Investigation of the Effect of Therapeutic Plasma Exchange for TAFRO Syndrome: A Pilot Study.

TAFRO syndrome is a rare systemic inflammatory disorder with a fatal course. Nevertheless, a definitive treatment strategy has not yet been established. Anti-inflammatory therapies, including glucocorticoid treatment and immunosuppressants, have not been satisfactory. Therefore, new treatment options are needed for patients with TAFRO syndrome. The effectiveness of therapeutic plasma exchange (TPE) has mainly been reported in several case reports. In this case series study, we investigated the effect of TPE on TAFRO syndrome. We reviewed six consecutive cases with TAFRO syndrome treated at Shinshu University Hospital. All of them underwent TPE. A significant improvement in mean blood pressure, albumin, total bilirubin, and C-reactive protein was observed after TPE. Furthermore, early TPE treatment was suggested to have an impact on the prognosis. More intensive studies are needed to emphasize the overall conclusion obtained that TPE can be an effective/acceptable treatment option for TAFRO syndrome.

Impact of Vascular Access Flow Suppression Surgery on Cervical Artery Circulation: A Retrospective Observational Study.

Vascular access (VA) flow suppression surgery augments VA flow resistance and can increase other circulation flows hindered by high-flow VA. However, whether VA flow suppression surgery affects cervical circulation has rarely been reported. We aimed to determine the effect of VA flow suppression surgery on the cervical circulation in patients with high-flow VA. This single-center, retrospective, observational study included 85 hemodialysis patients who underwent VA flow suppression surgery at the Kanno Dialysis and Access Clinic between 2009 and 2018. Blood flow in the VA, bilateral vertebral arteries, and common carotid artery was measured before and after VA flow suppression surgery. The VA flow decreased from 1548 mL/min to 693 mL/min postoperatively. The flow of the vertebral artery on the VA side increased from 55 mL/min to 81 mL/min. The flow in the bilateral common carotid arteries also increased. Patients whose symptoms improved postoperatively showed better improvement in the vertebral artery on the VA side. VA flow suppression surgery in patients with high-flow VA increases the flow of the vertebral artery on the VA side and of the bilateral common carotid arteries. High-flow VA can hinder the vertebral and common carotid circulation.

Investigation of Clinical Features and Association between Vascular Endothelial Injury Markers and Cytomegalovirus Infection Associated with Thrombotic Microangiopathy in Patients with Anti-Neutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitis: Case-Based Research.

Anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) can occasionally trigger thrombotic microangiopathy (TMA). Cytomegalovirus (CMV) may be reactivated during intensive immunosuppressive therapy for AAV and cause TMA. Therefore, we aimed to evaluate the clinical features of and the association between vascular endothelial injury markers and TMA due to CMV in patients with AAV. A 61-year-old female was diagnosed with AAV and severe kidney injury. Immunosuppressive therapy gradually improved her symptoms and laboratory findings. However, 2 weeks after induction therapy initiation, she exhibited altered consciousness, a significant decrease in platelet count, and hemolytic anemia, resulting in a TMA diagnosis. Plasma exchange did not improve TMA findings and routine screening test revealed CMV infection. Ganciclovir injection improved the infection and TMA findings. Consequently, we diagnosed her with CMV-induced TMA. Both AAV and CMV may induce severe vascular endothelial injury, potentially leading to TMA development. CMV-induced TMA should be considered when TMA develops during induction therapy against AAV. Moreover, of the three serum markers of vascular injury-serum sulfatides, soluble thrombomodulin, and pentraxin 3-serum sulfatides may be associated with the development of TMA, and a high level of soluble thrombomodulin may be associated with the development of CMV viremia during the clinical course of AAV.

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.

Lysinuric protein intolerance exhibiting renal tubular acidosis/Fanconi syndrome in a Japanese woman.

