Focal segmental glomerulosclerosis and pregnancy. Minimal change disease

Focal segmental glomerulosclerosis (FSGS) is one of the forms of idiopathic nephrotic syndrome, accounting for 10-15% of cases among children with idiopathic nephrotic syndrome. characteristic feature- the presence of glomeruli in part ( focal lesion) segmental (not in all capillary loops) mesangial sclerosis.

Causes of focal segmental sclerosis:

Idiopathic, including as a result of progressive minimal change disease

Tumor

Severe obesity

Chronic transplant rejection

pyelonephritis, medicinal nephritis reflux nephropathy

Along with possible viral etiology (as in HIV) and exposure to exogenous toxins (heroin), the role of genetic factors(there are differences in the frequency of occurrence depending on race). A particular mechanism is discussed in obesity - increased glomerular filtration and hemodynamic factors lead to glomerular damage. Perhaps hyperfiltration in the remaining nephrons is the main factor in the development of FSGS in obstructive pyelonephritis, reflux nephropathy.

Table 3. Characteristics of focal segmental glomerulosclerosis
‘Pathogenesis	Primary Damage epithelial cells glomerulus. There is an analogy with the mechanism of damage in minimal change disease (MCD) due to the cytotoxic effect of lymphokines. Proteinuria in both MMI and FSGS is due to the loss of the anionic layer (negative charge) basement membrane glomeruli, which makes it permeable to protein. These facts, some general histological characteristics, and repeated biopsies in patients with idiopathic nephrotic syndrome support that one of the variants of FSGS is its development as a result of MCD progression. At the same time, IgM-nephropathy is considered as an intermediate stage - focal-segmental mesangioproliferative GN with deposition of IgM and the C3 complement component, clinically manifested by nephrotic syndrome
Clinical and morphological characteristic	Often signs of nephrotic syndrome, less often the only manifestation of the disease may be proteinuria, not reaching the degree of nephrotic. In some cases, the disease is preceded respiratory infection. Only in some patients, already at the first manifestations of the disease, along with nephrotic syndrome, arterial hypertension, hematuria, and an increase in blood creatinine occur. Typically, a gradual increase in proteinuria from mild to severe, with the addition of other symptoms of nephrotic syndrome, over several weeks and even months. Steroid resistance is important diagnostic feature both idiopathic FSGS and transition from steroid-responsive minimal change disease to FSGS
Morphological peculiarities	AT initial stages only glomeruli of juxtamedullary nephrons are affected, with time the process spreads. In non-sclerosed glomeruli, histological changes are similar to those seen in MMI. Light microscopy: either normal glomeruli or slight proliferation of mesangial cells. Immunofluorescent study: there is no luminescence or in some patients a nonspecific luminescence of IgM and the C3-component of complement is found in areas of sclerosis. Electron microscopy. Diffuse thickening of podocytes. A sign of FSGS is the presence of segmental sclerosis in part of the glomeruli; the number of glomeruli with sclerosis does not matter for the diagnosis. Another important feature for distinguishing from minimal change disease is atrophy of the tubular epithelium, infiltration and fibrosis of the interstitium.
Forecast	FSGS refers to GN with a poor prognosis. Most patients with therapy-resistant nephrotic syndrome develop CKD within 1–20 years. More favorable course in patients with proteinuria that does not reach the severity of nephrotic. Steroid-sensitive patients within 5-10 years have no signs of progression. The presence of remissions caused by prednisolone or cytostatics improves the prognosis of the disease. After a kidney transplant, in 20-30% of patients, the disease recurs in the transplanted kidney, usually within a month after transplantation. This fact is considered as confirmation of the presence of some circulating toxin (lymphokine) that directly damages the glomeruli. Return of FSGS leads to graft loss in "/g-"/g of these patients, in most of them the initial disease progressed to failure within 3 years

The diagnosis of FSGS is established by kidney biopsy. Since idiopathic nephrotic syndrome in children is most often a manifestation of minimal change disease and is sensitive to steroids, a biopsy is performed if remission does not occur after 8 weeks of taking prednisolone at a therapeutic dose.

Segmental sclerosis may also be found in the outcome of focal or poststreptococcal glomerulonephritis, and may also be a consequence of intraglomerular hypertension. In these conditions, there is no clinic of nephrotic syndrome, and podocytes are not diffusely changed, as in idiopathic FSGS, but only in foci of sclerosis.

