Kidney Topics
Types of Kidney Diseases
Alport syndrome is caused by genetic disorders affecting the kidneys, ears, eyes, and the vasculature. It is caused by inheriting certain genes responsible for producing collagen, which provides structural support to certain tissues in the body, including the tiny filters in the kidneys called glomeruli. Those that inherit the genes associated with Alport syndrome will have collagen abnormalities that lead to the symptoms associated with this syndrome.
Alport Syndrome can be categorized into three forms based on the inheritance of genes.
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XLAS (X-linked Alport Syndrome): Inherited in an X-linked dominant pattern, meaning that a mutation on the X chromosome causes it and affects mostly males. About 80% of males with XLAS develop hearing loss by their teenage years.
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ARAS (autosomal recessive Alport Syndrome): Inherited in an autosomal recessive pattern, meaning that it requires two copies of the mutated gene (one from each parent) to develop the condition. ARAS affects males and females equally.
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ADAS (autosomal dominant Alport Syndrome): Inherited in an autosomal dominant pattern, meaning that it requires only one copy of the mutated gene to develop the condition. ADAS is the rarest form of Alport Syndrome.
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X-linked Alport syndrome is the more severe form and makes up about 85% of all cases of Alport syndrome. It commonly affects young boys. In contrast, autosomal (dominant and recessive) Alport syndrome is less severe and affects both boys and girls equally.
Signs and symptoms of Alport syndrome may include:
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Foamy urine due to protein present in urine
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Red or brown-colored urine due to blood present in urine
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Hearing loss
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Vision impairment and cataract formation
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Swelling (edema) throughout the body, including legs and face
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Weight gain due to excess fluid in the body
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High blood cholesterol
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High blood pressure
Alport syndrome causes damage to the glomeruli and leads to a loss of proteins from the blood, which can cause high blood pressure and fluid build-up in the body. People with Alport syndrome may also experience hearing loss, vision changes, and vascular complications. Regular checkups for eye exams and hearing tests are essential to monitor changes. Rarely, Alport syndrome can cause aortic aneurysm, for which a screening heart echocardiogram can be evaluated.
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Alport syndrome is usually suspected based on medical history, clinical examination, vision and hearing tests, and blood and urine tests. Usually, a genetic test and kidney biopsy will be required to confirm the diagnosis. A kidney biopsy is a procedure where a doctor takes a small sample of kidney tissue using a needle and studies the sample under a microscope.
There is no cure for Alport syndrome, but there are ways to manage the condition and slow down the progression of kidney damage. These include medications to control blood pressure and fluid levels, as well as a low-salt diet to help manage kidney function. Medications can manage blood pressure, reduce protein in the urine, and slow the progression of kidney failure in children with Alport syndrome. In severe cases, patients with Alport syndrome may need to undergo dialysis or kidney transplantation to manage their kidney function.
See your healthcare provider regularly to monitor your kidney function and manage related symptoms such as hearing, vision, and heart complications. Follow your healthcare provider’s recommendations for treatment and care for Alport Syndrome.
The likelihood of developing kidney failure with Alport syndrome depends on several factors, including the type and severity of the genetic mutation, the presence of other medical conditions, and whether you are receiving appropriate medical treatment and care. Some people with Alport syndrome may experience mild kidney damage that does not progress to kidney failure. In contrast, others may experience more severe kidney damage that requires dialysis or a kidney transplant to manage. In some cases, Alport syndrome may lead to progressive kidney failure in the late teens to early adulthood.
Receiving regular medical checkups and following your doctor’s recommendations for treatment and care will help to slow down the progression of kidney damage and manage any related symptoms. If you have Alport syndrome and are concerned about your risk of developing kidney failure, it is important to speak with your healthcare provider for more information.New treatments and therapies are being developed for kidney diseases. Ask your doctor if you want to participate in a clinical trial for Alport Syndrome. More information is available at: www.enrollmypatient.org
When kidneys become unhealthy over time, it can lead to chronic kidney disease (CKD). There are many causes of CKD, but the more common ones include long-standing diabetes, high blood pressure (some may argue that high blood pressure is the result of kidney disease), inflammation of the filters of the kidney, obesity, heart disease, some familial or genetic conditions, excessive use of certain medications (like anti-inflammatory medicine), etc.
