- 著者
- Yoshitaka Furuto Hirotsugu Hashimoto Akio Namikawa Teppei Morikawa Yuko Shibuya
- 出版者
- The Japanese Society of Internal Medicine
- 雑誌
- Internal Medicine (ISSN:09182918)
- 巻号頁・発行日
- pp.1023-22, (Released:2023-03-15)
- 参考文献数
- 25
- 被引用文献数
- 2
IgG4-related membranous nephropathy (MN) is often refractory to glucocorticoid (GC) therapy, and treatment remains unclear. We herein report a 67-year-old Japanese man with IgG4-related MN and tubulointerstitial nephritis. A post-gastroscopy antibody test revealed Helicobacter pylori infection. After eradication, his proteinuria decreased indefinitely. We started prednisolone (30 mg/day), long-term GCs, and immunosuppressant therapy. However, remission proved challenging to achieve, with persistent proteinuria present at 1.0-2.0 g/gCr. We performed multitarget therapy for refractory IgG4-related MN, achieving proteinuria remission (<0.3 g/gCr). Multitarget therapy with low-dose GCs can resolve refractory IgG4-related MN through remission induction of proteinuria and minimize the risks associated with GC therapy.
1 0 0 0 OA An Autopsy Case of Familial Neuronal Intranuclear Inclusion Disease with Dementia and Neuropathy
- 著者
- Nanaka Yamaguchi Tatsuo Mano Ryo Ohtomo Hiroyuki Ishiura M. Asem Almansour Harushi Mori Junko Kanda Yuichiro Shirota Kenichiro Taira Teppei Morikawa Masako Ikemura Yasuo Yanagi Shigeo Murayama Jun Shimizu Yasuhisa Sakurai Shoji Tsuji Atsushi Iwata
- 出版者
- The Japanese Society of Internal Medicine
- 雑誌
- Internal Medicine (ISSN:09182918)
- 巻号頁・発行日
- vol.57, no.23, pp.3459-3462, 2018-12-01 (Released:2018-12-01)
- 参考文献数
- 10
- 被引用文献数
- 6 20
Neuronal intranuclear inclusion disease (NIID) is a rare neurodegenerative disease with marked variety in its clinical manifestations. While characteristic neuroimaging and skin biopsy findings are important clues to the diagnosis, autopsy studies are still important for confirming the exact disease features. We herein report the case of a patient who received an antemortem diagnosis of familial NIID with dementia-dominant phenotype that was later confirmed by an autopsy. Our report is the first to document a case of autopsy-confirmed NIID involving both cognitive impairment and sensorimotor neuropathy.