IgA nephropathy natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Rim Halaby, M.D. [2]

Natural History

The clinical course of IgA nephropathy varies widely between patients. Although it is generally regarded as a benign disease, emerging data has shown that progression to ESRD and death are more common than originally believed. Some patients rapidly progress into ESRD; but the majority experience a stable kidney function following diagnosis. Commonly, the progression of IgA nephropathy is slower than other notorious glomerular disease. Approximately 20-30% of patients with IgA nephropathy progress to ESRD after 10 years and up to 30-50% of patients develop ESRD over 20 years.

Complications

Complications of IgA nephropathy are generally those of renal injury.[1][2][3]

Prognosis

IgA nephropathy follows an unpredictable clinical course with debatable prognostic factors. Although considered a benign disease in comparison to other forms of glomerulonephritis, new data show that up to 20-30% of patients with IgA nephropathy progress to ESRD after 10 years.[1] It is important, however, to note that experts concede that the true prognosis of IgA nephropathy is poorly established because the diagnosis by biopsy is often made late during stage 3-4 chronic kidney disease.[3] The approximately 10-year renal survival following diagnosis ranges between 67-94%, based on the findings of 5 major trials from Germany, France, UK, Japan, and Australia and 1 meta-analysis from USA.[4][5][6][7][8]


Several studies have analyzed factors associated with prognosis of IgA nephropathy. In 2011, Berthoux and colleagues established 3 main factors that have been attributed to be the core predictors of outcome when studying 332 patients with IgA nephropathy over 13 years[9]:

Proteinuria > 1g/24 hrs

Severe pathologic lesions with a global optical score ≥ 8

Hypertension > 140/90 mmHg

Proteinuria is the most important prognostic factor with a “dose-dependent” effect[10]


In 2011, Berthoux et al. calculated absolute renal risk (ARR) of dialysis or death.[9] The absence of all 3 risk factors was associated with a 96% prediction of survival without hemodialysis.[9] As ARR increased, survival prediction decreased, where the presence of all 3 risk factors was associated with only 36% prediction of survival without the need for dialysis.[9]

In one major meta-analysis that involved a database of 148 patients with IgA nephropathy between 1973 and 1995, Radford and colleagues[3] suggested a “glomerular score” based on previous findings from the literature that consists of the summation of 6 components:

  • Mesangial hypercellularity
  • Mesangial matrix increase
  • Glomerular sclerosis
  • Capillary narrowing or disruption
  • Cellular crescents
  • Fibrous adhesions


Observational, cross-sectional, and cohort studies to date have shown the following data to be significantly associated with progression of IgA nephropathy into ESRD and worse outcome. However, the significance of the following is variable and has not been consistent in the literature.

Genetic:

Clinical:

Biochemical:

Histopathological:

  • Mesangial hypercellularity and diffuse mesangial proliferation[16][17][18]
  • Tubular atrophy


Age remains a controversial predictor of outcome for patients with IgA nephropathy. While some studies showed that younger age is associated with worse outcomes[3], these findings were not consistent in the literature and at times, completely opposing.[16][4]

Biological Biomarkers of Progression
Change in Serum Marker Biological Marker
Increased
Decreased

