{"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":"<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":""},"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}
{"title":"Parvovirus B19-related membranoproliferative glomerulonephritis presenting with positive glomerular staining for nephritis-associated plasmin receptor: a case report and review of the literature.","authors":"Haruka Takahashi, Yukihiro Wada, Takuya Yamazaki, Kazuhiro Takeuchi, Tetsuya Abe, Shokichi Naito, Togo Aoyama, Takashi Sano, Rika Moriya, Takashi Oda, Yasuo Takeuchi","doi":"10.1007/s13730-024-00956-1","DOIUrl":"https://doi.org/10.1007/s13730-024-00956-1","url":null,"abstract":"<p><p>Several cases of glomerulonephritis occurring after infection with human parvovirus B19 (PVB19) have been reported. However, the pathogenesis and clinicopathological features of PVB19-related glomerulonephritis remain elusive. We describe the case of a 34 year-old woman who showed nephrotic syndrome and microscopic hematuria 10 days after PVB19 infection. Blood pressure and renal function were within normal ranges. Laboratory tests showed positive results for anti-PVB19 immunoglobulin (Ig)M antibody and complement 3 (C3) hypocomplementemia. Antibody to streptolysin O (ASO) was slightly elevated, but bacterial cultures yielded no colonies. Light microscopy of renal biopsy was compatible with membranoproliferative glomerulonephritis (MPGN). Immunofluorescence microscopy showed intense staining for C3 and faint staining for IgG on the glomerular capillary wall and paramesangial area. Electron micrography showed subendothelial electron-dense deposits (EDDs), but hump-shaped subepithelial EDDs were not evident. PBV19-DNA was absent from renal tissue. Moreover, glomeruli showed positive staining for nephritis-associated plasmin receptor (NAPlr) and plasmin activity with similar distribution. Around 6 months after PVB19 infection, levels of anti-PVB19 IgM antibody spontaneously tuned negative with an apparent reduction of proteinuria and improvement of hypocomplementemia, although ASO level remained unchanged. This appears to represent the first description of positive glomerular staining for NAPlr in MPGN after PVB19 infection. Based on a review of 27 cases, including our own case, the MPGN lesions could be attributable to PVB19 infection. Clinicopathological features of this case were incompatible with post-streptococcal acute glomerulonephritis. We presume that a PBV19-derived glomerular pathogen that cross-reacts with anti-NAPlr antibody might be involved in the development of PVB19-related MPGN.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2024-12-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142827555","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}
{"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":"https://doi.org/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":""},"PeriodicalIF":1.0,"publicationDate":"2024-12-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142823984","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}
{"title":"Diffuse large B-cell lymphoma with rapid kidney enlargement after induction of hemodialysis in a patient with IgG4-related disease.","authors":"Shintaro Hara, Daisuke Morita, Ryoko Shibata, Yuki Yasui, Yoshiki Naito, Noriyoshi Fukushima, Seiya Kato, Noriko Uesugi, Yasuhiro Abe, Kosuke Masutani","doi":"10.1007/s13730-024-00957-0","DOIUrl":"https://doi.org/10.1007/s13730-024-00957-0","url":null,"abstract":"<p><p>A 76-year-old Japanese man was incidentally diagnosed with a pancreatic head tumor on computed tomography after surgery for colon cancer. He underwent pancreatoduodenectomy and was diagnosed with IgG4-related autoimmune pancreatitis. Concurrent chronic kidney disease gradually progressed and chronic hemodialysis was introduced 2 years later. Six months after the introduction of hemodialysis, follow-up abdominal computed tomography revealed marked enlargement of bilateral kidneys compared with previous images. Blood tests revealed persistent high IgG and IgG4 levels, and IgG4-related kidney disease was suspected. Thus, percutaneous kidney biopsy was performed. No evidence of IgG4-related kidney disease was detected, and a diagnosis of diffuse large B-cell lymphoma was made. Six courses of combination chemotherapy consisting of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone was effective, and the patient achieved and maintained complete remission for five years. This case highlights the need to consider the possible development of malignant lymphoma within several years after IgG4-related disease, especially in cases of autoimmune pancreatitis.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2024-12-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142823980","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}
