Focal segmental glomerulosclerosis

Focal segmental glomerulosclerosis (FSGS): diagnosis and treatment

Glomerulosclerosis is a disease characterized by damage to the glomeruli, which leads to a violation of the filtering ability of the kidneys. According to the World Health Organization, this pathology affects 1% to 3% of people worldwide. According to a study published in The Lancet, glomerulosclerosis is one of the most common causes of chronic kidney disease. Therefore, the search for new effective methods of treatment is an important task of modern medicine, and stem cell therapy is one of the most promising areas in this area.

Focal segmental glomerulosclerosis

Many diseases can affect kidney function by attacking and damaging the glomeruli (tiny structures that filter and purify the blood). Such diseases are called glomerular. A fairly common of these is focal segmental glomerulosclerosis. FSGS often leads to kidney failure, which requires replacement therapy (dialysis).

The term “focal” means focal, that is, some glomeruli undergo a scarring process, while the rest remain normal. “Segmental” indicates that only one part of a particular glomerulus is damaged. Focal segmental glomerulosclerosis affects both children and adults. Men get sick slightly more often than women.

Classification of glomerulosclerosis

The main FSGS classification system is based on the results of light microscopy – the analysis of tissues taken by biopsy. Depending on the histological picture, the following forms of the disease are distinguished:

• classic (occurs in 42% of patients);
• perihilar (26%);
• Tip Lesion or peripheral (17%);
• collapsing (11%);
• cellular (3%).

The prognosis of focal segmental glomerulosclerosis largely depends on the histological form. Collapsing FSGS is considered the most dangerous for patients, it quickly leads to terminal renal failure. The least dangerous is peripheral.

By the nature of the origin, 4 types of FSGS are distinguished:

• Primary. It starts suddenly. Accompanied by progressive damage to the renal glomeruli. Occurs on its own, with no known or obvious cause.
• Genetic. Caused by genetic breakdowns in podocytes (glomerular cells). It can occur in families or be sporadic (random) in nature.
• Secondary. Causes of this type of glomerulosclerosis include: obesity, loss of one kidney, diabetes mellitus (diabetic FSGS), arterial hypertension, HIV, sickle cell anemia, autoimmune diseases (systemic lupus erythematosus and hemorrhagic vasculitis).
• Unspecified etiology. FSGS that develops in the absence of a genetic or other identifiable cause without nephrotic syndrome.

Determining the form of the disease helps to choose the right tactics for the treatment of glomerulosclerosis.

Symptoms and manifestations of glomerulosclerosis

With FSGS, many patients experience cycles of remission and relapse. Some people are asymptomatic. Others have the following symptoms:

• swelling of the eyes, arms, legs, abdomen;
• weight gain due to the accumulation of excess fluid in the body;
• high blood pressure;
• increased levels of protein in the urine, foamy urine;
• low levels of protein in the blood (hypoalbuminemia);
• high levels of creatinine in the blood due to the fact that the kidneys cannot filter the blood properly.

Some patients may also experience a decrease in thyroid hormone levels. This condition can be controlled by taking synthetic thyroid hormones.

Diagnosis of glomerulosclerosis

The following methods are commonly used to diagnose FSGS:

• history taking and physical examination of the patient;
• urinalysis for protein levels;
• a blood test to determine the level of protein, cholesterol and creatinine;
• glomerular filtration rate analysis;
• ultrasound examination of the kidneys;
• kidney biopsy (taking a small sample of tissue for laboratory analysis).

Genetic testing is also sometimes performed to identify hereditary diseases that can provoke the development of glomerulosclerosis.

Effective treatment of focal segmental glomerulosclerosis

Depending on the type and severity of the disease, the following methods may be used to treat FSGS:

• taking ARBs and ACE inhibitors to control blood pressure;
• corticosteroid therapy to treat symptoms of nephrotic syndrome;
• taking diuretics to reduce swelling;
• taking immunosuppressants (drugs that suppress the function of the immune system) to treat steroid-resistant forms of FSGS and nephrotic syndrome;
• kidney dialysis;
• kidney transplant.

Unfortunately, there has not yet been a significant breakthrough in the medical treatment of renal pathology. Currently available approaches can only delay the progression of the disease. To increase the life expectancy of patients, methods such as dialysis and transplantation are used. However, dialysis cannot completely replace kidney function. And an acute shortage of donors and the potential risk of rejection of a transplanted kidney limits the possibilities of transplantation. Moreover, in autoimmune forms of FSGS, there is a very high probability of recurrence of the disease after transplantation.

The growing number of patients with renal pathology is becoming one of the urgent problems of modern healthcare. Today, active attempts are being made to find innovative methods of treating nephrological diseases that could restore damaged tissues and improve the quality of life of patients. Stem cell therapy is a promising technology in this area.

New methods of treatment of glomerulosclerosis

Cellular therapy is a modern therapeutic strategy that can help slow the progression of kidney failure in FSGS.

Accumulated experimental and clinical data suggest that the development of glomerulosclerosis is largely related to the number of podocytes in the glomeruli of the kidneys. Progressive forms of FSGS are usually accompanied by podocytopenia, the loss of cells that cover the capillaries in the glomeruli. These cells play a large role in the ability of the kidneys to filter blood.

Mature podocytes are highly differentiated cells that practically do not divide. Therefore, their loss can lead to irreversible impairment of the filtering function of the kidneys. Advances in experimental nephrology have shown that destroyed podocytes can be partly replaced by bone marrow stem cells.

Mesenchymal stem cells (MSCs), which are found in bone marrow, adipose tissue, and blood, are one of the most effective types of cell population for activating regeneration processes in a damaged kidney. An analysis of more than 70 scientific sources has shown that it is MSCs that give the best result in the treatment of glomerulosclerosis. According to the results of preclinical studies, the introduction of mesenchymal stem cells stimulates the regeneration of renal tissue and reduces the level of synthesis of inflammatory cytokines.

Another area of application of stem cells is to prevent the recurrence of glomerulosclerosis after kidney transplantation, which occurs quite often. The scientific literature describes a case of therapy with allogeneic bone marrow mesenchymal stem cells (MSCs) of a 13-year-old patient. A teenager developed a relapse of FSGS after a kidney transplant and standard treatment failed. After three MSC infusions in a patient:

• stable kidney function was maintained;
• the level of a number of inflammatory factors in the blood serum decreased;
• the target for proteinuria (the amount of protein in the urine) was achieved without plasmapheresis.

Stem cell treatment was not accompanied by any side effects.

All these data prove that cell therapy can be an effective treatment for kidney diseases. If you are interested in the possibility of stem cell treatment, please contact our medical coordinator by phone or using the feedback form.

Best clinics for glomerulosclerosis treatment

MedTour company cooperates with the best clinics for the treatment of kidney diseases around the world. We now offer a unique opportunity for stem cell therapy for FSGS. Call or fill out the feedback form to contact the medical coordinator. He will answer all your questions and select the clinic that is most suitable for the treatment of your disease.

The best specialists for the treatment of glomerulosclerosis

Andrey Kovalchuk, biotechnologist, PhD, director of the stem cell bank of LLC “Genome”, head of the bank of umbilical cord blood, tissues and human cells on the basis of Gorbachevsky TNMU, spoke about the achievements and features of regenerative medicine.

To learn more about the possibilities of cell therapy for kidney diseases, get a free consultation from a MedTour coordinating doctor.

Frequently asked Questions

What diet should be followed for glomerulosclerosis?

Dietary recommendations include: reducing salt and fat intake, and reducing protein intake (including meat, chicken, fish and eggs) to 0.5 per kg of body weight.

What is the difference between glomerulonephritis and glomerulosclerosis?

Glomerulonephritis is an inflammation of the membrane tissue in the kidneys that serves as a filter that clears the blood of metabolic waste and excess fluid. Glomerulosclerosis is the process of scarring of tiny blood vessels in the kidneys.

Can stem cells help kidney disease?

There are a number of studies that prove that mesenchymal stem cells play an important role in kidney regeneration. They are introduced into the body to help repair damaged kidney tissue and reduce inflammation. In addition, stem cells are being used in conjunction with organ transplants to eliminate the need for lifelong immunosuppressive drugs.

Published: 10.04.2023

Updated: 18.04.2023

Medical author, Medical editor:

Olena Kursabaieva

Medical author, Medical editor:

Natalia Segen

Information on this webpage verified by the medical expert

Medical Doctor, oncologist, PHD student

Kateryna Maliarchuk