{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,28]],"date-time":"2026-02-28T09:23:39Z","timestamp":1772270619252,"version":"3.50.1"},"reference-count":51,"publisher":"MDPI AG","issue":"21","license":[{"start":{"date-parts":[[2021,10,22]],"date-time":"2021-10-22T00:00:00Z","timestamp":1634860800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"National Institute of Health","award":["DK078244"],"award-info":[{"award-number":["DK078244"]}]},{"name":"National Institute of Health","award":["DK082753"],"award-info":[{"award-number":["DK082753"]}]},{"name":"IGA Nephropathy Foundation of America","award":["gift"],"award-info":[{"award-number":["gift"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Patients with IgA vasculitis (IgAV), an immune complex-mediated disease, may exhibit kidney involvement\u2014IgAV 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, &lt;1 month, n = 14), Group 2 (G2, 1\u20136 months, n = 58), and Group 3 (G3, &gt;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 \u00b1 8%). In the patients with an interval &gt;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 &lt;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.<\/jats:p>","DOI":"10.3390\/jcm10214851","type":"journal-article","created":{"date-parts":[[2021,10,22]],"date-time":"2021-10-22T09:18:09Z","timestamp":1634894289000},"page":"4851","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["IgA Vasculitis with Nephritis in Adults: Histological and Clinical Assessment"],"prefix":"10.3390","volume":"10","author":[{"given":"Lingyun","family":"Lai","sequence":"first","affiliation":[{"name":"Division of Nephrology, Fudan University Huashan Hospital, Shanghai 200040, China"},{"name":"Department of Microbiology, University of Alabama at Birmingham, Birmingham, AL 35294, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shaojun","family":"Liu","sequence":"additional","affiliation":[{"name":"Division of Nephrology, Fudan University Huashan Hospital, Shanghai 200040, China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maria","family":"Azrad","sequence":"additional","affiliation":[{"name":"Department of Nutrition, University of Alabama at Birmingham, Birmingham, AL 35294, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stacy","family":"Hall","sequence":"additional","affiliation":[{"name":"Department of Microbiology, University of Alabama at Birmingham, Birmingham, AL 35294, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chuanming","family":"Hao","sequence":"additional","affiliation":[{"name":"Division of Nephrology, Fudan University Huashan Hospital, Shanghai 200040, China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9211-6670","authenticated-orcid":false,"given":"Jan","family":"Novak","sequence":"additional","affiliation":[{"name":"Department of Microbiology, University of Alabama at Birmingham, Birmingham, AL 35294, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bruce A.","family":"Julian","sequence":"additional","affiliation":[{"name":"Department of Medicine, University of Alabama at Birmingham, Birmingham, AL 35294, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lea","family":"Novak","sequence":"additional","affiliation":[{"name":"Department of Pathology, University of Alabama at Birmingham, Birmingham, AL 35294, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2021,10,22]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"598","DOI":"10.1097\/BOR.0b013e32833af608","article-title":"Henoch-Sch\u00f6nlein purpura","volume":"22","author":"Saulsbury","year":"2010","journal-title":"Curr. Opin. Rheumatol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"679","DOI":"10.2215\/CJN.06710810","article-title":"Henoch-Sch\u00f6nlein purpura nephritis: Pathophysiology, treatment, and future strategy","volume":"6","author":"Davin","year":"2011","journal-title":"Clin. J. Am. Soc. Nephrol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"563","DOI":"10.1038\/nrneph.2014.126","article-title":"Henoch-Sch\u00f6nlein purpura nephritis in children","volume":"10","author":"Davin","year":"2014","journal-title":"Nat. Rev. Nephrol."},{"key":"ref_4","unstructured":"Jennette, J.C., Olson, J.L., Schwartz, M.M., and Silva, F.G. (2007). IgA nephropathy and Henoch-Sch\u00f6nlein purpura nephritis. Heptinstall\u2019s Pathology of the Kidney, Lippincott, Williams and Wilkins."