Morphologically characterized by segmental glomerulosclerosis (individual segments of the glomeruli are sclerosed) parts of the glomeruli (focal changes); the remaining glomeruli are intact at the onset of the disease. Immunohistochemical examination reveals IgM. Often this morphological type of changes is difficult to distinguish from the "minimal changes" of the glomerulus, the possibility of the transition of the latter to FSGS is discussed. There is an opinion, not shared by all authors, that this different severity options or different stages of the same disease, united by the term "idiopathic nephrotic syndrome".

As with minimal changes in the glomeruli, in FSGS the main pathology is the defeat of epithelial cells (podocytes), detected only by electron microscopy, and the possible role of the same factors responsible for both vascular permeability and "podocytosis" is discussed. However, in FSGS, changes in podocytes that are unable to replicate gradually lead to the development of sclerosis. Confirmation of the possible role of circulating pathological factor a description of a woman with steroid-resistant FSGS who gave birth to two children with proteinuria and hypoalbuminemia may serve; in both children, both proteinuria and nephrotic syndrome disappeared 2 and 3 weeks after birth, respectively.

FSGS is a rather rare variant of GN, observed in 5-10% of adult patients with CGN (according to our clinic, over the past 20 years - in 6%). It is clinically characterized by nephrotic syndrome (in our observations, HC was observed in 91 out of 135 patients, i.e. in 67% of cases) or persistent proteinuria, in most patients it is combined with hematuria (although gross hematuria is rare), in half - with arterial hypertension. It is observed in 15-20% of patients with NS, more often in children in whom FSGS is the most common cause steroid-resistant NS.

What is glomerulosclerosis? This is a pathological process or type of nephropathy, in which sclerotic changes and hyalinosis occur in individual renal glomeruli.

In turn, sclerosis is a process of replacing the normal renal tissue to the connecting. Hyalinosis is a type of dystrophy in which dense protein masses are deposited in the tissues.

Classification

There are 2 main forms:

1. Focal segmental glomerulosclerosis.
2. Diabetic glomerulosclerosis or nephropathy (nodular, diffuse and exudative)

According to the causes of occurrence, primary (idiopathic) glomerulosclerosis is isolated, which is typical for children. younger age. Occurs spontaneously without obvious reasons. Secondary glomerulosclerosis develops with progression various kinds diseases of the kidneys, cardiovascular system.

The reasons

Allocate the following reasons that contribute to the development of this pathological process:

• Acute or chronic glomerulonephritis.
• Idiopathic nephrotic syndrome.
• Diabetes.
• Atherosclerosis of the vessels of the kidneys.
• Side effects of drugs.
• Autoimmune kidney disease.
• Severe liver damage.
• Hypertonic disease.

It should be noted that primary glomerulosclerosis develops extremely rarely. Much more often this secondary process, which is a consequence of the above diseases.

Symptoms

The often asymptomatic course of the disease leads to late diagnosis. Glomerulosclerosis has long latency period when there are practically no symptoms. Pain syndrome may present as pain in lumbar region or lower abdomen. But the process can proceed without pain.

The clinical manifestations of glomerulosclerosis are dominated by nephrotic syndrome, which is characterized by the presence of protein in the urine, edema throughout the body, a decrease in protein in the blood, and hypercholesterolemia.

Edema appears on the face, in the area of ​​​​the eyelids, ascites, hydrothorax, hydropericardium also develop over time. Generalized edema can lead to hypovolemic shock in the patient. In some patients, there is an increase arterial pressure and blood appears in the urine. As a result, kidney function is significantly impaired, which leads to oliguria.

Diagnostics

The diagnosis of focal segmental glomerulosclerosis is usually based on clinical picture, anamnestic information, examination data, as well as the results of laboratory and instrumental studies.

Required research:

• General analysis of blood and urine.
• Biochemical blood test (urea, creatinine, protein content)
• Urinalysis according to Nechiporenko and Zemnitsky.
• Ultrasound of organs abdominal cavity and small pelvis.
• Radioisotope study of the kidneys and urinary system.
• Urodynamic study.
• Kidney biopsy.
• MRI of the abdominal organs.

