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New Insights into the Pathogenesis and Therapies of IgA Nephropathy

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A special issue of Journal of Clinical Medicine (ISSN 2077-0383). This special issue belongs to the section "Nephrology & Urology".

Deadline for manuscript submissions: closed (31 March 2022) | Viewed by 64781

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Prof. Dr. Hitoshi Suzuki

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Guest Editor

Department of Nephrology, Juntendo University Urayasu Hospital, Chiba, Japan
Interests: IgA nephropathy; immune complex; chronic kidney disease; hypertension
Special Issues, Collections and Topics in MDPI journals

Prof. Dr. Jan Novak

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Department of Microbiology, University of Alabama at Birmingham, Birmingham, AL 35294, USA
Interests: glycosylation in health and disease; chronic inflammatory diseases; glomerular diseases; IgA nephropathy; gly-comics; infectious diseases; HIV-1 glycomics
Special Issues, Collections and Topics in MDPI journals

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Dear Colleagues,

IgA nephropathy (IgAN) is the most common form of primary glomerulonephritis worldwide and a frequent cause of end-stage renal disease (ESRD). Up to 20%–40% of IgAN patients progress to ESRD within 20 years after diagnosis. Moreover, life expectancy is reduced in patients with IgAN by a decade. Thus, IgAN is an important public health problem. Better understanding of the pathogenesis of IgAN and the related genetic, immunological, and cellular susceptibility factors is needed to enable development of effective disease-specific therapy.

Pathologic assessment of a renal-biopsy specimen is the current “gold standard” for diagnosis of IgAN as well as assessment of disease severity and prognosis. However, the findings may be impacted by the time between disease onset and diagnostic renal biopsy as well as by prior medications. Thus, renal biopsy provides a snapshot in time as the inherent risks associated with renal biopsy discourage from repeat biopsy. Minimally invasive approaches, such as those based on liquid biopsy biomarkers (e.g., blood, urine), are needed for monitoring of disease progression, responses to treatments, and selection of IgAN patients who would benefit from participation in new clinical trials.

Although the understanding of IgAN pathogenesis has expanded significantly since the initial disease description in 1968, disease-specific therapy to slow or prevent the progression of IgAN to ESRD is still available now. To develop a curative treatment, new strategies for early diagnosis, disease-specific targets, and methods for assessment of clinical responses in clinical trials need to be identified and developed.

In this Special Issue on ‘New Insights into the Pathogenesis and Therapies of IgA Nephropathy’, we seek clinical and experimental studies with emphasis on early diagnosis, prognosis, disease pathogenesis, determination of disease activity, and new strategies for treatment for IgAN. Both original research papers and comprehensive review papers are welcome.

Dr. Hitoshi Suzuki
Dr. Jan Novak
Guest Editors

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Manuscripts should be submitted online at mdpi.longhoe.net by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Journal of Clinical Medicine is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2600 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

IgA nephropathy
immune complex
galactose-deficient IgA1
complement
biomarker
B cell
renal pathology
genetic factor
corticosteroid
immunosuppressant

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New Insights into the Pathogenesis and Therapies of IgA Nephropathy—Second Volume in Journal of Clinical Medicine (3 articles)

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Editorial

Jump to: Research, Review

3 pages, 203 KiB

Open AccessEditorial

Special Issue: New Insights into the Pathogenesis and Therapies of IgA Nephropathy

by Hitoshi Suzuki and Jan Novak

J. Clin. Med. 2022, 11(15), 4378; https://doi.org/10.3390/jcm11154378 - 28 Jul 2022

Cited by 3 | Viewed by 1609

Abstract

IgA nephropathy (IgAN) is the most common form of primary glomerulonephritis worldwide [...] Full article

(This article belongs to the Special Issue New Insights into the Pathogenesis and Therapies of IgA Nephropathy)

Research

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9 pages, 1423 KiB

Open AccessArticle

Galactose-Deficient IgA1 as a Candidate Urinary Marker of IgA Nephropathy

by Yusuke Fukao, Hitoshi Suzuki, ** Sug Kim, Kyung Hwan Jeong, Yuko Makita, Toshiki Kano, Yoshihito Nihei, Maiko Nakayama, Mingfeng Lee, Rina Kato, Jer-Ming Chang, Sang Ho Lee and Yusuke Suzuki

