IgA nephropathy history and symptoms
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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.
Diagnosis
Kidney Biopsy
Immunofluorescence microscopy is required for the diagnosis of IgA nephropathy. IgA nephropathy histopathological findings may vary among individuals and with disease progression. The histopathological hallmark of IgA nephropathy is the predominant presence of mesangial stains for IgA in comparison to the much less intense stains for IgG or C3. IgA1 deposits may be present anywhere along the mesangium, and even in the glomerular capillary wall.
IgA nephropathy may have any of the following 6 findings on light microscopy (in increasing order of severity):
- Normal appearing biopsy
- Focal mesangial hypercellularity
- Diffuse mesangial hypercellularity
- Focal proliferative glomerulonephritis (most common finding on diagnostic biopsy)
- Diffuse proliferative glomerulonephritis
- Chronic sclerosing glomerulonephritis
On electron microscopy, mesangial deposits are most commonly seen. However, depositions on capillary walls are possible; they herald worse prognosis.
The following variations may also be found; but they are not exclusive of IgA nephropathy[4]:
- Segmental endocapillary proliferation
- Segmental Glomerulosclerosis and adhesions
- Tubular atrophy and interstitial fibrosis
- Glomerular crescent surrounding the glomerular tuft
References
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 4.0 4.1 Wyatt RJ, Julian BA (2013). "IgA nephropathy". N Engl J Med. 368 (25): 2402–14. doi:10.1056/NEJMra1206793. PMID 23782179.