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Pediatric IgA Nephropathy Clinical Presentation

  • Author: Mohammad Ilyas, MD, FAAP; Chief Editor: Craig B Langman, MD  more...
 
Updated: Feb 27, 2014
 

History

Immunoglobulin A (IgA) nephropathy (IgAN) is characterized by recurrent episodes of macroscopic hematuria accompanied by upper respiratory tract infections or persistent asymptomatic microscopic hematuria with or without proteinuria. IgA nephropathy is frequently classified as primary (idiopathic) or secondary (associated with some other known condition).

Primary IgA nephropathy

Although the clinical presentation of IgA nephropathy varies from asymptomatic urinary abnormalities to acute renal failure, 5 different clinical syndromes are generally recognized.

The most common presentation (approximately 60-80%) of IgA nephropathy is asymptomatic microscopic urinary abnormalities with one or more episodes of intermittent gross hematuria. The recurrent macroscopic hematuria often associated with upper respiratory infection (viral pharyngitis) is traditionally regarded as the hallmark of childhood IgA nephropathy, compared with poststreptococcal glomerulonephritis (PSGN), in which hematuria usually occurs 1-2 weeks after infection. The hematuria is usually painless, but loin pain has been reported. Blood pressure may be within the reference range or elevated. Renal clearance function is within the reference range or reduced.

The second most common presentation (approximately 26%) is asymptomatic microscopic hematuria with or without mild proteinuria, hypertension, or reduced renal clearance function.

Acute nephritic presentation (approximately 12%) with heavy proteinuria, normal or low clearance function, and normal or high blood pressure is the third most common presentation.

Nephrotic syndrome may be the initial presentation in as many as 10% of patients.

Rarely, IgA nephrology may present as an acute crescentic glomerulonephritis with oliguria, edema, and hypertension.

Secondary IgA nephropathy

When renal mesangial IgA deposition occurs because of another specific clinical condition (secondary IgA nephrology), the history of that disease or signs and symptoms related to the primary condition may be present.

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Physical

In the early stages of primary IgA nephropathy, no physical signs may be observed. However, early diagnosis might be suggested by a urinalysis that reveals microscopic hematuria with or without proteinuria.

Hypertension is infrequent, is mild to moderate, and is usually a late presentation of disease.

Edema due to nephrosis is reported in approximately 10% of patients.

If renal function is compromised at presentation, the patients may have signs of uremic syndrome, anemia, pallor, and lethargy.

If IgA nephrology is secondary to underlying disease, such Henoch-Schönlein purpura (HSP) or systemic lupus erythematosus (SLE), the signs and symptoms of that specific primary disease may be apparent.

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Causes

The cause of primary IgA nephropathy is unknown. The conditions producing secondary mesangial IgA deposition include the following:

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Contributor Information and Disclosures
Author

Mohammad Ilyas, MD, FAAP Assistant Professor of Pediatrics, University of Florida College of Medicine; Consulting Staff, Department of Pediatrics, Section of Nephrology, Wolfson Children Hospital and Shands Hospital Jacksonville

Mohammad Ilyas, MD, FAAP is a member of the following medical societies: American Academy of Pediatrics, American Society of Nephrology

Disclosure: Nothing to disclose.

Coauthor(s)

Richard Neiberger, MD, PhD Director of Pediatric Renal Stone Disease Clinic, Associate Professor, Department of Pediatrics, Division of Nephrology, University of Florida College of Medicine and Shands Hospital

Richard Neiberger, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, American Federation for Medical Research, American Medical Association, American Society of Nephrology, American Society of Pediatric Nephrology, Christian Medical and Dental Associations, Florida Medical Association, International Society for Peritoneal Dialysis, International Society of Nephrology, National Kidney Foundation, New York Academy of Sciences, Shock Society, Sigma Xi, Southern Medical Association, Southern Society for Pediatric Research, Southwest Pediatric Nephrology Study Group

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Adrian Spitzer, MD Clinical Professor Emeritus, Department of Pediatrics, Albert Einstein College of Medicine

Adrian Spitzer, MD is a member of the following medical societies: American Academy of Pediatrics, American Federation for Medical Research, American Pediatric Society, American Society of Nephrology, American Society of Pediatric Nephrology, International Society of Nephrology, Society for Pediatric Research

Disclosure: Nothing to disclose.

