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Abstract: TH-PO712

Minimal Change Nephrotic Syndrome in an Elderly Female with Acute Onset Type 1 Diabetes

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Trials

Author

  • Koga, Shinichiro, Section for Nephrology & Hypertension, Department of Medicine, Tokyo Metropolitan Police Hospital, Tokyo, Japan
Introduction

Concurrent type 1 diabetes (T1D) and idiopathic nephrotic syndrome (NS) is rare, and most previously reported cases were in children. Recently, a younger adult who developed minimal change NS (MCN) several months after acute-onset of T1D was reported 1). Moreover, human leukocyte antigen (HLA) A24, Bw52, Bw61, DR2 and DR9 has been described to be associated with both T1D and renal disease 2).

Case Description

An Asian 78-year-old female with anti-glutamic acid decarboxylase (GAD) 65 antibody-positive acute onset T1D, anti-thyroperoxidase antibody-positive Hashimoto thyroiditis, and fatty liver disease, diagnosed them three years ago then treated with mdi insulin and po levothyroxine 3), was admitted again to our section due to bilateral leg edema as well as remarkable body weight gain. Her serum albumin was decreased to 1.8 mg/dL. Laboratory data show Hb 12.2 g/dL, TP 4.7 g/dL, Cr 1.11 mg/dL, eGFR 36.8 ml/min/1.73m2, Na 132 mEq/L, K 5.7 mEq/L, Tchol 353 mg/dL, LDL-c 165 mg/dL, fT4 0.90 ng/dL, TSH 13.81 mU/L, HbA1c 6.9 %, normo-complement proteins, D-dimer 9.23 μg/mL, UACR < 30 mg/gCr, UP 7.9 g/gCr with selectivity index 0.18, β2-microglobulin 27 mg/L. Serum antibodies were positive in GAD 604 U/mL, TPO 168 IU/mL, Tg 123 IU/mL, whereas was negative in MPO/PR3-ANCA, GBM, nor staff as paraproteinemia. No deep vein thrombosis nor malignancy was shown in non-enhanced computed tomography imaging. Kidney biopsy detected minor glomerular abnormalities with 1/10 obsolescence glomeruli; No Kimmelstiel-Wilson nodule was shown. Diagnosis of MCN was made with incidence described above. Her HLA typing was A24, A26, B62, B54, and DR9.
Given methylpredonisolone (mPSL) 500 mg/day intravenously for three days, then PSL 40 mg/day orally, CR was acheived but fastly relapsed twice so added po 75 mg cyclosporin A. Five weeks after admission, she discharged with po 25mg PSL plus 75 mg cyclosporin A, mdi 50 units/day insulin with po 50 mg SGLT2i ipraglifrozin, and 25 μg levothyroxin.

Discussion

The patient maintained remission with cyclosprin A; not required temporal hemodialysis 1)2) nor LDL-apheresis 1). HLA subtype was partially coincide in aged case 2) but not in young adult case 1). Th 1/2 predominance in T1D/MCN should have discussed 1)2).
References. 1) Nishizono R, BMC Nephrol 2020;21:410. 2) Kagiyama S, Am J Nephrol 1999;19:369-72. 3) Koga S, 17th Intl Conf Endocr 2016 poster # PT04-02-17, program book p.203.