IgA nephropathy history and symptoms: Difference between revisions

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{{CMG}}
==Overview==
==Overview==
==History and Symptoms==
==Clinical Presentation==
The classic presentation (in 40-50% of the cases) is episodic frank [[hematuria]] which usually starts within a day of an [[upper respiratory tract infection]] (hence ''synpharyngitic'', as opposed to [[post-streptococcal glomerulonephritis]] which occurs some time after an initial infection). Flank pain can also occur. The frank hematuria resolves after a few days, though the [[microscopic hematuria]] persists. These episodes occur on an irregular basis, and in most patients, this eventually stops (although it can take many years). Renal function usually remains normal, though rarely, [[acute renal failure]] may occur (see below). This presentation is more common in younger adults.  
IgA nephropathy has a heterogeneous unpredictable clinical course. However, nephritic syndrome with gross hematuria in a young male patient is the most common clinical presentation for IgA nephropathy.  Classically, it follows an upper respiratory or gastrointestinal tract infection. The diagnosis of IgA nephropathy is not always obvious clinically; where only 30-40% of patients have such typical presentation.<ref name="pmid15930092">{{cite journal| author=Barratt J, Feehally J| title=IgA nephropathy. | journal=J Am Soc Nephrol | year= 2005 | volume= 16 | issue= 7 | pages= 2088-97 | pmid=15930092 | doi=10.1681/ASN.2005020134 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15930092 }} </ref>  In comparison, another 30-40% of patients with IgA nephropathy remain asymptomatic and are only found to have microscopic hematuria.<ref name="pmid15930092">{{cite journal|author=Barratt J, Feehally J| title=IgA nephropathy. | journal=J Am Soc Nephrol | year= 2005 |volume= 16 | issue= 7 | pages= 2088-97 | pmid=15930092 | doi=10.1681/ASN.2005020134 | pmc= |url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15930092 }} </ref>


A smaller proportion (20-30%), usually the older population, have microscopic hematuria and [[proteinuria]] (less than 2 grams of protein per 24 hours). These patients may not have any symptoms and are only picked up if a doctor decides to take a urine sample. Hence, the disease is picked up more commonly in situations where screening of urine is compulsory, e.g. school children in Japan.  
Unlike poststreptococcal glomerulonephritis (PSGN), which is also a glomerular disease with similar clinical presentation, IgA nephropathy is called “synpharyngitic” because it typically becomes clinically evident within 2-3 days during the infectious episode vs. PSGN which becomes clinically evident a few weeks after a streptococcal infection.


Very rarely (5% each), the presenting history is:
Adult patients with IgA nephropathy have a less obvious course of disease.  The most common presentation of IgA nephropathy among patients of older age groups is microscopic hematuria, proteinuria, and hypertension that might or might not be symptomatic.  The presence and intensity of these signs and symptoms vary and may be present in isolation or in combination.<ref name="pmid9596083">{{cite journal| author=Wyatt RJ, Julian BA, Baehler RW, Stafford CC, McMorrow RG, Ferguson T et al.| title=Epidemiology of IgA nephropathy in central and eastern Kentucky for the period 1975 through 1994. Central Kentucky Region of the Southeastern United States IgA Nephropathy DATABANK Project. | journal=J Am Soc Nephrol | year= 1998 | volume= 9 | issue= 5 | pages= 853-8 | pmid=9596083 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9596083 }} </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>
* [[Nephrotic syndrome]] (excessive protein loss in the urine, associated with an excellent prognosis)
* [[Acute renal failure]] (either as a complication of the frank hematuria, when it usually recovers, or due to [[rapidly progressive glomerulonephritis]] which often leads to [[chronic renal failure]])
* [[Chronic renal failure]] (no previous symptoms, presents with [[anemia]], [[hypertension]] and other symptoms of renal failure,  in people who probably had longstanding undetected microscopic hematuria and/or proteinuria)


A variety of systemic diseases are associated with IgA nephropathy such as [[liver failure]], [[coeliac disease]], [[rheumatoid arthritis]], [[Reiter's disease]], [[ankylosing spondylitis]] and [[HIV]]. Diagnosis of IgA Nephropathy and a search for any associated disease occasionally reveals such an underlying serious systemic disease. Occasionally, there are simultaneous symptoms of [[Henoch-Schönlein purpura]]; see below for more details on the association.
Only 10% of patients with IgA nephropathy present with nephrotic syndrome; usually these patients have pathological findings of minimal-change disease on biopsy.<ref name="pmid23782179">{{cite journal| author=Wyatt RJ, Julian BA| title=IgA nephropathy. | journal=N Engl J Med | year= 2013 | volume= 368 | issue= 25 | pages= 2402-14 | pmid=23782179 | doi=10.1056/NEJMra1206793 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23782179 }} </ref> Finally, only 10% of the patients with IgA nephropathy present with renal failure at diagnosis.


==References==
==References==

Revision as of 14:59, 21 October 2013

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

Overview

Clinical Presentation

IgA nephropathy has a heterogeneous unpredictable clinical course. However, nephritic syndrome with gross hematuria in a young male patient is the most common clinical presentation for IgA nephropathy. Classically, it follows an upper respiratory or gastrointestinal tract infection. The diagnosis of IgA nephropathy is not always obvious clinically; where only 30-40% of patients have such typical presentation.[1] In comparison, another 30-40% of patients with IgA nephropathy remain asymptomatic and are only found to have microscopic hematuria.[1]

Unlike poststreptococcal glomerulonephritis (PSGN), which is also a glomerular disease with similar clinical presentation, IgA nephropathy is called “synpharyngitic” because it typically becomes clinically evident within 2-3 days during the infectious episode vs. PSGN which becomes clinically evident a few weeks after a streptococcal infection.

Adult patients with IgA nephropathy have a less obvious course of disease. The most common presentation of IgA nephropathy among patients of older age groups is microscopic hematuria, proteinuria, and hypertension that might or might not be symptomatic. The presence and intensity of these signs and symptoms vary and may be present in isolation or in combination.[2][3]

Only 10% of patients with IgA nephropathy present with nephrotic syndrome; usually these patients have pathological findings of minimal-change disease on biopsy.[4] Finally, only 10% of the patients with IgA nephropathy present with renal failure at diagnosis.

References

  1. 1.0 1.1 Barratt J, Feehally J (2005). "IgA nephropathy". J Am Soc Nephrol. 16 (7): 2088–97. doi:10.1681/ASN.2005020134. PMID 15930092.
  2. Wyatt RJ, Julian BA, Baehler RW, Stafford CC, McMorrow RG, Ferguson T; et al. (1998). "Epidemiology of IgA nephropathy in central and eastern Kentucky for the period 1975 through 1994. Central Kentucky Region of the Southeastern United States IgA Nephropathy DATABANK Project". J Am Soc Nephrol. 9 (5): 853–8. PMID 9596083.
  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.
  4. Wyatt RJ, Julian BA (2013). "IgA nephropathy". N Engl J Med. 368 (25): 2402–14. doi:10.1056/NEJMra1206793. PMID 23782179.

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