Glomerulonephritis or inflammatory lesions of renal pelvis can occur in a variety of ways. One of the rare clinical cases is the focal segmental glomerulosclerosis, which, according to statistics, is detected in 5-10% of patients who have chronic inflammation of the kidneys.
Focal-segmental glomerulosclerosis
Focal-segmental glomerulosclerosis is called a special form of renal inflammation, which is manifested by sclerotic lesions of individual glomerular segments. Pathology is found predominantly in male patients( 60%), less often in children. As a result of sclerosing the segments, the glomeruli shrivel.
There are several variants of focal segmental glomerulosclerosis:
- End - has favorable clinical characteristics, responds well to glucocorticosteroid therapy. According to the changes occurring in the kidneys, it is similar to nephropathies on the background of diabetes, amyloidosis, etc.;
- Cellular - has a distinctive pronounced cellular response, pathogenetic changes are similar to proliferative glomerulonephritis;
- Collapsing idiopathic FSSS - for this option, segmental, and sometimes global, capillary-glomerular collapse, which occurs against the background of wrinkling, is characteristic. Also characteristic of the idiopathic type of FSGS is visceral cell hypertrophy and hyperplasia. Often a similar variant of pathology in specialists is associated with the use of heroin or HBV infection. Unfortunately, to the modern therapeutic methods this form is very stable.
In most cases( 70%), focal-segmental renal sclerosis is accompanied by a nephrotic syndrome, which is difficult to respond to therapy and proceeds quite hard.
Causes of
The basis of the pathology for glomerulosclerosis of the focal segmental type is epithelial cell damage, which is revealed by examination with an electron microscope. Therefore the main etiological factors are the same reasons as in the development of subcytosis and excessive vascular permeability. Only with FSGS, changes that occur with podocytes provoke the development of sclerotic processes.
Although pathological morphological changes are of a moderate nature, its development is progressive and complete remission is almost never achieved. Especially complex are the clinical cases complicated by nephrotic syndrome.
Symptoms of
For focal segmental glomerulosclerosis, the symptomatology of nephrotic syndrome and persistent proteinuria, hypertension and hematuria is typical.
In other words, the pathology is characterized by such manifestations:
- Swelling on the face, lower back and extremities, in severe cases can be complicated by hydropericardium, ascites or hydrothorax;
- Anemia, which is characterized by pronounced weakness and paleness of the skin, dyspnea and tachycardia, flies, etc.;
- Skin changes, for nephrotic patients, typically blanching and excessive dryness, peeling of the covers;
- Gastralgic symptoms associated with nausea and vomiting reactions, lack of appetite, bloating and diarrhea, epigastric pain;
- Oliguria, manifested by a decrease in the daily volume of urine, and urine acquires a pronounced turbid consistency;
- A large amount of protein in excreted urine, why there are flocculent impurities in the biological fluid;
- Severe pain in the area of the location of the kidneys;
- Bloody impurities in urine;
- Rapid urination, often with a slight discharge of urine;Dizziness and headache;
- Hypertensive manifestations associated with ear noise and visual impairment, heart pain and increased heart rate, increased blood pressure.
Diagnostics
To establish an accurate analysis, a patient needs to undergo a thorough diagnosis, which involves ultrasound examination of ureters and kidneys, X-rays and biopsies, MRI and radioisotope diagnostics, uroflowmetry and urodynamic procedures. In addition, it will be necessary to submit a list of laboratory tests, such as a general urinalysis, and to determine the level of albumins and protein suspensions in urine.
Treatment of
FSCS therapy is often ineffective. For a rather long time( 2-9 months), it is recommended to take glucocorticoid drugs. From a third to a half of patients with prolonged corticosteroid treatment achieve a favorable response to the effects of drugs. If FSGS is of a family or secondary nature, then in such cases there is a special resistance to glucocorticoid drugs.
If an improvement in the condition or a relapse occurs, the use of Cyclosporine or Cyclophosphamide will help achieve remission. If the patient is resistant to glucocorticoids, and FSGS has a neglected form, then long-term therapy with ACE inhibitors is prescribed. Sometimes plasmapheresis with Tacrolimus is prescribed. If the glomerulosclerosis of the focal-segmental type is not complicated by the nephrotic syndrome, then antihypertensive drugs with antiproteinuric effect and retarded kidney failure are prescribed.
For a long time there was a theory that the use of immunosuppressants has no prospects, but now scientists have proved that prolonged therapy with such drugs can well lead to remission.
Forecasts and complications
Forecasts for focal-segmental sclerotic kidney damage are quite serious. If there is a nephrotic syndrome, then the picture is considered the most unfavorable, since such cases rarely yield to immunosuppressive treatment. Remissions in these patients occur in isolated cases, and life expectancy during the five-year period is only 70-73% of adult patients.
Approximately half of patients develop kidney failure during the 10-year period, and in 20% of patients even after treatment, its terminal stage is formed in about 2 years. If the patient becomes pregnant, this will only complicate the course of the pathological process, worsening the prognosis for the mother and fetus. Even in patients who had a kidney transplant, in 20-30% of cases there was a recurrence of FSGS.In children, the prognosis for cure is much more favorable.
Collapsing glomerulopathy, accompanied by collapse of glomerular capillaries, hyperplastic and hypertrophic epithelial cell changes, tubular microcasts, interstitial edema, etc., is the most unfavorable prognosis.
In a video about forkal segmental glomerulosclerosis: