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Case of immunotactoid glomerulopathy showing high responsiveness to steroids therapy despite severe pathological features

A 72-year-old woman presented with nephrotic proteinuria and moderate haematuria. Renal pathology was compatible with immunotactoid glomerulopathy (ITG), for which there is no consensus for appropriate therapy. We, therefore, postponed immunosuppressive therapy. After 4 years, the patient’s renal fu...

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Detalles Bibliográficos
Autores principales: Ohashi, Atsuki, Kumagai, Jiro, Nagahama, Kiyotaka, Fujisawa, Hajime
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BMJ Publishing Group 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6663160/
https://www.ncbi.nlm.nih.gov/pubmed/31350228
http://dx.doi.org/10.1136/bcr-2019-229751
Descripción
Sumario:A 72-year-old woman presented with nephrotic proteinuria and moderate haematuria. Renal pathology was compatible with immunotactoid glomerulopathy (ITG), for which there is no consensus for appropriate therapy. We, therefore, postponed immunosuppressive therapy. After 4 years, the patient’s renal function started to decline and renal pathology was re-evaluated, revealing a pathological change from mesangial proliferative glomerulonephritis to endocapillary proliferative glomerulonephritis. Treatment with oral prednisolone (30 mg/day) was initiated. Within 5 weeks, complete remission of proteinuria was obtained (proteinuria 6.02 g/gCr to 0.12 g/gCr), and the patient’s renal function stabilised. Generally, responsiveness to immunosuppressive therapy is poor in patients with ITG, and the present case represented a very rare clinical course. Some previous cases have indicated susceptibility to the therapy, regardless of the severity of renal damage. As a possible distinct entity that determines susceptibility to immunosuppressive therapy, we suggest the presence of a latent lymphoproliferative disease with no significant haematological symptoms.