Glomerular diseases最新文献

{"title":"Hypocomplementemic Urticarial Vasculitis Syndrome Masquerading as Systemic Lupus Erythematosus: A Case Report.","authors":"Jiten Prakash Mehta,&nbsp;Charley Qi Hua Jang,&nbsp;Peter Fahim,&nbsp;Minhtri Khac Nguyen,&nbsp;Jonathan Zuckerman,&nbsp;Rosha Mamita,&nbsp;Mohammad Kamgar","doi":"10.1159/000525942","DOIUrl":"https://doi.org/10.1159/000525942","url":null,"abstract":"<p><strong>Introduction: </strong>Hypocomplementemic urticarial vasculitis syndrome (HUVS) is an infrequent immune complex-mediated condition characterized by nonpruritic urticarial lesions, low serum complement levels, and autoantibodies, associated with systemic manifestations like arthralgia/arthritis, angioedema, ocular inflammation with conjunctivitis, episcleritis, uveitis, renal, gastrointestinal, and pulmonary involvement. HUVS and systemic lupus erythematosus (SLE) overlap and the criteria for identifying HUVS as an entity distinct from SLE are lacking. Despite the diagnostic criteria established by Schwartz et al. [<i>Curr Opin Rheumatol.</i> 2014;26(5):502-9], differentiation from SLE is sometimes difficult as patients often also fulfill the classification criteria of the American College of Rheumatology (ACR). The prognosis of HUVS depends on the organ system involved. Lung disease results in significant morbidity and mortality and is made worse by smoking. Kidney involvement with glomerulonephritis may ultimately result in end-stage renal disease with the need for kidney transplant. Death may also occur due to acute laryngeal edema.</p><p><strong>Case presentation: </strong>We pre-sent a case of a 40-year-old female who had a diagnosis of SLE, presented with severe odynophagia, was found to have an erythematous macular rash, and had acute kidney injury attributed to contrast-related injury and cardiorenal syndrome. After the resolution of the AKI, she continued to have hematuria and low-grade proteinuria that led to a kidney biopsy that aided in the diagnosis of HUVS.</p><p><strong>Discussion/conclusion: </strong>Given the rarity of this disease and the difficulty in differentiating HUVS from other rheumatological diseases such as SLE, further accumulation of cases is necessary to understand the best diagnostic modality for this entity.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"2 4","pages":"189-193"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/ee/c4/gdz-0002-0189.PMC9936762.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10772191","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"NELL1-Positive HIV-Associated Lupus-Like Membranous Nephropathy with Spontaneous Remission.","authors":"Kumar P Dinesh,&nbsp;Vivek Charu,&nbsp;Megan L Troxell,&nbsp;Nicole K Andeen","doi":"10.1159/000525541","DOIUrl":"https://doi.org/10.1159/000525541","url":null,"abstract":"<p><strong>Introduction: </strong>Kidney biopsy findings in patients with human immunodeficiency virus (HIV) are diverse, and optimal therapy for the various immune complex diseases in the setting of HIV is unknown.</p><p><strong>Case presentation: </strong>A man with well-controlled HIV developed nephrotic range proteinuria, and kidney biopsy revealed lupus-like glomerulonephritis with a predominantly membranous pattern of injury. He opted for conservative therapy and experienced spontaneous and sustained remission. Subsequent testing revealed neural epidermal growth factor-like 1 (NELL1)-positive glomerular immune deposits. NELL1-positive glomerular immune deposits were identified in a total of 2 of 5 tested HIV-associated membranous nephropathy (MN), which were morphologically dissimilar and one of which weakly co-expressed phospholipase A2 receptor (PLA2R).</p><p><strong>Discussion: </strong>This case suggests potentially different outcomes in patients with immune complex diseases in the setting of HIV based on disease etiology and histopathology. HIV-associated MN is occasionally NELL1-positive.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"2 4","pages":"184-188"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/9e/05/gdz-0002-0184.PMC9936763.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10772189","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Acknowledgement to Reviewers.","authors":"","doi":"10.1159/000527669","DOIUrl":"https://doi.org/10.1159/000527669","url":null,"abstract":"","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"2 4","pages":"194"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/0b/db/gdz-0002-0194.PMC9936755.