CEN Case Reports最新文献

Successful use of avacopan in a case of ANCA-associated vasculitis with treatment-resistant medium-sized vessel involvement.

IF 1

CEN Case Reports Pub Date : 2025-01-24 DOI: 10.1007/s13730-025-00965-8

Takaaki Tsuchiya, Yui Ohta, Masatoshi Oka, Kaoruko Fukushima, Shiho Matsuno, Noriko Yamanaka, Noriyuki Suzuki, Wako Yumura, Akiko Komatsu, Tomio Arai, Takashi Takei, Mitsuyo Itabashi

{"title":"Successful use of avacopan in a case of ANCA-associated vasculitis with treatment-resistant medium-sized vessel involvement.","authors":"Takaaki Tsuchiya, Yui Ohta, Masatoshi Oka, Kaoruko Fukushima, Shiho Matsuno, Noriko Yamanaka, Noriyuki Suzuki, Wako Yumura, Akiko Komatsu, Tomio Arai, Takashi Takei, Mitsuyo Itabashi","doi":"10.1007/s13730-025-00965-8","DOIUrl":"https://doi.org/10.1007/s13730-025-00965-8","url":null,"abstract":"We report the case of a 75-year-old woman who presented with fever, right back pain, paresthesia in the right extremities, erythema, purpura, and nodules. She had previously initiated dialysis due to rapidly progressive glomerulonephritis and was transferred to our hospital. Imaging studies revealed multiple cerebral and splenic infarcts and hemorrhage encapsulating the right kidney, likely due to microaneurysms in multiple renal arteries. High MPO-ANCA titers were observed, and a skin biopsy revealed granulomatous inflammation affecting medium-sized vessels, leading to a diagnosis of granulomatosis with polyangiitis (GPA) and ANCA-associated vasculitis (AAV) involving medium-sized vessels. Treatment began with intravenous pulse steroid therapy (methylprednisolone 1000 mg/day) and subsequent oral prednisolone (PSL) 40 mg (about 0.8 mg/kg) and intravenous cyclophosphamide (IVCY) at 250 mg. While her symptoms improved, she developed severe infections, including candidemia and febrile neutropenia. Consequently, we combined PSL with the C5a receptor antagonist avacopan, which allowed for PSL tapering and stabilized her disease. This case is significant as no previous reports of avacopan's efficacy in AAV with medium-sized vessel involvement suggest its potential effectiveness in such cases.","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2025-01-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143028043","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

An educational case of toluene intoxication: importance of kidney function and timing in diagnosis.

IF 1

CEN Case Reports Pub Date : 2025-01-22 DOI: 10.1007/s13730-025-00964-9

Masatomo Ogata, Naoto Tominaga, Satoru Morikubo, Tomohiko Inoue, Yugo Shibagaki, Masahiko Yazawa

{"title":"An educational case of toluene intoxication: importance of kidney function and timing in diagnosis.","authors":"Masatomo Ogata, Naoto Tominaga, Satoru Morikubo, Tomohiko Inoue, Yugo Shibagaki, Masahiko Yazawa","doi":"10.1007/s13730-025-00964-9","DOIUrl":"https://doi.org/10.1007/s13730-025-00964-9","url":null,"abstract":"Metabolic acidosis (MA) is common in daily clinical settings and requires evaluation not only by serum anion gap (AG) but also by urine AG (UAG) and urine osmolal gap (UOG) to investigate potential causes and determine appropriate treatment. Herein, we report an educational case of non-gap (normal AG) MA (pH 7.16, HCO3- 8.4, AG 11.6) with nausea and fatigue. The patient had three episodes of hospital admission with MA in the previous three months. Although serum lactate and ketone levels were negative, the AG levels were notably inconsistent (elevated or not) each time. Nevertheless, the patient was suspected to have toluene intoxication because of low UAG and high UOG levels in the non-gap acidosis phase. Eventually, he was diagnosed with toluene intoxication due to a significantly elevated urinary hippurate level (28.7 g/L) despite never admitting to using toluene. Additionally, he had a high AG only in the presence of significant kidney dysfunction, which suggests that the high AG was either due to kidney dysfunction or accumulated hippurate in the blood (when UOG was high). Thus, it should be noted that the use of serum AG alone may lead to incorrect determination of cause(s) of MA when kidney dysfunction coexists, and that UAG and UOG, in addition to AG, are necessary to determine correct diagnosis.","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2025-01-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143022241","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

