Paraprotein-related renal disease and amyloid

Paraprotein-related renal diseases present as renal impairment and proteinuria in mainly older patients. The underlying haematological conditions are often subtle and may only be detected after the diagnosis is made at renal biopsy. The major renal diseases caused by deposition of aggregated monoclonal immunoglobulins are: myeloma cast nephropathy, AL amyloidosis and light chain deposition disease. In all these diseases, treatment is based on cytotoxic regimens aimed at preventing further paraprotein or monoclonal free light chain production combined with general supportive care. Although haematological remission can be accompanied by long-term survival and renal improvement, in many cases the prognosis is poor.