The Pulsing Paradox: Successful Steroid Therapy in Infection-Related Glomerulonephritis.

The patterns of infection-related glomerulonephritis (IRGN) are rapidly changing in terms of age at presentation and sources of infection. The existing literature on the use of steroids in IRGN is inconsistent. A diabetic male in his sixties presented with features of anasarca, bilateral flank pain, and acute pulmonary edema. He had a non-healing ulcer over his right leg, with pus culture showing growth of methicillin-resistant Staphylococcus aureus (MRSA). Computed tomography (CT) of the kidneys, ureter, and bladder (KUB) showed features of bilateral pyelonephritis. The patient went on to develop acute renal failure and eventually required hemodialysis. A renal biopsy was performed, and features of IRGN with crescents were noted. Considering the presence of crescents in renal biopsy, a trial of steroids was given under antibiotic cover, which resulted in a near-complete resolution of renal failure.

Protein A immunoadsorption for anti-glomerular basement membrane nephritis.

Anti-glomerular basement membrane (GBM) nephritis is a rare autoimmune disorder characterized by acute and rapidly progressive glomerulonephritis. In this report, we present the case of a 52-year-old woman with anti-GBM nephritis who was treated with Staphylococcus Protein A immunoadsorption in combination with glucocorticoids and cyclophosphamide. After 8 cycles of immunoadsorption, the patient's anti-GBM antibodies decreased from 363 AU/mL to less than 20 AU/mL, accompanied by a dropped immunoglobin G level, although renal impairment persisted. We reviewed the therapeutic options for anti-GBM nephritis and compared plasma exchange, double filtration plasmapheresis, and immunoadsorption with regard to plasma consumption, allergic events, and plasma components loss. Protein A immunoadsorption appears to be a promising treatment modality for anti-GBM nephritis.

Lupus Nephritis Patterns and Response to Type I Interferon in Patients With DNASE1L3 Mutations: Report of Three Cases.

DNASE1L3 is an extracellular nuclease that digests chromatin released from apoptotic cells. DNASE1L3 mutations impair the enzyme function, enhance autoantibody production and type I interferon (IFN-I) responses, and cause different autosomal recessive phenotypes ranging from hypocomplementemic urticarial vasculitis syndrome to full-blown systemic lupus erythematosus (SLE). Kidney involvement in patients with DNASE1L3 mutations is poorly characterised. Herein, we describe the clinical course of three children with monogenic SLE due to DNASE1L3 mutations who developed refractory glomerulonephritis leading to kidney failure. They had different renal histopathological patterns (i.e., membranous, endo- and extra-capillary glomerulonephritis and thrombotic microangiopathy), all belonging to the lupus nephritis (LN) spectrum. One patient had a mixed phenotype, showing an overlap between SLE and ANCA-associated vasculitis. Using immunofluorescence, we detected glomerular expression of the IFN I-induced human myxovirus resistance protein 1 (MXA), which was particularly evident in glomerular endothelial cells. 2/3 patients had increased expression of interferon-stimulated genes in the peripheral blood and all three patients had reduced serum DNAse activity. Our findings suggest that DNASE1L3-related glomerulonephritis can be included in the spectrum of IFN I-mediated kidney disorders, and provide the rationale for IFN I-directed therapies in order to improve the poor outcome of this rare condition.

Rapidly Progressive Glomerulonephritis Secondary to Immunoglobulin M-associated Monoclonal Gammopathy of Renal Significance: A Report of a Rare Case.

Monoclonal gammopathy of undetermined significance (MGUS) is a premalignant condition characterized by monoclonal paraprotein production, with IgM and non-IgM variants. While IgM MGUS is often associated with lymphoid neoplasms, non-IgM MGUS can progress to multiple myeloma. Comorbidities include bone mineral density loss and renal complications, such as monoclonal gammopathy of renal significance (MGRS) and peripheral neuropathy. Cardiovascular risks are also elevated. Despite its significance, MGUS often goes undiagnosed due to its asymptomatic nature and overlap with age-related comorbidities. We present a case of IgM MGRS manifesting as rapidly progressive glomerulonephritis, highlighting the diagnostic challenges and clinical implications of MGUS-associated complications.

Anti-neutrophil Cytoplasmic Antibody-Negative Rapid Progressive Glomerulonephritis With Mild Pathological Presentation in an Older Patient: A Case Report.

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.

An Unusual Case of Alcoholic Liver Disease Associated with Secondary IgA Vasculitic Nephritis presenting as Rapidly Progressive Glomerulonephritis.

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.

Diagnosis of membranous nephropathy with Anti-GBM glomerulonephritis: a case series report.