{"title":"Fatal visceral disseminated varicella zoster virus infection during initial remission induction therapy in a patient with lupus nephritis: a case report and review of the literature.","authors":"Runa Takehara, Itaru Ebihara, Yoshifumi Honda, Norimasa Ooba, Hiromi Kurosawa, Chihiro Sato, Haruo Ohtani, Yutaka Tsutsumi, Masato Nose, Masaki Kobayashi","doi":"10.1007/s13730-024-00950-7","DOIUrl":"https://doi.org/10.1007/s13730-024-00950-7","url":null,"abstract":"<p><p>Visceral disseminated varicella zoster virus (VZV) infection is a severe complication, characterized by a notably high mortality rate. Herein, we present a case of a 36-year-old-man involving visceral disseminated VZV infection that emerged during remission induction therapy involving high-dose prednisolone (PSL), mycophenolate mofetil (MMF), and hydroxychloroquine for lupus nephritis. Two months after starting the immunosuppressive therapy, he experienced a rapid onset of severe upper abdominal pain. The following day, clinical manifestations and laboratory abnormalities rapidly deteriorated. Hyperferritinemia and hypertriglyceridemia, indicative of hemophagocytic lymphohistiocytosis (HLH), emerged, along with escalating liver and renal impairment and newly appeared disseminated intravascular coagulation, and multiple organ failure is suggested. The patient developed widespread blistering predominantly on the trunk and face, patient's condition failed to ameliorate, ultimately culminating in his demise a few hours later. At autopsy, the cutaneous lesions with blisters revealed positive immunostaining with anti-VZV antibody, and similar findings were detected in multiple organs. HLH was confirmed in lymph nodes. It is crucial to emphasize the awareness of visceral disseminated VZV, particularly in cases patients are undergoing concurrent PSL therapy alongside MMF for SLE. The progression of this fatal condition usually begins with abdominal pain, followed by a skin rash a few days later. The present case is the first to show evidence of HLH occurring as a result of visceral disseminated VZV infection. This disease is extremely rare but extremely serious, therefore, VZV-DNA should be measured in cases where you suspect this disease for early diagnosis and treatment.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2024-12-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142812006","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}
{"title":"Fanconi syndrome following administration of oral supplements containing red yeast rice: several months follow-up of three cases.","authors":"Reina Matsui-Hosoya, Koji Sato, Motohiro Yagasaki, Hitomi Hirose, Yusuke Fukao, Toshiki Kano, Hiroaki Io, Yusuke Suzuki","doi":"10.1007/s13730-024-00955-2","DOIUrl":"https://doi.org/10.1007/s13730-024-00955-2","url":null,"abstract":"<p><p>To date, the treatment strategy and long-term prognosis of acute kidney injury (AKI) after taking Benikoji CholesteHelp<sup>®</sup>, a red yeast rice supplement, remains unclear. We present three cases wherein renal dysfunction improved within a few months of supplement discontinuation, without immunosuppressive therapy. Case 1: A 59-year-old woman with a history of hypertension, treated with telmisartan (serum creatinine [sCr]: 0.65 mg/dL; estimated glomerular filtration rate [eGFR]: 71.3 mL/min/1.73 m<sup>2</sup>) and Benikoji CholesteHelp<sup>®</sup> for 7 months, developed Fanconi syndrome (FS) and severe renal impairment (sCr: 2.32 mg/dL; eGFR: 17.7 mL/min/1.73 m<sup>2</sup>). Renal biopsy and gallium-67 scintigraphy revealed no active drug-induced interstitial nephritis. Her condition improved significantly after supplement discontinuation. Her renal function gradually improved, with 3-month follow-up sCr and eGFR values of 0.96 mg/dL and 46.5 mL/min/1.73 m<sup>2</sup>, respectively; however, these were still worse than the pre-onset values. Case 2: A 48-year-old man had mild renal dysfunction (sCr: 1.12 mg/dL; eGFR: 56.76 mL/min/1.73 m<sup>2</sup>) after taking Benikoji CholesteHelp<sup>®</sup> for approximately 2 years; this was reversed upon supplement discontinuation. Case 3: A 47-year-old man with FS and mild renal dysfunction (sCr: 1.09 mg/dL; eGFR: 58.5 mL/min/1.73 m<sup>2</sup>) after taking Benikoji CholesteHelp<sup>®</sup> for approximately 4 months, showed notable improvement in FS after supplement discontinuation; however, the mild renal dysfunction persisted. The primary intervention in all cases was immediate supplement discontinuation, leading to rapid improvement in renal function, without need for immunosuppressive therapy. These findings increase our understanding of renal impairment caused by red yeast rice, with improvement after withdrawal, sometimes after several months.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2024-12-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142812033","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}