References

  1. 1.0 1.1 Haubitz M, Wittke S, Weissinger EM, Walden M, Rupprecht HD, Floege J; et al. (2005). "Urine protein patterns can serve as diagnostic tools in patients with IgA nephropathy". Kidney Int. 67 (6): 2313–20. doi:10.1111/j.1523-1755.2005.00335.x. PMID 15882273.
  2. Velo M, Lozano L, Egido J, Gutierrez-Millet V, Hernando L (1987). "Natural history of IgA nephropathy in patients followed-up for more than ten years in Spain". Semin Nephrol. 7 (4): 346–50. PMID 3445013.
  3. 3.0 3.1 3.2 3.3 3.4 3.5 3.6 3.7 Radford MG, Donadio JV, Bergstralh EJ, Grande JP (1997). "Predicting renal outcome in IgA nephropathy". J Am Soc Nephrol. 8 (2): 199–207. PMID 9048338.
  4. 4.0 4.1 4.2 4.3 Bogenschütz O, Bohle A, Batz C, Wehrmann M, Pressler H, Kendziorra H; et al. (1990). "IgA nephritis: on the importance of morphological and clinical parameters in the long-term prognosis of 239 patients". Am J Nephrol. 10 (2): 137–47. PMID 2349957.
  5. 5.0 5.1 Alamartine E, Sabatier JC, Guerin C, Berliet JM, Berthoux F (1991). "Prognostic factors in mesangial IgA glomerulonephritis: an extensive study with univariate and multivariate analyses". Am J Kidney Dis. 18 (1): 12–9. PMID 2063844.
  6. 6.0 6.1 Johnston PA, Brown JS, Braumholtz DA, Davison AM (1992). "Clinico-pathological correlations and long-term follow-up of 253 United Kingdom patients with IgA nephropathy. A report from the MRC Glomerulonephritis Registry". Q J Med. 84 (304): 619–27. PMID 1484940.
  7. 7.0 7.1 Ibels LS, Györy AZ (1994). "IgA nephropathy: analysis of the natural history, important factors in the progression of renal disease, and a review of the literature". Medicine (Baltimore). 73 (2): 79–102. PMID 8152367.
  8. 8.0 8.1 Nicholls KM, Fairley KF, Dowling JP, Kincaid-Smith P (1984). "The clinical course of mesangial IgA associated nephropathy in adults". Q J Med. 53 (210): 227–50. PMID 6463197.
  9. 9.0 9.1 9.2 9.3 Berthoux F, Mohey H, Laurent B, Mariat C, Afiani A, Thibaudin L (2011). "Predicting the risk for dialysis or death in IgA nephropathy". J Am Soc Nephrol. 22 (4): 752–61. doi:10.1681/ASN.2010040355. PMC 3065230. PMID 21258035.
  10. Radhakrishnan J, Cattran DC (2012). "The KDIGO practice guideline on glomerulonephritis: reading between the (guide)lines--application to the individual patient". Kidney Int. 82 (8): 840–56. doi:10.1038/ki.2012.280. PMID 22895519.
  11. Qin YH, Zhou TB, Su LN, Lei FY, Huang WF, Zhao YJ (2011). "Association between ACE polymorphism and risk of IgA nephropathy: a meta-analysis". J Renin Angiotensin Aldosterone Syst. 12 (3): 215–23. doi:10.1177/1470320310391835. PMID 21357308.
  12. 12.0 12.1 12.2 12.3 Syrjänen J, Mustonen J, Pasternack A (2000). "Hypertriglyceridaemia and hyperuricaemia are risk factors for progression of IgA nephropathy". Nephrol Dial Transplant. 15 (1): 34–42. PMID 10607765.
  13. 13.0 13.1 Xie J, Kiryluk K, Wang W, Wang Z, Guo S, Shen P; et al. (2012). "Predicting progression of IgA nephropathy: new clinical progression risk score". PLoS One. 7 (6): e38904. doi:10.1371/journal.pone.0038904. PMC 3375310. PMID 22719981.
  14. Abe T, Kida H, Yoshimura M, Yokoyama H, Koshino Y, Tomosugi N; et al. (1986). "Participation of extracapillary lesions (ECL) in progression of IgA nephropathy". Clin Nephrol. 25 (1): 37–41. PMID 3955907.
  15. 15.0 15.1 Haas M (1997). "Histologic subclassification of IgA nephropathy: a clinicopathologic study of 244 cases". Am J Kidney Dis. 29 (6): 829–42. PMID 9186068.