Lysinuric protein intolerance (LPI), caused by pathogenic variants of SLC7A7, is characterized by protein aversion, failure to thrive, hyperammonemia, and hepatomegaly. Recent studies have reported that LPI can cause multiple organ dysfunctions, including kidney disease, autoimmune deficiency, pulmonary alveolar proteinosis, and osteoporosis. We report the case of a 47-year-old Japanese woman who was initially diagnosed with renal tubular acidosis (RTA), Fanconi syndrome, and rickets. At the age of 3 years, she demonstrated a failure to thrive. Urinary amino acid analysis revealed elevated lysine and arginine levels, which were masked by pan-amino aciduria. She was subsequently diagnosed with rickets at 5 years of age and RTA/Fanconi syndrome at 15 years of age. She was continuously treated with supplementation of vitamin D3, phosphate, and bicarbonate. A renal biopsy at 18 years of age demonstrated diffuse proximal and distal tubular damage with endocytosis-lysosome pathway abnormalities. Distinctive symptoms of LPI, such as protein aversion and postprandial hyperammonemia were not observed throughout the patient's clinical course. The patient underwent a panel-based comprehensive genetic testing and was diagnosed with LPI. As the complications of LPI involve many organs, patients lacking distinctive symptoms may develop various diseases, including RTA/Fanconi syndrome. Our case indicates that proximal and distal tubular damages are notable findings in patients with LPI. The possibility of LPI should be carefully considered in the management of RTA/Fanconi syndrome and/or incomprehensible pathological tubular damage, even in the absence of distinctive symptoms; furthermore, a comprehensive genetic analysis is useful for diagnosing LPI.

A Case of an Elderly Woman Who Developed Corneal Perforation in the Clinical Course of Myeloperoxidase Positive Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.

Antineutrophil cytoplasmic antibody- (ANCA-) associated vasculitis (AAV) is a systemic vasculitis characterized by ANCA positivity and categorized into three main types: microscopic polyangiitis, granulomatosis with polyangiitis, and eosinophilic granulomatous with polyangiitis. Although AAV leads to systemic organ injury, such as of the lungs, kidneys, nerves, and skin, patients with AAV sometimes develop ocular lesions. Here, we report the case of an elderly woman who had been treated for AAV for seven years. She developed scleritis and relapsed twice, with elevation of serum disease markers such as ANCA titer and C-reactive protein. After the decline of these markers due to treatment with additional medication, her scleritis relapsed again and caused a corneal ulcer, which resulted in perforation without obvious marker elevation. She did not present with any symptoms of organ injury, except for ocular lesions. She was treated with surgery, followed by methylprednisolone and rituximab therapy. Subsequently, her ocular lesions and symptoms improved, and she did not relapse. AAV can cause various ocular manifestations. Although C-reactive protein and ANCA titers are useful markers of disease activity and the relapse of AAV complications, including ocular lesions, these markers do not always increase at the time of worsening ocular lesions. Therefore, it is important for clinicians treating patients with AAV to pay careful attention to serum data and physical findings, including the eyes.

A case of emphysematous pyelonephritis in an older man with poorly controlled type 2 diabetes mellitus.

Emphysematous pyelonephritis (EPN) is a necrotizing bacterial infection characterized by gas retention and a poor prognosis. We present the case of a 75-year-old man who was diagnosed early with EPN and received multidisciplinary treatment. He had poorly controlled type 2 diabetes mellitus (DM) and chronic kidney disease (CKD), and was treated with oral hypoglycemic drugs, including a sodium-glucose co-transporter-2 inhibitor. He experienced the onset of back pain in the midsection of his back, tenderness in the costovertebral angle, and a high fever (> 39 °C), accompanied by tachycardia, hypotension, and tachypnea. The patient was diagnosed with pyelonephritis and septic shock. Immediate measures encompassing empirical antibiotic therapy, administration of noradrenaline, blood glucose regulation, and urethral catheterization were implemented. However, due to the persistent fever (> 38 °C) and lack of improvement in his condition, abdominal computed tomography (CT) was repeated on the fourth day. This revealed the presence of gas around the right kidney parenchyma, leading to a diagnosis of EPN that had evolved from acute pyelonephritis. Subsequently, percutaneous drainage of the right kidney parenchyma was performed. Subsequently, multidisciplinary treatment was continued, and his condition gradually improved. Clinicians should evaluate abdominal CT when acute pyelonephritis does not improve within a few days of antibiotic therapy. Disease progression from acute pyelonephritis to EPN should be considered in patients with DM and CKD.

