen Organschäden kommt einer frü-hen Diagnosestellung und Behandlung entscheidende Bedeutung zu. ANCA können dabei als diagnostische Marker dienen, wobei die Diagnose möglichst immer histologisch gesichert werden sollte. Die Rolle von ANCA als Verlaufsparameter dagegen wird kontrovers diskutiert. Heutzutage haben die stadienadaptierten Therapieformen Induktions-oder Erhaltungstherapie das mit einer hohen Morbiditäts-und Mortalitätsrate behaftete Fauci-Schema verdrängt.
LeitthemaANCA (antineutrophile zytoplasmatische Antikörper)-assoziierte Vaskulitiden sind die häufigsten primären systemischen Vaskulitiden der kleinen Gefäße des Erwachsenen. Aufgrund ihrer teilweise gravierenden Folgen mit der Gefahr von irreversiblen Organschäden kommt einer frü-hen Diagnosestellung und Behandlung entscheidende Bedeutung zu. ANCA können dabei als diagnostische Marker dienen, wobei die Diagnose möglichst immer histologisch gesichert werden sollte. Die Rolle von ANCA als Verlaufsparameter dagegen wird kontrovers diskutiert. Heutzutage haben die stadienadaptierten Therapieformen Induktions-oder Erhaltungstherapie das mit einer hohen Morbiditäts-und Mortalitätsrate behaftete Fauci-Schema verdrängt.
A 26-year-old female with systemic lupus erythematosus was admitted because of dyspnea and progressive lower extremity edema. Laboratory testing showed blood urea nitrogen levels of 147 mg/dL, creatinine of 6.7 mg/dL, serum albumin of 1.7 g/dL and the daily protein loss was 12.7 g. Her C3 level was 60.4mg/dL and C4 level was 10.2 mg/dL. The antinuclear antibody titer was 1:320, with a homogeneous pattern, but she was negative for anti-dsDNA. ELISA testing for anti-PR3 antibodies and anti-MPO antibodies were all negative. She was also negative for circulating lupus anticoagulant. Renal biopsy revealed diffuse proliferation of glomerular cells, but immunofluorescent microscopy showed no immune deposits and electron microscopy revealed only scanty electron-dense deposits. She received 1 g/day of methylprednisolone intravenously for 3 days, followed by 60 mg/day of prednisolone. She was discharged with serum creatinine decreased to 4.7 mg/dL, and a great improvement in dyspnea. Diffuse proliferative lupus nephritis that contains little or no subendothelial deposits is rare. The differential diagnosis, possible mechanisms and treatment are discussed.
The kidneys are frequently targeted by pathogenic immune responses against renal autoantigens or by local manifestations of systemic autoimmunity. Recent studies in rodent models and humans have uncovered several underlying mechanisms that can be used to explain the previously enigmatic immunopathology of many kidney diseases. These mechanisms include kidney-specific damage-associated molecular patterns that cause sterile inflammation, the crosstalk between renal dendritic cells and T cells, the development of kidney-targeting autoantibodies and molecular mimicry with microbial pathogens. Conversely, kidney failure affects general immunity, causing intestinal barrier dysfunction, systemic inflammation and immunodeficiency that contribute to the morbidity and mortality of patients with kidney disease. In this Review, we summarize the recent findings regarding the interactions between the kidneys and the immune system.
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