This case report discusses the management of anti-neutrophil cytoplasmic antibodies (ANCA)-negative rapid progressive glomerulonephritis (RPGN) in a 68-year-old man with a complex medical history, presenting with fatigue, edema, and acute renal failure. Despite the absence of positive biomarkers for specific RPGN types, the clinical progression suggested microscopic polyangiitis, leading to intensive immunosuppressive therapy with cyclophosphamide and rituximab. The patient\'s condition was further complicated by the coexistence of nephritic and nephrotic syndromes, requiring nuanced management strategies, including prolonged hemodialysis. After initial treatment failure, remission was eventually achieved, allowing cessation of dialysis and significant recovery of renal function. This case highlights the challenges of diagnosing and managing ANCA-negative RPGN, particularly the importance of a tailored, dynamic approach to treatment in resource-limited settings. The recovery observed underscores the potential for renal function improvement even after prolonged periods of intensive therapy, reinforcing the need for persistence and adaptability in managing complex RPGN cases.

IgA nephropathy (IgAN) is a fairly common association with alcoholic liver disease. However, IgA vasculitis (IgAV) is quite an uncommon association with alcoholic liver cirrhosis and only a handful of cases have been reported in literature. Secondary IgAN usually presents in a docile manner, progressing slowly in about 5-25 years. It is usually responsive to steroid therapy, very rarely progressing to End-Stage Renal Disease. Here, we present a man in his late 50s, a known hypertensive and alcohol related liver-cirrhotic, who presented to our hospital with rash and rapidly progressive renal failure (RPRF). He was diagnosed with IgA nephritis with IgA vasculitis (IgAVN). His diagnosis was confirmed with skin and renal biopsy. He was started on renal replacement therapy for his renal failure and began oral steroid therapy. After administration of steroid therapy for 6 months, the patient recovered and was dialysis independent with stable renal parameters.

BACKGROUND: The concomitant occurrence of membranous nephropathy and anti-glomerular basement (anti-GBM) disease has been previously described but is extremely rare. However, delayed recognition or misdiagnosis leads to delayed treatment, resulting in worse renal and patient outcomes.
METHODS: We present 3 patients with rapidly progressive glomerulonephritis (RPGN), anti-GBM and serum-positive M-type phospholipase A2 receptor (anti-PLA2R) antibody. Renal biopsies revealed PLA2R-associated membranous nephropathy with anti-GBM glomerulonephritis. We analyzed the clinical and pathological characteristics and discussed that the correct diagnosis of membranous nephropathy with anti-GBM should rely on a combination of renal biopsy findings and serological testing. Despite aggressive treatment, one patient received maintenance hemodialysis, one patient progressed to CKD 3 stage, and the other patient died of cerebral infarction.
CONCLUSIONS: The simultaneous occurrence of membranous nephropathy and anti-GBM disease is extremely rare. The correct diagnosis of membranous nephropathy with anti-GBM relies on a combination of renal biopsy findings and serological testing. Early diagnosis is needed to improve the renal dysfunction.

A 75-year-old woman, with hypertension and atrial fibrillation but no prior renal history, presented to the hospital for chest discomfort and dyspnea. She was found to be in acute renal failure, with a serum creatinine of 5.1, increased from a baseline of 0.9, and urine analysis revealing proteinuria and hematuria with dysmorphic red blood cells. Subsequent work up was significant for positive perinuclear antineutrophil cytoplasmic antibody (p-ANCA) and myeloperoxidase antibodies. She underwent a renal biopsy, which revealed necrotizing crescents in 12 of 14 glomeruli, and she was diagnosed with rapidly progressive glomerulonephritis due to microscopic polyangiitis. Despite aggressive treatment with plasmapheresis, high-dose prednisone, and rituximab infusions, renal function worsened, and she required initiation of hemodialysis. She was ultimately discharged after a three-week admission, with plans to continue rituximab infusions and three times weekly hemodialysis in the outpatient setting. Due to her poor response to traditional therapies, initiation of a new targeted immunomodulator known as avacopan, a complement 5a receptor antagonist, was considered. Such targeted immunomodulators are also of particular interest as possible ways to reduce the risk of severe infection associated with current broad immunosuppressive modalities. In addition, when used in place of steroids, they reduce the morbidity associated with cumulative glucocorticoid toxicity. For patients with ANCA-associated vasculitis refractory to standard therapies, targeted immunomodulators such as avacopan should be considered as alternative or adjunct therapy.