When the kidneys don’t work correctly, they can often leak protein and red blood cells into the urine. Over time, excess water and waste products can build up in the body, blood pressure is affected, and red blood cell counts and vitamin D levels may be affected. If not treated, chronic kidney disease can lead to high blood pressure, heart disease, and other complications.
In the early stages of CKD, most people (up to 40%) may not have any symptoms and may not even be aware that they have kidney disease. Symptoms may only occur when the kidney function is very low.There are many things you can do to reduce your risk of developing CKD. Measures include maintaining a healthy weight with physical exercise and a healthy diet, quitting smoking, and following instructions for over-the-counter pain relievers, such as aspirin, ibuprofen, and acetaminophen (Tylenol). If you have CKD, then it is best to avoid anti-inflammatory medications like ibuprofen if you can. Also, try and avoid herbal remedies unless approved by your doctor.
As diabetes and high blood pressure are two of the most common causes of CKD, maintaining your blood pressure and blood sugars within the recommended range is a critical way to reduce the progression of kidney damage. A low-salt diet is recommended if you have high blood pressure, heart problems, or issues with water retention. Some kidney diseases require special medications that your kidney specialist will manage.
People with CKD often feel well and may not notice any symptoms. The only way to know if you have CKD is through blood and urine tests.
If you have high blood pressure, diabetes, heart disease, or a family history of kidney disease, talk to your healthcare professional. The best way to protect your kidneys is to start treatment when kidney disease is identified.
Sometimes, treatment of kidney disease and its risk factors may still not be enough to prevent progression. If kidney damage is severe, the kidneys will have lost their ability to function and can no longer support your body’s needs. This can result in kidney failure. Symptoms include high blood pressure, poor appetite, weakness/ fatigue, cramps, nausea, vomiting, itching, swelling of the legs, and difficulty breathing, among other things. In these cases, your specialist will discuss the option of kidney replacement therapy (KRT) with you to replace the lost kidney function. Typically the options include dialysis and/ or kidney transplantation.
Schedule an appointment with your doctor if you experience any signs or symptoms of kidney disease. Work with your doctor to control diseases or conditions that increase your risk of kidney disease.
New treatments and therapies are being developed for kidney diseases. Ask your doctor if you are interested in participating in a clinical trial for CKD. More information is available at: www.enrollmypatient.org
Focal segmental glomerulosclerosis (FSGS) is a collection of many types of kidney disorders characterized by scarring of glomeruli, which are tiny filters in the kidneys that help remove waste and excess fluids from the blood. FSGS is one of the leading causes of glomerular disease in the United States and is the second leading cause globally. The exact number of people with this disease is difficult to measure. However, new cases of FSGS have been increasing around the world.
“Focal” and “segmental” describe where the scarring occurs. Focal means that only a few glomeruli in the kidneys are affected. Segmental means only a portion of the individual glomerulus is scarred. Sclerosis means scar. Therefore, Focal Segmental Glomerulosclerosis affects a few of all glomeruli and only scars a portion of these glomeruli. This scarring ultimately results in the leakage of protein into the urine and progressive loss of kidney function.
There are many causes of FSGS. Some of the leading causes are:
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Primary FSGS: This is due to an autoimmune reaction and can respond to therapy if diagnosed early.
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Genetic FSGS: The disease occurs due to a defective gene. Several genes and genetic mutations have been identified that can lead to FSGS.
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APOL1-mediated kidney disease: One of the mutations includes a defect in the APOL1 gene which is more commonly seen in people of recent African descent. The presence of mutations in this gene protects against infection from trypanosomes but, unfortunately, increases susceptibility to kidney injury due to other events (other infections, inflammation, lupus), leading to kidney damage over the lifetime.
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Drug-induced FSGS: Certain medications and illicit drugs can cause FSGS.
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Viruses: Certain viral infections can result in FSGS.