Novel and highly reliable leak check tests for drop- and external pressure-type cell-free and concentrated ascites reinfusion therapy.

INTRODUCTION: For safe management of cell-free and concentrated ascites reinfusion therapy (CART), a highly reliable leak test for detecting ascites filter damage is essential. However, such a test has not been established for drop-type CART. METHODS: We devised two novel leak tests for drop- and external pressure-type CART, manual or pump pressurization methods, using high-pressure loading and pressure monitoring, and investigated their reliability. RESULTS: Both methods could easily load and maintain sufficiently high pressure (>400 Torr) on the hollow fibers for 2 min. No result deviation was noted between different operators. The pressure drops in both methods were identical and significantly lower than those in the leak test using a special CART machine, the e-CART. CONCLUSION: The reliability of our revised leak test is equivalent to that of the automatic leak test of e-CART. These highly reliable leak tests may contribute to safety in patients undergoing drop- and external pressure-type CART.

Immunological risk and complement genetic evaluations in early onset de novo thrombotic microangiopathy after living donor kidney transplantation: A Japanese multicenter registry.

BACKGROUND: Thrombotic microangiopathy (TMA) after kidney transplantation (KTx), particularly early onset de novo (dn) TMA, requires immediate interventions to prevent irreversible organ damage. This multicenter study was performed to investigate the allogeneic clinical factors and complement genetic background of dnTMA after KTx. METHODS: Perioperative dnTMA after KTx within 1 week after KTx were diagnosed based on pathological or/and hematological criteria at each center, and their immunological backgrounds were researched. Twelve aHUS-related gene variants were examined in dnTMA cases. RESULTS: Seventeen recipients (15 donors) were enrolled, and all dnTMA cases were onset within 72-h of KTx, and 16 of 17 cases were ABO incompatible. The implementation rate of pre-transplant plasmaphereses therapies were low, including cases with high titers of anti-A/anti-B antibodies. Examination of aHUS-related gene variants revealed some deletions and variants with minor allele frequency (MAF) in Japan or East Asian genome databases in genes encoding alternative pathways and complement regulatory factors. These variants was positive in 8 cases, 6 of which were positive in both recipient and donor, but only in one graft loss case. CONCLUSIONS: Although some immunological risks were found for dnTMA after KTx, only a few cases developed into TMA. The characteristic variations revealed in the present study may be novel candidates related to dnTMA in Japanese or Asian patients, but not pathogenic variants of aHUS. Future studies on genetic and antigenic factors are needed to identify factors contributing to dnTMA after KTx.

Anti-SARS-CoV-2 IgG antibody titer after BNT162b2 mRNA COVID-19 vaccination in Japanese patients who underwent renal replacement therapy, hemodialysis, peritoneal dialysis, and kidney transplantation.

BACKGROUND: Coronavirus disease (COVID-19) vaccination is recommended for patients undergoing renal replacement therapy (RRT), including hemodialysis (HD), peritoneal dialysis (PD), and kidney transplantation (KT). However, the difference in the immune response between RRT patients and healthy individuals after mRNA vaccines remains uncertain. METHODS: This retrospective observational study evaluated the anti-severe-acute-respiratory-syndrome-coronavirus-2 (anti-SARS-CoV-2) IgG antibody acquisition, titers and their changes, normal response rate (reaching titers of healthy individuals), factors associated with a normal response, and effectiveness of booster vaccination in Japanese RRT patients. RESULTS: Most HD and PD patients acquired anti-SARS-CoV-2 IgG antibodies after the second vaccination; however, their antibody titers and normal response rates (62-75%) were low compared with those of healthy subjects. Approximately 62% of KT recipients acquired antibodies, but the normal response rate was low (23%). Anti-SARS-CoV-2 IgG antibody waning occurred in the control, HD, and PD groups, while negative or very low titers remained in KT recipients. Third booster vaccination was effective in most HD and PD patients. However, the effect was mild in KT recipients - only 58% reached a normal response level. Multivariate logistic regression analyses demonstrated that younger age, higher serum albumin level, and RRT other than KT were significantly associated with a normal response after the second vaccination. CONCLUSIONS: RRT patients, particularly KT recipients, exhibited poor vaccine responses. Booster vaccination would be beneficial for HD and PD patients; however, its effect in KT recipients was mild. Further COVID-19 vaccinations using the latest vaccine or alternative procedures should be considered in RRT patients.

Newspaper Campaign to Promote Deceased Organ Donation: Who Can Appeal to the General Japanese Population?

BACKGROUND: Various interventions are reportedly effective in promoting organ donor registration. However, the identity of those who best serve as presenters to appeal to the general public is not known. METHODS: A campaign for organ donor registration was conducted through a local newspaper advertisement in Japan. The advertisement appeared in 439,733 copies of the newspaper on January 9, 2021. In addition to the main message, 6 different presenters with photos of their faces and quick response codes were listed in the advertisement, namely a urologist, transplant physician, nephrologist, dialysis physician, ophthalmologist, and kidney transplant recipient who was a nephrologist himself (ie, a recipient and nephrologist). Newspaper readers watched each video about deceased organ donation via the quick response codes, and the number of video views acquired 30 days after the appearance was the main outcome, which was assessed using YouTube analytics. The proportions (95% CI) of people who watched each video among 439,733 newspaper readers were compared among the 6 presenters. RESULTS: The analyzed videos were viewed 262 times. The video produced by the recipient and nephrologist had the highest number of views among the 6 presenters (proportion: 0.019% [95% CI, 0.015-0.023]), followed by the one produced by the dialysis physician (0.011% [95% CI, 0.008-0.014]), the nephrologist (0.010% [95% CI, 0.007-0.014]), the urologist (0.008% [95% CI, 0.006-0.012]), the transplant physician (0.006% [95% CI, 0.004-0.009]), and the ophthalmologist (0.005% [95% CI, 0.004-0.008]). CONCLUSIONS: The appeal by the recipient and the nephrologist reached the highest proportion of people who watched the video about deceased organ donation in Japan.

Predictive Factors of Cytomegalovirus Viremia during the Clinical Course of Anti-Neutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitis: A Single Center Observational Study.

We aim to elucidate factors to aid in the prediction of cytomegalovirus viremia during the treatment of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV). We conducted a single-center, retrospective, observational study of 35 patients with newly diagnosed AAV. Factors associated with the development of CMV viremia were investigated via a logistic regression analysis. The CMV antigenemia test was performed in 25 patients (71%), of whom 15 (60%) were diagnosed with CMV viremia. Of these 15 patients, 5 developed a CMV infection. The total protein, hemoglobin, platelet count and lymphocyte counts at the time of the CMV antigenemia test were significantly lower in patients who developed CMV viremia. In addition, total protein, hemoglobin, platelet count and lymphocyte count also presented significantly decreasing trends in the following order: patients who did not develop CMV viremia, patients who developed CMV viremia without any symptoms, and patients who developed CMV infection. All patients with CMV recovered. In conclusion, the total protein, hemoglobin, platelet count and lymphocyte count may be useful markers for the prediction of CMV viremia and infection after the start of induction of immunosuppressive therapy for patients with AAV.

Early detection of thiamine deficiency by non-thyroidal illness syndrome in a hemodialysis patient.

An 88-year-old male patient on maintenance hemodialysis (HD) therapy experienced gradual losses in appetite and liveliness during the course of 1 month. Physical examinations revealed no abnormalities. However, blood testing indicated non-thyroidal illness syndrome (NTIS) typically observed in patients with severe illness, with serum levels of thyroid stimulating hormone, free triiodothyronine, and free thyroxine of 0.17 µIU/mL, < 1.0 pg/mL, and 0.23 ng/dL, respectively. Brain magnetic resonance imaging to exclude the possibility of central hypothyroidism unexpectedly displayed slight abnormalities inside of the thalami that were characteristic of Wernicke's encephalopathy. Additional examination disclosed low serum thiamine of 20 ng/mL. Thiamine injections of 100 mg at every HD treatment rapidly restored his appetite, liveliness, and NTIS findings. HD patients are at a particularly high risk of thiamine deficiency (TD) and associated severe symptoms due to losses of thiamine during HD sessions. However, its non-specific initial symptoms, including decreases in appetite and liveliness, as well as undetectability in routine blood tests complicate early detection, resulting in underdiagnosis and more severe outcomes. In the present case, TD manifested only as non-specific symptoms and was ultimately revealed by the presence of NTIS, which was resolved with thiamine supplementation. Thus, NTIS might assist in the early detection of TD as an initial sign in HD patients.

