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Primary focal segmental glomerulosclerosis

Primary focal segmental glomerulosclerosis (FSGS) is a condition which refers to the scarring of the glomeruli (the filtering units of the kidneys). It is a progressive kidney disease which can lead to end stage renal disease (ESRD) in nearly 2.3% of the affected patients.

Symptoms

Usually, the patient with primary FSGS does not exhibit any symptoms in the initial stages. However, as the disease progresses, the below symptoms may be presented:

  • Swelling of the body parts such as hands, legs, feet, ankles, abdominal region and around the eyes due to the retention of fluids
  • Rapid weight gain associated with fluid retention
  • Cloudy urine due to the presence of proteins
  • High blood pressure and high cholesterol

If the disease progresses further, it can produce the symptoms of kidney failure such as a headache, decrease in appetite, itching, shortness of breath and nausea.

Take medical help if the above symptoms are noticed. It is important to seek medical help especially for the symptoms such as fever, pain during urination and a decrease in the urinary output.

Causes

FSGS which results without an obvious cause is termed as primary FSGS or idiopathic FSGS, unlike secondary FSGS which has multiple causes.

Risk factors

Primary focal segmental glomerulosclerosis can occur in both children and adults. The factors which can increase the risk of developing this condition include:

  • Male gender
  • Race which mainly includes African Americans

Complications

The complications which may occur if primary FSGS is not treated properly are:

  • Infections
  • Malnutrition
  • Neprhotic syndrome
  • Chronic kidney disease
  • End-stage kidney disease

Diagnosis

If the doctor suspects FSGS from the symptoms, then he or she will perform a physical examination of the patient to check for the presence of the following:

  • Abdominal pain
  • Ulcerations and infections in the related areas
  • Severe hypertension
  • Swelling of the body parts
  • Pleural effusion (build up of fluids in the space surrounding the lungs)

Further, the below tests may be necessary to detect if the person has primary focal segmental glomerulosclerosis:

  • Blood tests to check the amount of proteins, cholesterol and creatinine levels
  • Urine tests to detect the presence of proteins and blood
  • Glomerular filtration rate to determine the kidney function
  • Kidney biopsy which looks for abnormalities gives a definitive diagnosis
  • Imaging tests such as an ultrasound to identify if any defects are present
  • Genetic testing to check for the presence of genes related to kidney disease

Primary FSGS gives negative results for systemic lupus erythematosus, hepatitis B or C infections and vasculitis.

Treatment

It is very challenging to treat primary FSGS as the exact cause is not clear. Although there is no complete cure for FSGS, treatment only aims at delaying the progression of the condition and improving the symptoms.

Medicines such as corticosteroids and immuno-suppressive agents are given to calm down the immune system and prevent the attack of healthy glomeruli. Additionally, the below precautions are taken by the doctor during the management of primary FSGS:

  • Avoiding the use of cyclophosphamide in patients who are non-responsive
  • Monitoring for adverse affects during the therapy with corticosteroids
  • Monitoring for bone marrow suppression
  • Encouraging the patient to take adequate fluids to prevent hemorrhagic cystitis
  • Counseling the patient and the family members regarding the treatment options for ESRD

The other medicines used to manage primary FSGS are:

  • Statins to control the blood cholesterol levels
  • Anti-coagulant drugs to prevent the formation of blood clots
  • Blood pressure medicines to reduce the loss of proteins
  • Water pills (i.e. diuretics) to reduce swelling by removing excess salts and water from the body

If the condition has progressed to kidney failure then the treatment options include:

  • Continuous ambulatory peritoneal dialysis
  • Maintenance of hemodialysis
  • Live donor kidney transplantation

Additionally, the doctor will suggest restrictions in the diet which include:

  • Reduction of sodium content in the food
  • Low-fat diet
  • Low to moderate protein diet

Prevention

Preventive measures for primary FSGS are not known.

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