An important criterion for making a diagnosis is renal tissue biopsy. Only after microscopic examination you can definitely tell about the character pathological changes in the glomeruli of the kidneys.

In diagnostics diabetic nephropathy have their own characteristics. First of all, a glucose profile is required, as well as the level of sugar in the urine. Since diabetes is a systemic process that affects more than just the renal vessels, it is important to conduct research peripheral vessels. These include the vessels of the distal lower extremities and the retina in the first place.

Treatment

Treatment of focal segmental glomerulosclerosis directly depends on presence of nephrotic syndrome. With absence this syndrome sufficient treatment with ACE inhibitors, which lower blood pressure and thereby reduce the load on the renal vessels.

Also, these drugs slow down the excretion of protein in the urine and inhibit the progression of chronic kidney failure. Patients are also advised to follow a diet with a limited amount of protein, fluids and a reduced salt content.

In the presence of nephrotic syndrome, treatment is more stringent. Such patients must follow a diet. Appointed infusion therapy colloidal solutions or blood products. These include gelofusin, reopoliglkin, albumins, fresh frozen plasma. Diuretics are used to remove excess fluid ( furosemide, verospiron).

Also, these patients are shown immunosuppressive therapy , for which hormonal preparations. These include medicines from the group of glucocorticosteroids - prednisolone and methylprednisolone first of all. Treatment with corticosteroids is long-term and their intake should be continued in courses up to 6 months. With the ineffectiveness of steroid therapy, they switch to treatment with cytostatic drugs ( cyclophosphamide, methotrexate). Doses of cytostatics should not exceed therapeutic values, because they all have serious side effects. Sometimes antibacterial drugs are used in complex treatment.

There are some peculiarities in the treatment of diabetic glomerulosclerosis. In addition to the named diet, the patient must also reduce the intake of simple carbohydrates and animal fat.

The total calorie content of food must be reduced and divided into 5-6 meals. It is better not to limit fluid intake. The second feature is the precise control of blood glucose levels. If the sugar level remains within target values the disease will not progress.

The prognosis for glomerulosclerosis depends on the presence of the following factors:

• arterial hypertension.
• Hematuria.
• nephrotic syndrome.
• Resistance to glucocorticosteroid therapy.
• Decompensated diabetes mellitus.

Sometimes when good effect from complex treatment stable remission can be achieved. The frequency of remissions, unfortunately, is low and rarely exceeds the values ​​in 10% . Otherwise, the patient goes into renal failure.

In adults, the disease progresses more intensively than in children. Patients with decompensated renal failure can only be helped using methods such as hemodialysis and peritoneal dialysis. In the terminal stage, kidney transplantation is indicated.

The nephrotic syndrome is characterized mainly by proteinuria, which can be glomerular, tubular, and hyperproteinemic.

• Glomerular proteinuria develops with an increase in the permeability of the glomerular filter for proteins.
• Canalicular - in violation of the reabsorption of proteins in the proximal tubules.
• Hyperproteinemic - with an excess of protein in the blood (light chains of immunoglobulins).

Etiology, pathogenesis, pathological anatomy

Nephrotic syndrome develops only with glomerular proteinuria. The main diseases leading to nephrotic syndrome are:

1. minimal change disease
2. focal segmental glomerulosclerosis,
3. membranous nephropathy,
4. mesangiocapillary glomerulonephritis,
5. diabetic glomerulosclerosis,
6. amyloidosis.

Minimal change disease

Minimal change disease, or lipoid nephrosis, develops when there is an imbalance between subpopulations of T-lymphocytes. In most cases, the disease occurs without visible reasons(idiopathic variant), less often - with systemic diseases (lymphogranulomatosis, HIV infection, IgA nephropathy, Fabry disease) and the use of drugs (NSAIDs, rifampicin, interferon α, dextran-iron complex). Morphological changes are revealed only by electron microscopy. Detect edema with diffuse swelling of the processes of epithelial podocytes, vacuoles, lysomas and an increased number of organelles.