Chief Editor

Craig B Langman, MD The Isaac A Abt, MD, Professor of Kidney Diseases, Northwestern University, The Feinberg School of Medicine; Division Head of Kidney Diseases, The Ann and Robert H Lurie Children's Hospital of Chicago

Craig B Langman, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Nephrology, International Society of Nephrology

Disclosure: Received income in an amount equal to or greater than $250 from: Alexion Pharmaceuticals; Raptor Pharmaceuticals; Eli Lilly and Company; Dicerna<br/>Received grant/research funds from NIH for none; Received grant/research funds from Raptor Pharmaceuticals, Inc for none; Received grant/research funds from Alexion Pharmaceuticals, Inc. for none; Received consulting fee from DiCerna Pharmaceutical Inc. for none.

Additional Contributors

Deogracias Pena, MD Medical Director of Dialysis, Medical Director of Pediatric Nephrology and Transplantation, Cook Children's Medical Center; Clinical Associate Professor, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Medical Director of Pediatric Nephrology, Florida Hospital for Children

Deogracias Pena, MD is a member of the following medical societies: American Academy of Pediatrics, American Medical Association, American Society of Pediatric Nephrology

Disclosure: Nothing to disclose.

References
  1. D'Amico G. Natural history of idiopathic IgA nephropathy and factors predictive of disease outcome. Semin Nephrol. 2004 May. 24(3):179-96. [Medline].

  2. Li LS; Liu ZH. Epidemiologic data of renal diseases from a single unit in China: analysis based on 13,519 renal biopsies. Kidney Int. 2004 Sep. 66(3):920-3. [Medline].

  3. Novak J, Julian BA, Mestecky J, Renfrow MB. Glycosylation of IgA1 and pathogenesis of IgA nephropathy. Semin Immunopathol. 2012 May. 34(3):365-82. [Medline].

  4. Segarra A. [Progress in understanding the pathogenesis of IgA nephropathy: new perspectives for the near future?]. Nefrologia. 2010. 30(5):501-7. [Medline].

  5. Narita I, Gejyo F. Pathogenetic significance of aberrant glycosylation of IgA1 in IgA nephropathy. Clin Exp Nephrol. 2008 Oct. 12(5):332-8. [Medline].

  6. Suzuki H, Kiryluk K, Novak J, et al. The pathophysiology of IgA nephropathy. J Am Soc Nephrol. 2011 Oct. 22(10):1795-803. [Medline].

  7. Yu HH, Chu KH, Yang YH, et al. Genetics and immunopathogenesis of IgA nephropathy. Clin Rev Allergy Immunol. 2011 Oct. 41(2):198-213. [Medline].

  8. Novak J, Moldoveanu Z, Julian BA, et al. Aberrant glycosylation of IgA1 and anti-glycan antibodies in IgA nephropathy: role of mucosal immune system. Adv Otorhinolaryngol. 2011. 72:60-3. [Medline].

  9. [Guideline] Cattran DC, Coppo R, Cook HT, et al. The Oxford classification of IgA nephropathy: rationale, clinicopathological correlations, and classification. Kidney Int. 2009 Sep. 76(5):534-45. [Medline].

  10. Lai FM, Szeto CC, Choi PC, et al. Characterization of early IgA nephropathy. Am J Kidney Dis. 2000 Oct. 36(4):703-8. [Medline].

  11. Glassock RJ. Analyzing antibody activity in IgA nephropathy. J Clin Invest. 2009 Jun. 119(6):1450-2. [Medline]. [Full Text].