J. Clin. Med. 2022, 11(11), 3173; https://doi.org/10.3390/jcm11113173 - 2 Jun 2022

Cited by 7 | Viewed by 2174

Abstract

In patients with IgA nephropathy (IgAN), circulatory IgA1 and IgA1 in the mesangial deposits contain galactose-deficient IgA1 (Gd-IgA1). Some of the Gd-IgA1 from the glomerular deposits is excreted in the urine and thus urinary Gd-IgA1 may represent a disease-specific marker. We recruited 338 Japanese biopsy-proven IgAN patients and 120 patients with other renal diseases (disease controls). Urine samples collected at the time of renal biopsy were used to measure Gd-IgA1 levels using a specific monoclonal antibody (KM55 mAb). Urinary Gd-IgA1 levels were significantly higher in patients with IgAN than in disease controls. Moreover, urinary Gd-IgA1 was significantly correlated with the severity of the histopathological parameters in IgAN patients. Next, we validated the use of urinary Gd-IgA1 levels in the other Asian cohorts. In the Korean cohort, urinary Gd-IgA1 levels were also higher in patients with IgAN than in disease controls. Even in Japanese patients with IgAN and trace proteinuria (less than 0.3 g/gCr), urinary Gd-IgA1 was detected. Thus, urinary Gd-IgA1 may be an early disease-specific biomarker useful for determining the disease activity of IgAN. Full article

(This article belongs to the Special Issue New Insights into the Pathogenesis and Therapies of IgA Nephropathy)

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9 pages, 712 KiB

Open AccessArticle

IgA Vasculitis with Nephritis in Adults: Histological and Clinical Assessment

by Lingyun Lai, Shaojun Liu, Maria Azrad, Stacy Hall, Chuanming Hao, Jan Novak, Bruce A. Julian and Lea Novak

J. Clin. Med. 2021, 10(21), 4851; https://doi.org/10.3390/jcm10214851 - 22 Oct 2021

Cited by 4 | Viewed by 2051

Abstract

Patients with IgA vasculitis (IgAV), an immune complex-mediated disease, may exhibit kidney involvement—IgAV with nephritis (IgAVN). The kidney-biopsy histopathologic features of IgAVN are similar to those of IgA nephropathy, but little is known about histopathologic disease severity based on the interval between purpura onset and diagnostic kidney biopsy. We assessed kidney histopathology and clinical and laboratory data in a cohort of adult patients with IgAVN (n = 110). The cases were grouped based on the interval between the onset of purpura and kidney biopsy: Group 1 (G1, <1 month, n = 14), Group 2 (G2, 1–6 months, n = 58), and Group 3 (G3, >6 months, n = 38). Glomerular leukocytes were more common in G1 than in the other groups (p = 0.0008). The proportion of neutrophils among peripheral-blood leukocytes was the highest in the patients biopsied within a month after onset of purpura (G1: 71 ± 8%). In the patients with an interval >6 months, the neutrophil proportion was lower, 60%. Moreover, the glomerular mesangial proliferation score correlated with the serum total IgA concentration (p = 0.0056). In conclusion, IgAVN patients biopsied <1 month from purpura onset showed an elevated percentage of blood neutrophils and glomerular leukocytes, consistent with an acute-onset inflammatory reaction. In all IgAVN patients, the mesangial proliferation score correlated with the serum IgA level. Full article

(This article belongs to the Special Issue New Insights into the Pathogenesis and Therapies of IgA Nephropathy)

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11 pages, 1162 KiB

Open AccessArticle

The Role of Complement Component C3 Activation in the Clinical Presentation and Prognosis of IgA Nephropathy—A National Study in Children

by Małgorzata Mizerska-Wasiak, Agnieszka Such-Gruchot, Karolina Cichoń-Kawa, Agnieszka Turczyn, Jadwiga Małdyk, Monika Miklaszewska, Dorota Drożdż, Agnieszka Firszt-Adamczyk, Roman Stankiewicz, Agnieszka Rybi-Szumińska, Anna Wasilewska, Maria Szczepańska, Beata Bieniaś, Przemysław Sikora, Agnieszka Pukajło-Marczyk, Danuta Zwolińska, Monika Pawlak-Bratkowska, Marcin Tkaczyk, Jacek Zachwieja, Magdalena Drożyńska-Duklas, Aleksandra Żurowska, Katarzyna Gadomska-Prokop, Ryszard Grenda and Małgorzata Pańczyk-Tomaszewska Show full author list Hide full author list