Focal segmental glomerulosclerosis

Primary focal-segmental glomerulosclerosis is characterized by sclerosis and hyalinosis of individual loops of the glomerulus (hence segmental) in less than half of the glomeruli (focal), in most cases it is idiopathic, rarely develops with HIV infection, heroinism, lysosomal storage diseases. Secondary focal segmental glomerulosclerosis develops after the death of part of the renal parenchyma, leading to an increase in intraglomerular pressure, with congenital agenesis kidneys; after kidney resection with tubolointerstitial nephritis, sickle anemia. At histological examination find the fusion of the legs of podocytes and segmental sclerosis of the glomeruli, nodular and coarse-grained deposits of IgM and C3.

Membranous nephropathy

Membranous nephropathy is characterized by diffuse thickening of the basement membranes of the glomerular capillaries. The causes of primary membranous nephropathy are unknown. Secondary complicates the course systemic diseases (malignant neoplasms, SLE, hepatitis B) or develops with the introduction of penicillamine and gold preparations. On electron microscopy, early stages diseases reveal subepithelial deposits with protrusions of the lamina densa between them. Later, deposits form within the GBM, with diffuse and granular distribution of IgG along it without glomerular proliferation, exudation, and necrosis.

Mesangiocapillary glomerulonephritis

Mesangiocapillary glomerulonephritis is of two types, similar in their main morphological features(increase in the number of cells in the mesangium, expansion of the mesangial matrix, bypass and thickening of the basement membranes, lobularity of the glomeruli) and deposits differing in localization and composition. With type I deposits, subendothelial and mesangial, they contain C3, IgG or IgM. In type II, the deposits contain SZ, do not contain immunoglobulins, and are located inside the membranes. Mesangiocapillary glomerulonephritis is an immunocomplex disease that develops when infective endocarditis, HIV infection, hepatitis B and C, SLE and malignant neoplasms (leukemia and lymphoma).

Symptoms

The main symptom of nephrotic syndrome is proteinuria, usually greater than 2 g/m 2 . Proteinuria is caused by an increase in the permeability of the glomerular filter with damage to the glomerular basement membrane and filtration gaps between the legs of podocytes. The consequence of proteinuria is hypoalbuminemia, the level of which depends on the amount of albumin excreted in the urine. Hypoalbuminemia is also due to the breakdown of reabsorbed albumin in the proximal tubules and impaired albumin synthesis in the liver.

Leading clinical sign diseases are edema that occurs gradually, gradually increasing and often reaching the degree of anasarca with ascites, hydrothorax and hydropericardium. But the initial visit to the doctor may be associated with focal edema due to complaints of difficulty breathing (laryngeal edema or pleural effusion), retrosternal pain (hydropericardium), swollen knees (hydroarthrosis), abdominal pain (mesenteric edema), swelling of the scrotum.

Edema usually appears in the morning on the eyelids and face and in the knees in the late afternoon after walking. As the disease progresses, edema becomes permanent and massive, leading to stretching of the skin with the formation of pale atrophic areas - striae, especially on the abdomen and thighs. The pathogenesis of edema is complex. Fluid accumulates mainly due to hypoalbuminemia and changes in the ratio of hydraulic and oncotic pressure in the capillaries and interstitium (Starling forces) and an increase in tubular reabsorption of Na and water due to increased secretion of ADH and activation of the renin-angiotensin aldosterone mechanism.

Another important diagnostic feature of this syndrome is hyperlipoproteinemia. LDL levels and cholesterol are elevated in most patients, and VLDL and triglycerides - in the most severe cases. But the risk of atherosclerosis and coronary artery disease in nephrotic syndrome has not been proven. With nephrotic syndrome, blood clotting disorders usually develop due to a decrease in the activity of anticoagulant and fibrinolytic factors of serum proteinases. Coagulation disorders and episodic hypovolemia create a risk of PE, thrombosis of peripheral vessels, especially renal veins.

characteristic clinical symptoms are malaise, anorexia, weight gain, muscle atrophy, which can be masked by edema. Depending on the degree of production in patients with angiotensin II, a hypo-, normo- or hypertensive state is possible.

Complications

Complications develop with long-term nephrotic syndrome. These include:

• deficit nutrients, including protein deficiency, manifested by fragility of hair and nails, growth retardation, demineralization of bones;
• potassium deficiency syndrome;
• myopathy;
• decrease in metabolism.

In connection with the loss of immunoglobulins, infectious diseases often develop. Arterial hypertension can be complicated by damage to the heart and brain. But development is possible. orthostatic hypotension and hypovolemic shock, sometimes fatal.