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Adaptive FSGS: There are many causes of adaptive FSGS, such as diabetes, high blood pressure, obesity, birth defects in the kidney, and sickle cell anemia.
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Depending on the cause, FSGS can be seen in men and women of all ages and ethnicities. Children and young patients are more likely to have genetic forms of FSGS, whereas patients of recent African descent are more likely to have APOL1-mediated kidney disease. FSGS is also linked to various diseases and conditions, including diabetes, obesity, sickle cell disease, viral infections, and certain medications and drugs.
Signs and symptoms of focal segmental glomerulosclerosis or APOL1-mediated kidney disease may include:
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Foamy urine due to protein present in urine
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Swelling (edema) throughout the body, including legs and face
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Weight gain due to excess fluid in the body
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High blood cholesterol
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High blood pressure
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Fatigue
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There is no known preventative therapy for FSGS, but clinical trials are underway. However, following a healthy lifestyle that includes a low-fat and low-sodium diet will improve kidney health and could control symptoms of FSGS. It is essential to manage underlying diseases such as diabetes, sickle cell anemia, obesity, and viral infection to protect kidneys from developing FSGS.
FSGS is a pattern of injury to the kidney filters observed on a kidney biopsy. A kidney biopsy is a procedure where a doctor will take a small sample of kidney tissue using a needle and study the sample under a microscope. Your doctor will put these findings together with your clinical presentation to identify the underlying cause of the disease.
To confirm whether the FSGS seen on the biopsy is due to a genetic mutation in the APOL1 gene (APOL1-mediated kidney disease) or due to other genetic causes, genetic testing needs to be performed.
Your doctor will arrange for specific blood and urine tests along with a kidney ultrasound scan and a kidney biopsy to confirm the diagnosis and determine a possible cause.
Treatment for FSGS is dependent on the cause of the disease, either primary or secondary such as due to genetic cause.
Typically primary FSGS treatment involves immunosuppressive medications. Sometimes, several immunosuppressive medications may be required.
For APOL1-mediated kidney disease, several clinical trials are currently under way to find a treatment.
For secondary forms of the disease, treatment is usually directed at the underlying cause. For example, if your doctor suspects an infection causes the disease, treatment is targeted at the infection.
For any form of FSGS, your doctor will likely give you medications to control your blood pressure, reduce the amount of protein leaking into the urine, reduce swelling (using diuretics), maintain your cholesterol levels, and prevent blood clots (using anti-coagulant medications). Your doctor will also advise you to maintain a healthy lifestyle, follow a low-salt diet, quit smoking, and exercise regularly.
If the disease progresses or presents in an advanced stage, your doctor will discuss kidney replacement therapy options with you, including dialysis and kidney transplantation. Your doctor may also offer you the option of enrolling in a clinical trial.
It is important for anyone experiencing symptoms suspicious of FSGS to see a doctor for proper diagnosis and treatment. Early intervention may help prevent complications and improve the chances of a successful outcome.
Developing kidney failure is, unfortunately, a possibility for those suffering from FSGS. Depending on the underlying cause, about 40-50% of patients may not have an adequate response to treatment and may progress to kidney failure, in which case your doctor will discuss the options of dialysis and/or kidney transplantation.
