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14-September-2008 18:02:40 - IgA nephropathy IgA nephropathy Classification and external resources Immunoglobulin A ICD-9 583.9 OMIM 161950 DiseasesDB 1353 MedlinePlus 000466 eMedicine med/886 MeSH D005922 IgA nephropathy also known as IgA nephritis, IgAN, Berger's disease and synpharyngitic glomerulonephritis is a form of glomerulonephritis inflammation of the glomeruli of the kidney. This should not be confused with Buerger's disease, an unrelated condition. IgA nephropathy is the most common glomerulonephritis throughout the world. Primary IgA nephropathy is characterized by deposition of the IgA antibody in the glomerulus. There are other diseases associated with glomerular IgA deposits, the most common being Henoch-Schönlein purpura, which is considered by many to be a systemic form of IgA nephropathy. Henoch-Schönlein purpura presents with a characteristic skin rash, occurs more commonly in young adults 16-35 yrs old and is associated with a more benign prognosis than IgA nephropathy, which typically presents with hematuria in adults and may lead to chronic renal failure. Contents 1 Signs and symptoms 2 Diagnosis 3 Pathophysiology 4 Natural history 5 Therapy 6 Genetics 7 Prognosis 8 Epidemiology 9 History 10 References 11 External links Signs and symptoms 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. 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. schoolchildren in Japan. Very rarely 5% each, the presenting history is: 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, celiac 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. Diagnosis For an adult patient with isolated hematuria, tests such as ultrasound of the kidney and cystoscopy are usually done first to pinpoint the source of the bleeding. These tests would rule out kidney stones and bladder cancer, two other common urological causes of hematuria. In children and younger adults, the history and association with respiratory infection can raise the suspicion of IgA nephropathy directly. A urinalysis will show red blood cells, usually as red cell urinary casts. Proteinuria, usually less than 2 grams per day, also may be present. Other renal causes of isolated hematuria include thin basement membrane disease and Alport syndrome, the latter being a herary disease associated with hearing impairment. A kidney biopsy is necessary to confirm the diagnosis. The biopsy specimen shows proliferation of the mesangium, with IgA deposits on immunofluorescence and electron microscopy. However, all patients with isolated microscopic hematuria i.e. without associated proteinuria and with normal kidney function are not usually biopsied since this is associated with an excellent prognosis. Other blood tests done to aid in the diagnosis include CRP or ESR, complement levels, ANA, ANCA and LDH. Protein electrophoresis and immunoglobulin levels can show increased IgA1 in 30% to 50% of all patients. may be normal or reduced. Tests such as electrolytes, renal function creatinine, urea, total protein, serum albumin help in establishing the prognosis. Other tests such as bleeding time, full blood count, PT and PTT are done before performing a biopsy. Pathophysiology Immunostaining showing IgA in the glomerulus of a patient with Henoch-Schönlein nephritis. Immunostaining showing IgA in the glomerulus of a patient with Henoch-Schönlein nephritis. The disease derives its name from deposits of Immunoglobulin A IgA in a blotchy pattern in the mesangium on immunofluorescence, the heart of the renal glomerulus. As a rule, this affects the whole kidney. The tissue changes gradually from being hypercellular to depositing extracellular matrix proteins, and finally fibrosis. There is no clear known explanation for the accumulation of the IgA. Exogenous antigens for IgA have not been identified in the kidney, but it is possible that this antigen has been cleared before the disease manifests itself. It has also been proposed that IgA itself may be the antigen. A recently advanced theory focuses on abnormalities of the IgA1 molecule. IgA1 is one of the two immunoglobulin subclasses the other is IgD that is O-glycosylated on a number of serine and threonine residues in a special proline-rich hinge region. Deficiency of these sugars appears to lead to polymerisation of the IgA molecule in tissues, especially the glomerular mesangium. A similar mechanism has been claimed to underly Henoch-Schönlein purpura HSP, a vasculitis that mainly affects children and can feature renal involvement that is almost indistinguishable from IgA nephritis. From the fact that IgAN can recur after renal transplant it can be postulated that the disease is caused by a problem in the immune system rather than the kidney itself. Remarkably, the IgA1 that accumulates in the kidney does not appear to originate from the mucosa-associated lymphoid tissue MALT, which is the site of most upper respiratory tract infections, but from the bone marrow. This, too, suggests an immune pathology rather than direct interference by outside agents. 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 policy. Similarly, the local policy for performing kidney 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. 