Diagnostics

Urinalysis reveals significant proteinuria with excretion of 3.5 g or more of protein per day. In the urinary sediment, usually hyaline, waxy, granular and epithelial cell casts are detected. Proteinuria in glomerulonephritis can be combined with hematuria and leukocyturia.

The content of albumin in the blood decreases (less than 1.5-2.5 g%), the levels of α- and γ-globulins, adrenocortical and thyroid hormones, as well as transferrin, antistreptolysin-O, other Ig and complement. On the contrary, with systemic lupus erythematosus, the level of IgG is increased, with focal glomerulosclerosis it is reduced, with membranous glomerulonephritis, the level of C3 is normal. The content of urea nitrogen and creatinine in the blood serum depends on the degree of kidney damage.

Violation of blood clotting is due to the excretion of IX, XII and thrombolytic factors (urokinase and antithrombin III) in the urine and an increase in the content factor VIII, fibrinogen and platelets in serum. Loss of transferrin in the urine leads to the development of microcytic anemia. The nephrotic syndrome is characterized by an increase in serum total cholesterol, triglycerides, free and esterified cholesterol and phosphates. A sharply increased concentration of lipids is combined with severe hypoalbuminemia.

Diagnosis is based on clinical manifestations and laboratory research. However final diagnosis placed only after histological and electron microscopic examination of kidney biopsy specimens.

Differential Diagnosis:

• First of all, it is carried out between the primary nephrotic syndrome, which is characterized by severe proteinuria, severe biochemical changes in the serum and the relatively late development of renal failure, and the secondary nephrotic syndrome, in which renal failure is already present at the onset of the nephrotic syndrome or develops shortly thereafter.
• Minimal change disease is more common in children and is characterized by hypertension and azotemia.
• Membranoproliferative glomerulonephritis also develops mainly in children (60-80% of cases), occurs with gross hematuria, azotemia and arterial hypertension.
• Mesangiocapillary glomerulonephritis is detected more often in adult patients (75%), occurs with microhematuria in 20% and hypertension in 35% of cases.

Course and forecast

The course of nephrotic syndrome is long, the prognosis depends on the etiology. A better prognosis for diseases that can be treated with corticosteroids. Some of them, such as mesangiocapillary glomerulonephritis, may resolve on their own after 5-8 years. In minimal change disease, 90% of children and adults have a good prognosis. Relapses are common but treatable, and kidney failure usually does not develop.

Membranous nephropathy proceeds slowly, gradually progressing to renal failure in 50% of patients over 15-20 years, proteinuria or nephrotic syndrome may persist in 50% without impaired renal function. Focal-segmental glomerulosclerosis and mesangiocapillary glomerulonephritis are more severe, in which almost half of the patients develop within 8-10 years. Treatment with corticosteroids is usually not effective. Persistent remission observed in few patients (5%).

After kidney transplantation, relapses of nephrotic syndrome often develop in patients with focal segmental glomerulosclerosis, mesangiocapillary glomerulonephritis, systemic lupus erythematosus, Ig - nephropathy.

The prognosis usually worsens with infectious diseases, azotemia, arterial hypertension, severe azotemia, peripheral vascular thrombosis.

Treatment

Treatment of patients is carried out taking into account the nosological form that caused the nephrotic syndrome, the duration and characteristics of its course. It is not necessary to strictly limit the regimen and diet of patients.

Patients are prescribed physiotherapy exercises, daily walking up to 3-4 km, good nutrition with the content of animal protein in food up to 1 g / kg of body weight and a decrease in sodium chloride intake to 5 g / day. Medical treatment includes: treatment of the underlying disease, reduction of proteinuria and reduction of individual clinical manifestations.

Treatment of the underlying disease

In minimal change disease, primary therapy with prednisolone at a dose of 1-1.5 mg/kg orally for 4-6 weeks is recommended. Positive effect manifested by the cessation of proteinuria and an increase in diuresis. Subsequently, patients are transferred to maintenance therapy with prednisolone 2-3 mg/kg every other day for 4 weeks and then the dose is gradually reduced over the next 4 months. If patients do not respond to corticosteroids or develop frequent relapses, the appointment of prednisolone with cyclophosphamide 2-3 mg / kg / day for 3 weeks or chlorambucil 0.2 mg / kg / day for 12 weeks is recommended.