  16. 16.0 16.1 16.2 16.3 D'Amico G, Minetti L, Ponticelli C, Fellin G, Ferrario F, Barbiano di Belgioioso G; et al. (1986). "Prognostic indicators in idiopathic IgA mesangial nephropathy". Q J Med. 59 (228): 363–78. PMID 3749442.
  17. 17.0 17.1 17.2 Katafuchi R, Oh Y, Hori K, Komota T, Yanase T, Ikeda K; et al. (1994). "An important role of glomerular segmental lesions on progression of IgA nephropathy: a multivariate analysis". Clin Nephrol. 41 (4): 191–8. PMID 8026110.
  18. Rekola S, Bergstrand A, Bucht H (1989). "IGA nephropathy: a retrospective evaluation of prognostic indices in 176 patients". Scand J Urol Nephrol. 23 (1): 37–50. PMID 2922579.
  19. Torres DD, Rossini M, Manno C, Mattace-Raso F, D'Altri C, Ranieri E; et al. (2008). "The ratio of epidermal growth factor to monocyte chemotactic peptide-1 in the urine predicts renal prognosis in IgA nephropathy". Kidney Int. 73 (3): 327–33. doi:10.1038/sj.ki.5002621. PMID 17943082.
  20. Lundberg S, Qureshi AR, Olivecrona S, Gunnarsson I, Jacobson SH, Larsson TE (2012). "FGF23, albuminuria, and disease progression in patients with chronic IgA nephropathy". Clin J Am Soc Nephrol. 7 (5): 727–34. doi:10.2215/CJN.10331011. PMC 3338280. PMID 22383747.
  21. Asao R, Asanuma K, Kodama F, Akiba-Takagi M, Nagai-Hosoe Y, Seki T; et al. (2012). "Relationships between levels of urinary podocalyxin, number of urinary podocytes, and histologic injury in adult patients with IgA nephropathy". Clin J Am Soc Nephrol. 7 (9): 1385–93. doi:10.2215/CJN.08110811. PMC 3430952. PMID 22700887.
  22. Peters HP, van den Brand JA, Wetzels JF (2009). "Urinary excretion of low-molecular-weight proteins as prognostic markers in IgA nephropathy". Neth J Med. 67 (2): 54–61. PMID 19299847.
  23. Liu LL, Jiang Y, Wang LN, Liu N (2012). "Urinary mannose-binding lectin is a biomarker for predicting the progression of immunoglobulin (Ig)A nephropathy". Clin Exp Immunol. 169 (2): 148–55. doi:10.1111/j.1365-2249.2012.04604.x. PMC 3406374. PMID 22774989.
  24. Zwirner J, Burg M, Schulze M, Brunkhorst R, Götze O, Koch KM; et al. (1997). "Activated complement C3: a potentially novel predictor of progressive IgA nephropathy". Kidney Int. 51 (4): 1257–64. PMID 9083294.
  25. Camilla R, Suzuki H, Daprà V, Loiacono E, Peruzzi L, Amore A; et al. (2011). "Oxidative stress and galactose-deficient IgA1 as markers of progression in IgA nephropathy". Clin J Am Soc Nephrol. 6 (8): 1903–11. doi:10.2215/CJN.11571210. PMC 3156425. PMID 21784819.
  26. Shi Y, Chen W, Jalal D, Li Z, Chen W, Mao H; et al. (2012). "Clinical outcome of hyperuricemia in IgA nephropathy: a retrospective cohort study and randomized controlled trial". Kidney Blood Press Res. 35 (3): 153–60. doi:10.1159/000331453. PMC 3242707. PMID 22116196.
  27. Cheng GY, Liu DW, Zhang N, Tang L, Zhao ZZ, Liu ZS (2013). "Clinical and prognostic implications of serum uric acid levels on IgA nephropathy: a cohort study of 348 cases with a mean 5-year follow-up". Clin Nephrol. 80 (1): 40–6. doi:10.5414/CN107813. PMID 23391320.
  28. Vuong MT, Hahn-Zoric M, Lundberg S, Gunnarsson I, van Kooten C, Wramner L; et al. (2010). "Association of soluble CD89 levels with disease progression but not susceptibility in IgA nephropathy". Kidney Int. 78 (12): 1281–7. doi:10.1038/ki.2010.314. PMID 20811333.

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