A case of mucormycosis caused by Rhizopus microsporus in a renal transplant patient. 肾移植患者小孢子根霉引起毛霉病1例。

IF 1

CEN Case Reports Pub Date : 2025-01-20 DOI: 10.1007/s13730-025-00969-4

Miruzato Fukuda, Takayoshi Yokoyama, Morota Marie, Katsuyuki Miki, Masayuki Yamanouchi, Hiroki Mizuno, Yuki Oba, Noriko Inoue, Akinari Sekine, Kiho Tanaka, Eiko Hasegawa, Tatsuya Suwabe, Takehiko Wada, Kei Kono, Kenichi Ohashi, Yutaka Yamaguchi, Sho Ogura, Naoki Sawa, Yuki Nakamura, Yasuo Ishii, Yoshifumi Ubara

{"title":"A case of mucormycosis caused by Rhizopus microsporus in a renal transplant patient.","authors":"Miruzato Fukuda, Takayoshi Yokoyama, Morota Marie, Katsuyuki Miki, Masayuki Yamanouchi, Hiroki Mizuno, Yuki Oba, Noriko Inoue, Akinari Sekine, Kiho Tanaka, Eiko Hasegawa, Tatsuya Suwabe, Takehiko Wada, Kei Kono, Kenichi Ohashi, Yutaka Yamaguchi, Sho Ogura, Naoki Sawa, Yuki Nakamura, Yasuo Ishii, Yoshifumi Ubara","doi":"10.1007/s13730-025-00969-4","DOIUrl":"https://doi.org/10.1007/s13730-025-00969-4","url":null,"abstract":"A 54-year-old man who had been on the kidney donor register for 32 years received a kidney from a 9-year-old boy who had died of fulminant myocarditis. The post-operative course was poor, and hemodialysis was still needed after surgery. A kidney biopsy one hour after surgery showed a neutrophil-predominant inflammatory cell infiltrate localized to the peritubular capillaries (PTC) and acute tubular necrosis of the proximal tubule. Rhizopus species was detected in a perirenal white exudate taken 29 days postoperatively, and the transplanted kidney was removed on postoperative day 45. The removed kidney showed fungal collection formation localized in the arteries and endotheliitis and embolization of the arteries. Renal damage caused by mucormycosis due to Rhizopus microspores was diagnosed. Mucormycosis is a fungal infection with a strong vascular affinity that develops along the vascular wall but does not extend beyond it. The infection leads to arterial infarction and organ failure. This report presents a rare case in which mucormycosis, which had been nested within the peritubular capillaries of the donor kidney, proliferated under immunosuppressive treatment but did so only in the artery, leading to infarction of the kidney. Neutrophilic collections within the PTC on one hour biopsy may be helpful in the early diagnosis of fungal infections.","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2025-01-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143000938","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

Steroid-resistant nephrotic syndrome as paraneoplastic syndrome of Waldenström macroglobulinemia: a case report. 类固醇抵抗性肾病综合征作为Waldenström巨球蛋白血症的副肿瘤综合征1例。

IF 1

CEN Case Reports Pub Date : 2025-01-14 DOI: 10.1007/s13730-025-00968-5

Konosuke Hirano, Sayuri Shirai, Teppei Koyama, Ryuichiro Makinouchi, Shinji Machida, Katsuomi Matsui, Shigeki Kosugi, Yasushi Ariizumi, Yukiko Kanetsuna, Junki Koike, Naohiko Imai

{"title":"Steroid-resistant nephrotic syndrome as paraneoplastic syndrome of Waldenström macroglobulinemia: a case report.","authors":"Konosuke Hirano, Sayuri Shirai, Teppei Koyama, Ryuichiro Makinouchi, Shinji Machida, Katsuomi Matsui, Shigeki Kosugi, Yasushi Ariizumi, Yukiko Kanetsuna, Junki Koike, Naohiko Imai","doi":"10.1007/s13730-025-00968-5","DOIUrl":"https://doi.org/10.1007/s13730-025-00968-5","url":null,"abstract":"Reports of glomerulonephritis associated with lymphoproliferative disorders are common, but reports of minimal change disease (MCD) accompanying non-Hodgkin's lymphoma are rare. Here, we present a case of a 45-year-old woman diagnosed with primary Waldenström's macroglobulinemia (WM) during MCD treatment. Her kidney biopsy revealed endothelial cell injury in parts of the MCD. Subsequently, she developed steroid-resistant nephrotic syndrome and temporary acute kidney injury, requiring dialysis. Remission of the nephrotic syndrome was achieved after initiating combination therapy with bendamustine and rituximab for WM. The renal histological findings and treatment course suggest a causal relationship between MCD and WM in this case. The pathogenesis of MCD associated with WM may involve the release of glomerular permeability factors derived from B lymphocytes. Although mild WM is often managed with observation, steroid-resistant nephrotic syndrome associated with WM should raise suspicion of a paraneoplastic syndrome, necessitating active chemotherapy targeting WM as a critical treatment approach.","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2025-01-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142982885","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

