Low-density lipoprotein apheresis for PLA2R-related membranous glomerulonephritis accompanied by IgG4-related tubulointe
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CASE REPORT
Low‑density lipoprotein apheresis for PLA2R‑related membranous glomerulonephritis accompanied by IgG4‑related tubulointerstitial nephritis Yoko Nishizawa1 · Kazuho Honda2 · Yumi Aoyama1 · Yumi Hosoda1 · Tomomi Tamura1 · Ai Horimoto1 · Kiyotsugu Omae1 · Chieko Higuchi1 · Hiroshi Sakura1 · Kosaku Nitta3 · Tetsuya Ogawa1 Received: 12 March 2020 / Accepted: 6 June 2020 © Japanese Society of Nephrology 2020
Abstract IgG4-related disease preferentially involves the kidney by tubulointerstitial nephritis with IgG4-positive plasma cell filtration and/or membranous glomerulonephritis. We reported the case of a 68-year-old man with IgG4-related tubulointerstitial nephritis combined with antiphospholipase A2 receptor (PLA2R)-related membranous glomerulonephritis, in which distinguishing between idiopathic PLA2R-related and IgG4-related secondary membranous glomerulonephritis was difficult. We diagnosed him as having IgG4-related disease, based on a serum IgG4 level of 170 mg/dL and the presence of IgG4related parotiditis. On renal biopsy, there was tubulointerstitial nephritis with IgG4-positive plasma cell filtration, which was compatible with IgG4-related disease and membranous glomerulonephritis, with concomitant positive staining for PLA2R on immunofluorescence microscopy. The renal function immediately recovered after steroid treatment, probably because of the improvement in the tubulointerstitial lesions, but his nephrotic syndrome was steroid-resistant. Low-density lipoprotein (LDL) apheresis therapy was effective for membranous glomerulonephritis and increased his serum albumin from 1.4 to 2.8 g/dL. Although IgG4-related kidney disease usually accompanies secondary membranous glomerulonephritis, the positive PLA2R staining suggested a concomitant primary membranous glomerulonephritis. The recent treatment strategy, including LDL apheresis, for primary and secondary membranous glomerulonephritis was discussed briefly in this report. Keywords IgG4-related kidney disease · Membranous glomerulonephritis · Tubulointerstitial nephritis · PLA2R · LDL apheresis
Introduction IgG4-related disease (IgG4-RD) is a systemic inflammatory disorder affecting various organs [1] and the kidney is one of the most frequently involved [2]. Patients with IgG4-related kidney disease (IgG4-RKD) usually have high serum IgG4 * Yoko Nishizawa [email protected] 1
Department of Medicine, Tokyo Women’s Medical University Medical Center East, 2‑1‑10, Nishiogu, Arakawa, Tokyo 116‑8567, Japan
2
Department of Anatomy, Showa University School of Medicine, 1‑5‑8, Hatanodai, Shinagawa, Tokyo 142‑8555, Japan
3
Department of Nephrology, Tokyo Women’s Medical University, 8‑1, Kawadacho, Shinjyuku, Tokyo 162‑8666, Japan
level [1]. Histologically, the most common manifestation is plasma cell-rich tubulointerstitial nephritis (TIN) with storiform or bird’s eye fibrosis [3]. Moreover, IgG4-RKD is sometimes accompanied by a membranous glomerulonephritis (MGN) pattern of glomerular lesion. Because IgG4-related
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