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. CASE PRESENTATION: 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. CONCLUSION: 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.

Native and prosthetic valve infective endocarditis complicated by rapidly progressive glomerulonephritis and its diagnostic challenges and therapeutic implications.

Key Clinical Message: 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. Abstract: 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.

A Refractory Case of Rapidly Progressive Glomerulonephritis Due to Microscopic Polyangiitis.

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.

Very Slowly Progressive Microscopic Polyangiitis: Comparative Analysis with Rapidly Progressive Forms.

Microscopic polyangiitis (MPA) is predominantly characterized by rapidly progressive glomerulonephritis (RPGN) associated with myeloperoxidase anti-neutrophil cytoplasmic antibodies (MPO-ANCA). Nonetheless, up to 30% of cases of ANCA-associated vasculitis (AAV) may exhibit a more indolent progression toward renal failure, an aspect less frequently discussed and understood in medical literature. This study seeks to clarify the clinical and pathological distinctions between the slowly and rapidly progressive forms of MPA, thereby enhancing understanding of their distinct pathogeneses and treatment responses. We conducted a comparative analysis of two patients diagnosed with MPA under the 2022 American College of Rheumatology/the European Alliance of Associations for Rheumatology (ACR/EULAR) classification. Evaluations included laboratory tests such as serum creatinine levels, serology for MPO-ANCA, and renal biopsies. Patient 1 exhibited a mere 1.07% decrease in estimated glomerular filtration rate (eGFR) over 6 months, significantly below the RPGN threshold, and demonstrated sclerotic glomerular pathology without active inflammation. This patient also showed lower levels of MPO-ANCA, Birmingham Vasculitis Activity Score (BVAS), and C-reactive protein. Conversely, Patient 2 experienced an 89.9% reduction in eGFR over the same timeframe, accompanied by acute systemic inflammation. The comparative clinical analysis of these cases illuminates clear differences in disease activity. Slowly progressive MPA is marked by lesser disease activity that fosters chronic inflammation, leading to a more gradual decline in renal function. Early diagnosis, facilitated by initial measurements of MPO-ANCA, can enhance disease management and improve patient outcomes.

Trends in the Incidence of Renal Replacement Therapy due to Rapidly Progressive Glomerulonephritis in Japan, 2006-2021.

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.

Case report: renal recovery in Goodpasture's syndrome treated with rituximab.

Successful induction of remission in anti-glomerular basement membrane (anti-GBM) glomerulonephritis can be obtained by using rituximab as a first-line immunosuppressive agent. We report the case of a 20-year-old male patient with Goodpasture's (anti-GBM) syndrome, with poor prognostic factors at presentation including intra-alveolar hemorrhage and dialysis-dependent rapidly progressive glomerulonephritis. The diagnosis was confirmed on kidney biopsy and serology (anti-GBM antibody titer). Rituximab was used as the first-line immunosuppressive agent in combination with pulse corticosteroids and plasmapheresis, to avoid potential side effects of cyclophosphamide. Anti-GBM antibody titers became undetectable after initiating rituximab. No adverse events were reported, and the patient became dialysis-independent after 6 months. This case reports the successful remission of a patient with Goodpasture's syndrome after induction with rituximab.

Relevance of histopathological findings for predictive scoring of short-term treatment response to plasma exchange in severe ANCA-associated renal vasculitides.

Introduction: Rapidly progressive glomerulonephritis (RPGN) is characterized by a rapid loss of kidney function, affecting both renal and overall patient survival. Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a small vessel vasculitis affecting multiple organ systems including the kidney, and among most frequent causes of RPGN. We here aimed to validate a recently described scoring system for short-term treatment response to therapeutic plasma exchange (PLEX) in a well-characterized and independent cohort of severe renal AAV presenting with RPGN. Furthermore, we compared this scoring with established classification systems in renal AAV including histopathological findings. Methods: We here directly compare the scoring system with retrospective data about PLEX treatment in our own clinical practice and according to current recommendations in a cohort of 53 patients with severe AAV presenting with RPGN confirmed by kidney biopsy. Results: We here confirm that PLEX scoring is capable to identify patients at risk for short-term poor outcome in severe AAV presenting with RPGN (p<0.0001). Furthermore, multiple stepwise regression analysis revealed that the PLEX score with renal biopsy performed best to predict poor outcome in this patient population (p<0.0001). Conclusion: Our observations underscore the relevance of performing a kidney biopsy in this patient population that is often challenged in the setting of intensive care treatment, requirement of KRT with need for anticoagulation and bleeding risk. Therefore, validation of our observations and this recent scoring system for treatment response to PLEX in independent cohorts would be of great clinical relevance in the treatment of patients with severe AAV presenting with RPGN.

