Paraproteinemic kidney diseases
Synonym(s)
DefinitionThis section has been translated automatically.
Group of different, chronic kidney diseases (glomerular and/or interstitial nephropathies) associated with deposits of intact or fragmentary immunoglobulins (heavy and light chains), e.g. in multiple myeloma, plasmocytoma or Waldenstrom's disease.
ClassificationThis section has been translated automatically.
Paraproteinemic kidney diseases include (Kanzaki G et al. 2019):
- Monoclonal immunoglobulin depositiondisease (MIDD): At the time of diagnosis most patients already have renal failure.
- Nephropathy in AL or AH amyloidosis: 50% of patients develop nephropathy)
- Glomerulonephritis associated with type I cryoglobulinemia (membranoproliferative glomerulonephritis)
- Myeloma kidney (cast nephropathy; proliferative glomerulonephritis with monoclonal IgG deposits; PGNMID)
- Immunotactoid glomerulopathy and fibrillary glomerulonephritis: 1% of biopsied patients.
- As well as very rare:
- Monoclonal crystalline glomerulopathy
- Paraprotein-related thrombotic microangiopathy
- Membranous-like glomerulopathy with masked IgGκ deposits
EtiologyThis section has been translated automatically.
Of clinical relevance for nephrotoxic monoclonal proteins is that their nephrotoxicity is independent of whether they are generated by a malignant or non-malignant cell clone. In this respect, the non-prejudicial term: "monoclonal gammopathy of renal significance" has become established (see also Monoclonal gammopathy of indeterminate significance = MGUS). Based on these findings, therapeutic approaches with "clone-directed strategies" such as rituximab (directed against CD20-expressing B cells) and bortezomib (directed against plasma cell clones, see below proteasome inhibitors) have become established. These clone-adapted therapeutic approaches are also more effective in "nonmonoclonal protein-related kidney diseases" than the unspecific immunosuppressive therapeutic approaches (Leung N et al. (2018)
LiteratureThis section has been translated automatically.
- Huang X et al (2016) Bortezomib with dexamethasone as first-line treatment for AL amyloidosis withrenal involvement. Amyloid 23:51-57.
- Leung N et al (2018) Dysproteinemias and Glomerular Disease. Clin J Am Soc Nephrol 13:128-139.
- Luan H et al (2019) Cast nephropathy associated with monoclonal immunoglobulin M-secreting mucosa-associated lymphoid tissue B-cell lymphoma .Clin Nephrol doi: 10.5414/CN109682.
- Sallée M et al (2019) Myeloma cast nephropathy: the dusk of high cutoff haemodialysis. Lancet Haematol 6:e174-e176.