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 glomerulonephritis. 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. Therapy The ideal treatment for IgAN would remove IgA from the glomerulus and prevent further IgA deposition. This goal still remains a remote prospect. There are a few additional caveats that have to be considered while treating IgA nephropathy. IgA nephropathy has a very variable course, ranging from a benign recurrent hematuria up to a rapid progression to chronic renal failure. Hence the decision on which patients to treat should be based on the prognostic factors and the risk of progression. Also, IgA nephropathy recurs in transplants despite the use of ciclosporin, azathioprine or mycophenolate mofetil and steroids in these patients. There are persisting uncertainties, due to the limited number of patients included in the few controlled randomized studies performed to date, which hardly produce statistically significant evidence regarding the heterogeneity of IgA nephropathy patients, the diversity of study treatment protocols, and the length of follow-up. Patients with isolated hematuria, proteinuria 1 g/day and normal renal function have a benign course and are generally just followed up annually. In cases where tonsillitis is the precipitating factor for episodic hematuria, tonsillectomy has been claimed to reduce the frequency of those episodes. However, it does not reduce the incidence of progressive renal failure.1 Also, the natural history of the disease is such that episodes of frank hematuria reduce over time, independent of any specific treatment. Similarly, prophylactic antibiotics have not been proven to be beneficial. Dietary gluten restriction, used to reduce mucosal antigen challenge, also has not been shown to preserve renal function. Phenytoin has been also been tried without any benefit2 A subset of IgA nephropathy patients, who have minimal change disease on light microscopy and clinically have nephrotic syndrome, show an exquisite response to steroids, behaving more or less like minimal change disease. In other patients, the evidence for steroids is not compelling. Short courses of high dose steroids have been proven to lack benefit. However, in patients with preserved renal function and proteinuria 1-3.5 g/day, a recent prospective study has shown that 6 months regimen of steroids may lessen proteinuria and preserve renal function.3 However, the risks of long-term steroid use have to be weighed in such cases. It should be noted that the study had 10 years of patient follow-up data, and did show a benefit for steroid therapy; there was a lower chance of reaching end-stage renal disease renal function so poor that dialysis was required in the steroid group. Importantly, angiotensin-converting enzyme inhibitors were used in both groups equally. Cyclophosphamide had been used in combination with anti-platelet/anticoagulants in unselected IgA nephropathy patients with conflicting results. Also, the side effect profile of this drug, including long term risk of malignancy and sterility, made it an unfavorable choice for use in young adults. However, one recent study, in a carefully selected high risk population of patients with declining GFR, showed that a combination of steroids and cyclophosphamide for the initial 3 months followed by azathioprine for a minimum of 2 years resulted in a significant preservation of renal function.4 Other agents such as mycophenolate mofetil, ciclosporin and mizoribine have also been tried with varying results. A study from Mayo Clinic did show that long term treatment with omega-3 fatty acids results in reduction of progression to renal failure, without, however, reducing proteinuria in a subset of patients with high risk of worsening kidney function.5 However, these results have not been reproduced by other study groups and in two subsequent meta-analyses.67 However, fish oil therapy does not have the drawbacks of immunosuppressive therapy. Also, apart from its unpleasant taste and abdominal discomfort, it is relatively safe to consume. The events that tend to progressive renal failure are not unique to IgA nephropathy and non-specific measures to reduce the same would be equally useful. These include low-protein diet and optimal control of blood pressure. The choice of the antihypertensive agent is open as long as the blood pressure is controlled to desired level. However, Angiotensin converting enzyme inhibitors and Angiotensin II receptor antagonists are favoured due to their anti-proteinuric effect. Genetics Though various associations have been described, no consistent pattern pointing to a single susceptible gene has been yet identified. Associations described include those with C4 null allele, factor B Bf alleles, MHC antigens and IgA isotypes. ACE gene polymorphism D allele is associated with progression of renal failure, similar to its association with other causes of chronic renal failure. However, more than 90% of cases of IgA nephropathy are sporadic, with a few large pedigrees described from Kentucky and Italy Online 'Mendelian Inheritance in Man' OMIM 161950. 