UNASSIGNED: Concomitant native and prosthetic valve infective endocarditis (IE) is very rare, and both can rarely be complicated by rapidly progressive glomerulonephritis (RPGN). This diagnosis has therapeutic implications, as not all RPGN need immunosuppression therapy.
UNASSIGNED: Native and prosthetic valve infective endocarditis (IE) may be rarely complicated by rapidly progressive glomerulonephritis (RPGN). The diagnosis of IE as a cause of RPGN may be missed, and patients may be subjected to inappropriate immune suppressive therapy. Moreover, IE involving multi-valves has rarely been described, and there are only few case reports of simultaneous native and prosthetic valve endocarditis. Here, we present a case of 34-year-old female patient who has RPGN and whose initial workup missed IE. However, further workup revealed a diagnosis of native and prosthetic valve IE and our patient, who would have been subjected to inappropriate immune suppressive therapy, was treated with intravenous antibiotics alone and discharged with improvement.

Objective Patients with rapidly progressive glomerulonephritis (RPGN) are at a high risk of progression to end-stage kidney disease (ESKD), requiring renal replacement therapy (RRT). The present study examined recent trends in the incidence of RRT due to RPGN in Japan. Methods The number of patients with incident RRT due to RPGN by sex from 2006 to 2021 was extracted from the Japanese Society of Dialysis Therapy Registry. The incidence rates of RRT were calculated for four-year periods with the census population as the denominator. Standardized incidence ratios (SIRs) and age-specific incidence rates were also calculated. Results From 2006 to 2021, the crude number of patients with incident RRT due to RPGN increased by 34% and 58% in men and women, respectively. The SIRs decreased significantly in 2010-2013 relative to the first period (2006-2009) for both men (0.90 [95% confidence interval {CI} 0.85-0.96]) and women (0.92 [0.86-0.99]) but then increased to 1.01 (0.96-1.07) for men and 1.20 (1.13-1.27) for women in 2018-2021. In the older age groups (≥70 years old), age-specific incidence rates initially decreased in 2010-2013 but increased thereafter, peaking in 2018-2021. Conclusion From 2006 to 2021, the number of patients with incident RRT due to RPGN increased, with an increase in the age-specific incidence of RRT due to RPGN in the older age groups (≥70 years old), suggesting that the number of patients with incident RRT due to RPGN will continue to increase as the population ages in Japan.

BACKGROUND: Crescentic glomerulonephritis with syphilis infection is rare, and the mechanism underlying the formation of glomerular capillary wall damage-induced crescent has not been elucidated.
METHODS: A 62-year-old Japanese male showed edema, eruption, and rapid deterioration of the renal function after an acute syphilis infection. A renal biopsy showed crescentic glomerulonephritis with C3 deposition in the glomerular capillary wall, and immunostaining for anti-Treponema pallidum antibody was weakly positive in some interstitium and one glomerulus. Electron microscopy revealed the presence of string-shaped structures in the glomerular capillary walls. After treatment with penicillin followed by prednisolone, the renal function and urinary abnormalities, including Treponema pallidum protein, disappeared.
CONCLUSIONS: Crescentic glomerulonephritis associated with syphilis showed a string-shaped deposition in the glomerular capillary and urinary Treponema pallidum protein excretion, and was effectively treated with penicillin and prednisolone.

