CEN Case Reports最新文献

Successful treatment of MPO-ANCA positive crescentic IgA nephropathy/IgA vasculitis with nephritis potentially triggered by a COVID-19 vaccine in a young adult female using corticosteroids, rituximab, and avacopan.

IF 1

CEN Case Reports Pub Date : 2025-04-09 DOI: 10.1007/s13730-025-00991-6

Ken Kaseda, Ryou Terakawa, Rena Matsui, Minoru Yasukawa, Shinichiro Asakawa, Shigeyuki Arai, Osamu Yamazaki, Yoshifuru Tamura, Ryuji Ohashi, Shigeru Shibata, Yoshihide Fujigaki

{"title":"Successful treatment of MPO-ANCA positive crescentic IgA nephropathy/IgA vasculitis with nephritis potentially triggered by a COVID-19 vaccine in a young adult female using corticosteroids, rituximab, and avacopan.","authors":"Ken Kaseda, Ryou Terakawa, Rena Matsui, Minoru Yasukawa, Shinichiro Asakawa, Shigeyuki Arai, Osamu Yamazaki, Yoshifuru Tamura, Ryuji Ohashi, Shigeru Shibata, Yoshihide Fujigaki","doi":"10.1007/s13730-025-00991-6","DOIUrl":"https://doi.org/10.1007/s13730-025-00991-6","url":null,"abstract":"An 18-year-old female presented with palpable purpura nine months before her hospital admission, which first appeared 1 month after receiving a COVID-19 vaccine and recurred intermittently. One month prior to admission, she developed macrohematuria, abdominal pain, and a loss of appetite. Occult blood in urine had been noted during high school health check-ups. Upon admission, she continued to have macrohematuria, along with renal dysfunction and a nephritic urinalysis, serum myeloperoxidase-anti-neutrophil cytoplasmic antibodies (MPO-ANCA) positivity. A renal biopsy revealed crescentic glomerulonephritis with mesangial and endocapillary hypercellularity, and dominant IgA deposition and electron-dense deposits in the mesangial regions. The diagnosis was IgA nephropathy (IgAN) or IgA vasculitis with nephritis (IgAVN), with a possible overlap of MPO-ANCA-associated glomerulonephritis. Treatment began with methylprednisolone pulse therapy and prednisolone. After the diagnosis, rituximab (RTX) and avacopan were added to the regimen. Within two months, renal function, hematuria, and MPO-ANCA levels had normalized, and proteinuria was almost fully resolved by 13 months. If IgAN/IgAVN and ANCA-associated vasculitis were indeed triggered by the COVID-19 vaccination in this case, it is plausible that both conditions share similar pathologic mechanisms. This case emphasizes the need for a reliable laboratory method to detect pathogenic ANCA to guide both induction and maintenance therapy. Further investigation into the effectiveness of the ANCA-associated glomerulonephritis treatment protocol including corticosteroids, RTX, and avacopan in managing crescentic IgAN/IgAVN could offer valuable insights into improving patient care.","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2025-04-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143810004","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Gastrointestinal calciphylaxis: a rare and devastating complication in end-stage kidney disease. 胃肠道钙化症：终末期肾病罕见的破坏性并发症。

IF 1

CEN Case Reports Pub Date : 2025-04-01 Epub Date: 2024-11-14 DOI: 10.1007/s13730-024-00944-5

Amirhossein Aarabi, Karthik Kumar

{"title":"Gastrointestinal calciphylaxis: a rare and devastating complication in end-stage kidney disease.","authors":"Amirhossein Aarabi, Karthik Kumar","doi":"10.1007/s13730-024-00944-5","DOIUrl":"10.1007/s13730-024-00944-5","url":null,"abstract":"Calciphylaxis, also known as calcific uremic arteriolopathy, is a rare and severe condition that predominantly affects individuals with end-stage kidney disease (ESKD). It manifests through vascular calcifications that precipitate tissue necrosis, with cutaneous manifestations being common. However, gastrointestinal involvement is an infrequent but gravely serious occurrence. In this report, we present an atypical case of gastrointestinal (GI) calciphylaxis in a 66-year-old woman undergoing peritoneal dialysis. The patient's initial symptoms of abdominal pain and turbid peritoneal fluid raised suspicions of bacterial peritonitis. Despite therapeutic interventions, the patient's condition deteriorated. Computed tomography (CT) imaging revealed a perforation in the caecal wall, necessitating emergency surgical intervention. Histopathology post-surgery confirmed the diagnosis of GI calciphylaxis. The patient's postoperative trajectory was fraught with complications, ultimately leading to a fatal outcome. This case highlights the importance of vigilance for calciphylaxis in the differential diagnosis when abdominal pain develops in patients with ESKD, regardless of the presence of typical skin symptoms. Prompt detection and an integrated care approach are essential for improved prognosis. Further research is needed to find more efficacious treatments for this devastating illness.","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"297-300"},"PeriodicalIF":1.0,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11958865/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142615409","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}