IgA vasculitis with nephritis accompanied by pulmonary tuberculosis: a case report. IgA血管炎伴肾炎并发肺结核1例。

IF 1

CEN Case Reports Pub Date : 2025-01-09 DOI: 10.1007/s13730-025-00966-7

Eriko Abe, Ryu Kobayashi, Rio Matsuoka, Tomohiko Kanaoka, Shoji Yamanaka, Satoshi Fujii, Junki Koike, Takashi Oda, Hiromichi Wakui, Kouichi Tamura

{"title":"IgA vasculitis with nephritis accompanied by pulmonary tuberculosis: a case report.","authors":"Eriko Abe, Ryu Kobayashi, Rio Matsuoka, Tomohiko Kanaoka, Shoji Yamanaka, Satoshi Fujii, Junki Koike, Takashi Oda, Hiromichi Wakui, Kouichi Tamura","doi":"10.1007/s13730-025-00966-7","DOIUrl":"https://doi.org/10.1007/s13730-025-00966-7","url":null,"abstract":"A 69-year-old Japanese man developed abdominal pain, purpura, proteinuria, and hematuria while receiving treatment for pulmonary tuberculosis. A skin biopsy revealed IgA-positive leukocytoclastic vasculitis, and a renal biopsy showed IgA-positive mesangial proliferative glomerulonephritis with crescent formation. Based on these findings, we diagnosed IgA vasculitis with nephritis (IgAVN) and initiated treatment. The patient's abdominal symptoms improved following factor XIII supplementation and corticosteroids. Corticosteroids were administered, and after 5 months, the proteinuria was in complete remission. Although IgAVN often follows a prior infection, it is rarely complicated by tuberculosis. In this case, staining for galactose-deficient IgA1, which is specifically positive in IgA nephropathy and IgAVN, was positive. Nephritis-associated plasmin receptor staining was also positive, suggesting some involvement of infectious glomerulonephritis. Therefore, the patient was considered to have IgAVN associated with pulmonary tuberculosis. In adult-onset cases, IgAVN is often severe. This patient was presented with adult-onset nephrosis and International Study of Kidney Disease in Children grade IIIb IgAVN, suggesting a poor prognosis. Therefore, we immediately initiated treatment with corticosteroids, factor XIII supplementation, a renin-aldosterone-system inhibitor, and a sodium-glucose cotransporter 2 inhibitor. The patient recovered uneventfully with no worsening of tuberculosis.","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2025-01-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142944936","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

Successful treatment with bortezomib for refractory cryoglobulinemic vasculitis triggered by ischemic non-obstructive coronary artery disease. 硼替佐米成功治疗缺血性非阻塞性冠状动脉疾病引发的难治性冷球蛋白性血管炎。

IF 1

CEN Case Reports Pub Date : 2025-01-06 DOI: 10.1007/s13730-024-00963-2

Yui Ohta, Takaaki Tsuchiya, Masatoshi Oka, Moriaki Tachibana, Yoshitaka Kondo, Kaoruko Fukushima, Shiho Matsuno, Noriko Yamanaka, Noriyuki Suzuki, Akiko Komatsu, Hirofumi Rokutan, Wako Yumura, Tomio Arai, Akihito Ishigami, Mitsuyo Itabashi, Takashi Takei

