Light chain disease
Synonym(s)
MIDD; Monoclonal immunoglobulin deposition disease
DefinitionThis section has been translated automatically.
Kidney disease associated with a non-fibrillary deposition of monoclonal immunoglobulin fragments (light chains).
Occurrence/EpidemiologyThis section has been translated automatically.
2/3 of light chain diseases are caused by multiple myeloma.
EtiopathogenesisThis section has been translated automatically.
In multiple myeloma, the light chains accumulate in the lysosomes and cannot be degraded by proteases. The resulting atrophy of the proximal tubule cells is one of the decisive factors for renal insufficiency.
ManifestationThis section has been translated automatically.
Mean age of onset of light chain disease: 55 years
Clinical featuresThis section has been translated automatically.
The light chain disease can show one or more of the following symptoms:
- renal insufficiency (90%)
- Rapid loss of renal function (70%)
- proteinuria (84%)
- Nephrotic syndrome(40%)
- Microhaematuria (30%)
- Heart and liver involvement (30%)
Complication(s)This section has been translated automatically.
The light chain disease can lead to severe renal failure.
LiteratureThis section has been translated automatically.
- Hirashio S et al, (2018) A case of immunotactoid glomerulopathy with false-negative IgG staining. BMC Nephrol 19:143.
- Karasawa K et al, (2018) Successful treatment with rituximab of immunotactoid glomerulopathy exhibiting nephrotic syndrome. Clin Nephrol 90:222-226.
- Motwani SS et al (2016) Paraprotein-Related Kidney Disease: Glomerular Diseases Associated with Paraproteinemias. Clin J Am Soc Nephrol 11:2260-2272.
- Nasr SH et al (2017) DNAJB9 Is a Specific Immunohistochemical Marker for Fibrillary Glomerulonephritis. Kidney Int Rep 3:56-64.
- Sehgal R et al (2017) Fibrillary Glomerulonephritis in Primary Sjogren's Syndrome: A Rare Cause of Renal Failure. Clin Med Res 15(3-4):100-105.