Comparative analysis of humoral responses to BNT162b2 vaccine among patients with hematologic disorders and organ transplant recipients.

Vaccination against SARS-COV-2 is considered the most promising approach to curbing the pandemic. Patients with an immunocompromised state, such as those with hematological malignancies and organ transplantation recipients, are considered more susceptible to infection, but these at-risk patients were underrepresented in early clinical trials for vaccination. Although a growing body of studies suggests that the humoral response to COVID-19 vaccination in each of these at-risk groups of patients may be suboptimal in comparison to healthy controls, a clinical and strategic information for the further comparative analysis among these groups is not fully described. The humoral responses after two doses of BNT162b2 vaccination were evaluated in a total of 187 patients either with allogeneic hematopoietic transplantation, with renal transplantation, with anti-CD20 antibody therapy, or with anti-CD38 antibody therapy, and in 66 healthy controls. The early response at one to three months after vaccination was significantly inferior among patients with renal transplantation, patients with anti-CD20 antibody therapy, and patients with anti-CD38 antibody therapy in comparison to healthy control. But the patients with allogeneic hematopoietic transplantation showed early humoral response comparable to healthy control. The late response at 6 months after vaccination was still suboptimal among patients with renal transplantation and patients with anti-CD20 therapy. Among our patient group, renal transplant recipients had the lowest antibody titers after vaccination regardless of timing of vaccination. Patients who had received allogeneic hematopoietic transplantation attained a comparable serological response to the control group especially if they are vaccinated >300 days after transplantation, but the response was suboptimal if the vaccination was within 300 days after transplantation. Our results may provide policy makers with critical information for the further stratification of at-risk groups, helping contribute to a better allocation of resources, including additional booster vaccination.

A nationwide analysis of renal and patient outcomes for adults with lupus nephritis in Japan.

BACKGROUND: The prognosis of lupus nephritis (LN) has improved following the introduction of effective immunosuppressive therapy and progress in supportive care. This study examined recent renal and patient prognosis for adults with LN in Japan. METHODS: We conducted a nationwide retrospective cohort study of LN patients who received a renal biopsy between 2007 and 2012 that were registered in the Japan Renal Biopsy Registry. Of 623 registered adults with LN from 25 institutions and their affiliated or community hospitals, 489 were eligible for this study. RESULTS: The median age at renal biopsy was 39 years, and 82.2% of patients were female. Renal biopsies were performed in 348 patients with new-onset LN, 106 with relapse LN, and 35 with refractory LN. The distribution of ISN/RPS 2003 Classes was as follows: I 1.6%; II 5.3%; III (± V) 27.0%; IV (± V) 47.0%; V 18.4%; VI 0.6%. During the median observation period of 63.8 months, 36 patients (7.3%) reached a doubling of serum creatinine or end-stage kidney disease (ESKD), and 28 patients (5.7%) died. The 5 year renal and patient survival rates were 93.9% and 94.7%, respectively. Multivariate analysis revealed body mass index (BMI) and estimated glomerular filtration rate (eGFR) were independent risk factors for a doubling of serum creatinine in ESKD. Age and eGFR were independent risk factors for death. CONCLUSION: Recent prognosis for adults with LN are relatively good in Japan. Risk factors for impaired renal function are BMI and eGFR at renal biopsy, while age and eGFR are risk factors for death.

Brain MRI detection of early Wernicke's encephalopathy in a hemodialysis patient.

A 93-year-old hemodialysis patient became hospitalized for thiamine deficiency-induced appetite loss and ultimately experienced consciousness disorder diagnosed as Wernicke's encephalopathy (WE). Since brain MRI on admission was retrospectively found to display faint WE findings, careful brain MRI assessment is recommended in hemodialysis patients with appetite loss alone.

Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis Developing Pancreatic Lesion and Diabetes Mellitus: A Case Report and Review of the Literature.

