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A large-sized bubbling appearance of the glomerular basement membrane in a patient with pulmonary limited AL amyloidosis and a past history of lupus nephritis

Abstract

We report an unusual pathological finding, a large-sized bubbling appearance of the glomerular basement membrane (GBM), in a patient with pulmonary limited AL amyloidosis and a past history of lupus nephritis. The first renal biopsy specimen from 10 years ago, when systemic lupus erythematosus was diagnosed, demonstrated mild mesangial proliferation and subepithelial deposits (WHO classification: III + V). Light microscopy of the current biopsy using periodic acid methenamine silver (PAMS) stain demonstrated a large-sized bubbling appearance of the GBM; however, very weak immunoglobulin and complement deposition was observed in immunofluorescence studies. Routine electron microscopy demonstrated partial subendothelial expansion with electron-lucent materials, but no electron-dense deposits or amyloid fibrils. Electron microscopy with PAMS stain revealed electron-lucent endothelial scalloping, including some cellular components and microspheres in the GBM; however, it is not clear if these materials are derived from endothelial cells. One possibility is that these unique findings represent a recovery phase of lupus membranous nephritis; another is that these findings correspond to a new disease entity.

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Acknowledgments

The authors were supported by a grant from the Amyloidosis Research Committee of the Intractable Diseases Division in the Ministry of Health, Labour and Welfare of Japan. We thank Dr. M. Ueda and Dr. Y. Ando for their analysis of anti-λ antibody staining (Fig. 1c) and amyloid staining (Fig. 1d) in the pulmonary biopsy specimen.

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Correspondence to Hirokazu Imai.

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Suga, N., Miura, N., Uemura, Y. et al. A large-sized bubbling appearance of the glomerular basement membrane in a patient with pulmonary limited AL amyloidosis and a past history of lupus nephritis. Clin Exp Nephrol 15, 970–975 (2011). https://doi.org/10.1007/s10157-011-0528-8

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  • DOI: https://doi.org/10.1007/s10157-011-0528-8

Keywords

  • Large-sized bubbling appearance
  • Very weak immunoglobulin deposition
  • PAMS
  • Pulmonary limited AL amyloidosis
  • Lupus membranous nephritis