  12. Hasbargen JA, Copley JB. Utility of skin biopsy in the diagnosis of IgA nephropathy. Am J Kidney Dis. 1985 Aug. 6(2):100-2. [Medline].

  13. Berthoux F, Mohey H, Laurent B, Mariat C, Afiani A, Thibaudin L. Predicting the risk for dialysis or death in IgA nephropathy. J Am Soc Nephrol. 2011 Apr. 22(4):752-61. [Medline]. [Full Text].

  14. Reich HN, Troyanov S, Scholey JW, Cattran DC. Remission of proteinuria improves prognosis in IgA nephropathy. J Am Soc Nephrol. 2007 Dec. 18(12):3177-83. [Medline].

  15. Kunz R, Friedrich C, Wolbers M, Mann JF. Meta-analysis: effect of monotherapy and combination therapy with inhibitors of the renin angiotensin system on proteinuria in renal disease. Ann Intern Med. 2008 Jan 1. 148(1):30-48. [Medline].

  16. Donadio JV Jr, Larson TS, Bergstralh EJ, Grande JP. A randomized trial of high-dose compared with low-dose omega-3 fatty acids in severe IgA nephropathy. J Am Soc Nephrol. 2001 Apr. 12(4):791-9. [Medline]. [Full Text].

  17. Appel GB, Waldman M. The IgA nephropathy treatment dilemma. Kidney Int. 2006 Jun. 69(11):1939-44. [Medline].

  18. Floege J, Eitner F. Present and future therapy options in IgA-nephropathy. J Nephrol. 2005 Jul-Aug. 18(4):354-61. [Medline].

  19. Manno C, Torres DD, Rossini M, Pesce F, Schena FP. Randomized controlled clinical trial of corticosteroids plus ACE-inhibitors with long-term follow-up in proteinuric IgA nephropathy. Nephrol Dial Transplant. 2009 Dec. 24(12):3694-701. [Medline].

  20. Ballardie FW, Roberts IS. Controlled prospective trial of prednisolone and cytotoxics in progressive IgA nephropathy. J Am Soc Nephrol. 2002 Jan. 13(1):142-8. [Medline].

  21. Tumlin JA, Lohavichan V, Hennigar R. Crescentic, proliferative IgA nephropathy: clinical and histological response to methylprednisolone and intravenous cyclophosphamide. Nephrol Dial Transplant. 2003 Jul. 18(7):1321-9. [Medline].

  22. Maes BD, Oyen R, Claes K, et al. Mycophenolate mofetil in IgA nephropathy: results of a 3-year prospective placebo-controlled randomized study. Kidney Int. 2004 May. 65(5):1842-9. [Medline].

  23. Tang SC, Tang AW, Wong SS, Leung JC, Ho YW, Lai KN. Long-term study of mycophenolate mofetil treatment in IgA nephropathy. Kidney Int. 2010 Mar. 77(6):543-9. [Medline].

  24. Frisch G, Lin J, Rosenstock J, Markowitz G, et al. Mycophenolate mofetil (MMF) vs placebo in patients with moderately advanced IgA nephropathy: a double-blind randomized controlled trial. Nephrol Dial Transplant. 2005 Oct. 20(10):2139-45. [Medline].

  25. Cattran DC. Current status of cyclosporin A in the treatment of membranous, IgA and membranoproliferative glomerulonephritis. Clin Nephrol. 1991. 35 Suppl 1:S43-7. [Medline].

  26. Béné MC, Faure GC, Hurault de Ligny B, de March AK. Clinical involvement of the tonsillar immune system in IgA nephropathy. Acta Otolaryngol Suppl. 2004 Dec. 10-4. [Medline].

  27. Xie Y, Chen X, Nishi S, Narita I, Gejyo F. Relationship between tonsils and IgA nephropathy as well as indications of tonsillectomy. Kidney Int. 2004 Apr. 65(4):1135-44. [Medline].