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) affects small blood vessels and causes severe systemic organ injury commonly affecting the lungs and kidney. However, gastrointestinal, especially pancreatic, lesions are rare. We report the case of a 67-year-old Japanese man diagnosed with myeloperoxidase (MPO) AAV who developed pancreatic lesions and diabetes mellitus. The patient was admitted to our hospital due to fever, cough, and weight loss. He developed progressive glomerulonephritis, lung nodules, and pancreatic swelling and mass. Additionally, laboratory examination revealed positive MPO-ANCA and elevated glycated hemoglobin A1c, which were suggestive of diabetes mellitus. Renal biopsy revealed necrotizing crescentic glomerulonephritis and vasculitis in the small arteries. Endoscopic ultrasound-guided fine needle aspiration of the pancreas was performed, and histological findings suggested the possibility of pancreatic vasculitis and parenchymal injury. The patient was diagnosed with AAV, which was managed with glucocorticoids. This improved the renal function and pancreatic lesions. Furthermore, blood glucose levels improved despite treatment with glucocorticoids. These findings suggest that AAV-related pancreatic lesions worsened glycemic control. However, glucocorticoid therapy improved vasculitis and pancreatic lesions, which resulted in improved glycemic control.

Serum Sulfatide Levels as a Biomarker of Active Glomerular Lesion in Patients with Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis: A Single Center Pilot Study.

Sulfatides are glycosphingolipids that are associated with coagulation and platelet aggregation. Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) activates platelet function and often leads to thrombotic complications. These facts suggest an association between serum sulfatides and AAV. We aimed to clarify the significance of serum sulfatide levels in patients with AAV. We conducted a retrospective, single-center, observational pilot study that included 35 patients who developed AAV and 10 control patients who were candidates for living-donor kidney transplantation. We compared serum sulfatide levels between the control and AAV patients. We analyzed the differences in serum sulfatide levels among four classes (focal, crescentic, mixed, and sclerotic class) of glomerular lesions that were categorized by histopathologic classification of ANCA-associated glomerulonephritis. Serum sulfatide levels in patients with AAV were significantly lower than those in the controls. Serum sulfatide levels were significantly different between the four classes. Additionally, serum sulfatide levels in the crescentic class were significantly lower than those in the other classes. Serum sulfatide levels were significantly correlated with albumin, cholesterol, C-reactive protein, and pentraxin 3. In conclusion, serum sulfatide levels are significantly correlated with inflammation, reflecting crescentic glomerulonephritis, which is an active glomerular lesion in AAV patients.

Relationship between glomerular number in fresh kidney biopsy samples and light microscopy samples.

BACKGROUND: On-site evaluation of fresh kidney biopsy (FKB) samples at the time of biopsy is useful to verify that adequate specimens are acquired. However, some cases present poor correlation between glomerular number in FKB samples and light microscopy (LM) samples. We examined the usefulness of such on-site evaluation. METHODS: We conducted a retrospective cross-sectional observational study (n = 129) to assess the correlation between glomerular number in FKB samples and LM samples and the associated factors hindering the evaluation. RESULTS: There was a significant positive correlation between glomerular number in FKB samples and LM samples. The median ratio of glomerular number (LM samples/FKB samples) was 0.74. According to this ratio, cases were divided into three groups: reasonable estimation (65 cases), underestimation (32 cases), and overestimation (32 cases). Comparing the reasonable and underestimation groups, significant differences were detected in the extent of interstitial fibrosis and tubular atrophy (IFTA) and interstitial inflammation. Logistic regression analysis demonstrated that IFTA and interstitial inflammation were significantly associated with the underestimation. Moreover, the cortex length of FKB samples correlated with glomerular number in LM samples regardless of tubulointerstitial lesions. CONCLUSIONS: Glomerular number determined during on-site evaluation can be a reference for the actual number of glomeruli in LM samples. Since tubulointerstitial lesions make it difficult to recognize glomeruli in FKB samples, the possibility of underestimation for cases with possibly severe tubulointerstitial lesions should be considered. In such cases, evaluation of cortex length of FKB samples may substitute for evaluating glomeruli on-site.