J. Clin. Med. 2021, 10(19), 4405; https://doi.org/10.3390/jcm10194405 - 26 Sep 2021

Cited by 8 | Viewed by 2780

Abstract

The aim of the study was to evaluate the influence of the intensity of mesangial C3 deposits in kidney biopsy and the serum C3 level on the clinical course and outcomes of IgAN in children. The study included 148 children from the Polish Pediatric IgAN Registry, diagnosed based on kidney biopsy. Proteinuria, creatinine, IgA, C3 were evaluated twice in the study group, at baseline and the end of follow-up. Kidney biopsy was categorized using the Oxford classification, with a calculation of the MEST-C score. The intensity of IgA and C3 deposits were rated from 0 to +4 in immunofluorescence microscopy. The intensity of mesangial C3 > +1 deposits in kidney biopsy has an effect on renal survival with normal GFR in children with IgAN. A reduced serum C3 level has not been a prognostic factor in children but perhaps this finding should be confirmed in a larger group of children. Full article

(This article belongs to the Special Issue New Insights into the Pathogenesis and Therapies of IgA Nephropathy)

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14 pages, 1412 KiB

Open AccessArticle

The Impact of Obesity on the Severity of Clinicopathologic Parameters in Patients with IgA Nephropathy

by Yu Ah Hong, Ji Won Min, Myung Ah Ha, Eun Sil Koh, Hyung Duk Kim, Tae Hyun Ban, Young Soo Kim, Yong Kyun Kim, Dongryul Kim, Seok Joon Shin, Won Jung Choi, Yoon Kyung Chang, Suk Young Kim, Cheol Whee Park, Young Ok Kim, Chul Woo Yang and Hye Eun Yoon

J. Clin. Med. 2020, 9(9), 2824; https://doi.org/10.3390/jcm9092824 - 31 Aug 2020

Cited by 12 | Viewed by 2800

Abstract

Several studies reported the effect of obesity on the progression of IgA nephropathy (IgAN). However, the impact of obesity on the clinicopathologic presentation of IgAN remains uncertain. This is a retrospective cross-sectional study from eight university hospitals in South Korea. Patients were categorized into three groups using the Asia-Pacific obesity classification based on body mass index (BMI). Clinical and histopathologic data at the time of renal biopsy were analyzed. Among 537 patients with IgAN, the obese group was more hypertensive and had lower estimated glomerular filtration rate and more proteinuria than other groups. The histologic scores for mesangial matrix expansion (MME), interstitial fibrosis, tubular atrophy, and mesangial C3 deposition differed significantly between the three groups. Among these histopathologic parameters, BMI was independently positively associated with MME score on multivariable linear regression analysis (p = 0.028). Using multivariable logistic regression analysis, the obese group was independently associated with higher MME scores compared to the normal weight/overweight group (p = 0.020). However, BMI was not independently associated with estimated glomerular filtration rate or proteinuria on multivariable analysis. Obesity was independently associated with severe MME in patients with IgAN. Obesity may play an important pathogenetic role in mesangial lesions seen in IgAN. Full article

(This article belongs to the Special Issue New Insights into the Pathogenesis and Therapies of IgA Nephropathy)

