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Currently, multiple treatment options are available; no one therapy is appropriate for all patients. J Nephrol. Long-term renal survival and related risk factors in patients with IgA nephropathy: results from a cohort of 1155 cases in a Chinese adult population. [Full Text]. [52], Some patients with post-transplantation IgA nephropathy present with microscopic hematuria and proteinuria, while others have only positive histologic findings. 69(11):1939-44. . [Medline]. [30], The Supportive Versus Immunosuppressive Therapy for the Treatment of Progressive IgA Nephropathy (STOP-IgAN) trial showed that after 3 years, full clinical remission had occurred in 5% of patients in the supportive-care group, as compared with 17% of patients who received immunosuppression with steroids plus cyclophosphamide followed by azathioprine. Vecchio M, Bonerba B, Palmer SC, Craig JC, Ruospo M, Samuels JA, et al. 2019 Jan 8;16:2. doi: 10.1186/s12986-018-0328-1. 69(11):1939-44. [41], CD19 B-cells are increased in IgA nephropathy. Treatment of IgA nephropathy: Recent advances and prospects. 2015 Nov. 88 (5):974-89. doi: 10.1016/j.nephro.2018.02.010. As a result, the kidneys begin to let substances such as blood and protein leak into the urine. eCollection 2020. Watanabe H, Goto S, Kondo D, Takata T, Yamazaki H, Hosojima M, et al. [23, 36]  The KDIGO guidelines do not recommend use of mycophenolate mofetil. American Federation for Clinical Research, International Society for Peritoneal Dialysis, Southern Society for Clinical Investigation. 35(2):194-201. PLoS One. N Engl J Med. Kidney transplantation is effective in patients with IgA nephropathy, but the disorder frequently recurs after transplantation (20-60%). Clinical predictors (UptoDate: Treatment and Prognosis of IgA Nephropathy [Accessed 6 April 2020]): High serum creatinine / reduced eGFR Hypertension (> 140/90 mmHg) Proteinuria > 1 g/24 h for over 6 months Nat Rev Nephrol. In general there is no benefit gained by adding corticosteroids to renin-angiotensin blockade when proteinuria < 1 g/day. [38]. J Investig Med. 2004 Jan. 15(1):157-63. [Medline]. Tibor Fulop, MD, PhD, FACP, FASN Professor of Medicine, Department of Medicine, Division of Nephrology, Medical University of South Carolina College of Medicine; Attending Physician, Medical Services, Ralph H Johnson VA Medical Center Recent international collaborative efforts have led to important discoveries that have improved our understanding of some of the key steps involved in the immunopathogenesis of IgAN. Dr. Anass Ahmed Qasem 2. The study found that adding calcitriol to a renin-angiotensin system inhibitor resulted in a safe decrease in proteinuria. [Medline]. Kidney Int. Kidney Int. Gharavi AG, Yan Y, Scolari F, et al. Please enable it to take advantage of the complete set of features! Many diseases can affect your kidney function by attacking and damaging the glomeruli, the tiny filtering units inside your kidney where blood is cleaned. 2012 Oct. 23(10):1753-1760. 70:S56-62. IgA nephropathy is one of the most common causes of glomerulonephritis in the world and is characterized histologically by the deposition of polymeric forms of IgA within the mesangium and along glomerular capillary walls [].The binding of IgA to putative Fc receptors on the surface of mesangial cells leads to mesangial hypercellularity and production of proinflammatory cytokines. [Full Text]. Klahr S, Levey AS, Beck GJ, et al. [Medline]. Low-protein diets have been recommended to slow the rate of progression of many nephropathies. Nephrol Dial Transplant 2011; 26:3237. Intensive Supportive Care plus Immunosuppression in IgA Nephropathy. [Medline]. 2010 Mar. Am J Kidney Dis 1998; 31:713. Effects of dietary protein restriction on the progression of advanced renal disease in the Modification of Diet in Renal Disease Study. It affects millions of people worldwide. Fellström BC, Barratt J, Cook H, et al. J Am Soc Nephrol. Tonsillectomy may limit the production of degalactosylated IgA1 by reducing mucosal-associated lymphoid tissue (MALT). 2019 Jul. Sohail Abdul Salim, MD, FASN, FACP is a member of the following medical societies: American College of Physicians, American Society of Nephrology, Mississippi State Medical Association, Renal Physicians AssociationDisclosure: Nothing to disclose. 33(5):857-65. [30]  Use of mycophenolate mofetil has been controversial, as studies have been small in size and studies have reported negative results. Low α-defensin gene copy number increases the risk for IgA nephropathy and renal dysfunction. Ai Z, Li M, Liu W, Foo JN, Mansouri O, Yin P, et al. Inhibitors of these cytokines are potential therapeutic targets. The benefits and risks of steroids for the treatment of IgA nephropathy remain uncertain. Douglas Somers, MD Assistant Professor, Department of Internal Medicine, Division of Nephrology, University of Iowa Medical Center, Douglas Somers, MD is a member of the following medical societies: American Society of Nephrology. 