  28. Rasche FM, Schwarz A, Keller F. Tonsillectomy does not prevent a progressive course in IgA nephropathy. Clin Nephrol. 1999 Mar. 51(3):147-52. [Medline].

  29. Coppo R, Roccatello D, Amore A, et al. Effects of a gluten-free diet in primary IgA nephropathy. Clin Nephrol. 1990 Feb. 33(2):72-86. [Medline].

  30. Rostoker G, Desvaux-Belghiti D, Pilatte Y, et al. High-dose immunoglobulin therapy for severe IgA nephropathy and Henoch-Schönlein purpura. Ann Intern Med. 1994 Mar 15. 120(6):476-84. [Medline].

  31. Krebs S, Omer B, Omer TN, Fliser D. Wormwood (Artemisia absinthium) for poorly responsive early-stage IgA nephropathy: a pilot uncontrolled trial. Am J Kidney Dis. 2010 Dec. 56(6):1095-9. [Medline].

  32. Abe S. Pregnancy in IgA nephropathy. Kidney Int. 1991 Dec. 40(6):1098-102. [Medline].

  33. Choy BY, Chan TM, Lai KN. Recurrent glomerulonephritis after kidney transplantation. Am J Transplant. 2006 Nov. 6(11):2535-42. [Medline].

  34. Odum J, Peh CA, Clarkson AR, et al. Recurrent mesangial IgA nephritis following renal transplantation. Nephrol Dial Transplant. 1994. 9(3):309-12. [Medline].

  35. Ponticelli C, Traversi L, Feliciani A, Cesana BM, Banfi G, Tarantino A. Kidney transplantation in patients with IgA mesangial glomerulonephritis. Kidney Int. 2001 Nov. 60(5):1948-54. [Medline].

  36. Geddes CC, Rauta V, Gronhagen-Riska C, Bartosik LP, Jardine AG, Ibels LS. A tricontinental view of IgA nephropathy. Nephrol Dial Transplant. 2003 Aug. 18(8):1541-8. [Medline].

  37. Szeto CC, Lai FM, To KF, et al. The natural history of immunoglobulin a nephropathy among patients with hematuria and minimal proteinuria. Am J Med. 2001 Apr 15. 110(6):434-7. [Medline].

  38. Wakai K, Kawamura T, Endoh M, et al. A scoring system to predict renal outcome in IgA nephropathy: from a nationwide prospective study. Nephrol Dial Transplant. 2006 Oct. 21(10):2800-8. [Medline].

  39. Alamartine E, Sabatier JC, Berthoux FC. Comparison of pathological lesions on repeated renal biopsies in 73 patients with primary IgA glomerulonephritis: value of quantitative scoring and approach to final prognosis. Clin Nephrol. 1990 Aug. 34(2):45-51. [Medline].

  40. Rekola S, Bergstrand A, Bucht H. Deterioration of GFR in IgA nephropathy as measured by 51Cr-EDTA clearance. Kidney Int. 1991 Dec. 40(6):1050-4. [Medline].

  41. Donadio JV, Bergstralh EJ, Grande JP, Rademcher DM. Proteinuria patterns and their association with subsequent end-stage renal disease in IgA nephropathy. Nephrol Dial Transplant. 2002 Jul. 17(7):1197-203. [Medline].

 
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Glomerulus with mesangial hypercellularity and intact capillary loops. Trichrome Stain, original magnification 400x. Image courtesy of Patrick D Walker, MD.
Mesangial deposits of immunoglobulin A (IgA). Fluoresceinated Anti-IgA Antibody, Immunofluorescence microscopy, original magnification 400x. Image courtesy of Patrick D Walker, MD.
Electron photomicrograph showing mesangial electron dense deposits (arrow). Uranyl acetate and lead citrate stain, original magnification 12,000x. Image courtesy of Patrick D Walker, MD.
 
 
 
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