Sulfatides are a type of sulfated glycosphingolipid that are secreted with lipoproteins into the serum. These molecules are involved in the inflammatory pathway of vessels in addition to coagulation and platelet aggregation. Previous studies have proposed that sulfatides play a pivotal role in regulating inflammation-related disorders. Systemic vasculitis (SV) diseases are generally caused by autoimmune diseases and often involve kidney vasculitis, which may lead to rapidly progressive kidney dysfunction and end-stage kidney disease. Our earlier pilot study revealed that the level of serum sulfatides (SSs) was significantly decreased in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV), a representative disease-causing SV with kidney involvement (SVKI), especially in patients exhibiting active crescentic findings on kidney biopsy. To further explore the clinical significance of an association between SS and SVKI, we analyzed and compared the SS level of patients with various SVKI diseases in this retrospective cohort study. Among patients admitted to our hospital between 2008 and 2021, we ultimately enrolled 26 patients with IgA vasculitis (IgAV), 62 patients with AAV, and 10 patients with anti-glomerular basement membrane disease (GBM) as examples of SVKI diseases, as well as 50 patients with IgA nephropathy (IgAN) and 23 donors for living kidney transplantation as controls. The mean ± standard deviation SS level in the donor, IgAN, IgAV, AAV, and GBM groups was 8.26 ± 1.72, 8.01 ± 2.21, 6.01 ± 1.73, 5.37 ± 1.97, and 2.73 ± 0.99 nmol/mL, respectively. Analysis of patients in the SVKI disease group showed that those with the crescentic class kidney biopsy finding exhibited a significantly lower SS level than did those with other class biopsy features. Additionally, the SS level had a higher detection ability for SVKI patients with crescentic class kidney biopsy findings (area under the receiver operating characteristic curve 0.90, 95% confidence interval 0.82-0.99) than did several other predictor candidates. Our results indicate that the SS level is decreased in more severe SVKI diseases and may be associated with active glomerular lesions in SVKI kidney biopsy samples.

Granulomatous polyangiitis (GPA) is a rare autoimmune disease that can involve multiple systems throughout the body, including the ear, nose, upper and lower respiratory tracts. It is classified as an antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Telitacicept is a novel recombinant fusion protein targeting B-lymphocyte stimulator (BLyS). Telitacicept can inhibit the development and maturation of abnormal B cells by blocking BLyS, and inhibit the production of antibodies by abnormal plasma cells by blocking APRIL (A proliferation-inducing ligand), which is expected to become a new drug for the treatment of GPA. We report a 64-year-old man diagnosed at our hospital with GPA involving multiple systems including kidneys, lungs, nose and ears. Renal involvement was severe, with a clinical characteristic of rapidly progressive glomerulonephritis and a pathologic manifestation of crescentic nephritis with plasma cell infiltration. The patient was treated with hormones, immunoglobulins and cyclophosphamide (CYC) with the addition of telitacicept and a rapid reduction in hormone dosage. The patient\'s renal function improved significantly within a short period of time, and his hearing and lung lesions improved significantly. At the same time, he did not develop serious infections and other related complications. Our report suggests that short-term control of the patient\'s conditions is necessary in GPA patients with organ-threatening disease. Telitacicept combined with CYC and glucocorticoids may be an induction therapy with safety and feasibility. However, more clinical trials are needed to validate the efficacy and safety of the therapeutic regimen.

ANCA-associated Vasculitides (AAV) are characterized by small vessel necrotizing inflammation and can present with multisystem organ involvement, including organ/life threatening manifestations of rapidly progressive glomerulonephritis and diffuse alveolar haemorrhage, where immediate and aggressive intervention is needed to prevent further organ damage. Although, the rationale of plasma exchange (PLEX) in AAV is strong, through removing the pathogenic ANCAs; target either myeloperoxidase (MPO) or proteinase 3 (PR3), and other inflammatory molecules, especially in the initiation when the immunosuppressive treatment is no sufficient to prevent the organ damage, overall impact on patient outcomes is not well-established, while the risk of infections seems to be higher in the PLEX-treated patients. A comprehensive overview of the challenges and uncertainties surrounding the use of PLEX in the management of AAV will be reviewed, providing the current practice recommendations guiding treatment decisions.