Your doctor will also discuss the risk of the disease recurring after kidney transplantation (this risk is often high in those with primary FSGS but unlikely for genetic or secondary causes).New treatments and therapies are being developed for kidney diseases. Ask your doctor if you are interested in participating in a clinical trial for Focal Segmental Glomerulosclerosis. More information is available at: www.enrollmypatient.org
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American Association of Kidney Patients (AAKP): Provides educational materials and support for those affected by FSGS. AAKP Education & Resources for FSGS
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National Kidney Foundation (NKF): Offers information on FSGS, including causes, symptoms, and treatment options. NKF Focal Segmental Glomerulosclerosis (FSGS)
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National Institute of Health (NIH): Features detailed and comprehensive articles and research updates on FSGS. NIH FSGS Overview
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Kidney Health Australia: Provides information on FSGS for patients in Australia. FSGS Information (Kidney Health Australia)
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Kidney Research UK: Offers insights into FSGS for patients in the United Kingdom. Focal and Segmental Glomerulosclerosis (Kidney Research UK)
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Canadian Glomerulonephritis Registry: Delivers resources and support for Canadian patients dealing with FSGS. FSGS Resources for Patients (Canadian Glomerulonephritis Registry)
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IgA nephropathy is a kidney disease that occurs when abnormal IgA proteins are produced by the body and are deposited in the filters (glomeruli) of our kidneys. IgA proteins (or IgA immunoglobulins) are proteins that are made by our immune system to fight infections. IgA deposition causes inflammation and can cause leakage of blood and protein in the urine. It eventually damages and scars the kidneys over time.We do not fully understand what causes the production of the abnormal IgA proteins or why they only deposit in the kidneys’ filters. We know that there is an association with certain infections, liver disease, coeliac disease, and other conditions. There may also be a genetic predisposition to this disease.
IgA nephropathy is known to be more common among people of Asian (Far East) and European ancestry. It can occur at any age, but it is more commonly seen in younger ages starting in the teens to late 30s. IgA nephropathy occurs more often among males than females and those with a family history of this disease.
Depending on your kidney function and the activity of the disease, you might experience different symptoms. You may notice episodes of blood in the urine, where the urine appears tea-colored or like ‘coca-cola.’ This may often occur after a throat or stomach infection and sometimes after heavy exercise. Often, blood in the urine may not be visible to your naked eye, but your doctor can perform a urine test that can detect small amounts of blood and protein in the urine.
Sometimes this may only be discovered when you have a routine medical exam for other reasons. You may also have high blood pressure, which can reflect kidney damage. Sometimes, the disease presents as ‘IgA vasculitis’ and can involve other organs as well. In this case, you may notice a rash, especially on the legs, diarrhea, leg swelling, joint pains, and the features described above. Some symptoms that may suggest the disease is at a late stage include nausea, vomiting, fatigue, loss of appetite, or leg swelling.
Your doctor will probably request a blood test, urine test, ultrasound of the kidney, and often a kidney biopsy. A kidney biopsy provides a definitive diagnosis. No blood test can diagnose this condition.
Treatments will vary for each patient depending on the level of protein in your urine as well as the kidney biopsy report. There is no specific cure for IgA nephropathy, but we can take measures to slow the disease’s progression and damage to the kidneys.
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Maintaining near-normal blood pressure and reducing the amount of protein leak in the urine are the cornerstones of current treatment.
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Standard treatment options for IgA nephropathy include medications like angiotensin inhibitors or angiotensin II receptor blockers.
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Some select patients may require treatments with steroids that are available under various formulations.
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There is increasing evidence of using sodium-glucose cotransporter 2 inhibitors (SGLT2 inhibitors).
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Your doctor may also prescribe cholesterol-lowering drugs such as statins if this is indicated.
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If your kidney disease is very severe, your doctor will discuss your options for kidney replacement therapy (such as dialysis or a kidney transplant).
Leading a healthy lifestyle is important in supportive care treatment for IgA nephropathy. Your doctor will advise you to eat a balanced diet, reduce salt intake, exercise regularly, quit smoking, and reduce consumption of alcohol within recommended limits. It can be helpful to monitor your blood pressure at home regularly.Yes, several clinical trials are going on evaluating the efficacy of new agents for the treatment of IgA nephropathy. If you would like more information regarding the trials, you can find it here: www.enrollmypatient.org
Currently, there is no proven method to prevent IgA nephropathy, but there are medications available that can delay the progression of the disease. If you have a family history of IgA nephropathy, talk to your doctor about how to keep your kidneys healthy.
IgA vasculitis, formerly Henoch-Schonlein purpura, is the most common childhood systemic vasculitis (inflammation of small-sized blood vessels). The disease develops after IgA antibodies deposit in the walls of small blood vessels, which results in inflammation.