},{"key":"ref_5","doi-asserted-by":"crossref","unstructured":"Hastings, M.C., Rizk, D.V., Kiryluk, K., Nelson, R., Zahr, R.S., Novak, J., and Wyatt, R.J. (2021). IgA vasculitis with nephritis: Update of pathogenesis with clinical implications. Pediatr. Nephrol., 1\u201315.","DOI":"10.1007\/s00467-021-04950-y"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"823","DOI":"10.1046\/j.1523-1755.2001.059003823.x","article-title":"What is the difference between IgA nephropathy and Henoch-Sch\u00f6nlein purpura nephritis?","volume":"59","author":"Davin","year":"2001","journal-title":"Kidney Int."},{"key":"ref_7","unstructured":"Jennette, J.C., Olson, J.L., Schwartz, M.M., and Silva, F.G. (1998). IgA nephropathy and Henoch-Sch\u00f6nlein syndrome. Heptinstall\u2019s Pathology of the Kidney, Lippincott-Raven Publishers."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"170","DOI":"10.1046\/j.1365-4362.2003.01769.x","article-title":"Henoch-Sch\u00f6nlein purpura nephritis","volume":"42","author":"Fervenza","year":"2003","journal-title":"Int. J. Dermatol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"373","DOI":"10.1016\/S0272-6386(88)80028-3","article-title":"Is Henoch-Sch\u00f6nlein purpura the systemic form of IgA nephropathy?","volume":"12","author":"Waldo","year":"1988","journal-title":"Am. J. Kidney Dis."},{"key":"ref_10","unstructured":"Kher, K.K., Schnapper, H.W., and Makker, S.P. (2006). IgA nephropathy and Henoch-Sch\u00f6nlein purpura nephritis. Clinical Pediatric Nephrology, Informa UK Ltd.. [2nd ed.]."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"635","DOI":"10.1038\/modpathol.3880364","article-title":"Grading of acute and chronic renal lesions in Henoch-Sch\u00f6nlein purpura","volume":"14","author":"Szeto","year":"2001","journal-title":"Mod. Pathol."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"546","DOI":"10.1038\/ki.2009.168","article-title":"The Oxford classification of IgA nephropathy: Pathology definitions, correlations, and reproducibility","volume":"76","author":"Roberts","year":"2009","journal-title":"Kidney Int."},{"key":"ref_13","first-page":"229","article-title":"Henoch-Sch\u00f6nlein purpura (IgA vasculitis): Rapid evidence review","volume":"102","author":"Reamy","year":"2020","journal-title":"Am. Fam. Physician"},{"key":"ref_14","first-page":"437","article-title":"Anaphylactoid purpura. II. Immunofluorescent and electron microscopic studies of the glomerular lesions","volume":"19","author":"Urizar","year":"1968","journal-title":"Lab. Investig."},{"key":"ref_15","first-page":"939","article-title":"IgA glomerular deposits in renal disease","volume":"1","author":"Berger","year":"1969","journal-title":"Transplant. Proc."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"326","DOI":"10.1136\/bmj.3.5875.326","article-title":"Glomerular deposition of properdin in Henoch-Sch\u00f6nlein syndrome and idiopathic focal nephritis","volume":"3","author":"Evans","year":"1973","journal-title":"Br. Med. J."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"1373","DOI":"10.1016\/j.ekir.2018.07.021","article-title":"Clinical characteristics and treatment patterns of children and adults with IgA nephropathy or IgA vasculitis: Findings from the CureGN study","volume":"3","author":"Selewski","year":"2018","journal-title":"Kidney Int. Rep."},{"key":"ref_18","doi-asserted-by":"crossref","unstructured":"Pillebout, E., Jamin, A., Ayari, H., Housset, P., Pierre, M., Sauvaget, V., Viglietti, D., Deschenes, G., Monteiro, R.C., and Berthelot, L. (2017). Biomarkers of IgA vasculitis nephritis in children. PLoS ONE, 12.","DOI":"10.1371\/journal.pone.0188718"},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"1432","DOI":"10.1172\/JCI109998","article-title":"Selective deposition of immunoglobulin A1 in immunoglobulin A nephropathy, anaphylactoid purpura nephritis, and systemic lupus erythematosus","volume":"66","author":"Conley","year":"1980","journal-title":"J. Clin. Investig."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"700","DOI":"10.1016\/j.kint.2017.10.019","article-title":"IgA nephropathy and IgA vasculitis with nephritis have a shared feature involving galactose-deficient IgA1-oriented pathogenesis","volume":"93","author":"Suzuki","year":"2018","journal-title":"Kidney Int."},{"key":"ref_21","first-page":"2246","article-title":"Alterations in the O-linked glycosylation of IgA1 in children with Henoch-Sch\u00f6nlein purpura","volume":"24","author":"Saulsbury","year":"1997","journal-title":"J. Rheumatol."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"930","DOI":"10.1093\/ndt\/13.4.930","article-title":"Abnormal IgA glycosylation in Henoch-Sch\u00f6nlein purpura restricted to pa-tients with clinical nephritis","volume":"13","author":"Allen","year":"1998","journal-title":"Nephrol Dial Transplant."