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.8 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. Epidemiology Men are affected three times as often as women. There is also a striking geographic variation in the prevalence of IgA nephropathy throughout the world. It is the most common glomerular disease in the Far East and Southeast Asia, comprising almost half of all the patients with glomerular disease. However, it comprises only about 25% of the proportion in European and about 10% among North Americans, with African-Americans having a very low prevalence of about 2%. A confounding factor in this analysis is the existing policy of screening and use of kidney biopsy as an investigative tool. School children in Japan undergo routine urinalysis as do Army recruits in Singapore and any suspicious abnormality is pursued with a kidney biopsy, which might partly explain the high incidence of IgA nephropathy in those countries. History Heberden first described the disease in 1801 in a 5-year-old child with abdominal pain, hematuria, hematochezia, and purpura of the legs. In 1837, Johann Schönlein described a syndrome of purpura associated with joint pain and urinary precipitates in children. Eduard Henoch, a student of Schönlein's, further associated abdominal pain and renal involvement with the syndrome. Jean Berger and Hinglais, in 1968, were the first to describe IgA deposition in this form of glomerulonephritis hence, Berger's disease.9 References ^ Xie Y, Chen X, Nishi S, Narita I, Gejyo F 2004. Relationship between tonsils and IgA nephropathy as well as indications of tonsillectomy. Kidney Int. 65 4: 1135-44. doi:10.1111/j.1523-1755.2004.00486.x. PMID 15086452. ^ Clarkson AR, Seymour AE, Woodroffe AJ, McKenzie PE, Chan YL, Wootton AM 1980. Controlled trial of phenytoin therapy in IgA nephropathy. Clin. Nephrol. 13 5: 215-8. PMID 6994960. ^ Kobayashi Y, Hiki Y, Kokubo T, Horii A, Tateno S 1996. Steroid therapy during the early stage of progressive IgA nephropathy. A 10-year follow-up study. Nephron 72 2: 237-42. PMID 8684533. ^ Ballardie FW, Roberts IS 2002. Controlled prospective trial of prednisolone and cytotoxics in progressive IgA nephropathy. J. Am. Soc. Nephrol. 13 1: 142-8. PMID 11752031. ^ Donadio JV, Bergstralh EJ, Offord KP, Spencer DC, Holley KE 1994. A controlled trial of fish oil in IgA nephropathy. Mayo Nephrology Collaborative Group. N. Engl. J. Med. 331 18: 1194-9. PMID 7935657. ^ Strippoli GF, Manno C, Schena FP 2003. An evidence-based survey of therapeutic options for IgA nephropathy: assessment and criticism. Am. J. Kidney Dis. 41 6: 1129-39. PMID 12776264. ^ Dillon JJ 1997. Fish oil therapy for IgA nephropathy: efficacy and interstudy variability. J. Am. Soc. Nephrol. 8 11: 1739-44. PMID 9355077. ^ Bartosik LP, Lajoie G, Sugar L, Cattran DC 2001. Predicting progression in IgA nephropathy. Am. J. Kidney Dis. 38 4: 728-35. PMID 11576875. ^ Berger J, Hinglais N 1968. Les depots intercapillaires d'IgA-IgG. J Urol Nephrol 74: 694-5. External links The Foundation for IgA Nephropathy v d e Urinary system - Pathology - Urologic disease N00-N39, 580-599 Abdominal Kidney/ nephropathy Glomerulus Nephritis/ glomerulonephritis by structure: Membranoproliferative glomerulonephritis - Membranous glomerulonephritis/Membranous nephritis - IgA nephropathy/glomerulonephritis by disease: Post-streptococcal glomerulonephritis - Lupus nephritis other: Rapidly progressive glomerulonephritis - Nephritic syndrome Nephrosis/ noninflammatory Glomerulosclerosis Focal segmental glomerulosclerosis, Diabetic nephropathy/glomerulosclerosis - Nephrotic syndrome Minimal change disease - Familial renal amyloidosis Tubulointerstitial/ Renal tubule Interstitial nephritis Pyelonephritis, Danubian endemic familial nephropathy Uropathy Obstructive uropathy, Hydronephrosis, Pyonephrosis Inborn errors of renal tubular transport Renal tubular acidosis, Gitelman syndrome Reflux nephropathy - Nephrogenic diabetes insipidus - Renal papillary necrosis Renal failure Acute renal failure Acute tubular necrosis - Chronic renal failure Other Renal osteodystrophy - Nephroptosis - Abderhalden-Kaufmann-Lignac syndrome vascular Renal artery stenosis, Hypertensive nephropathy, Renovascular hypertension Ureter Ureteritis - Ureterocele - Megaureter Pelvic Bladder Cystitis Interstitial cystitis, Trigonitis - Neurogenic bladder - Vesicointestinal fistula - Vesicoureteral reflux Urethra Urethritis Non-gonococcal urethritis - Urethral syndrome - Urethral stricture Other/general Urinary tract infection - Retroperitoneal fibrosis - Urolithiasis Kidney stone, Renal colic See also congenital, neoplasia, symptoms/signs Retrieved from http://en..org/wiki/IgA_nephropathy Categories: Kidney diseases Views Article Discussion this page History Personal tools Log in / create account Navigation Main page Contents Featured content Current events Random article Search Go Search Interaction Community portal Recent changes Contact Donate to Help Toolbox What links here Related changes Upload file Special pages Printable version Permanent link Cite this page Languages Deutsch Español Français Italiano 日本語 Polski Português Suomi Svenska This page was last modified on 18 August 2008, at 18:42
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