{"title":"Successful treatment with bortezomib for refractory cryoglobulinemic vasculitis triggered by ischemic non-obstructive coronary artery disease.","authors":"Yui Ohta, Takaaki Tsuchiya, Masatoshi Oka, Moriaki Tachibana, Yoshitaka Kondo, Kaoruko Fukushima, Shiho Matsuno, Noriko Yamanaka, Noriyuki Suzuki, Akiko Komatsu, Hirofumi Rokutan, Wako Yumura, Tomio Arai, Akihito Ishigami, Mitsuyo Itabashi, Takashi Takei","doi":"10.1007/s13730-024-00963-2","DOIUrl":"https://doi.org/10.1007/s13730-024-00963-2","url":null,"abstract":"Type I and mixed cryoglobulinemic vasculitis differ in pathophysiology, clinical presentation, and therapeutic response. We report a case of refractory cryoglobulinemic vasculitis diagnosed following ischemic non-obstructive coronary artery disease (INOCA). The patient presented with dyspnea, as well as abdominal pain due to ischemic enteritis, purpura, and renal failure requiring dialysis. Despite the patient's IgG λ-type monoclonal gammopathy of undetermined significance (MGUS) and negative hepatitis C virus, the presence of rheumatoid factor (RF) activity and the possibility of IgM involvement were suggested by cryoglobulin analysis and strong glomerular IgM deposition. The condition was diagnosed as mixed cryoglobulinemia, and various immunomodulatory treatments, including methylprednisolone, rituximab and plasmapheresis, were administered without achieving cryoglobulin negativity. However, treatment with bortezomib and dexamethasone ultimately led to cryoglobulin negativity and clinical improvement although the patient was not weaned off dialysis, resulting in remission of the cryoglobulinemic vasculitis. This case suggests that bortezomib, a proteasome inhibitor, may be a promising treatment for refractory cryoglobulinemic vasculitis.","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2025-01-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142930776","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

The application of omalizumab in children with atopic diseases complicated by nephrotic syndrome: a case report. 奥玛珠单抗在儿童特应性疾病并发肾病综合征中的应用：1例报告

IF 1

CEN Case Reports Pub Date : 2024-12-31 DOI: 10.1007/s13730-024-00962-3

Yi Mao, Jialu Lu, Lei Yin, Chong Liu, Jinhong Wu, Youying Mao

{"title":"The application of omalizumab in children with atopic diseases complicated by nephrotic syndrome: a case report.","authors":"Yi Mao, Jialu Lu, Lei Yin, Chong Liu, Jinhong Wu, Youying Mao","doi":"10.1007/s13730-024-00962-3","DOIUrl":"https://doi.org/10.1007/s13730-024-00962-3","url":null,"abstract":"Nephrotic syndrome (NS) and atopic diseases are linked through shared immunological pathways, with allergic triggers often contributing to NS relapses, particularly in immunoglobulin E (IgE)-mediated pathways. Omalizumab, a humanized monoclonal antibody targeting free and cell-bound IgE, is commonly used in treating atopic diseases. We presented a pediatric case with a history of eczema, asthma, and recurrent atopic rhinitis, who first developed NS at age three, responding well to steroid therapy. The patient experienced four NS relapses, three of which were associated with significant atopic symptoms, elevated eosinophil counts, and increased IgE levels, suggesting allergic triggers. After the latest relapse achieved remission, steroid therapy was discontinued, and Omalizumab was initiated two months later. The patient received monthly subcutaneous Omalizumab (300 mg) with regular urine protein monitoring. To date, the patient has shown marked improvement in atopic symptoms, sustained NS control, and reduced steroid dependence. This case highlights the potential of Omalizumab in treating NS triggered by allergic reactions, further prospective pilot studies and randomized controlled trials are warranted to rigorously evaluate its efficacy and safety.","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2024-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142909491","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

A case of neuron-derived neurotrophic factor-positive, syphilis-related membranous nephropathy that achieved spontaneous remission. 一例神经源性神经营养因子阳性，梅毒相关的膜性肾病实现自发缓解。

IF 1

CEN Case Reports Pub Date : 2024-12-28 DOI: 10.1007/s13730-024-00960-5

Yuko Yoshida, Kenji Ueki, Yuta Matsukuma, Akihiro Tsuchimoto, Eri Ataka, Hirofumi Okamoto, Kumiko Torisu, Yuichirou Hara, Hirofumi Ikeda, Takanari Kitazono, Toshiaki Nakano

