Abstract
Non-amyloid glomerulopathies characterized by Congo-red negative organized immunoglobulin (Ig) deposits display two characteristic patterns by electron microscopy (EM); either randomly arranged (pseudo-amyloid) fiibrils or microtubules usually larger and ordered in parallel arrays 1–23. It is currently unclear whether this morphological distinction is relevant. Certain investigators consider that such a distinction is of no clinical or pathophysiological basis, and propose that fiibrillar and microtubular Ig deposits are merely variants of the same glomerulopathy, referred to as fiibrillary-immunotactoid GN3,1 5,1 g,2o They also advocate that this diagnosis should be limited to patients with primary renal disorders, thus excluding patients with monoclonal gammopathy, systemic diseases or lymphoproliferative disorders3,7. Others suggest that distinguishing immunotactoid (IT) GN and FGN is critical 10,12–14,19 In immunotactoid GN, defiined by the presence of orderly arranged microtubules usually more than 30 nm in width, glomerular Ig are often monotypic. Immunotactoid GN may be associated with monoclonal gammopathy and/or hematological malignancy with improved renal prognosis after chemotherapy6,10,13,14 On the contrary, in FGN the diameter of fiibrils is less than 30 nm and these patients present without underlying hematological disease5,8,12,13,22,23 Moreover, a predominance of polyclonal IgG4 in glomerular deposits has been reported in FGN 12,16,23, but not in immunotactoid GN3,6,14.
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Bridoux, F. et al. (2003). Glomerulonephritis with organized immunoglobulin deposits: fibrillar and microtubular deposits are associated with distinct immunological features. In: Touchard, G., Aucouturier, P., Hermine, O., Ronco, P. (eds) Monoclonal Gammopathies and the Kidney. Springer, Dordrecht. https://doi.org/10.1007/978-94-017-0191-4_15
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DOI: https://doi.org/10.1007/978-94-017-0191-4_15
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