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"2067","DOI":"10.1007\/s00467-007-0623-y","article-title":"Serum levels of galactose-deficient IgA in children with IgA nephropathy and Henoch-Schoenlein purpura","volume":"22","author":"Lau","year":"2007","journal-title":"Ped. Nephrol."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"162","DOI":"10.5114\/ceji.2018.77386","article-title":"Serum GDIgA1 levels in children with IgA nephropathy and Henoch-Sch\u00f6nlein nephritis","volume":"43","author":"Gajewski","year":"2018","journal-title":"Cent. Eur. J. Immunol."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"1481","DOI":"10.1016\/j.transproceed.2019.01.122","article-title":"Evaluation of IgA1 O-glycosylation in Henoch-Sch\u00f6nlein purpura nephritis using mass spectrometry","volume":"51","author":"Nakazawa","year":"2019","journal-title":"Transplant. Proc."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"134","DOI":"10.1159\/000102455","article-title":"IgA nephropathy and Henoch-Schoenlein purpura nephritis: Aberrant glycosylation of IgA1, formation of IgA1-containing immune complexes, and activation of mesangial cells","volume":"157","author":"Novak","year":"2007","journal-title":"Contrib. Nephrol."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"1148","DOI":"10.1038\/sj.ki.5002185","article-title":"Patients with IgA nephropathy have increased serum galactose-deficient IgA1 levels","volume":"71","author":"Moldoveanu","year":"2007","journal-title":"Kidney Int."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1038\/ki.2011.16","article-title":"Aberrant glycosylation of IgA1 is inherited in both pediatric IgA nephropathy and Henoch-Sch\u00f6nlein purpura nephritis","volume":"80","author":"Kiryluk","year":"2011","journal-title":"Kidney Int."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"1717","DOI":"10.1016\/j.ekir.2019.08.015","article-title":"Autoantibodies specific for galactose-deficient IgA1 in IgA vasculitis with nephritis","volume":"4","author":"Suzuki","year":"2019","journal-title":"Kidney Int. Rep."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"73","DOI":"10.1172\/JCI5535","article-title":"Circulating immune complexes in IgA nephropathy consist of IgA1 with galactose-deficient hinge region and antiglycan antibodies","volume":"104","author":"Tomana","year":"1999","journal-title":"J. Clin. Investig."},{"key":"ref_31","first-page":"1668","article-title":"Aberrantly glycosylated IgA1 in IgA nephropathy patients is recognized by IgG antibodies with restricted heterogeneity","volume":"119","author":"Suzuki","year":"2009","journal-title":"J. Clin. Investig."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"3278","DOI":"10.1681\/ASN.2014101044","article-title":"Somatic mutations modulate autoantibodies against galactose-deficient IgA1 in IgA nephropathy","volume":"27","author":"Huang","year":"2016","journal-title":"J. Am. Soc. Nephrol."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"2017","DOI":"10.1681\/ASN.2018111156","article-title":"Glomerular immunodeposits of patients with IgA nephropathy are enriched for IgG autoantibodies specific for galactose-deficient IgA1","volume":"30","author":"Rizk","year":"2019","journal-title":"J. Am. Soc. Nephrol."},{"key":"ref_34","doi-asserted-by":"crossref","unstructured":"Moldoveanu, Z., Suzuki, H., Reily, C., Satake, K., Novak, L., Xu, N., Huang, Z.-Q., Knoppova, B., Khan, A., and Hall, S. (2021). Experimental evidence of pathogenic role of IgG autoantibodies in IgA nephropathy. J. Autoimmun., 118.","DOI":"10.1016\/j.jaut.2021.102593"},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"504","DOI":"10.1111\/j.1523-1755.2005.67107.x","article-title":"IgA1-containing immune complexes in IgA nephropathy differentially affect proliferation of mesangial cells","volume":"67","author":"Novak","year":"2005","journal-title":"Kidney Int."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"3451","DOI":"10.1093\/ndt\/gfr448","article-title":"IgA1 immune complexes from pediatric patients with IgA nephropathy activate cultured human mesangial cells","volume":"26","author":"Novak","year":"2011","journal-title":"Nephrol. Dial. Transplant."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"622","DOI":"10.1097\/01.ASN.0000115401.07980.0C","article-title":"Glycosylation and size of IgA1 are essential for interaction with mesangial transferrin receptor in IgA nephropathy","volume":"15","author":"Moura","year":"2004","journal-title":"J. Am. Soc. Nephrol."