Complete remission of DnaJ homolog subfamily B member 9-positive fibrillary glomerulonephritis following steroid monotherapy in an elderly Japanese woman. 2021

Takahiro Uchida, and Shuuhei Komatsu, and Takashi Sakai, and Aki Kojima, and Sachiko Iwama, and Kentaro Sugisaki, and Takashi Oda
Department of Nephrology and Blood Purification, Kidney Disease Center, Tokyo Medical University Hachioji Medical Center, 1163 Tatemachi, Hachioji, Tokyo, 193-0998, Japan.

A 74-year-old Japanese woman was referred to our department because of anasarca and massive proteinuria. She was clinically diagnosed with nephrotic syndrome, and renal biopsy showed membranoproliferative glomerulonephritis accompanied by marked glomerular infiltration with macrophages and full-house immunofluorescence glomerular deposition. Furthermore, randomly arranged nonbranching fibrils, approximately 12 nm in diameter, were found by electron microscopy, and immunostaining for DnaJ homolog subfamily B member 9 (DNAJB9), a recently identified diagnostic biomarker of fibrillary glomerulonephritis (FGN), showed positive result, thereby confirming the diagnosis of FGN. Steroid treatment was initiated, and she obtained complete remission of nephrotic syndrome and has maintained it. FGN is an uncommon form of glomerular disease, and reported cases of DNAJB9-positive FGN among Asians, particularly among Japanese population, are rare. There have been no established therapeutic regimens and its renal prognosis is generally unfavorable. The present case suggests that some patients with FGN can achieve favorable clinical outcomes through steroid monotherapy.

UI MeSH Term Description Entries
D007564 Japan A country in eastern Asia, island chain between the North Pacific Ocean and the Sea of Japan, east of the Korean Peninsula. The capital is Tokyo. Bonin Islands
D008565 Membrane Proteins Proteins which are found in membranes including cellular and intracellular membranes. They consist of two types, peripheral and integral proteins. They include most membrane-associated enzymes, antigenic proteins, transport proteins, and drug, hormone, and lectin receptors. Cell Membrane Protein,Cell Membrane Proteins,Cell Surface Protein,Cell Surface Proteins,Integral Membrane Proteins,Membrane-Associated Protein,Surface Protein,Surface Proteins,Integral Membrane Protein,Membrane Protein,Membrane-Associated Proteins,Membrane Associated Protein,Membrane Associated Proteins,Membrane Protein, Cell,Membrane Protein, Integral,Membrane Proteins, Integral,Protein, Cell Membrane,Protein, Cell Surface,Protein, Integral Membrane,Protein, Membrane,Protein, Membrane-Associated,Protein, Surface,Proteins, Cell Membrane,Proteins, Cell Surface,Proteins, Integral Membrane,Proteins, Membrane,Proteins, Membrane-Associated,Proteins, Surface,Surface Protein, Cell
D005260 Female Females
D005921 Glomerulonephritis Inflammation of the renal glomeruli (KIDNEY GLOMERULUS) that can be classified by the type of glomerular injuries including antibody deposition, complement activation, cellular proliferation, and glomerulosclerosis. These structural and functional abnormalities usually lead to HEMATURIA; PROTEINURIA; HYPERTENSION; and RENAL INSUFFICIENCY. Bright Disease,Kidney Scarring,Glomerulonephritides,Scarring, Kidney
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000368 Aged A person 65 years of age or older. For a person older than 79 years, AGED, 80 AND OVER is available. Elderly
D013256 Steroids A group of polycyclic compounds closely related biochemically to TERPENES. They include cholesterol, numerous hormones, precursors of certain vitamins, bile acids, alcohols (STEROLS), and certain natural drugs and poisons. Steroids have a common nucleus, a fused, reduced 17-carbon atom ring system, cyclopentanoperhydrophenanthrene. Most steroids also have two methyl groups and an aliphatic side-chain attached to the nucleus. (From Hawley's Condensed Chemical Dictionary, 11th ed) Steroid,Catatoxic Steroids,Steroids, Catatoxic
D016896 Treatment Outcome Evaluation undertaken to assess the results or consequences of management and procedures used in combating disease in order to determine the efficacy, effectiveness, safety, and practicability of these interventions in individual cases or series. Rehabilitation Outcome,Treatment Effectiveness,Clinical Effectiveness,Clinical Efficacy,Patient-Relevant Outcome,Treatment Efficacy,Effectiveness, Clinical,Effectiveness, Treatment,Efficacy, Clinical,Efficacy, Treatment,Outcome, Patient-Relevant,Outcome, Rehabilitation,Outcome, Treatment,Outcomes, Patient-Relevant,Patient Relevant Outcome,Patient-Relevant Outcomes
D050956 HSP40 Heat-Shock Proteins A family of heat-shock proteins that contain a 70 amino-acid consensus sequence known as the J domain. The J domain of HSP40 heat shock proteins interacts with HSP70 HEAT-SHOCK PROTEINS. HSP40 heat-shock proteins play a role in regulating the ADENOSINE TRIPHOSPHATASES activity of HSP70 heat-shock proteins. DnaJ Protein,HSP40 Heat-Shock Protein,HSP40 Protein,Heat-Shock Proteins 40,DnaJ Proteins,HSP40 Protein Family,HSP40 Proteins,Heat Shock Protein 40 Family,Heat-Shock Protein 40,HSP40 Heat Shock Protein,HSP40 Heat Shock Proteins,Heat Shock Protein 40,Heat Shock Proteins 40,Heat-Shock Protein, HSP40,Heat-Shock Proteins, HSP40,Protein, DnaJ,Protein, HSP40,Protein, HSP40 Heat-Shock,Proteins, HSP40 Heat-Shock
D018832 Molecular Chaperones A family of cellular proteins that mediate the correct assembly or disassembly of polypeptides and their associated ligands. Although they take part in the assembly process, molecular chaperones are not components of the final structures. Chaperones, Molecular,Chaperone, Molecular,Molecular Chaperone

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