{"title":"Granulomatosis with polyangiitis with rapidly progressive glomerulonephritis treated with a multipronged approach-a case based review.","authors":"Ujjwal Madan, Vishesh Goel, Jignesh Shah, Hameed Ahmad, Clarissa Cassol, Amr Edrees","doi":"10.1007/s13730-024-00959-y","DOIUrl":"10.1007/s13730-024-00959-y","url":null,"abstract":"<p><p>Granulomatosis with polyangiitis is an ANCA-associated vasculitis that involves small to medium-sized vessels. The extent of renal involvement varies, which is also associated with disease prognosis, with aggressive renal involvement having worse outcomes. Rapidly progressive glomerulonephritis with severe inflammatory features and extensive crescent formation can be challenging to treat. Usually, induction regimes utilize a combination of pulse dose methylprednisolone followed by rituximab or cyclophosphamide. Resistant diseases pose additional treatment challenges, and individualized treatment regimens have been described without accumulated outcome data. Cyclophosphamide, rituximab, azathioprine, methotrexate, and mycophenolate with or without plasmapheresis have been variably used, but there is a lack of consensus on a standardized regime in literature. Our case adds to the existing literature on the treatment-refractory granulomatosis with polyangiitis, which was treated with high-dose corticosteroid in combination with rituximab, low-dose cyclophosphamide, plasmapheresis, and brief use of hemodialysis. It also reiterates that the use of a variety of low-dose cyclophosphamide with rituximab could be beneficial for treatment-refractory cases or patients with severe renal involvement, in addition to better tolerance with low dose cyclophosphamide in comparison with full-dose cyclophosphamide.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"567-572"},"PeriodicalIF":0.7,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12307261/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142863524","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Multiple relapses of sigmoid volvulus in a patient on automated peritoneal dialysis: the role of an elongated sigmoid colon and peritoneal dialysis as potential predisposing factors.","authors":"Keitaro Uehara, Shunsuke Yamada, Yusuke Mizuuchi, Takanari Kitazono, Toshiaki Nakano","doi":"10.1007/s13730-024-00930-x","DOIUrl":"10.1007/s13730-024-00930-x","url":null,"abstract":"","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"507-508"},"PeriodicalIF":0.7,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12307848/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142280881","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"A case of syphilis-associated membranous nephropathy with NDNF detected by immunohistochemistry, not by mass spectrometry: case report and literature review.","authors":"Emi Sakamoto, Keiki Shimada, Kazuhiro Takeuchi, Hideaki Kuno, Haruka Yamada, Minami Suzuki, Daisuke Katagiri, Akira Shimizu, Hideki Takano","doi":"10.1007/s13730-025-00997-0","DOIUrl":"10.1007/s13730-025-00997-0","url":null,"abstract":"<p><p>Syphilis is an infectious disease caused by the spirochete Treponema pallidum and is increasingly prevalent worldwide, with a rapid increase in reported cases in Japan in recent years. Syphilis is characterized by skin lesions, lymphadenopathy, and hepatosplenomegaly but is rarely manifested by renal disorders, including membranous nephropathy (MN). Neuron-derived neurotrophic factor (NDNF) is an antigen specific for syphilis-associated MN. This report describes a case of NDNF-related MN after syphilis infection in a 42-year-old male who developed nephrotic syndrome 3 months after infection. Renal biopsy under light microscopy revealed mild mesangial proliferation without spike formation in the glomerular basement membrane. Immunofluorescence and electron microscopy revealed granular deposits of IgM, IgG, C1q, and C3 on the capillary walls, with subepithelial hump-like electron-dense deposits (EDDs), consistent with stage I MN. Immunohistochemistry confirmed the presence of NDNF. In contrast, other common antigens related to primary MN, such as M-type phospholipase A2 receptor and thrombospondin type I domain-containing 7A, were negative on mass spectrometry. The patient achieved remission with antibiotic therapy alone. This case and the literature review on NDNF-related MN highlight the relevance of NDNF as a syphilis-associated MN antigen and demonstrate that antibiotic therapy alone without immunosuppressive drugs can lead to remission. Mass spectrometry can accurately identify MN antigens; however, immunostaining is more effective in cases where EDDs are segmental and antigen concentration is low. Our findings indicate that NDNF testing should be performed in cases of proteinuria associated with syphilis to help guide antibiotic therapy and reduce immunosuppression.