A case report of progressive multifocal leukoencephalopathy during steroid treatment for ANCA-associated renal vasculiti
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CASE REPORT
A case report of progressive multifocal leukoencephalopathy during steroid treatment for ANCA‑associated renal vasculitis Takashi Tawara1 · Hirayasu Kai1 · Mikiko Kageyama1 · Tomoki Akiyama1 · Takahiro Matsunaga1 · Aki Sakuma1 · Ryota Ishii1 · Ryouya Tsunoda1 · Tetusya Kawamura1 · Akiko Fujita1 · Shuzo Kaneko1 · Naoki Morito1 · Chie Saito1 · Joichi Usui1 · Kunihiro Yamagata1 Received: 10 February 2020 / Accepted: 15 April 2020 © Japanese Society of Nephrology 2020
Abstract Case report: an 80-year-old woman presented with rapidly progressive glomerulonephritis and was admitted to our hospital. Myeloperoxidase-specific antineutrophil cytoplasmic antibody (MPO-ANCA) was positive. We diagnosed ANCA-associated renal vasculitis (ANCA-RV). Treatment was initiated with intravenous methylprednisolone pulse therapy, followed by prednisolone (PSL) at 30 mg/day. We gradually reduced the PSL dose to 7.5 mg/day over 6 months. At that time, the patient developed disturbances of consciousness which progressed subacutely. MRI revealed regions of patchy white matter with an increased signal on T2-weighted, fluid attenuated inversion recovery (FLAIR) sequences and diffusion-weighted sequences. JC virus DNA was detected in the cerebrospinal fluid (CSF) by polymerase chain reaction (PCR), leading to a diagnosis of progressive multifocal leukoencephalopathy (PML). PML is a rare infectious demyelinating disease of the central nervous system caused by JC virus infection, occurring in highly immunosuppressed individuals such as HIV-infected patients and patients using some biological agents, and having a very poor prognosis. In the present case, PML may have been associated with steroid use, although there are very few case reports of PML in patients taking only steroids. We report progressive multifocal leukoencephalopathy during steroid treatment of ANCA-RV. When patients show progressive disturbance of consciousness during treatment for ANCA-RV, we need to take PML into consideration for differential diagnosis. Keywords Progressive multifocal leukoencephalopathy · ANCA-associated renal vasculitis · Steroid treatment · Rapidly progressive glomerulonephritis
Introduction Antineutrophil cytoplasmic antibody (ANCA)-associated renal vasculitis (ANCA-RV) is a small-vessel vasculitis in the kidney characterized by ANCA-positive, rapidly progressive glomerulonephritis with hematuria, proteinuria and progressive loss of renal function over a short period of time (weeks or months). ANCA-RV pathology manifests as necrotizing crescentic glomerulonephritis without immune complex deposition [1, 2]. Its systemic clinical features are chronic lung fibrosis, peripheral reticulation, honeycombing and interstitial pneumonia, purpuric rash, leukocytoclastic * Kunihiro Yamagata k‑[email protected] 1
Division of Clinical Medicine, Department of Nephrology, Faculty of Medicine, University of Tsukuba, 1‑1‑1 Tennoudai, Tsukuba, Ibaraki 305‑8575, Japan
vasculitis, peripheral neuropathy, and gastrointestinal bleeding [2]. Initial immuno
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