Unlike PGNMID-IgG, the light chain variant (PGNMID-LC) showed only monoclonal light chain deposits. All 17 patients had either underlying monoclonal gammopathy of renal significance (MGRS) or multiple myeloma. This was in contrast to PGNMID-IgG in which more than two-thirds of patients did not have MGRS or B/plasma cell neoplasm. All 6 patients who had anti-plasma cell therapy with complete hematologic response had renal response.
Although PGNMIC-LC is considered a variant of PGNMID-IgG, its hematological underlying is more like light chain deposition disease (LCDD).
PGNMID-LC show large subendothelial deposits by LM resembling wire loop in lupus nephritis. PGNMID-IgG usually has small subendothelial deposits best demonstrated by EM (Image from Nasr et al.) |