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Review

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12 pages, 1241 KiB

Open AccessReview

Crescents and IgA Nephropathy: A Delicate Marriage

by Hernán Trimarchi, Mark Haas and Rosanna Coppo

J. Clin. Med. 2022, 11(13), 3569; https://doi.org/10.3390/jcm11133569 - 21 Jun 2022

Cited by 12 | Viewed by 2770

Abstract

IgA nephropathy (IgAN) is a progressive disease with great variability in the clinical course. Among the clinical and pathologic features contributing to variable outcomes, the presence of crescents has attracted particular interest as a distinct pathological feature associated with severity. Several uncontrolled observations have led to the general thought that the presence and extent of crescents was a prognostic indicator associated with poor outcomes. However, KDIGO 2021 guidelines concluded that either the presence or the relative number of crescents should not be used to determine the progression of IgAN nor should they suggest the choice of immunosuppression. Our aim is to report and discuss recent data on the debated issue of the value of active (cellular and fibrocellular) crescents in the pathogenesis and clinical progression of IgAN, their predictive value, and the impact of immunosuppression on renal function. We conclude that the value of crescents should not be disregarded, although this feature does not have an independent predictive value for progression in IgAN, particularly when considering immunosuppressed patients. An integrated overall evaluation of crescents with other active MEST scores, clinical data, and novel biomarkers must be considered in achieving a personalized therapeutic approach to IgAN patients. Full article

(This article belongs to the Special Issue New Insights into the Pathogenesis and Therapies of IgA Nephropathy)

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12 pages, 994 KiB

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New Treatment Strategies for IgA Nephropathy: Targeting Plasma Cells as the Main Source of Pathogenic Antibodies

by Dita Maixnerova, Delphine El Mehdi, Dana V. Rizk, Hong Zhang and Vladimir Tesar

J. Clin. Med. 2022, 11(10), 2810; https://doi.org/10.3390/jcm11102810 - 16 May 2022

Cited by 18 | Viewed by 8360

Abstract

Immunoglobulin A nephropathy (IgAN) is a rare autoimmune disorder and the leading cause of biopsy-reported glomerulonephritis (GN) worldwide. Disease progression is driven by the formation and deposition of immune complexes composed of galactose-deficient IgA1 (Gd-IgA1) and Gd-IgA1 autoantibodies (anti-Gd-IgA1 antibodies) in the glomeruli, where they trigger complement-mediated inflammation that can result in loss of kidney function and end-stage kidney disease (ESKD). With the risk of progression and limited treatment options, there is an unmet need for therapies that address the formation of pathogenic Gd-IgA1 antibody and anti-Gd-IgA1 antibody-containing immune complexes. New therapeutic approaches target immunological aspects of IgAN, including complement-mediated inflammation and pathogenic antibody production by inhibiting activation or promoting depletion of B cells and CD38-positive plasma cells. This article will review therapies, both approved and in development, that support the depletion of Gd-IgA1-producing cells in IgAN and have the potential to modify the course of this disease. Ultimately, we propose here a novel therapeutic approach by depleting CD38-positive plasma cells, as the source of the autoimmunity, to treat patients with IgAN. Full article

(This article belongs to the Special Issue New Insights into the Pathogenesis and Therapies of IgA Nephropathy)

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21 pages, 12147 KiB

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The Contribution of Complement to the Pathogenesis of IgA Nephropathy: Are Complement-Targeted Therapies Moving from Rare Disorders to More Common Diseases?

by Felix Poppelaars, Bernardo Faria, Wilhelm Schwaeble and Mohamed R. Daha

J. Clin. Med. 2021, 10(20), 4715; https://doi.org/10.3390/jcm10204715 - 14 Oct 2021

Cited by 25 | Viewed by 4835

Abstract

Primary IgA nephropathy (IgAN) is a leading cause of chronic kidney disease and kidney failure for which there is no disease-specific treatment. However, this could change, since novel therapeutic approaches are currently being assessed in clinical trials, including complement-targeting therapies. An improved understanding of the role of the lectin and the alternative pathway of complement in the pathophysiology of IgAN has led to the development of these treatment strategies. Recently, in a phase 2 trial, treatment with a blocking antibody against mannose-binding protein-associated serine protease 2 (MASP-2, a crucial enzyme of the lectin pathway) was suggested to have a potential benefit for IgAN. Now in a phase 3 study, this MASP-2 inhibitor for the treatment of IgAN could mark the start of a new era of complement therapeutics where common diseases can be treated with these drugs. The clinical development of complement inhibitors requires a better understanding by physicians of the biology of complement, the pathogenic role of complement in IgAN, and complement-targeted therapies. The purpose of this review is to provide an overview of the role of complement in IgAN, including the recent discovery of new mechanisms of complement activation and opportunities for complement inhibitors as the treatment of IgAN. Full article

(This article belongs to the Special Issue New Insights into the Pathogenesis and Therapies of IgA Nephropathy)

