Background: Idiopathic membranous nephropathy (IMN) is one of the most common causes of nephrotic syndrome in adults. A proportion of patients will experience spontaneous remission and the decision to offer immunosuppression is guided by the presence of adverse prognostic features. Data relating to the efficacy of different immunosuppressive protocols is lacking, in particular there are little data available on the efficacy or benefits of an intravenous (IV) cyclophosphamide-based regimen. Since 2010, our unit has been using a treatment regimen based on IV cyclophosphamide and oral prednisolone for patients with IMN associated with adverse prognostic features. The outcomes of these patients were compared with a historic cohort of similar patients who did not receive immunosuppressive therapy.
Methods: Between January 2010 and 2014, a total of 41 patients were treated with pulse IV cyclophosphamide and oral prednisolone. The historical comparator group included 47 similar patients diagnosed between 2006 and 2010 who did not receive immunosuppression. Two-year follow-up data were collected. The primary outcome measure was time to remission of nephrotic syndrome (defined as normalization of serum albumin). Secondary outcomes included rate of progression of kidney disease as well as incidence of treatment-related adverse events.
Results: As compared with supportive care alone, treatment with IV cyclophosphamide and oral prednisolone was associated with a significantly higher number of patients achieving remission. Within 18 months of therapy, 74% of treated patients had achieved a normal serum albumin level. Though there was a trend towards a more rapid decline in estimated glomerular filtration rate in the untreated cohort, this did not reach statistical significance. The IV cyclophosphamide-based regimen was well tolerated, with few significant treatment-associated side effects.
Conclusion: IV cyclophosphamide is a safe and effective treatment for IMN.
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http://dx.doi.org/10.1093/ckj/sfw152 | DOI Listing |
CEN Case Rep
January 2025
Department of Nephrology and Dialysis, Tokyo Metropolitan Institute for Geriatrics and Gerontology, 35-2 Sakae-Cho, Itabashi, Tokyo, 173-0015, Japan.
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.
View Article and Find Full Text PDFOral Oncol
January 2025
Department of Oncology, Xiang'an Hospital of Xiamen University, Xiamen, People's Republic of China.
This case report describes a 4.5-year-old girl diagnosed with a rare Undifferentiated Small Round Cell Sarcoma (USRCS) originating in the parapharyngeal space with multiple lung metastases. Diagnostic workups, including imaging, immunohistochemistry, and genetic sequencing, identified the tumor as an unclassified subtype of USRCS.
View Article and Find Full Text PDFActa Oncol
January 2025
Comprehensive Cancer Center Munich and Department of Medicine III, University Hospital, LMU Munich, Munich, Germany; Bavarian Cancer Research Center (BZKF), Munich, Germany.
Background: The prognosis of patients with advanced soft tissue sarcoma (STS) remains dismal. Trofosfamide (TRO) has been proposed as a well-tolerated oral maintenance therapy. This retrospective analysis aims to determine the value of this therapy.
View Article and Find Full Text PDFMedicine (Baltimore)
January 2025
Division of Nephrology, Department of Medicine, National University Hospital, Singapore.
Rationale: We report the efficacy of combination prednisolone and intravenous (IV) rituximab as an immunosuppressive regimen for a young male presenting with extensive venous thromboembolism including a submassive pulmonary embolism secondary to life-threatening nephrotic syndrome from very high risk anti-phospholipase-A2 receptor (PLA2R) positive membranous nephropathy. Initial treatment was with mechanical thrombectomy and anticoagulation. Thereafter, oral prednisolone was initiated to induce remission, during a period of uninterrupted anticoagulation.
View Article and Find Full Text PDFAm J Hematol
January 2025
Hematopoietic Stem Cell Transplant and Cellular Therapy Program, Division of Hematology/Oncology, Department of Medicine, Chao Family Comprehensive Cancer Center, University of California Irvine, Irvine, California, USA.
Oral budesonide exerts local effects with negligible systemic glucocorticoid activity, due to rapid first-pass metabolism, therefore, could potentially be efficacious in preventing gastrointestinal (GI) acute GVHD (aGVHD). We explored the use of budesonide, added to posttransplant cyclophosphamide (PTCy), tacrolimus, and mycophenolate mofetil, for prevention of GI aGVHD after allogeneic hematopoietic stem cell transplantation (AHSCT) in a prospective observational study and treated 80 patients with a median age of 53 years (range 19-74). Results were compared with a publicly available CIBMTR dataset of 646 patients who received PTCy-based GVHD prophylaxis (CIBMTR Study # GV17-02) (control).
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