Muneharu Yamada, Masataka Nagashima, Ken Aoki, Takahiko Hoshino, Rie Fujii, Takahiro Uchida, Yutaka Yamaguchi, Takashi Oda
Immunotactoid glomerulopathy (ITG) is diagnosed by identifying organized microtubules on electron microscopy and it can therefore be missed when they are not demonstrated. A 48-year-old woman with hematuria, nephrotic-range proteinuria, and a faint IgG-κ monoclonal protein underwent an initial kidney biopsy, which showed endocapillary proliferative glomerulonephritis with membranoproliferative features and scant subendothelial deposits, but no diagnostic microtubules. With worsening renal dysfunction and a limited corticosteroid response, a repeat biopsy revealed IgG3-κ-restricted deposits and non-branching, hollow-core microtubules, confirming ITG associated with monoclonal gammopathy of renal significance. Rituximab-based B cell-directed therapy was followed by sustained remission with prednisolone tapered to 2.5 mg every other day.