引用次数: 0

Identification of CUBN variants in triplets with a 20-year history of proteinuria. 在有 20 年蛋白尿病史的三胞胎中鉴定 CUBN 变体。

IF 1

CEN Case Reports Pub Date : 2025-04-01 Epub Date: 2024-08-05 DOI: 10.1007/s13730-024-00919-6

Natsumi Yamamura-Miyazaki, Nana Sakakibara, Kandai Nozu, Yuko Shima, Kenichi Satomura, Satoko Yamamoto, Minato Baba, Kaori Fujiwara, Katsusuke Yamamoto, Toshimi Michigami

{"title":"Identification of CUBN variants in triplets with a 20-year history of proteinuria.","authors":"Natsumi Yamamura-Miyazaki, Nana Sakakibara, Kandai Nozu, Yuko Shima, Kenichi Satomura, Satoko Yamamoto, Minato Baba, Kaori Fujiwara, Katsusuke Yamamoto, Toshimi Michigami","doi":"10.1007/s13730-024-00919-6","DOIUrl":"10.1007/s13730-024-00919-6","url":null,"abstract":"CUBN encodes cubilin, which plays a role in the reabsorption of glomerular-filtered albumin in the proximal tubule. CUBN-related proteinuria was recently established as a new disease concept and may be present in proteinuric cases that were previously undiagnosed either genetically or histologically. We herein report a case of triplets diagnosed with chronic benign proteinuria due to CUBN variants 20 years after its onset. The proband, the first child of triplets, tested positive for urinary protein several times during the neonatal period. A urine screening test at 3 years old was positive. Proteinuria persisted for years within a non-nephrotic range. Kidney biopsy at 8 years old revealed minor glomerular abnormalities. Renin-angiotensin system inhibitors were started for albumin-based proteinuria but were ineffective. Since the two other triplets had similar courses, analyses of the NPHS1/2 and WT1 genes were performed but revealed no abnormalities. The triplets transitioned to adult care at 15 years old. CUBN-related proteinuria was reported in 2020; therefore, we re-analyzed their DNA samples and identified compound heterozygous variants in CUBN in all three triplets. The molecular diagnosis of CUBN-related proteinuria will save patients from unnecessary treatments and concerns about renal prognosis.","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"145-150"},"PeriodicalIF":1.0,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11958850/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141888563","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}

引用次数: 0

Chronic kidney disease associated with extremely premature birth and extremely low birth weight may progress through the burden of growth. 与极早产和极低出生体重有关的慢性肾病可能会在生长过程中逐渐加重。

IF 1

CEN Case Reports Pub Date : 2025-04-01 Epub Date: 2024-10-05 DOI: 10.1007/s13730-024-00931-w

Chinatsu Onodera, Ken Ishikawa, Hiroshi Sugawara, Saeko Nishimi, Hiromi Furukawa, Akira Takada, Manami Akasaka, Megumi Kobayashi