This treatment is usually effective, but cytotoxic drugs cause numerous side effects(suppression of gonadal function, immunity, cystitis, carcinogenicity), especially in prepubertal age. Perhaps the appointment instead of alkylating agents of cyclosporine orally at 5 mg / kg / day in two divided doses. Cyclosporine causes remission in 60-80% of cases, but after dose reduction, relapse is possible.

Nephrotic syndrome with membranous nephropathy goes away without treatment in 40%, in 35-40% it proceeds in waves - with relapses and remissions, in the remaining 20-25% it persists constantly, while kidney function is gradually impaired, and after 10-15 years it develops terminal renal failure. The effectiveness of glucocorticoids, cyclophosphamide, chlorambucil and cyclosporine has not been proven. In terminal chronic renal failure is indicated.

Treatment of focal segmental glomerulosclerosis is not effective. Proteinuria can be reduced by an 8-week course of prednisolone and cyclophosphamide. Recovery glomerular filtration and a reduction in proteinuria is possible with ciclosporin at doses used in the treatment of minimal change disease. Remission is usually short-lived, relapse develops quickly. There is no evidence of the effectiveness of the treatment of nephrotic syndrome with anticoagulants and antithrombotic agents.

With mesangiocapillary glomerulonephritis, treatment with glucocorticoids in ultra-high doses ("pulse therapy"), alternating with chlorambucil, is effective. Prednisolone is prescribed at 1000 mg IV for 3 days, and then inside at 0.4 mg/kg/day for 27 days; chlorambucil - orally at 0.2 mg / kg / day. The course of treatment is 6 months. Perhaps the appointment of antiplatelet agents (dipyridamole 200-400 mg / day and aspirin 300-500 mg / day).

Reducing proteinuria and reducing clinical manifestations

The complex of treatment includes ACE inhibitors, which can reduce proteinuria and lipemia. But in severe renal dysfunction, they can exacerbate hyperkalemia.

With massive edema and ascites, it is advisable to treat with thiazide diuretics. But they can lead to hypokalemia and metabolic alkalosis. Therefore, when prescribing them, potassium preparations should be additionally used. In addition, diuretics large doses reduce plasma volume, which can lead to deterioration of kidney function and the risk of thrombosis.

With severe hypovolemia, threatening the development of hypovolemic shock, infusions of plasma or albumin are necessary. Arterial hypertension treated with diuretics, ACE inhibitors and calcium antagonists. Bacteriuria, endocarditis, peritonitis and other foci of infection require timely detection and intensive treatment.

NSAIDs reduce proteinuria, probably by reducing blood flow to the glomeruli, but they do not affect the activity of the process. Given the ability of these drugs to cause sodium and water retention, hyperkalemia and other side effects, their use is limited.

Glomerulonephritis or inflammatory lesion renal pelvis may flow into various options. One of the rare clinical cases is the focal segmental glomerulosclerotic form, which, according to statistics, is detected in 5-10% of patients with chronic inflammation kidneys.

Focal segmental glomerulosclerosis

Focal segmental glomerulosclerosis is called special form renal inflammation, which is manifested by sclerotic lesions of individual glomerular segments. Pathology is found predominantly in male patients (in 60%), less often in children. As a result of sclerosis of the segments, the glomeruli shrink.

With the progression of the pathological process, the glomerular structures of the cortical substance undergo sclerosis. In the tubules, the formation of protein-fatty epithelial dystrophy occurs, symptoms of apoptosis appear, the appearance of hyaline in the lumen.

There are several variants of focal segmental glomerulosclerosis:

• Terminal - has favorable clinical characteristics responds well to glucocorticosteroid therapy. According to the changes occurring in the kidneys, it is similar to nephropathies against the background of diabetes, amyloidosis, etc.;
• Cellular - has a characteristic pronounced cellular reaction, pathogenetic changes are similar to proliferative glomerulonephritis;
• Collapsing idiopathic FSGS - this variant is characterized by segmental, and sometimes global capillary-glomerular collapse, which occurs against the background of wrinkling. Also characteristic of the idiopathic type of FSGS is visceral cell hypertrophy and hyperplasia. Often, a similar variant of the pathology among specialists is associated with the use of heroin or HBV infection. Unfortunately, modern therapeutic methods given form very stable.