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"647-652"},"PeriodicalIF":0.7,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12307845/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144076159","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Tsukamurella conjunctivitidis peritonitis diagnosed by 16S rRNA gene sequencing in a patient undergoing peritoneal dialysis.","authors":"Masato Habuka, Kosuke Matsunaga, Asa Ogawa, Takashi Yaguchi, Suguru Yamamoto, Yoshinari Tanabe","doi":"10.1007/s13730-024-00958-z","DOIUrl":"10.1007/s13730-024-00958-z","url":null,"abstract":"<p><p>Peritoneal dialysis (PD)-associated peritonitis remains a serious and life-threatening complication in patients undergoing PD. Majority of peritonitis cases are caused by bacteria, with coagulase-negative Staphylococcus being the most common cause. Tsukamurella species are obligate aerobic gram-positive bacilli found in various environments; however, peritonitis caused by Tsukamurella species in association with PD is rare, with few reports of infections caused by T. conjunctivitidis, including PD-associated peritonitis. We describe a rare case of peritonitis caused by T. conjunctivitidis in a patient undergoing PD. A 62-year-old man undergoing PD was referred to our hospital for fever and abdominal pain lasting for 4 days and was diagnosed with PD-associated peritonitis based on abdominal pain, cloudy effluent, elevated white blood cell count in the peritoneal fluid, and presence of gram-positive rods in the effluent culture. Yellow-grayish, dry, membrane-like colonies were observed on a blood agar plate. The isolated strain was identified as T. conjunctivitidis through 16S rRNA gene sequencing. Because the PD-associated peritonitis was refractory to antibiotics, the PD catheter was removed, and the patient was switched to hemodialysis on day 21 of admission. Clinicians should consider peritonitis caused by T. conjunctivitidis as a differential diagnosis in cases where diagnosis using routine examination is challenging. Notably, 16S rRNA gene sequencing is a useful diagnostic tool for identifying T. conjunctivitidis. Further analyses of similar cases are required to understand the characteristics of such infections and establish adequate diagnostic methods and treatment regimens.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"553-557"},"PeriodicalIF":0.7,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12307253/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142823984","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Genetic and protein structure prediction analyses identify a rare pathogenic PKD1 variant causing autosomal dominant polycystic kidney disease.","authors":"Takamitsu Shiiya, Hirofumi Watanabe, Ryo Aida, Tadashi Otsuka, Ryohei Kaseda, Suguru Yamamoto, Yoshikatsu Kaneko, Shin Goto, Ichiei Narita","doi":"10.1007/s13730-025-00985-4","DOIUrl":"10.1007/s13730-025-00985-4","url":null,"abstract":"<p><p>Autosomal dominant polycystic kidney disease (ADPKD) is one of the most common monogenic kidney disorders. The diagnosis of ADPKD requires imaging findings showing multiple kidney cysts or genetic testing, in cases where a family history is unknown. We report a patient diagnosed with ADPKD based on the identification of a rare PKD1 variant. The patient was a 41-year-old female in whom cysts and calcification in the kidneys were incidentally detected. Whole-exome sequencing identified a rare PKD1 variant (NM_001009944.3: c.11557G > A/p.E3853K). Protein structure prediction of the PKD1-PKD2 complex showed that the variant may be pathogenic, leading to the diagnosis of autosomal dominant polycystic kidney disease. A detailed family history revealed that her relatives also had ADPKD, further supporting the diagnosis of ADPKD. Comprehensive genetic analysis and protein structure prediction led to the diagnosis of ADPKD and the identification of rare causative genes. These methods are useful for diagnosing hereditary kidney diseases of unknown etiologies.