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10755288","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Monoclonal Gammopathy of Renal Significance: Histomorphological Spectrum at a Tertiary Care Center.","authors":"Adarsh Barwad,&nbsp;Varun Bajaj,&nbsp;Geetika Singh,&nbsp;Amit Kumar Dinda,&nbsp;Ranjit Kumar Sahoo,&nbsp;Lalit Kumar,&nbsp;Sanjay Kumar Agarwal","doi":"10.1159/000526244","DOIUrl":"https://doi.org/10.1159/000526244","url":null,"abstract":"<p><strong>Introduction: </strong>The term monoclonal gammopathy of renal significance (MGRS) has been described to include patients with renal manifestations associated with circulating monoclonal proteins with or without a clonal lymphoproliferation (B-cell or plasma cell) and not meeting diagnostic criteria for an overt hematological malignancy. A host of MGRS-associated lesions have been described that involve various renal compartments. Our study describes the histomorphological spectrum of MGRS cases at our center in the last 5 years and description as per the classification system of the International Kidney and Monoclonal Gammopathy Research Group (IKMG).</p><p><strong>Material and methods: </strong>Retrospective analysis was carried out of all the renal biopsies with characteristic monoclonal immunoglobulin lesions for histopathological diagnosis between years 2015 and 2020 and reviewed by two independent pathologists.</p><p><strong>Results: </strong>Most patients in the study belonged to the fifth decade, with a median age of 50 years (mean 50.14 ± 10.43) range (24-68 years) with a male preponderance. Most patients presented with proteinuria as the sole manifestation (66.6%). Many of the patients (48%) had an M spike by serum protein electrophoresis or urinary protein electrophoresis with an abnormal serum free light chain assay (60.8%). AL amyloidosis was the most common diagnosis observed on histopathological evaluation (68.7%), followed by light chain deposition disease (10.4%).</p><p><strong>Conclusion: </strong>MGRS lesions are infrequently encountered in the practice of nephropathology and pose a diagnostic challenge due to the limitation of a congruent clinical or hematological picture. A thorough histological examination with immunofluorescence and electron microscopy often precipitates in the right diagnosis and prompts timely management.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"2 4","pages":"153-163"},"PeriodicalIF":0.0,"publicationDate":"2022-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/22/0f/gdz-0002-0153.PMC9936767.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10766710","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"IgA Nephropathy: A Tale of 3 Continents.","authors":"Sydney Chi-Wai Tang","doi":"10.1159/000521511","DOIUrl":"10.1159/000521511","url":null,"abstract":"","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"2 1","pages":"1-3"},"PeriodicalIF":0.0,"publicationDate":"2021-12-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/36/5b/gdz-0002-0001.PMC9677710.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9243516","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Experimentally Induced Anti-Myeloperoxidase Vasculitis Is Not Attenuated in Factor B or VISTA Deficient Mice.","authors":"Fernanda Flórez-Barrós, Simon J Freeley, El Li Tham, Michael G Robson","doi":"10.1159/000521233","DOIUrl":"10.1159/000521233","url":null,"abstract":"<p><strong>Background: </strong>Anti-neutrophil cytoplasmic antibody vasculitis is characterized by antibodies to myeloperoxidase or proteinase 3. Previous work in murine anti-myeloperoxidase vasculitis has shown a role for the alternative pathway complement component factor B and the anaphylatoxin C5a. However, mice deficient in properdin, which stabilizes the alternative pathway convertase, were not protected. V-Type immunoglobulin domain-containing suppressor of T-cell activation (VISTA)-deficient mice were protected in the nephrotoxic nephritis model but the role of VISTA in anti-myeloperoxidase vasculitis is unknown.</p><p><strong>Objectives: </strong>This study had 2 aims. First, we attempted to reproduce previous findings on the role of factor B in anti-myeloperoxidase vasculitis. Second, we examined the role of VISTA in this model, in order to see if the protection in the nephrotoxic nephritis model extended to anti-myeloperoxidase vasculitis.</p><p><strong>Methods: </strong>Anti-myeloperoxidase vasculitis was induced in wild type, factor B, or VISTA deficient mice. Disease was assessed by quantifying glomerular crescents and macrophages, in addition to albuminuria and serum creatinine.</p><p><strong>Results: </strong>When wild type and factor B deficient mice were compared, there were no differences in any of the histological or biochemical parameters of disease assessed. Similarly, when wild type or VISTA deficient mice were compared, there were no differences.</p><p><strong>Conclusions: </strong>Factor B deficient mice were not protected which is in contrast to previous studies. Therefore alternative pathway activation is not essential in this model, under the conditions used in this study. VISTA deficient mice were not protected, suggesting that therapies targeting VISTA may not be effective in vasculitis.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"2 2","pages":"83-88"},"PeriodicalIF":0.0,"publicationDate":"2021-11-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9670022/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10681225","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Deconvolution of Focal Segmental Glomerulosclerosis Pathophysiology Using Transcriptomics Techniques.","authors":"Dries Deleersnijder,&nbsp;Amaryllis H Van Craenenbroeck,&nbsp;Ben Sprangers","doi":"10.1159/000518404","DOIUrl":"https://doi.org/10.1159/000518404","url":null,"abstract":"<p><strong>Background: </strong>Focal segmental glomerulosclerosis is a histopathological pattern of renal injury and comprises a heterogeneous group of clinical conditions with different pathophysiology, clinical course, prognosis, and treatment. Nevertheless, subtype differentiation in clinical practice often remains challenging, and we currently lack reliable diagnostic, prognostic, and therapeutic biomarkers. The advent of new transcriptomics techniques in kidney research poses great potential in the identification of gene expression biomarkers that can be applied in clinical practice.</p><p><strong>Summary: </strong>Transcriptomics techniques have been completely revolutionized in the last 2 decades, with the evolution from low-throughput reverse-transcription polymerase chain reaction and in situ hybridization techniques to microarrays and next-generation sequencing techniques, including RNA-sequencing and single-cell transcriptomics. The integration of human gene expression profiles with functional in vitro and in vivo experiments provides a deeper mechanistic insight into the candidate genes, which enable the development of novel-targeted therapies. The correlation of gene expression profiles with clinical outcomes of large patient cohorts allows for the development of clinically applicable biomarkers that can aid in diagnosis and predict prognosis and therapy response. Finally, the integration of transcriptomics with other \"omics\" modalities creates a holistic view on disease pathophysiology.</p><p><strong>Key messages: </strong>New transcriptomics techniques allow high-throughput gene expression profiling of patients with focal segmental glomerulosclerosis (FSGS). The integration with clinical outcomes and fundamental mechanistic studies enables the discovery of new clinically useful biomarkers that will finally improve the clinical outcome of patients with FSGS.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"1 4","pages":"265-276"},"PeriodicalIF":0.0,"publicationDate":"2021-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/c6/95/gdz-0001-0265.PMC9677714.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10680810","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"An Introduction to Stereology with Applications to the Glomerulus.","authors":"Kevin V Lemley","doi":"10.1159/000519719","DOIUrl":"https://doi.org/10.1159/000519719","url":null,"abstract":"<p><strong>Background: </strong>Stereology is the science of inferring quantitative features of 3-dimensional structures from lower dimensional samples of those structures (probes). It is a statistical discipline and therefore may seem intimidating to many potential users. Without a proper understanding of its principles, though, errors may be made in the quantitative reporting of structural research results.</p><p><strong>Summary: </strong>This review article attempts to explain and justify the basic principles of stereology as applied to the glomerulus in a simple and accessible way. A few common errors in application are described. The strengths and weaknesses of \"biased\" (model-based) stereology are described as well as the basics of design-based (\"unbiased\") stereology.</p><p><strong>Key messages: </strong>Stereology is a useful body of theory and practices when quantitation of structural histological features of the glomerulus is desired.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"1 4","pages":"294-301"},"PeriodicalIF":0.0,"publicationDate":"2021-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/69/a5/gdz-0001-0294.PMC9677735.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10674908","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Current Understanding of Clinical Manifestations of COVID-19 in Glomerular Disease.","authors":"Allison Shimmel,&nbsp;Salma Shaikhouni,&nbsp;Laura Mariani","doi":"10.1159/000518276","DOIUrl":"https://doi.org/10.1159/000518276","url":null,"abstract":"<p><strong>Background: </strong>The novel coronavirus disease (COVID-19), also known as severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), is an evolving pandemic with significant mortality. Information about the impact of infection on glomerular disease patients in particular has been lacking. Understanding the virus's effect in glomerular disease is constantly changing. This review article summarizes the data published thus far on COVID-19 and its manifestations in pre-existing and de novo glomerular disease.