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Immunotactoid or fibrillary glomerulopathy

ORPHA91137
Synonym(s) Immunotactoid or fibrillary glomerulonephritis
Prevalence <1 / 1 000 000
Inheritance Not applicable
Age of onset Adult
ICD-10
  • N03.6
OMIM -
UMLS -
MeSH -
MedDRA -

Summary

Immunotactoid glomerulopathy (ITG, see this term) and fibrillary glomerulopathy (FGP, see this term) are very rare glomerular diseases characterized by monoclonal microtubular and polyclonal fibrillar deposits respectively in the mesangium. They both present clinically with nephrotic range proteinuria, hematuria and renal insufficiency leading to renal failure in many cases. ITG is more likely to manifest with underlying lymphoproliferative disease, hypocomplementemia, dysproteinemia, monoclonal gammopathy or occult cryoglobulinemia. FGP is 10 fold more frequent than ITG.

Expert reviewer(s)

  • Dr Laure Hélène NOEL

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