Plasma exchange in patients with ANCA-associated vasculitis.

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.

Combination treatment with telitacicept, cyclophosphamide and glucocorticoids for severe Granulomatous polyangiitis: a case report and literature review.

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.

Serum sulfatide level is associated with severe systemic vasculitis with kidney involvement.

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.

Crescentic glomerulonephritis associated with syphilis: a case report and review of the literature.

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. CASE PRESENTATION: 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.

Renal involvement in eosinophilic granulomatosis with polyangiitis.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a necrotizing vasculitis, which typically affects small-to medium-sized blood vessels. It is characterized by the presence of tissue infiltrates rich in eosinophils, along with the formation of granulomatous lesions. About 40% of cases have positive anti-neutrophil cytoplasm antibodies (ANCA), with predominant perinuclear staining, and anti-myeloperoxidase (anti-MPO) specificity in about 65% of cases. Typical manifestations of EGPA include the late onset of asthma, nasal and sinus-related symptoms, peripheral neuropathy, and significant eosinophilia observed in the peripheral blood. In contrast to granulomatosis with polyangiitis and microscopic polyangiitis, renal involvement in EGPA is less frequent (about 25%) and poorly studied. Necrotizing pauci-immune crescentic glomerulonephritis is the most common renal presentation in patients with ANCA-positive EGPA. Although rarely, other forms of renal involvement may also be observed, such as eosinophilic interstitial nephritis, mesangial glomerulonephritis, membranous nephropathy, or focal sclerosis. A standardized treatment for EGPA with renal involvement has not been defined, however the survival and the renal outcomes are usually better than in the other ANCA-associated vasculitides. Nonetheless, kidney disease is an adverse prognostic factor for EGPA patients. Larger studies are required to better describe the renal involvement, in particular for patterns different from crescentic glomerulonephritis, and to favor the development of a consensual therapeutic approach. In this article, in addition to personal data, we will review recent findings on patient clinical phenotypes based on ANCA, genetics and the impact of biological drugs on disease management.

Atypical form of Goodpasture's disease.

Goodpasture's disease and anti-glomerular basement membrane nephritis (anti-GBM nephritis) are rare autoimmune small vessel vasculitis predominantly affecting young men. Goodpasture's disease plays an important part in differential diagnosis of pulmonary - renal syndrome. The evidence of circulating autoantibodies, a typical histological appearance of the kidney biopsy with finding of the crescent glomerulonephritis and clinical presentation of nephritic syndrome play an important role in diagnosis. Our case report describes a case of a young male with anti-GBM nephritis that presents as rapidly progressive glomerulonephritis (RPGN) with progression to dialysis-dependent renal failure. The atypical sign of the case was development of nephrotic syndrome with volume-dependent hypertension. The case was complicated by heparin-induced thrombocytopenia. During combined immunosuppressive therapy with plasmapheresis, the condition of the patient has stabilized. However, renal function did not recover. This previously fatal disease has nowadays a very good survival rate because of immunosuppresion therapy, plasmapheresis and hemodialysis.

A Fatal Case of Rapidly Progressive Glomerulonephritis With Two Anti-neutrophil Cytoplasmic Antibodies and Anti-glomerular Basement Membrane Antibody: A Description of Autopsy Findings.

A 79-year-old man presented with dyspnea upon exertion, marked renal dysfunction, proteinuria, and hematuria. He was diagnosed with rapidly progressive glomerulonephritis. Serological tests were positive for MPO-ANCA, PR3-ANCA, and anti-GBM antibodies. Since the anti-GBM antibody titer was significantly higher than the ANCA titer and the renal dysfunction was severe, we initially assumed anti-GBM disease and started treatment. Due to poor general condition, a definitive diagnosis could not be made by renal biopsy. Corticosteroid therapy, plasmapheresis, and cyclophosphamide treatment were performed. However, renal function did not improve, and hemodialysis was required. He died of sepsis during treatment. An autopsy was performed with the consent of the family. Renal pathological examination revealed fibrocellular crescent formation in the glomeruli. Immunofluorescence revealed no major deposition in the glomeruli, suggesting ANCA-associated nephritis but not anti-GBM disease. Gross pathological findings of the abdominal aorta showed that a part of the artificial blood vessel had formed a pseudoaneurysm and abscess. There is no evidence of inflammatory cell infiltration or vasculitis in the alveoli. Pathological findings in the other organs did not suggest vasculitis. The renal prognosis of this case could have been improved with appropriate treatment if early diagnosis by renal biopsy had been made. There have been case reports of triple-seropositive rapid progressive glomerulonephritis (RPGN). We report a rare autopsy case of triple-seropositive RPGN.