</p><p><strong>Summary: </strong>While patients with glomerular disease may be at higher risk of severe COVID-19 due to their immunosuppressed status, some data suggest that a low amount of immunosuppression may be helpful in mitigating the systemic inflammatory response which is associated with high mortality rates in COVID-19. There have been a few case reports on COVID-19 causing glomerular disease relapse in patients. Multiple mechanisms have been proposed for kidney injury, proteinuria, and hematuria in the setting of COVID-19. More commonly, these are caused by direct tubular injury due to hemodynamic instability and hypoxic injury. However, the cytokine storm induced by COVID-19 may trigger common post-viral glomerular disease such as IgA nephropathy, anti-GBM, and ANCA vasculitis that have also been described in COVID-19 patients. Collapsing glomerulopathy, a hallmark of HIV-associated nephropathy, is being reported SARS-CoV-2 cases, particularly in patients with high-risk APOL1 alleles. Direct viral invasion of glomerular structures is hypothesized to cause a podocytopathy due to virus's affinity to ACE2, but evidence for this remains under study.</p><p><strong>Key messages: </strong>Infection with SARS-CoV-2 may cause glomerular disease in certain patients. The mechanism of de novo glomerular disease in the setting of COVID-19 is under study. The management of patients with existing glomerular disease poses unique challenges, especially with regard to immunosuppression management. Further studies are needed to inform clinician decisions about the management of these patients during the COVID-19 pandemic.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"1 4","pages":"250-264"},"PeriodicalIF":0.0,"publicationDate":"2021-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1159/000518276","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10660537","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Recurrent Glomerular Diseases in Renal Transplantation with Focus on Role of Electron Microscopy.","authors":"Surya V Seshan,&nbsp;Steven P Salvatore","doi":"10.1159/000517259","DOIUrl":"https://doi.org/10.1159/000517259","url":null,"abstract":"<p><strong>Background: </strong>The common causes of renal transplant complications include active or chronic rejection process, infections, and toxicity but also recurrent or de novo diseases, which play an important role in affecting long-term graft function or graft loss.</p><p><strong>Summary: </strong>Recurrent disease in renal transplantation is defined as recurrence of the original kidney disease leading to end-stage kidney disease. They comprise a heterogeneous group of predominantly glomerular and some tubulointerstitial and vascular lesions, which include primary kidney diseases (e.g., focal segmental glomerulosclerosis, membranous glomerulonephritis, and IgA nephropathy) or those secondary to systemic autoimmune, metabolic, and infectious processes that can range from subclinical to clinically overt acute, subacute, or chronic clinical presentations. In addition to the knowledge of prior renal disease and routine/periodic serum and urine testing for kidney function, a complete transplant renal biopsy examination is essential in the identification and differentiation of these diseases. The time of onset and severity of these diseases depend on the underlying etiopathogenetic mechanisms and the varied rates of recurrence in the early or late posttransplant period, often being modified by the current immunosuppressive protocols and other donor and recipient predisposing characteristics.</p><p><strong>Key messages: </strong>Transplant kidney biopsy findings provide diagnostic accuracy and prognostic information regarding the potential for reversibility along with detection of unsuspected or clinically symptomatic recurrent diseases, with any concomitant rejection process or toxicity, for appropriate therapeutic decision-making. Routine electron microscopy in transplant kidney biopsies is a valuable tool in recognizing fully developed or early/subtle features of evolving recurrent diseases, often during the subclinical phases, in for cause or surveillance allograft biopsies.</p>","PeriodicalId":73177,"journal":{"name":"Glomerular diseases","volume":"1 4","pages":"205-236"},"PeriodicalIF":0.0,"publicationDate":"2021-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1159/000517259","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10680809","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}