{"title":"A case of neuron-derived neurotrophic factor-positive, syphilis-related membranous nephropathy that achieved spontaneous remission.","authors":"Yuko Yoshida, Kenji Ueki, Yuta Matsukuma, Akihiro Tsuchimoto, Eri Ataka, Hirofumi Okamoto, Kumiko Torisu, Yuichirou Hara, Hirofumi Ikeda, Takanari Kitazono, Toshiaki Nakano","doi":"10.1007/s13730-024-00960-5","DOIUrl":"https://doi.org/10.1007/s13730-024-00960-5","url":null,"abstract":"Neuron-derived neurotrophic factor (NDNF) was discovered as a target antigen in membranous nephropathy (MN) caused by syphilis. However, there have been few reports of NDNF-positive MN in Japan. A 19-year-old female patient was admitted to our hospital with nephrotic syndrome and acute kidney injury. After admission, she presented with a skin rash and was serologically positive for syphilis. Kidney biopsy showed MN positive not only for immunoglobulin (Ig) G, but also for IgA and complement C1q. IgG subclass analysis revealed positivity for IgG1 and IgG3. Immunohistochemistry for glomerular NDNF was positive, and a diagnosis of syphilis-related MN was confirmed. Prior to treatment of the infection, her urinary protein decreased to achieve complete remission and her kidney function improved. Benzylpenicillin was administered, and no recurrence of nephrotic syndrome or kidney dysfunction was observed thereafter. This case illustrates the importance of considering syphilis infection when encountering a case of nephrotic syndrome with skin rash. In addition, urinalysis of syphilitic patients should be performed to avoid missing MN that may be in spontaneous remission. Further elucidation of the pathogenesis of NDNF-positive, syphilis-related MN in Japan is needed.","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2024-12-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142892388","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

Effective combination of corticosteroid and cyclosporine A for immunoglobulin A nephropathy with membranoproliferative glomerulonephritis features: a case report. 皮质类固醇和环孢素A有效联合治疗免疫球蛋白A肾病伴膜增生性肾小球肾炎1例

IF 1

CEN Case Reports Pub Date : 2024-12-28 DOI: 10.1007/s13730-024-00961-4

Saeko Hatanaka, Go Kanzaki, Kentaro Koike, Hiroyuki Ueda, Nanae Matsuo, Yukio Maruyama, Nobuo Tsuboi, Takashi Yokoo

{"title":"Effective combination of corticosteroid and cyclosporine A for immunoglobulin A nephropathy with membranoproliferative glomerulonephritis features: a case report.","authors":"Saeko Hatanaka, Go Kanzaki, Kentaro Koike, Hiroyuki Ueda, Nanae Matsuo, Yukio Maruyama, Nobuo Tsuboi, Takashi Yokoo","doi":"10.1007/s13730-024-00961-4","DOIUrl":"https://doi.org/10.1007/s13730-024-00961-4","url":null,"abstract":"Immunoglobulin A nephropathy (IgAN) is the most common primary glomerulonephritis worldwide with heterogeneous histopathological phenotypes. Although IgAN with membranoproliferative glomerulonephritis (MPGN)-like features has been reported in children and adults, treatment strategies for this rare IgAN subtype have not been established. Here, we present the case of a 56-year-old man with no history of kidney disease who initially presented with nephrotic syndrome. Renal biopsy revealed MPGN-like features with a negative serological workup for secondary causes. Immunofluorescent staining was predominantly positive for IgA in the glomerular mesangial and capillary walls. Galactose-deficient IgA1 staining showed a distribution pattern similar to IgA staining. Electron microscopy revealed disorganized structural deposits in the mesangial and subendothelial regions. Based on clinical and histopathological findings, the patient was diagnosed with primary IgAN. The nephrotic syndrome resolved completely after six months of combined corticosteroids and cyclosporine A (CsA) therapy. Although corticosteroids and CsA were tapered off, hematuria and proteinuria remained in complete remission for years of follow-up. This case demonstrates the importance of recognizing IgAN with MPGN-like features as a histopathological subtype that may benefit from intensive immunosuppressive therapy.","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2024-12-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142892389","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

Granulomatosis with polyangiitis with rapidly progressive glomerulonephritis treated with a multipronged approach-a case based review. 肉芽肿病合并多血管炎合并快速进展的肾小球肾炎的多管齐下治疗-基于病例的回顾。

IF 1

CEN Case Reports Pub Date : 2024-12-20 DOI: 10.1007/s13730-024-00959-y

Ujjwal Madan, Vishesh Goel, Jignesh Shah, Hameed Ahmad, Clarissa Cassol, Amr Edrees

{"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":"https://doi.org/10.1007/s13730-024-00959-y","url":null,"abstract":"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.","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2024-12-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142863524","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