IgA nephropathy (Berger's disease)

IgA nephropathy (Berger's disease) was first described in 1968 as glomerulonephritis occurring in the form of recurrent hematuria. Currently, IgA nephropathy occupies one of the leading places among adult patients with chronic glomerulonephritis undergoing hemodialysis.

In most cases, IgA nephropathy manifests itself in childhood, more often in boys. It is characterized by mesangial proliferation in the glomeruli of the kidneys due to the deposition of immunoglobulin A. In the works of recent years, an association with the predominance of class II histocompatibility antigens HLA-DR has been established in most patients.

Cause of IgA nephropathy (Berger's disease)unknown.

Pathogenesis of IgA nephropathy (Berger's disease)is associated with the peculiarities of IgA metabolism, consisting of two light and two heavy chains. In IgA nephritis, the synthesis of only light chains is sharply increased. In pathogenesis, an increase in IgA synthesis in the bone marrow in response to a viral infection and abnormal production of IgA by mononuclear tonsillar mucosa are important. The deposition of immune complexes containing IgA occurs mainly in the renal mesangium. Studies in recent years have shown that mesangial proliferation is associated with the activity of cytokines: IL-1, IL-6, FNO-a, platelet growth factor P, and vascular growth factor.

Morphology of IgA nephropathy (Berger's disease).Confirmation of the diagnosis of IgA nephritis is provided by morphological changes in the kidneys. A kidney biopsy is mandatory. Light microscopy typically reveals focal or diffuse mesangial proliferation - mesangioproliferative glomerulonephritis. However, the diagnosis of IgA nephropathy is impossible based on light microscopy alone. Immunofluorescence microscopy is required.

Immunofluorescence microscopy reveals the presence of IgA (light chain) deposits in the renal mesangium, often in combination with the complement fraction C3, sometimes IgG or IgM. Hypercellularity of the mesangium is characteristic - mesangioproliferative glomerulonephritis.

There may be segmental glomerular sclerosis, which indicates advanced disease. In advanced IgA nephropathy, tubular atrophy and interstitial fibrosis are pronounced.

Symptoms of IgA nephropathy (Berger's disease) . The onset of the disease is often in childhood. The first signs of the disease remain hidden for a long time. Macro- or microhematuria is first detected against 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 compared to 2-3 weeks in acute poststreptococcal glomerulonephritis. There is no edema or hypertension. Kidney function is not impaired. The renal process progresses slowly, and in patients with periodically occurring macrohematuria, the degree of damage to the kidney tissue is less than in children with almost constant hematuria and proteinuria. As the disease progresses, kidney damage develops under the influence of the secreted cytokines IL-1a, IL-6, y-interferon.

As the disease progresses in older children, arterial hypertension and pronounced proteinuria join in, which are unfavorable prognostic criteria. A decrease in renal function in more than half of patients is observed after 10-12 years. However, spontaneous remissions may be observed more often in children than in adults.

Diagnosis of IgA nephropathy (Berger's disease) .

• Recurrent macro- and/or microhematuria against the background of acute infections.
• Elevated levels of IgA in serum and saliva.
• The presence of light chains of the IgA subclass in a kidney biopsy by immunofluorescence microscopy.

Treatment of IgA nephropathy (Berger's disease) .A regime without any special restrictions. A gluten-free diet with the exclusion of products from wheat, rye, oats, millet with replacement by rice, buckwheat, corn.

Antibacterial therapy of infectious diseases.

Prognosis. It is important to assess the prevalence of the proliferative process and the degree of sclerotic changes both in the glomerulus and interstitium for prognosticating the course of IgA nephropathy and the effectiveness of therapy. The addition of arterial hypertension against the background of long-term IgA nephropathy worsens the prognosis, and in the future, in most cases, chronic renal failure develops in adults.

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