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"612-617"},"PeriodicalIF":0.7,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12307831/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143630205","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"A case of IgA nephropathy associated with Crohn's disease treated with enteric-coated budesonide.","authors":"Ryuto Yoshida, Takashin Nakayama, Akinori Hashiguchi, Tatsuhiko Azegami, Kaori Hayashi","doi":"10.1007/s13730-025-01001-5","DOIUrl":"10.1007/s13730-025-01001-5","url":null,"abstract":"<p><p>The gut-kidney axis has recently gained attention as a pathogenesis of IgA nephropathy (IgAN). In fact, the efficacy of target-release formulation of budesonide for IgAN has been reported in recent studies. On the other hand, there have been no reports yet on the efficacy of enteric-coated budesonide for IgAN complicated with inflammatory bowel disease. We report a case of IgAN with comorbid Crohn's disease (CD) treated with a combination of enteric-coated budesonide and methylprednisolone pulse therapy. The patient was followed for 2 years. He showed initial reduction in proteinuria following treatment. However, after discontinuation of enteric-coated budesonide therapy, proteinuria recurred despite well-controlled CD. Enteric-coated budesonide combined with methylprednisolone pulse therapy may be effective in short-term reduction of proteinuria while minimizing systemic steroid side effects in IgAN associated with CD. However, the tendency for relapse suggests the need for long-term management strategies. Therefore, further research is needed to establish optimal treatment protocols for this patient population.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"659-664"},"PeriodicalIF":0.7,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12307832/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144186654","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"A case of rapidly progressive IgA nephropathy after SARS-CoV-2 vaccination, successfully treated with cyclophosphamide.","authors":"Risa Masaki, Ayaka Mori, Haruki Ouchi, Hiroyuki Tsukada, Satoshi Furuse, Akiko Fujii, Naobumi Mise","doi":"10.1007/s13730-025-00993-4","DOIUrl":"10.1007/s13730-025-00993-4","url":null,"abstract":"<p><p>A 61-year-old woman with a 10-year history of type 1 diabetes mellitus was referred to our nephrology department due to rapid kidney function deterioration. Despite good blood glucose control, her serum creatinine (Cr) concentration rose from 0.77 mg/dL to 2.40 mg/dL over one year. She had microscopic hematuria for several years, and an increase in urinary protein was observed over the past year. One month before the onset of kidney function exacerbation, she received the fourth vaccination against SARS-CoV-2. At referral, her urinary examination showed proteinuria of 4.73 g/g Cr and microscopic hematuria. MPO-ANCA, PR3-ANCA, and anti-basal membrane antibodies were negative. A kidney biopsy revealed IgA nephropathy demonstrating focal mesangioproliferative glomerulonephritis with cellular crescents (M0E1S1T0-C1). The patient underwent six courses of intravenous cyclophosphamide administration in addition to steroid pulse therapy. One year after starting the treatment, her serum Cr improved to approximately 2 mg/dL, and urinary protein decreased to 0.6 g/g Cr.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"641-646"},"PeriodicalIF":0.7,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12307264/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143977940","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"An end-stage kidney disease patient undergoing dialysis treatment for 50 years with dialysis-related amyloidosis in multiple organs.","authors":"Toru Ito, Takashi Kato, Suguru Yamamoto, Hisaki Shimada, Hideyuki Kabasawa, Shin Goto, Riuko Ohashi, Ichiei Narita","doi":"10.1007/s13730-025-01003-3","DOIUrl":"10.1007/s13730-025-01003-3","url":null,"abstract":"<p><p>Advancements in dialysis treatment have led to an increase in the number of patients undergoing long-term dialysis. These patients often experience a decline in activities of daily living (ADL) owing to various chronic kidney disease-related conditions such as mineral and bone disorders, dialysis-related amyloidosis (DRA), and cardiovascular diseases. A male patient began hemodialysis at the age of 22 years because of end-stage kidney disease caused by chronic glomerulonephritis and continued dialysis treatment for 50 years. The patient died at 72 years of age from sepsis, ischemic colitis, and pneumonia. During the course of treatment, the patient frequently underwent surgeries for carpal tunnel syndrome and destructive spondyloarthropathy, which led to a decline in ADL. An autopsy revealed β<sub>2</sub>-microglobulin amyloid deposits in multiple organs, including the heart, lungs, and intestines. Long-term dialysis treatment results in ADL impairment owing to osteoarticular disorders caused by DRA, and systemic organ deposits are potentially related to organ failures such as ischemic colitis and pneumonia. Elucidating the pathophysiology of DRA and developing more effective treatments may improve ADL and prognosis in patients undergoing long-term dialysis.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"665-673"},"PeriodicalIF":0.7,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12307826/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144198339","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Takayasu arteritis-associated refractory hypertension induces nephrotic syndrome through glomerular microangiopathy.","authors":"Tomoya Nakayamada, Kensei Taguchi, Chikei Natori, Nao Nakamura, Makiko Fujii, Yuya Yamashita, Sakuya Ito, Kei Fukami","doi":"10.1007/s13730-024-00952-5","DOIUrl":"10.1007/s13730-024-00952-5","url":null,"abstract":"<p><p>Takayasu arteritis (TAK) is a systemic inflammatory condition characterized by vasculitis in mainly the aorta and their branches; however, few reports have demonstrated glomerulonephritis and subsequent nephrotic syndrome in patients with TAK. We encountered a 69-year-old woman with TAK who developed nephrotic syndrome owing to uncontrolled hypertension. Kidney biopsy demonstrated endotheliosis, aberrant proliferation of vascular smooth muscle cells, and concentric intimal hyperplasia without any clues of vasculitis. Treatment with sacubitril/valsartan reduced proteinuria and increased serum albumin without affecting renal function, which continued to suppress blood pressure and prevent recurrence of nephrotic syndrome over 2 years.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"527-533"},"PeriodicalIF":0.7,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12307251/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142794514","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Delayed rectum perforation by a peritoneal dialysis catheter in a peritoneal dialysis patient: a case report and literature review.","authors":"Mayu Iwata, Tadashi Uramatsu, Ryosuke Sakamoto, Kenta Torigoe, Ayuko Yamashita, Shinichi Abe, Yuki Ota, Mineaki Kitamura, Atsuhiro Maeda, Yasushi Mochizuki, Kumiko Muta, Ryoichi Imamura, Tomoya Nishino","doi":"10.1007/s13730-025-00971-w","DOIUrl":"10.1007/s13730-025-00971-w","url":null,"abstract":"<p><p>A 60-year-old woman with diabetic nephropathy was diagnosed with end-stage kidney disease and underwent peritoneal dialysis (PD) (X-3 years). Due to decreased residual renal function, she commenced combined PD and hemodialysis (HD) for 6 h once a week (Jan. X year). Seven months later, she visited her maintenance dialysis facility because of fluid leakage from the pubic region and abdominal pain during the dialysate infusion. Tests of the leakage fluid revealed a high glucose concentration, leading to discontinuation of PD and transfer to HD three times a week. She had been referred to a general hospital in her city, where vesical and uterine fistulas were excluded. Consequently, she was referred to our department to investigate the cause of the leakage. She was diagnosed with peritonitis by a physician and was receiving treatment. The lumen of the PD catheter was brownish, indicating possible contamination, leading us to plan its removal. At the time of catheter removal, laparoscopy and intraoperative lower gastrointestinal endoscopy revealed a rectal perforation at the tip of the PD catheter; this was removed after suturing the damaged area. This was a rare case of delayed perforation of the rectum by a PD catheter, during combined PD and HD therapy.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"596-603"},"PeriodicalIF":0.7,"publicationDate":"2025-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12307860/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143413575","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}