389 (10084):2117-2127. [Medline]. Kovács T, Vas T, Kövesdy CP, Degrell P, Nagy G, Rékási Z, et al. If UPCR is > 1.5 g/day despite maximum renin-angiotensin blockade for 4-6 months, steroids can be added, but not if the GFR is < 25 mL/min/1.73m2. Predicting progression of IgA nephropathy: new clinical progression risk score. [25]. J Am Soc Nephrol. Floege J, Feehally J. Appel GB, Waldman M. The IgA nephropathy treatment dilemma. Lancet. Rodrigues JC, Haas M, Reich HN. 8600 Rockville Pike Fish oil/prescription-strength omega-3 fatty acids: Fish oil has shown some promise in patients with … The conditions that affect your glomeruli are called glomerular diseases. 2016 Jun 28. However, the review concluded that the optimal management of IgA nephropathy remains uncertain, and larger controlled trials are needed. Recurrent disease was identified in only 2 of 28 patients during follow-up of 61.61 ± 47.23 months. 318 (5):432-442. Ferraro PM, Ferraccioli GF, Gambaro G, Fulignati P, Costanzi S. Nephrol Dial Transplant. Yu L, Jiang L, Zhou XJ, et al. James H Sondheimer, MD, FACP, FASN Professor of Medicine, Division Chief, Nephrology and Hypertension, Department of Medicine, Wayne State University School of Medicine; Medical Director, DaVita Kresge Dialysis (Detroit) 2017 Aug 1. 27(7):2806-13. Patients in the treatment group experienced an increased rate of steroid-related events including acne, hypertension, cushingoid features, mood swings, and hirsutism. 2017 May 27. 41:914–920. This impairs their filtering function. 2016 Aug. 29 (4):463-8. Christie P Thomas, MBBS, FRCP, FASN, FAHA Professor, Department of Internal Medicine, Division of Nephrology, Departments of Pediatrics and Obstetrics and Gynecology, Medical Director, Kidney and Kidney/Pancreas Transplant Program, University of Iowa Hospitals and Clinics [Medline]. New strategies and perspectives on managing IgA nephropathy. The Oxford classification of IgA nephropathy: rationale, clinicopathological correlations, and classification. /viewarticle/931857 The immunosuppressive treatment Cochrane review published in 2011, identified 1196 relevant citations from relevant medical databases, 1159 citations were excluded because they were not RCTs, did not include patients with biopsy-proven IgA nephropathy, had less than 75% of patients with IgA nephropathy in the trial, or were the wrong intervention. We systematically searched MEDLINE, EMBASE, and the Cochrane Library for randomized, controlled trials of corticosteroid therapy for IgA nephropathy published between 1966 and … Treatment focuses on slowing the disease and preventing complications. IgA nephropathy, identified 50 years ago in France, is the most frequent glomerular disease worldwide. IgA nephropathy, the most common cause of glomerulonephritis, is linked to 6q22-23. Conclusions: Supportive therapies, including angiotensin blockade, should be considered as first-line therapy for patients with urine protein >0.5 g/day and/or blood pressure >140/90 mm Hg. IgA Nephropathy is a relatively common kidney disease. General recommendations include the following: In patients with isolated hematuria (ie, without proteinuria or hypertension) monitor with urinalysis, renal function testing, and blood pressure measurement. Modification of Diet in Renal Disease Study Group. Rituximab had no significant effect on GFR, proteinuria, galactose-deficient IgA1 levels, or IgG autoantibodies. Am J Kidney Dis. The authors conclude that the results support the hypothesis that mucosal immune dysfunction has a significant role in the pathogenesis of IgA nephropathy. [23]  If proteinuria is > 1 g/day, then 125/75 mm Hg should be the goal. [Full Text]. Pei Pei Huang, Dan Hua Shu, Zhen Su, Sheng Nan Luo, Fei Fei Xu, Fan Lin. What is IgA Nephropathy? The role of direct renin inhibitors and mineralocorticoid receptor antagonists have not been evaluated in a randopmize controlled trial. If UPCR is persistently 0.75-1.5 g/day, the short-term benefits of steroid ues remain uncertain. [27]  In a randomized, controlled trial in 44 patients with biopsy-proven IgA nephropathy who had proteinuria and normal or moderately reduced renal function, Kaplan-Meier renal survival after 7 years was 92% in patients treated with enalapril versus 55% in the control group (P < 0.05). Kidney Int. 2016 May 23. Nat Genet. IgA has been covered extensively on the RFN before including the history, pathogenesis, diagnosis, features and treatment: collected in this group of posts. Glassock RJ. [28]. Mona Brake, MD is a member of the following medical societies: American College of Physicians, American Society of NephrologyDisclosure: Nothing to disclose. 