Symptoms depend on in which organ’s blood vessels the IgA antibodies are deposited. One common place is the skin's blood vessels on the buttocks and legs. In these cases, a palpable red-dotted rash called purpura develops. Abdominal pain and joint pain develop when the blood vessels of the intestines and joints are affected. Most children will completely recover from IgA vasculitis. Very rarely can the disease be severe, leading to kidney damage.
IgA vasculitis can affect anyone. However, it is most commonly seen in young children between 3 and 7 years and more commonly affects boys. Those with a family history of IgA vasculitis are more likely to develop it. Many children develop this disease after an upper respiratory tract infection.
Signs and symptoms of IgA vasculitis may include:
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Raised red/purple rash (purpura)
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It is caused by leaking blood vessels in the skin. It usually appears on the legs and buttocks. Sometimes can also occur on the arms, trunk, and face. The rash is not itchy or painful.
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Abdominal pain
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It can be mild to severe. Blood may be present in the stool. In rare cases, intussusception can occur, which means the bowel folds onto itself, causing a blockage.
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Joint pain and swelling
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Blood in the urine
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Swelling of the testicles in boys
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Seizures- rare
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Pneumonia-rare
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Rash, abdominal pain, and joint pain are the first symptoms, preceded by an upper respiratory tract infection weeks prior.
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IgA vasculitis is diagnosed clinically after a thorough history, physical examination, and a tissue biopsy (skin or kidney biopsy). Your doctor will order some laboratory studies to help exclude other diagnoses and to evaluate kidney function. Tests may include:
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Blood tests
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Urine tests
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Gastrointestinal and kidney ultrasound scans
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Skin biopsy of the rash
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Kidney biopsy
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For the most part, IgA vasculitis is a self-limiting disease, meaning patients will recover independently within several weeks or less without treatment. To date, no therapy is known to shorten the duration of IgA vasculitis. Your doctor may recommend medications, such as anti-inflammatory medications, to treat and relieve symptoms such as abdominal pain, joint pain, and swelling. If the kidneys are involved, the treatment goal will be to prevent chronic kidney disease. In case of severe or recurrent disease, your doctor may prescribe medications to suppress the immune system.
The likelihood of developing kidney failure with IgA vasculitis is rare. Patients should have close follow up with their doctors for six months to look for signs of kidney disease. Adults are at a higher risk of developing kidney disease than children. In rare cases, kidney failure develops, requiring long-term hemodialysis until a kidney is available for transplantation.
New treatments and therapies are being developed for kidney diseases. Ask your doctor if you want to participate in a clinical trial for IgA vasculitis. More information is available at: www.enrollmypatient.org
Lupus nephritis is a kidney disorder that occurs as a result of systemic lupus erythematosus (SLE), an autoimmune disease. In lupus nephritis, the body's immune system attacks the kidney tissues, causing inflammation and kidney damage. This can lead to proteinuria (excessive protein in the urine), hematuria (blood in the urine), high blood pressure, and swelling in the legs and feet. If left untreated, lupus nephritis can lead to end-stage kidney disease, requiring dialysis or kidney transplantation.
People who have systemic lupus erythematosus (SLE) are at risk for developing lupus nephritis. SLE is more common in women than in men, and it often develops during the childbearing years. Lupus nephritis can occur at any age, but it is more commonly diagnosed in people in their 20s or 30s. Other factors that may increase the risk of developing lupus nephritis include:
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Genetics: Certain genetic factors may make some people more susceptible to developing SLE and lupus nephritis.
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Ethnicity: Lupus nephritis is more common in people of African, Hispanic, and Asian descent than in people of European descent. In the United States, 1 out of every 250 African American women will develop lupus. However, among all patients with SLE, the risk of developing lupus nephritis was significantly higher in men, younger individuals, and Hispanics.
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Gender: Women are more likely than men to develop SLE. Nine out of ten people who have lupus are women. As noted before, among those with SLE, lupus nephritis is more common in men than women.
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Smoking: Smoking may increase the risk of developing SLE and lupus nephritis.
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Family history: People with a family history of SLE or lupus nephritis may be at a higher risk of developing the condition.