{"title":"Severe influenza A viral pneumonia in a hemodialysis patient: successful treatment with steroid pulse therapy.","authors":"Hiroki Ito, Sadatoshi Ito, Takuo Hirose, Tomoyoshi Kimura, Takefumi Mori, Sadayoshi Ito","doi":"10.1007/s13730-024-00951-6","DOIUrl":"https://doi.org/10.1007/s13730-024-00951-6","url":null,"abstract":"<p><p>Seasonal influenza is prevalent globally, particularly during winter months. It is well documented that this disease causes severe, often fatal complications in hemodialysis patients. While numerous reports have focused on novel influenza viruses, there is a paucity of case reports detailing seasonal influenza viral infections in this patient population. This case presents a 71-year-old male undergoing hemodialysis who developed severe seasonal influenza A pneumonia despite receiving the influenza vaccine and early antiviral treatment. Initially presenting with fever, cough, and myalgia, the patient was diagnosed with influenza A virus infection and hospitalized due to heightened risk associated with dialysis and an elevated inflammatory response. Despite treatment with two different antiviral medications, his condition deteriorated, leading to ARDS (acute respiratory distress syndrome). The administration of steroid pulse therapy resulted in significant clinical improvement. This case underscores the severe nature of influenza virus-related illnesses in dialysis patients, even with vaccination and early antiviral intervention. It also suggests the potential benefit of early steroid pulse therapy in managing severe influenza pneumonia in high-risk individuals.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2024-12-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142806297","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}
CEN Case ReportsPub Date : 2024-12-11DOI: 10.1007/s13730-024-00949-0
Chen-Xing Zhang, Lei Yin, You-Ying Mao, Zheng-Yu Zhou, Wei Zhou
{"title":"Microscopic polyangiitis in pediatric systemic lupus erythematosus: a unique presentation of pulmonary-renal syndrome and case report of an overlap syndrome.","authors":"Chen-Xing Zhang, Lei Yin, You-Ying Mao, Zheng-Yu Zhou, Wei Zhou","doi":"10.1007/s13730-024-00949-0","DOIUrl":"https://doi.org/10.1007/s13730-024-00949-0","url":null,"abstract":"<p><p>Secondary vasculitis is encountered in about one-third of all cases of systemic lupus erythematosus (SLE). Skin is most commonly involved in lupus-related small vasculitis. Although antineutrophil cytoplasmic autoantibodies (ANCA) associated vasculitis (AAV) is relatively uncommon, it can be the most dangerous manifestation associated with high mortality. SLE and AAV are separate diseases with different pathophysiologies and an overlap syndrome has only been reported a few times in previous literature. We present a unique case of a pediatric patient of pulmonary-renal syndrome, presenting with pulmonary alveolar hemorrhage and rapidly progressive glomerulonephritis. Serological and biopsy findings were suggestive of SLE and AAV occurring, simultaneously. Renal biopsy demonstrated necrotizing and crescentic glomerulonephritis, superimposed on diffuse segmental proliferative lupus glomerulonephritis class IV. The presentations of autoimmune diseases and vasculitis can be multi-systemic. Considering overlap syndromes, especially in patients with underlying connective tissue disease or systemic vasculitis, is vital for prompt therapy and prevention of morbidity in this population.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2024-12-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142806291","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}
{"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":"https://doi.org/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":""},"PeriodicalIF":1.0,"publicationDate":"2024-12-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142794514","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}
{"title":"Ceftriaxone encephalopathy in a very elderly dialysis patient.","authors":"Yumiko Nakamura, Ryo Yamamoto, Yuya Shioda, Takatsugu Iwashita, Taisuke Shimizu, Akito Maeshima, Hajime Hasegawa, Tomonari Ogawa","doi":"10.1007/s13730-024-00953-4","DOIUrl":"https://doi.org/10.1007/s13730-024-00953-4","url":null,"abstract":"<p><p>Ceftriaxone is widely used clinically but it can potentially cause ceftriaxone encephalopathy in individuals who are on dialysis. We describe ceftriaxone encephalopathy in a dialysis patient. The 87-year-old Japanese woman had a 9-year dialysis history. She was admitted to our department with right subcostal pain and lower back pain and was diagnosed with renal cyst hemorrhage and infection. Post-admission, we treated her with ceftriaxone (CTRX) 2 g/day and bed rest, which improved her symptoms. On the 7th hospitalization day, she began to exhibit incoherent speech and unresponsiveness; by day 10, her consciousness level had decreased to grade III and myoclonus appeared. Brain MRI and a cerebrospinal fluid examination ruled out cerebrovascular disease and encephalitis. EEG showed triphasic waves predominantly in the frontal lobe. Suspecting ceftriaxone encephalopathy, we switched the antibiotic to cefazolin 1 g/day. By day 12, the myoclonus decreased and the patient was able to communicate. By day 14, her consciousness level had improved to her admission level. We treated ceftriaxone encephalopathy in a dialysis patient. Although CTRX is generally thought to not require dose reduction in patients with renal failure (due to its hepatic excretion), caution is necessary as overdosage in dialysis patients can lead to myoclonus and encephalopathy.</p>","PeriodicalId":9697,"journal":{"name":"CEN Case Reports","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2024-12-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142799422","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}