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"1503","DOI":"10.1681\/ASN.2014101000","article-title":"Current Understanding of the role of complement in IgA nephropathy","volume":"26","author":"Maillard","year":"2015","journal-title":"J. Am. Soc. Nephrol."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"993","DOI":"10.1053\/j.ajkd.2006.02.178","article-title":"Predictors of outcome in Henoch-Sch\u00f6nlein nephritis in children and adults","volume":"47","author":"Coppo","year":"2006","journal-title":"Am. J. Kidney Dis."},{"key":"ref_40","first-page":"230","article-title":"Circulating immune complexes containing IgA, IgG and IgM in patients with primary IgA nephropathy and with Henoch-Sch\u00f6nlein nephritis. Correlation with clinical and histologic signs of activity","volume":"18","author":"Coppo","year":"1982","journal-title":"Clin. Nephrol."},{"key":"ref_41","first-page":"583","article-title":"IgA1 and IgA2 immune complexes in primary IgA nephropathy and Henoch-Sch\u00f6nlein nephritis","volume":"57","author":"Coppo","year":"1984","journal-title":"Clin. Exp. Immunol."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"2277","DOI":"10.1093\/ndt\/12.11.2277","article-title":"Long-term prognosis of Henoch-Sch\u00f6nlein nephritis in adults and children","volume":"12","author":"Coppo","year":"1997","journal-title":"Nephrol. Dial. Transplant."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"11","DOI":"10.1136\/bmj.2.6078.11","article-title":"Prognosis of Henoch-Sch\u00f6nlein nephritis in children","volume":"2","author":"Counahan","year":"1977","journal-title":"BMJ"},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"1795","DOI":"10.1681\/ASN.2011050464","article-title":"The pathophysiology of IgA nephropathy","volume":"22","author":"Suzuki","year":"2011","journal-title":"J. Am. Soc. Nephrol."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"790","DOI":"10.1038\/ki.2012.197","article-title":"The level of galactose-deficient IgA1 in the sera of patients with IgA nephropathy is associated with disease progression","volume":"82","author":"Zhao","year":"2012","journal-title":"Kidney Int."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"1579","DOI":"10.1681\/ASN.2012010053","article-title":"Autoantibodies targeting galactose-deficient IgA1 associate with progression of IgA nephropathy","volume":"23","author":"Berthoux","year":"2012","journal-title":"J. Am. Soc. Nephrol."},{"key":"ref_47","doi-asserted-by":"crossref","unstructured":"Maixnerov\u00e1, D., Ling, C., Hall, S., Reily, C., Brown, R., Neprasova, M., Suchanek, M., Honsova, E., Zima, T., and Novak, J. (2019). Galactose-deficient IgA1 and the corresponding IgG autoantibodies predict IgA nephropathy progression. PLoS ONE, 14.","DOI":"10.1371\/journal.pone.0219947"},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"969","DOI":"10.1046\/j.1523-1755.2001.060003969.x","article-title":"Mesangial IgA1 in IgA nephropathy exhibits aberrant O-glycosylation: Observations in three patients","volume":"60","author":"Allen","year":"2001","journal-title":"Kidney Int."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"1077","DOI":"10.1046\/j.1523-1755.2001.0590031077.x","article-title":"Mass spectrometry proves under-O-glycosylation of glomerular IgA1 in IgA nephropathy","volume":"59","author":"Hiki","year":"2001","journal-title":"Kidney Int."},{"key":"ref_50","first-page":"629","article-title":"IgA1-secreting cell lines from patients with IgA nephropathy produce aberrantly glycosylated IgA1","volume":"118","author":"Suzuki","year":"2008","journal-title":"J. Clin. Investig."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"5330","DOI":"10.1074\/jbc.M113.512277","article-title":"Cytokines alter IgA1 O-glycosylation by dysregulating C1GalT1 and ST6GalNAc-II enzymes","volume":"289","author":"Suzuki","year":"2014","journal-title":"J. Biol. Chem."}],"container-title":["Journal of Clinical Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2077-0383\/10\/21\/4851\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T07:20:55Z","timestamp":1760167255000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2077-0383\/10\/21\/4851"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,10,22]]},"references-count":51,"journal-issue":{"issue":"21","published-online":{"date-parts":[[2021,11]]}},"alternative-id":["jcm10214851"],"URL":"https:\/\/doi.org\/10.3390\/jcm10214851","relation":{},"ISSN":["2077-0383"],"issn-type":[{"value":"2077-0383","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,10,22]]}}}