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==Overview==
==Overview==
==Natural History==
==Natural History==
Since IgA nephropathy commonly presents without symptoms through abnormal findings on [[urinalysis]], there is considerable possibility for variation in any population studied depending upon the [[Screening (medicine)|screening]] policy. Similarly, the local policy for performing kidney [[needle aspiration biopsy|biopsy]] assumes a critical role; if it is a policy to simply observe patients with isolated [[hematuria]], a group with a generally favourable [[prognosis]] will be excluded. If, in contrast, all such patients are biopsied, then the group with isolated [[microscopic hematuria]] and isolated mesangial IgA will be included and ‘improve’ the prognosis of that particular series.  
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.<ref name="pmid15882273">{{cite journal| author=Haubitz M, Wittke S, Weissinger EM, Walden M, Rupprecht HD, Floege J et al.| title=Urine protein patterns can serve as diagnostic tools in patients with IgA nephropathy. | journal=Kidney Int | year= 2005 | volume= 67 | issue= 6 | pages= 2313-20 | pmid=15882273 | doi=10.1111/j.1523-1755.2005.00335.x | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15882273 }} </ref> Some patients rapidly progress into ESRD; but the majority experience a stable kidney function following diagnosis.<ref name="pmid15882273">{{cite journal| author=Haubitz M, Wittke S, Weissinger EM, Walden M, Rupprecht HD, Floege J et al.| title=Urine protein patterns can serve as diagnostic tools in patients with IgA nephropathy. | journal=Kidney Int | year= 2005 | volume= 67 | issue= 6 | pages= 2313-20 | pmid=15882273 | doi=10.1111/j.1523-1755.2005.00335.x | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15882273 }} </ref>
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<ref name="pmid15882273">{{cite journal| author=Haubitz M, Wittke S, Weissinger EM, Walden M, Rupprecht HD, Floege J et al.| title=Urine protein patterns can serve as diagnostic tools in patients with IgA nephropathy. | journal=Kidney Int | year= 2005 | volume= 67 | issue= 6 | pages= 2313-20 | pmid=15882273 | doi=10.1111/j.1523-1755.2005.00335.x | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15882273 }} </ref> and up to 30-50% of patients develop ESRD over 20 years.<ref name="pmid3445013">{{cite journal| author=Velo M, Lozano L, Egido J, Gutierrez-Millet V, Hernando L| title=Natural history of IgA nephropathy in patients followed-up for more than ten years in Spain. | journal=Semin Nephrol | year= 1987 | volume= 7 | issue= 4 | pages= 346-50 | pmid=3445013 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3445013 }} </ref><ref name="pmid9048338">{{cite journal| author=Radford MG, Donadio JV, Bergstralh EJ, Grande JP| title=Predicting renal outcome in IgA nephropathy. | journal=J Am Soc Nephrol | year= 1997 | volume= 8 | issue= 2 | pages= 199-207 | pmid=9048338 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9048338 }} </ref>


Nevertheless, IgA nephropathy, which was initially thought to be a benign disease, has been shown to have a not-so-benign long term outcome. Though most reports describe IgA nephropathy as having an indolent evolution towards either healing or renal damage, a more aggressive course is occasionally seen associated with extensive crescents, and presenting as [[acute renal failure]]. In general, the entry into [[chronic renal failure]] is slow as compared to most other [[glomerulonephritis]] – occurring over a time scale of 30 years or more (in contrast to the 5 to 15 years in other ğğglomerulonephritisüü). This may reflect the earlier diagnosis made due to frank hematuria.
Complete remission, i.e. a normal urinalysis, occurs rarely in adults, in about 5% of cases.  Thus, even in those with normal [[renal function]] after a decade or two, urinary abnormalities persist in the great majority. In contrast, 30 – 50% of children may have a normal urinalysis at the end of 10 years.  However, given the very slow evolution of this disease, the longer term (20 – 30 years) outcome of such patients is not yet established.
Overall, though the renal survival is 80 – 90% after 10 years, at least 25% and may be up to 45% of adult patients will eventually develop end stage renal disease.
==Complications==
==Complications==
*[[Acute nephritic syndrome]] or [[nephrotic syndrome]]
*[[Acute nephritic syndrome]] or [[nephrotic syndrome]]

Revision as of 15:10, 21 October 2013

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

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.[1] Some patients rapidly progress into ESRD; but the majority experience a stable kidney function following diagnosis.[1] 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[1] and up to 30-50% of patients develop ESRD over 20 years.[2][3]

Complications

Prognosis

Male gender, proteinuria (especially > 2 g/day), hypertension, smoking, hyperlipidemia, older age, familial disease and elevated creatinine concentrations are markers of a poor outcome. Frank hematuria has shown discordant results with most studies showing a better prognosis, perhaps related to the early diagnosis, except for one group which reported a poorer prognosis. Proteinuria and hypertension are the most powerful prognostic factors in this group[2]. There are certain other features on kidney biopsy such as interstitial scarring which are associated with a poor prognosis. ACE gene polymorphism has been recently shown to have an impact with the DD genotype associated more commonly with progression to renal failure.

References

  1. 1.0 1.1 1.2 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. 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.

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