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22 pages, 2701 KiB

Open AccessReview

Pathogenesis of IgA Nephropathy: Current Understanding and Implications for Development of Disease-Specific Treatment

by Barbora Knoppova, Colin Reily, R. Glenn King, Bruce A. Julian, Jan Novak and Todd J. Green

J. Clin. Med. 2021, 10(19), 4501; https://doi.org/10.3390/jcm10194501 - 29 Sep 2021

Cited by 35 | Viewed by 9257

Abstract

IgA nephropathy, initially described in 1968 as a kidney disease with glomerular “intercapillary deposits of IgA-IgG”, has no disease-specific treatment and is a common cause of kidney failure. Clinical observations and laboratory analyses suggest that IgA nephropathy is an autoimmune disease wherein the kidneys are damaged as innocent bystanders due to deposition of IgA1-IgG immune complexes from the circulation. A multi-hit hypothesis for the pathogenesis of IgA nephropathy describes four sequential steps in disease development. Specifically, patients with IgA nephropathy have elevated circulating levels of IgA1 with some O-glycans deficient in galactose (galactose-deficient IgA1) and these IgA1 glycoforms are recognized as autoantigens by unique IgG autoantibodies, resulting in formation of circulating immune complexes, some of which deposit in glomeruli and activate mesangial cells to induce kidney injury. This proposed mechanism is supported by observations that (i) glomerular immunodeposits in patients with IgA nephropathy are enriched for galactose-deficient IgA1 glycoforms and the corresponding IgG autoantibodies; (ii) circulatory levels of galactose-deficient IgA1 and IgG autoantibodies predict disease progression; and (iii) pathogenic potential of galactose-deficient IgA1 and IgG autoantibodies was demonstrated in vivo. Thus, a better understanding of the structure–function of these immunoglobulins as autoantibodies and autoantigens will enable development of disease-specific treatments. Full article

(This article belongs to the Special Issue New Insights into the Pathogenesis and Therapies of IgA Nephropathy)

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20 pages, 1525 KiB

Open AccessReview

Aberrantly Glycosylated IgA1 in IgA Nephropathy: What We Know and What We Don’t Know

by Yukako Ohyama, Matthew B. Renfrow, Jan Novak and Kazuo Takahashi

J. Clin. Med. 2021, 10(16), 3467; https://doi.org/10.3390/jcm10163467 - 5 Aug 2021

Cited by 27 | Viewed by 4528

Abstract

IgA nephropathy (IgAN), the most common primary glomerular disease worldwide, is characterized by glomerular deposition of IgA1-containing immune complexes. The IgA1 hinge region (HR) has up to six clustered O-glycans consisting of Ser/Thr-linked N-acetylgalactosamine usually with β1,3-linked galactose and variable sialylation. Circulating levels of IgA1 with abnormally O-glycosylated HR, termed galactose-deficient IgA1 (Gd-IgA1), are increased in patients with IgAN. Current evidence suggests that IgAN is induced by multiple sequential pathogenic steps, and production of aberrantly glycosylated IgA1 is considered the initial step. Thus, the mechanisms of biosynthesis of aberrantly glycosylated IgA1 and the involvement of aberrant glycoforms of IgA1 in disease development have been studied. Furthermore, Gd-IgA1 represents an attractive biomarker for IgAN, and its clinical significance is still being evaluated. To elucidate the pathogenesis of IgAN, it is important to deconvolute the biosynthetic origins of Gd-IgA1 and characterize the pathogenic IgA1 HR O-glycoform(s), including the glycan structures and their sites of attachment. These efforts will likely lead to development of new biomarkers. Here, we review the IgA1 HR O-glycosylation in general and the role of aberrantly glycosylated IgA1 in the pathogenesis of IgAN in particular. Full article

(This article belongs to the Special Issue New Insights into the Pathogenesis and Therapies of IgA Nephropathy)

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11 pages, 1171 KiB

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History of IgA Nephropathy Mouse Models

by Batoul Wehbi, Virginie Pascal, Lina Zawil, Michel Cogné and Jean-Claude Aldigier