2006 Jun. This website also contains material copyrighted by 3rd parties. Clipboard, Search History, and several other advanced features are temporarily unavailable. [Full Text]. 2:16002. Coppo R, Troyanov S, Camilla R, et al. 2017 Feb. 28 (2):691-701. 8 (345):345ra88. Front Pharmacol. 2016 Feb 11. [10]   In contrast, KDIGO guidelines suggest that tonsillectomy not be used for IgA nephropathy. Epub 2008 Aug 6. National Library of Medicine JAMA. COVID-19: What Do We Know About Hypercoagulation? [30]  Currently, most experts reserve tonsillectomy for patients who have tonsillar infection or tonsillitis. IgA nephropathy is the most common cause of primary (idiopathic) glomerulonephritis in the developed world. Tang SC, Tang AW, Wong SS, et al. Am J Kidney Dis. 2009 Jan. 53 (1):26-32. 77(6):543-9. Epidemiology of IgA nephropathy in central and eastern Kentucky for the period 1975 through 1994. Moderator's view: Treatment of IgA nephropathy-getting comfortable with uncertainty. Azathioprine: Ineffective and possibly even harmful, Calcineurin Inhibitors: Possibly effective; nephrotoxic and not studied in randomized controlled trials, Adrenocorticotropic hormone (ACTH) gel (Acthar gel): A prospective pilot study of ACTH gel in a dose of 80 units subcutaneously twice weekly for 6 months reported that patients with  urinary protein >1 g/24-hour and eGFR >30 ml/min had a significant reduction in 24-hour urinary protein with stable eGFR at 12-month follow-up. Effect of tonsillectomy and its timing on renal outcomes in Caucasian IgA nephropathy patients. Light microscopy of a glomerulus from a patient with immunoglobulin A nephropathy showing increased mesangial matrix and cellularity. Use of immunosuppression should be determined by considering the rate of progression, comorbidities, and whether alarming features are present on biopsy. Christie P Thomas, MBBS, FRCP, FASN, FAHA is a member of the following medical societies: American College of Physicians, American Heart Association, American Society of Nephrology, Royal College of PhysiciansDisclosure: Nothing to disclose. Epub 2019 Feb 13. Gutiérrez E, Zamora I, Ballarín JA, Arce Y, Jiménez S, Quereda C, et al. The KDIGO guidelines suggest not treating with corticosteroids combined with cyclophosphamide or azathioprine unless the patient has crescentic IgA nephropathy with rapidly deteriorating kidney function. J Am Soc Nephrol. Control of proteinuria is prudent, since there is a mostly linear association between the severity of proteinuria and decline in estimated glomerular filtration rate (GFR). Prevention and treatment information (HHS). Kidney disease usually cannot be cured. Since the efficacy of mycophenolate mofetil (MMF) to treat immunoglobulin A (IgA) nephropathy is controversial, we extended our original study by following 40 Chinese patients with established IgA nephropathy for 6 years. [34]. [Medline]. [26], Angiotensin-converting enzyme inhibitors (ACEIs) or angiotensin receptor blockers (ARBs) are the preferred agents for lowering blood pressure and decreasing proteinuria. [Medline]. [Medline]. [39]  A phase 3 trial of targeted-release budesonide, the NefigArd trial, is currently in progress. [Medline]. Am J Kidney Dis. [Medline]. Since the efficacy of mycophenolate mofetil (MMF) to treat immunoglobulin A (IgA) nephropathy is controversial, we extended our original study by following 40 Chinese patients with established IgA nephropathy for 6 years. Kidney Int. J Am Soc Nephrol. Tonsillectomy is appropriate only for patients with recurrent tonsillar infections. Clinical predictors (UptoDate: Treatment and Prognosis of IgA Nephropathy [Accessed 6 April 2020]): High serum creatinine / reduced eGFR Hypertension (> 140/90 mmHg) Proteinuria > 1 g/24 h for over 6 months Long-Term Outcomes of IgA Nephropathy Presenting with Minimal or No Proteinuria. IgA Nephropathy. Nephrol Dial Transplant. Cochrane Database Syst Rev. [Medline]. Changes in the diagnosis of glomerular diseases in east China: a 15-year renal biopsy study. [Full Text]. Manno C, Torres DD, Rossini M, et al. Moderator's view: Treatment of IgA nephropathy-getting comfortable with uncertainty. Until less toxic therapies for IgAN are available, treatment with corticosteroids will need to be made in the context of conflicting evidence, and should likely be limited to patients at highest risk of disease progression who understand the significant risk of adverse events. Long-term outcome of IgA nephropathy patients with recurrent macroscopic hematuria. 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