{"title":"Chronic kidney disease associated with extremely premature birth and extremely low birth weight may progress through the burden of growth.","authors":"Chinatsu Onodera, Ken Ishikawa, Hiroshi Sugawara, Saeko Nishimi, Hiromi Furukawa, Akira Takada, Manami Akasaka, Megumi Kobayashi","doi":"10.1007/s13730-024-00931-w","DOIUrl":"10.1007/s13730-024-00931-w","url":null,"abstract":"Chronic kidney disease associated with low birth weight and/or premature birth (L/P-CKD) in infants may result from a decreased number of nephrons at birth. These infants may develop acute kidney injury due to exposure to nephrotoxic substances or other events during nephrogenesis in early infancy. Nonetheless, L/P-CKD progression remains unclear. We present three cases of L/P-CKD diagnosed after neonatal intensive care unit (NICU) discharge. Three patients were born extremely prematurely (gestational age, 24-26 weeks) with extremely low birth weight (606-906 g). They were admitted to the NICU (117-311 days) anad received several nephrotoxic medications during the early postnatal period. They showed elevated serum creatinine levels at 4 weeks after birth, which decreased to normal levels at NICU discharge. Proteinuria was first detected during adolescence (10-15 years) on annual school urine screening, with a remarkable increase in their height (18 - 50.8 cm), without known episodes of urinary tract infection, dehydration, lifestyle-related issues, such as excessive salt/protein intake, and extreme lack of exercise that might have caused kidney damage. Their kidneys were smaller than normal on renal ultrasonography. Open renal biopsy findings indicated glomerulomegaly and perihilar glomerulosclerosis in two of the three patients, suggesting glomerular hypertension. The remarkable differences between the body height before CKD and the timing of diagnosis of CKD could contribute to the progress of CKD. Long-term follow-up of low birth weight and extremely premature infants, from NICU discharge until adulthood, should be established.","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"224-229"},"PeriodicalIF":1.0,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11958864/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142379133","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}

引用次数: 0

A case of PLA2R-positive membranous nephropathy with subsequent development of IgG4-related disease. 一例 PLA2R 阳性膜性肾病，随后发展为 IgG4 相关疾病。

IF 1

CEN Case Reports Pub Date : 2025-04-01 Epub Date: 2024-11-08 DOI: 10.1007/s13730-024-00941-8

Fumiaki Tanemoto, Imari Mimura, Hiroyuki Abe, Masaomi Nangaku

{"title":"A case of PLA2R-positive membranous nephropathy with subsequent development of IgG4-related disease.","authors":"Fumiaki Tanemoto, Imari Mimura, Hiroyuki Abe, Masaomi Nangaku","doi":"10.1007/s13730-024-00941-8","DOIUrl":"10.1007/s13730-024-00941-8","url":null,"abstract":"Membranous nephropathy (MN) is a common cause of adult-onset nephrotic syndrome. It is also known as a minor but established renal manifestation of Immunoglobulin G4-related disease (IgG4-RD). Previous reports suggest that MN can also be an initial manifestation of IgG4-RD, all of which are phospholipase A2 receptor (PLA2R)-negative MN. We describe a case of PLA2R-positive MN that subsequently developed other manifestations of IgG4-RD. A 60-year-old male with nephrotic syndrome was diagnosed as primary MN with positive staining for PLA2R on the initial renal biopsy, which remained in partial remission with supportive therapy using angiotensin II receptor blocker (ARB) without steroid. About 1 year later, a renal mass was detected during an annual checkup, and contrast-enhanced computed tomography revealed low-density masses in bilateral kidneys and the head of the pancreas. The findings of endoscopic biopsy of the pancreatic mass were consistent with autoimmune pancreatitis (AIP) and the second renal biopsy showed the findings of MN with tubulointerstitial nephritis, both of which led to a diagnosis of IgG4-RD. The second renal biopsy also showed positive PLA2R. The patient received oral glucocorticoid therapy for IgG4-RD, which improved IgG4-related AIP and renal masses and also resulted in complete remission of MN. To our knowledge, this is the first reported case of PLA2R-positive MN with subsequent development of IgG4-RD. It is sometimes difficult to determine whether PLA2R-positive MN occurring with IgG4-RD is primary MN or secondary MN associated with IgG4-RD. The possibility of developing IgG4-RD should be considered even when preceding MN is PLA2R-positive, suggesting of primary MN.","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"280-290"},"PeriodicalIF":1.0,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11958855/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142602295","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}

引用次数: 0

Clinical manifestations and renal pathology of ethylene glycol. 乙二醇的临床表现和肾脏病理学。

IF 1

CEN Case Reports Pub Date : 2025-04-01 Epub Date: 2024-08-12 DOI: 10.1007/s13730-024-00921-y