J. Clin. Med. 2021, 10(14), 3142; https://doi.org/10.3390/jcm10143142 - 16 Jul 2021

Cited by 8 | Viewed by 4302

Abstract

IgA nephropathy (IgAN) is the most common primary glomerulonephritis in the world. It was first described in 1968 by Jean Berger and Nicole Hinglais as the presence of intercapillary deposits of IgA. Despite this simple description, patients with IgAN may present very broad clinical features ranging from the isolated presence of IgA in the mesangium without clinical or biological manifestations to rapidly progressive kidney failure. These features are associated with a variety of histological lesions, from the discrete thickening of the mesangial matrix to diffuse cell proliferation. Immunofluorescence on IgAN kidney specimens shows the isolated presence of IgA or its inconsistent association with IgG and complement components. This clinical heterogeneity of IgAN clearly echoes its complex and multifactorial pathophysiology in humans, inviting further analyses of its various aspects through the use of experimental models. Small-animal models of IgAN provide the most pertinent strategies for studying the multifactorial aspects of IgAN pathogenesis and progression. Although only primates have the IgA1 subclass, several murine models have been developed in which various aspects of immune responses are deregulated and which are useful in the understanding of IgAN physiopathology as well as in the assessment of IgAN therapeutic approaches. In this manuscript, we review all murine IgAN models developed since 1968 and discuss their remarkable contribution to understanding the disease. Full article

(This article belongs to the Special Issue New Insights into the Pathogenesis and Therapies of IgA Nephropathy)

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19 pages, 624 KiB

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An Update on the Current State of Management and Clinical Trials for IgA Nephropathy

by Chee Kay Cheung, Arun Rajasekaran, Jonathan Barratt and Dana V. Rizk

J. Clin. Med. 2021, 10(11), 2493; https://doi.org/10.3390/jcm10112493 - 4 Jun 2021

Cited by 35 | Viewed by 10087

Abstract

IgA nephropathy remains the most common primary glomerular disease worldwide. It affects children and adults of all ages, and is a leading cause of end-stage kidney disease, making it a considerable public health issue in many countries. Despite being initially described over 50 years ago, there are still no disease specific treatments, with current management for most patients being focused on lifestyle measures and renin-angiotensin-aldosterone system blockade. However, significant advances in the understanding of its pathogenesis have been made particularly over the past decade, leading to great interest in develo** new therapeutic strategies, and a significant rise in the number of interventional clinical trials being performed. In this review, we will summarise the current state of management of IgAN, and then describe major areas of interest where new therapies are at their most advanced stages of development, that include the gut mucosal immune system, B cell signalling, the complement system and non-immune modulators. Finally, we describe clinical trials that are taking place in each area and explore future directions for translational research. Full article

(This article belongs to the Special Issue New Insights into the Pathogenesis and Therapies of IgA Nephropathy)

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14 pages, 1387 KiB

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IgA Vasculitis and IgA Nephropathy: Same Disease?

by Evangeline Pillebout

J. Clin. Med. 2021, 10(11), 2310; https://doi.org/10.3390/jcm10112310 - 25 May 2021

Cited by 29 | Viewed by 6864

Abstract

Many authors suggested that IgA Vasculitis (IgAV) and IgA Nephropathy (IgAN) would be two clinical manifestations of the same disease; in particular, that IgAV would be the systemic form of the IgAN. A limited number of studies have included sufficient children or adults with IgAN or IgAV (with or without nephropathy) and followed long enough to conclude on differences or similarities in terms of clinical, biological or histological presentation, physiopathology, genetics or prognosis. All therapeutic trials available on IgAN excluded patients with vasculitis. IgAV and IgAN could represent different extremities of a continuous spectrum of the same disease. Due to skin rash, patients with IgAV are diagnosed precociously. Conversely, because of the absence of any clinical signs, a renal biopsy is practiced for patients with an IgAN to confirm nephropathy at any time of the evolution of the disease, which could explain the frequent chronic lesions at diagnosis. Nevertheless, the question that remains unsolved is why do patients with IgAN not have skin lesions and some patients with IgAV not have nephropathy? Larger clinical studies are needed, including both diseases, with a common histological classification, and stratified on age and genetic background to assess renal prognosis and therapeutic strategies. Full article

(This article belongs to the Special Issue New Insights into the Pathogenesis and Therapies of IgA Nephropathy)

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