Immunoglobulin deposition diseases

Immunoglobulin deposition diseases are rare and unusual plasma cell dyscrasias. A tissue biopsy is required for diagnosis. Since the kidney is involved in most cases, a kidney biopsy usually provides the diagnosis. It is important to distinguish these disorders as their clinical presentation often mimics multiple myeloma and amyloidosis. The degree of renal involvement at presentation may vary from mild renal impairment to severe renal dysfunction requiring renal replacement therapy. The underlying lymphoproliferative or clonal plasma cell disorder usually has a low tumor burden compared to conditions such as multiple myeloma or Waldenstrom’s macroglobulinemia. The identification of the underlying hematological disorder guides therapeutic intervention. There has been significant improvement in the outcome of these patients, since the availability of novel agents such as immunomodulatory drugs, proteasome inhibitors, and with autologous stem cell transplantation. In this review, we address clinical presentation and outcomes with light-chain and heavy-chain monoclonal immunoglobulin deposition disease, Type I cryoglobulinemia, proliferative glomerulonephritis with monoclonal IgG deposits, C3 monoclonal-associated glomerulonephritis, immunotactoid glomerulonephropathy, Fanconi syndrome, and light-chain crystallopathy.