Shogo Taira, Shiori Tamayose, Tasuku Kikumura, Morikuni Nishihira

{"title":"Clinical manifestations and renal pathology of ethylene glycol.","authors":"Shogo Taira, Shiori Tamayose, Tasuku Kikumura, Morikuni Nishihira","doi":"10.1007/s13730-024-00921-y","DOIUrl":"10.1007/s13730-024-00921-y","url":null,"abstract":"Ethylene glycol (EG) poisoning is a critical medical emergency often associated with suicide attempts in adults. EG is metabolized by alcohol dehydrogenase, leading to the formation of toxic metabolites that cause metabolic acidosis, renal failure, hypocalcemia, aciduria, and disorders of the central nervous and cardiovascular systems. Calcium oxalate, a metabolite of EG, contributes to acute tubular necrosis. Despite limited reports on human renal pathology, we present a case detailing renal pathology following EG ingestion. A 44-year-old male, admitted due to loss of consciousness, had ingested a lethal dose of EG. Blood tests indicated metabolic acidosis, while urinary examination revealed calcium oxalate crystals. Continuous renal replacement therapy corrected the acidosis; however, nephrogenic diabetes insipidus subsequently developed. A renal biopsy on day 31 revealed calcium oxalate crystal deposition and tubulointerstitial damage. Notably, various stages of crystal deposition, adherence, and degradation were observed. This case underscores the importance of considering EG poisoning in cases of unexplained metabolic acidosis and renal dysfunction, with renal biopsy serving as a valuable diagnostic tool. Understanding the renal effects of EG is essential for timely intervention and effective management of poisoning cases.","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"157-161"},"PeriodicalIF":1.0,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11958893/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141970697","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}

引用次数: 0

Macroscopic hematuria-associated severe acute kidney injury triggered by kidney stone formation in a patient with thin basement membrane and no history of microscopic hematuria. 一名基底膜较薄且无镜下血尿病史的患者因肾结石形成引发的镜下血尿相关性重度急性肾损伤。

IF 1

CEN Case Reports Pub Date : 2025-04-01 Epub Date: 2024-10-17 DOI: 10.1007/s13730-024-00942-7

Shuzo Kaneko, Ririko Murata, Ainori Hoshimoto, Rina Hisada, Makiko Harano, Emi Anno, So Hagiwara, Eri Imai, Michio Nagata

{"title":"Macroscopic hematuria-associated severe acute kidney injury triggered by kidney stone formation in a patient with thin basement membrane and no history of microscopic hematuria.","authors":"Shuzo Kaneko, Ririko Murata, Ainori Hoshimoto, Rina Hisada, Makiko Harano, Emi Anno, So Hagiwara, Eri Imai, Michio Nagata","doi":"10.1007/s13730-024-00942-7","DOIUrl":"10.1007/s13730-024-00942-7","url":null,"abstract":"Macroscopic hematuria (MH)-associated acute kidney injury (AKI) is a rare condition that causes acute tubular damage due to severe glomerular bleeding with MH. A 66-year-old Japanese woman with no significant past medical history was referred for severe kidney injury with oliguric MH. Her prior medical checkup results showed no occult blood in her urine. Seven days earlier, she had experienced transient severe acute right lumbar back pain. On admission, her serum urea nitrogen was 147 mg/dL, serum creatinine (sCr) 18.3 mg/dL, urinary red blood cells (RBCs) > 100/hpf, urinary protein 28.8 g/gCr, with no hydronephrosis in either kidney, but two stones were found in the right kidney and right ureteropelvic junction. At the start of her hemodialysis, the patient was treated with high-dose steroids because of suspected rapidly progressive glomerulonephritis. A renal biopsy of the left kidney showed acute tubular injury with massive RBC casts filling the tubular lumen. Glomerulitis was not detected, but electron microscopy revealed diffuse glomerular thin basement membrane (TBM). Despite immediate steroid discontinuation, the patient's renal function and MH improved, and she was weaned from hemodialysis. The stones resolved 2 months after onset, but microscopic hematuria persisted for 7 months post-onset. The sCr level was fixed at 1.1 mg/dL 20 months post-onset. This is the first report of MH-AKI in a TBM without the risk of MH-AKI development, such as bleeding tendency or iron overload. In this TBM, a colic attack of the renal urinary tract induced glomerular bleeding, and intolerance to hematuria may have caused severe tubular damage.","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"246-252"},"PeriodicalIF":1.0,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11958900/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142459021","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}

引用次数: 0

Acute tubulointerstitial nephritis following coronavirus disease 2019 mRNA vaccination: a pediatric case report. 接种冠状病毒 2019 mRNA 疫苗后出现急性肾小管间质性肾炎：一例儿科病例报告。

IF 1

CEN Case Reports Pub Date : 2025-04-01 Epub Date: 2024-10-31 DOI: 10.1007/s13730-024-00945-4

Gakushi Eguchi, Miki Murakoshi, Futaba Miyaoka, Asami Shimbo, Hitoshi Irabu, Toru Kanamori, Tomohiro Udagawa, Tomohiro Morio, Masaki Shimizu

{"title":"Acute tubulointerstitial nephritis following coronavirus disease 2019 mRNA vaccination: a pediatric case report.","authors":"Gakushi Eguchi, Miki Murakoshi, Futaba Miyaoka, Asami Shimbo, Hitoshi Irabu, Toru Kanamori, Tomohiro Udagawa, Tomohiro Morio, Masaki Shimizu","doi":"10.1007/s13730-024-00945-4","DOIUrl":"10.1007/s13730-024-00945-4","url":null,"abstract":"Coronavirus disease 2019 (COVID-19) mRNA vaccines have been linked to various kidney adverse events including acute tubulointerstitial nephritis (ATIN). This report describes a 15-year-old female who developed persistent fever and fatigue 54 days after receiving her second dose of the BNT162b2 2 SARS-CoV-2 vaccine. She presented with elevated serum creatinine and urinary β2-microglobulin (β2MG) levels. Kidney biopsy revealed mononuclear infiltrate with some eosinophils, confirming the diagnosis of ATIN. Repeatedly positive lymphocyte transformation test results for the vaccine suggested a relationship between the vaccine and interstitial nephritis. Initially, treatment with prednisolone was effective. However, an increase in urinary β2MG level was observed 7 months later, and the introduction of mycophenolate mofetil (MMF) allowed for the gradual reduction and eventual cessation of prednisolone. This case represents one of the rare pediatric instances of ATIN following COVID-19 vaccination. MMF can be an effective alternative in corticosteroid-dependent cases.","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"261-265"},"PeriodicalIF":1.0,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11958856/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142557264","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}

引用次数: 0

Effective management of necrotizing crescentic glomerulonephritis using an aggressive combination therapy including avacopan in a patient double-seropositive for anti-GBM antibodies and ANCA: a case report. 病例报告：对一名抗 GBM 抗体和 ANCA 双血清阳性患者采用包括阿伐戈班在内的积极综合疗法有效治疗坏死性新月体肾小球肾炎。

IF 1

CEN Case Reports Pub Date : 2025-04-01 Epub Date: 2024-09-10 DOI: 10.1007/s13730-024-00929-4

Rina Tanaka, Takumi Toishi, Reiji Masaki, Hideaki Aihara, Sumie Sakamoto, Mari Ikeda, Tomohiko Inoue, Atsuro Kawaji, Masatoshi Matsunami, Junko Fukuda, Mamiko Ohara, Hiroshi Kuji, Daisuke Ichikawa, Tomo Suzuki