In most cases (70%), focal segmental renal sclerosis is accompanied by nephrotic syndrome, which is difficult to respond to therapy and is quite difficult.

Classification of focal segmental glomerulosclerosis

The reasons

The basis of the pathology in focal segmental glomerulosclerosis is epithelial cell damage, which is detected during the study electron microscope. Therefore, the main etiological factors the same causes are considered as with the development of podocytosis and excessive vascular permeability. Only with FSGS, the changes that occur with podocytes provoke the development of sclerotic processes.

Although pathologies occur morphological changes moderate character, its development is progressive and complete remission is almost never achieved. Particularly difficult clinical cases complicated by nephrotic syndrome.

Symptoms

For focal segmental glomerulosclerosis, symptoms of nephrotic syndrome and persistent proteinuria are typical, hypertension and .

In other words, the pathology is characterized by such manifestations:

• , lower back and limbs, in severe cases may be complicated by hydropericardium, ascites or hydrothorax;
• Anemia, which is characterized by severe weakness and pallor of the skin, shortness of breath and tachycardia, flies, etc.;
• Skin changes, blanching and excessive dryness, peeling of the integument are typical for nephrotics;
• Gastralgic symptoms associated with nausea and vomiting, lack of appetite, bloating and diarrhea, epigastric pain;
• Oliguria, manifested by a decrease in the daily volume of urine, and the urine acquires a pronounced cloudy consistency;
• A large number, why in biological fluid flocculent impurities are present;
• Expressed pain in the area of ​​​​the kidneys;
• Bloody impurities in urine;
• Frequent urination, often with little urine output;
• Dizziness and headaches;
• Hypertensive manifestations associated with tinnitus and visual disturbances, heart pain and increased heart rate, increased blood pressure.

Diagnostics

To establish precise analysis the patient needs to undergo a thorough diagnosis, which involves ultrasound examination ureters and kidneys, x-rays and biopsies, and radioisotope diagnostics, uroflowmetry and urodynamic procedures. In addition, you will need to submit a list laboratory tests like general research urine, as well as to determine the level of albumin and protein suspensions in the composition of urine.

Treatment

FSGS therapy is often ineffective. For quite a long time (2-9 months) it is recommended to take glucocorticoid drugs. From a third to a half of patients with long-term corticosteroid treatment achieve a favorable response to the action of the drugs. If FSGS is familial or secondary, then in such cases there is a special resistance to glucocorticoid drugs.

If improvement is achieved or a relapse occurs, then the use of Cyclosporine or Cyclophosphamide will help achieve remission. If the patient has resistance to glucocorticoids, and FSGS has a neglected form, then long-term therapy ACE inhibitors. Sometimes plasmapheresis with Tacrolimus is prescribed. If glomerulosclerosis of the focal segmental type is not complicated by nephrotic syndrome, then antihypertensive drugs with an antiproteinuric effect and slowing down the development of kidney failure are prescribed.

For a long time there was a theory that the use of immunosuppressants has no prospects, but now scientists have managed to prove that long-term therapy similar drugs may well lead to remission.

The duration of glucocorticosteroid therapy determines the frequency of remission. For this, patients are prescribed Prednisolone (1-1.2 mg / kg daily dose) for a 2-3-month course, then the dosage of the drug begins to be gradually reduced.

Predictions and Complications

The prognosis for focal segmental sclerotic kidney disease is quite serious. If there is a nephrotic syndrome, then the picture is considered the most unfavorable, since such cases rarely respond to immunosuppressive treatment. Remissions in these patients occur in isolated cases, and life expectancy over a five-year period is only 70-73% of adult patients.

Approximately half of patients develop kidney failure over a 10-year period, and in 20% of patients, even after treatment, its terminal stage develops 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 received a kidney transplant, FSGS recurred in 20-30% of cases. In children, the prognosis for recovery is much more favorable.

The most extremely unfavorable prognosis is characterized by collapsing glomerulopathy, which is accompanied by collapse of glomerular capillaries, hyperplastic and hypertrophic epithelial cell changes, tubular microcysts, interstitial edema, etc.
On the video about forcal-segmental glomerulosclerosis: