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Lupusnephritis mit Kristallstrukturen in glomerulären Ablagerungen

Der Pathologe volume 24, pages 433–438 (2003)Cite this article

Zusammenfassung

Die Lupusnephritis ist ein bekanntes Phänomen beim systemischen Lupus erythematodes (SLE). Wir untersuchten die Nierenbiopsie einer 30-jährigen Frau mit SLE. Die Anamnese zeigte einen klinisch typischen SLE mit generalisierten Symptomen mit positivem ANA- und Anti-ds-DNA-Titer ohne Nachweis von Lupuskoagulans, des Rheumafaktors, von Kryoglobulinen sowie ohne Antiphospholipid-Antikörper. Eine Paraproteinämie war weder für IgG noch für IgA oder IgM nachzuweisen. Mit Hilfe der Lichtmikroskopie, der Elektronenmikroskopie sowie der Immunelektronenmikroskopie wurden elektronendichte Ablagerungen in subepithelialer, subendothelialer und mesangialer Lokalisation nachgewiesen. Zusätzlich fanden wir sowohl in den elektronendichten Ablagerungen, als auch im angrenzenden Mesangium Kristalle eines elektronendichten Materials. Diese kristallinen Strukturen sind aus IgG und κ-Leichtketten zusammengesetzt, während IgM, IgA sowie λ-Leichtketten mittels Immunelektronenmikroskopie nicht nachzuweisen waren.

Unseres Wissens stellen wir den ersten Fall einer Patientin mit SLE und Lupusnephritis vor, bei der kristalline Strukturen innerhalb der elektronendichten Ablagerungen in verschiedenen glomerulären Kompartimenten nachzuweisen waren.

Abstract

Lupus nephritis is a common phenomenon in Systemic Lupus Erythematosus (SLE). We analyzed a renal biopsy of a 30-year-old woman with SLE. The clinical history showed a typical SLE with generalized symptoms without demonstrable lupus coagulant, positive for anti-nuclear antibodies and anti-ds-DNA antibodies but negative for rheumatoid factor, cryoglobulins and antiphospholipid antibodies. A paraproteinemia for IgA, IgG and IgM was not detectable. Using light, electron and immunoelectron microscopy electron-dense deposits were noted in subepithelial, subendothelial and mesangial position. Most remarkably, the electron-dense deposits and mesangial areas in the vicinity of deposits contained an electron-dense crystalline material. The crystalline structures were composed of IgG and kappa light chains, while they were negative for IgM, IgA and lambda light chains, as demonstrated by immunoelectron microscopy.

As far as we know, this is the first case of lupus nephritis with crystalline structures. Since we could not detect cryoglobulinemia or paraproteinemia, other mechanisms possibly favor organization of macromolecular structures.

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Author information

Affiliations

  1. Gemeinschaftspraxis für Pathologie, Trier

    M. Otto, S. Bertz & J. Kriegsmann

  2. Institut für Pathologie, Universität Mainz

    T. Hansen

  3. Institut für Pathologie, Ulleval Hospital, Oslo, Norwegen

    E. H. Strøm & S. H. Brorson

  4. Institut für Pathologie, Kantonsspital, Basel, Schweiz

    M. J. Mihatsch

  5. Gemeinschaftspraxis für Pathologie, Moltkestraße 32, 54292, Trier

    M. Otto

Authors
  1. M. Otto
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  2. T. Hansen
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  3. S. Bertz
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  4. E. H. Strøm
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  5. S. H. Brorson
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  6. M. J. Mihatsch
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  7. J. Kriegsmann
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Correspondence to M. Otto.

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Otto, M., Hansen, T., Bertz, S. et al. Lupusnephritis mit Kristallstrukturen in glomerulären Ablagerungen. Pathologe 24, 433–438 (2003). https://doi.org/10.1007/s00292-003-0658-1

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Schlüsselwörter

  • Lupusnephritis
  • Systemischer Lupus erythematodes
  • Kristalline Strukturen
  • Elektronendichte Ablagerungen
  • Immunglobuline

Keywords

  • Lupus nephritis
  • Systemic lupus erythematodes
  • Crystal structures
  • Electron-dense deposits
  • Immuoglobulins