{"title":"Effective management of necrotizing crescentic glomerulonephritis using an aggressive combination therapy including avacopan in a patient double-seropositive for anti-GBM antibodies and ANCA: a case report.","authors":"Rina Tanaka, Takumi Toishi, Reiji Masaki, Hideaki Aihara, Sumie Sakamoto, Mari Ikeda, Tomohiko Inoue, Atsuro Kawaji, Masatoshi Matsunami, Junko Fukuda, Mamiko Ohara, Hiroshi Kuji, Daisuke Ichikawa, Tomo Suzuki","doi":"10.1007/s13730-024-00929-4","DOIUrl":"10.1007/s13730-024-00929-4","url":null,"abstract":"The prognosis of anti-glomerular basement membrane (anti-GBM) nephritis, often accompanied by the presence of antineutrophil cytoplasmic antibodies (ANCA), is poor, and even with aggressive therapeutic approaches, kidney replacement therapy (KRT) is typically required. Here, we present a case of necrotizing crescentic glomerulonephritis in a patient double-seropositive for anti-GBM antibodies and ANCA who successfully achieved dialysis independence following aggressive treatment, including avacopan. The patient was a 77-year-old woman with rapidly progressive glomerulonephritis and double seropositivity for myeloperoxidase-ANCA and anti-GBM antibodies. A kidney biopsy revealed diffuse cellular crescents with necrosis and immunoglobin (Ig)G1 and IgG3 positivity on immunofluorescence staining, leading to a histological diagnosis of anti-glomerular basement membrane nephritis. Our treatment approach involved a novel combination of glucocorticoids, rituximab, low-dose cyclophosphamide, and plasma exchange complemented by avacopan. Temporary hemodialysis was required, and the patient successfully discontinued dialysis after 12 sessions despite a poor histological prognosis. This case underscores the significance of considering aggressive therapeutic strategies, including avacopan, for severe anti-GBM nephritis, even in the absence of lung involvement, to avert the need for KRT.","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"183-187"},"PeriodicalIF":1.0,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11958878/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142280879","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}

引用次数: 0

A case of ANCA-negative pauci-immune crescentic glomerulonephritis with lung adenocarcinoma with mediastinal involvement successfully treated by corticosteroid and radiation therapy. 一例 ANCA 阴性、伴有肺腺癌且纵隔受累的新月体肾小球肾炎病例，通过皮质类固醇和放射治疗获得成功。

IF 1

CEN Case Reports Pub Date : 2025-04-01 Epub Date: 2024-08-20 DOI: 10.1007/s13730-024-00925-8

Yuna Onozawa, Masahiro Koizumi, Yosuke Nakagawa, Go Ogura, Masayuki Oki, Takehiko Wada, Masafumi Fukagawa

{"title":"A case of ANCA-negative pauci-immune crescentic glomerulonephritis with lung adenocarcinoma with mediastinal involvement successfully treated by corticosteroid and radiation therapy.","authors":"Yuna Onozawa, Masahiro Koizumi, Yosuke Nakagawa, Go Ogura, Masayuki Oki, Takehiko Wada, Masafumi Fukagawa","doi":"10.1007/s13730-024-00925-8","DOIUrl":"10.1007/s13730-024-00925-8","url":null,"abstract":"Pauci-immune crescentic glomerulonephritis (PICGN) is one of the pathologies causing rapidly progressive glomerulonephritis, often associated with anti-neutrophil cytoplasmic antibody (ANCA); however, in 10-30% of cases, ANCAs are negative. While a relatively large number of cases of ANCA-positive PICGN complicated with malignancy have been previously reported, the number of cases of ANCA-negative PICGN with malignancy is limited. The prognosis for such cases was poor, and many patients died within a relatively short period. Here, we report the case of ANCA-negative PICGN complicated with malignancy successfully treated by corticosteroid and radiation therapy. A 63-year-old Japanese man was admitted to our hospital due to spiking fevers in the previous 3 months. Based on the findings of imaging and pathological tests, he was diagnosed with locally advanced lung adenocarcinoma with mediastinal involvement. After admission, his renal function rapidly deteriorated, and urinalysis showed heavy proteinuria. In serological tests, serology for autoantibodies, including ANCAs, was negative. The kidney biopsy revealed PICGN with prominent endocapillary proliferation. We administered corticosteroid therapy for glomerulonephritis and subsequent radiation therapy for lung carcinoma, both of which were effective. He has been alive without progression of malignancy or kidney disease for 5 years after discharge. In patients with malignancy presenting with acute deterioration of kidney function, although infrequent, one of the conceivable pathological conditions to consider is ANCA-negative PICGN associated with malignancy. In such cases, even with negative antibodies such as ANCA, pathological examination is warranted, and a combination of anti-tumor therapy and immunosuppressive therapy is expected to be effective.","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":"171-177"},"PeriodicalIF":1.0,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11958877/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142003721","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}

引用次数: 0