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IgA-nephropathy (Berger's disease)
Last reviewed: 23.04.2024
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IgA-nephropathy (Berger's disease) was described for the first time in 1968 as glomerulonephritis, which occurs as a recurrence of hematuria. Currently, IgA-nephropathy occupies one of the first places among adult patients with chronic glomerulonephritis, who are on hemodialysis.
In most cases, IgA-nephropathy manifests itself in childhood, more often in boys. Characterized by mesangial proliferation in renal glomeruli due to the deposition of immunoglobulin A. In recent years, most patients have established an association with the predominance of histocompatibility antigens of class II HLA-DR.
The cause of IgA-nephropathy (Berger's disease) is unknown.
The pathogenesis of IgA-nephropathy (Berger's disease) is associated with the peculiarities of IgA metabolism, consisting of two light and two heavy chains. With IgA-nephritis, the synthesis of only light chains is dramatically increased. In the pathogenesis, an increase in the synthesis of IgA in the bone marrow in response to a viral infection and abnormal production of IgA by mononuclears of the tonsillar mucosa is important. The deposition of immune complexes containing IgA occurs predominantly in the mesanga of the kidneys. Recent studies have shown that mesangial proliferation is associated with the activity of cytokines: IL-1, IL-6, FNO-a, platelet-derived growth factor P, and vascular growth factor.
Morphology of IgA-nephropathy (Berger's disease). Confirmation of the diagnosis with IgA-nephritis is the morphological changes in the kidneys. Kidney biopsy is mandatory. With light microscopy, focal or diffuse mesangial proliferation, mesangioproliferative glomerulonephritis, is characteristic. However, only according to light microscopy the diagnosis of IgA-nephropathy is impossible. Immunofluorescence microscopy is required.
According to the immunofluorescence microscopy data, the presence of IgA (light chain) deposits in the mesanga of the kidneys, often in combination with the complementarity C3 fraction, sometimes IgG or IgM. Characteristic hypercellularity of mesangium is mesangioproliferative glomerulonephritis.
There may be segmental sclerosis of the glomeruli, which indicates a far-advanced disease. With far-gone IgA-nephropathy, tubular atrophy and interstitial fibrosis are prominent.
Symptoms of IgA-nephropathy (Berger's disease) . The onset of the disease is more common in childhood. The first signs of the illness remain hidden for a long time. For the first time there is a macro- or microhematuria on the background of acute respiratory viral infection with pharyngitis, tonsillitis. In this case, the interval between the infectious process and hematuria is usually 1-2 days in comparison with 2-3 weeks in acute poststreptococcal glomerulonephritis. Edema and hypertension do not. The kidney function is not broken. The renal process progresses slowly, and in patients with periodically occurring macrogematuria the degree of renal tissue damage is less than in children who have almost constant hematuria and proteinuria. With the progression of the disease, renal lesions develop under the influence of secreted cytokines IL-1a, IL-6, y-interferon.
As the disease develops, hypertension, pronounced proteinuria, which are unfavorable prognostic criteria, is attached to older children. Reduction of renal functions in more than half of patients is observed after 10-12 years. However, in children more often than in adults, spontaneous remissions can occur.
Diagnosis of IgA-nephropathy (Berger's disease) .
- Recurrent macro- and / or microhematuria on the background of acute infections.
- Elevated levels of IgA in serum and in saliva.
- The presence of light chains of the IgA subclass in the kidney biopsy under immunofluorescence microscopy.
Treatment of IgA-nephropathy (Berger's disease) . Mode without special restrictions. The diet is agglutinous with the exception of products from wheat, rye, oats, millet, with rice, buckwheat, corn replaced.
Antibacterial therapy of infectious diseases.
Forecast. Important for predicting the course of IgA nephropathy and the effectiveness of therapy is to assess the prevalence of the proliferative process and the degree of sclerotic changes in both the glomerulus and interstitium. Attachment of arterial hypertension against the background of a long-term current IgA nephropathy worsens the prognosis, in the future, in most cases, adults develop chronic renal failure.
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