Berger's disease
Contents:
Description:
Berger's disease - a disease which is characterized by existence of mezangialny deposits of IgA in kidneys at patients with a recurrent hamaturia at the kept function of kidneys.
Disease frequency significantly varies in various geographical zones; men get sick by 3-4 times more often than women. Genetic aspects. At 50% of patients define HLA-DR4. Patomorfologiya In a mezangiya the diffusion, sometimes incorrectly distributed IgA deposits. Existence of IgM or IgG is possible.
Often meet a focal and segmented glomerulonephritis with proliferation a mezangiya. Swelling a mezangiya can be the only find.
Etiologies of Berger:
Origins of a disease of Berger are studied a little. The etiological role of a viral infection on the basis of communication of the palindromias with acute feverish diseases (pharyngitis) given about development of an IgA-nephropathy in a renal transplant is supposed. Hyperproduction of the antigen which is (not identified) on mucous membranes induces synthesis of the antibodies (on the basis of polymeric and monomeric IgA) having antimezangialny activity, reacting with antigens of gastrointestinal flora, food antigens with formation of the circulating cell-bound immune complexes. The circulating IgA containing the cell-bound immune complexes more often found in the period of an exacerbation of a disease damage renal balls. There are data on genetic predisposition to an IgA-nephropathy.
Symptoms of a disease of Berger:
Berger's disease develops preferential at the age of 15-30 years, considerably more often (by 3 - 4 times) meets at men, is characterized by the gross hematuria attacks proceeding quite often with dull aches in a waist, the mialgiya recuring against the background of pharyngitis (at fever height - a so-called sinfaringitny hamaturia). Sometimes these attacks are followed by passing OPN. The proteinuria is usually minimum also a vysokoselektivna. The ABP at most of patients within norm. Permanent increase in the IgA level of blood serum (at 60% of patients) with increase in a share of polymeric IgA is characteristic at the normal content of other immunoglobulins and Sz-fraction of a complement.
In a third of cases (is more often at adults) the atypical options of a disease which are shown a resistant microhematuria, a considerable proteinuria, a recurrent ostronefritichesky syndrome with a gross hematuria, a nephrotic syndrome, a malignant hypertension are observed.
Disease at typical option is more often persistent, high-quality. At a massive proteinuria and other atypical options the disease quite often can progress. The irreversible terminal renal failure develops on average in 10 years from a disease debut at 10-20% of patients.
Allocate 2 forms of a disease:
recurrent gross hematuria (at 90% of patients),
asymptomatic microhematuria with a weak proteinuria.
Sometimes the disease is shown by OPN, HPN, heavy arterial hypertension or a nephrotic syndrome (less than 20%).
The clinical symptomatology appears in 1-2 days after a feverish disease with damage of mucous membranes of upper respiratory tracts, intestines. Unlike postinfectious glomerulonephritis hamaturia matches a feverish state.
The weak proteinuria (less than 1 g/days), hamaturia are characteristic of a disease. Levels of creatinine and a complement in serum are not changed, IgA is raised.
The current and the forecast are variable 20-year survival - about 50%. At a smaller part of patients the renal failure develops.
The factors predetermining the bad forecast: advanced age at the beginning of a disease, the expressed proteinuria, arterial hypertension and detection of semilunums or a segmented sclerosis at a kidney biopsy.
Treatment of a disease of Berger:
Treatment depends on a cause of illness, and also a look and weight of symptoms. A main objective of treatment is control of symptoms. High blood pressure can be unmanageable, and it, as a rule, is the most important aspect of treatment. For decrease in the ABP komplekny schemes of antihypertensives can be involved. Corticosteroids, immunodepressants and other drugs can be used for treatment of some of the reasons of a chronic glomerulonephritis.
Restrictions of salt, liquid, proteins and some other components in a diet for simplification of a state, control of high blood pressure and displays of a renal failure can be recommended.
In hard cases renal dialysis and change of a donor kidney is recommended.