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Damage and inflammation of the kidneys in lupus nephritis cause a variety of signs and symptoms. Some of the common signs and symptoms include:
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Proteinuria: This means excessive amounts of protein in the urine, which can cause foamy urine.
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Hematuria: This means blood in the urine, which may appear pink or red.
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Edema: This means swelling in the legs, ankles, or feet.
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High blood pressure: This is a common complication of lupus nephritis.
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Fatigue: Feeling tired or weak all the time.
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Loss of appetite: Not feeling hungry or having a decreased appetite.
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Weight gain: Unexplained weight gain may occur due to fluid retention.
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Skin rash: A butterfly-shaped rash on the face (both cheeks symmetrically affected) is a common sign of SLE, which can be associated with lupus nephritis.
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Joint pain: Pain and stiffness in the joints can be a symptom of SLE.
If you have any of these symptoms, it is important to talk to your doctor, who may order tests to check your kidney function and determine if you have lupus nephritis. Early diagnosis and treatment can help to prevent further kidney damage and improve outcomes for patients.
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Lupus nephritis is treated with a combination of medications and lifestyle changes. The goal of treatment is to control inflammation, preserve kidney function by blocking your body’s immune cells from attacking the kidneys, and prevent complications. Some common treatments for lupus nephritis:
Immunosuppressive medications: These medications are critically essential and help suppress the immune system and reduce kidney inflammation. They include corticosteroids, and immunosuppressive drugs, such as mycophenolate mofetil, cyclophosphamide, and azathioprine.
Blood pressure medications: High blood pressure is a common symptom of lupus nephritis and can further damage the kidneys. Medications to treat blood pressure and reduce proteinuria can help control blood pressure and protect the kidneys.
Diet and lifestyle changes: A low-salt, low-fat diet can help reduce blood pressure and control weight gain. Regular exercise can also help manage blood pressure and maintain overall health.
Dialysis or kidney transplant: In severe cases of lupus nephritis, dialysis or kidney transplant may be necessary to replace kidney function.
It's essential to work closely with your doctor to develop a personalized treatment plan for your specific needs. Regular follow-up appointments and laboratory tests are necessary to monitor kidney function and adjust treatment. With proper treatment and management, many people with lupus nephritis can maintain kidney function and live normal life.
The risk of developing kidney failure (also known as end-stage renal disease) due to lupus nephritis varies depending on the severity of the disease and how well it is managed. With proper treatment and management, many people with lupus nephritis can preserve kidney function and avoid kidney failure. However, in some cases, kidney failure may occur despite treatment.
The risk of kidney failure is higher in people with severe or active lupus nephritis, high blood pressure, high levels of protein in their urine, or impaired kidney function at the time of diagnosis. Patients with kidney failure secondary to SLE represent 1.5% of all patients on dialysis in the United States. Other factors that can increase the risk of kidney failure include a delay in diagnosis and treatment, poor adherence to treatment, and other medical conditions that can affect the kidneys. Patients with SLE account for 3% of all kidney transplantations in the United States.
Regular follow-up appointments with your doctor and laboratory tests are necessary to monitor kidney function and adjust treatment as needed. Early diagnosis and prompt treatment of lupus nephritis can help reduce the risk of kidney damage and improve outcomes.
New treatments and therapies are being developed for kidney diseases. Ask your doctor if you want to participate in a clinical trial for Lupus Nephritis. More information is available on: www.enrollmypatient.org
Membranous nephropathy is an autoimmune disease in which the body’s immune system targets and attacks the glomeruli. The glomeruli become leaky, leading to abnormal loss of protein in the urine (proteinuria) and a decrease in the ability of the kidneys to filter waste products and excess fluid from the blood.
There are two main types of membranous nephropathy. Primary membranous nephropathy is a condition limited to the kidneys due to an autoimmune process. It is the most common form of membranous nephropathy. In contrast, secondary membranous nephropathy refers to cases of membranous nephropathy related to an underlying systemic disease or condition such as infections (such as hepatitis, parasitic infection, or others), cancer, drugs, toxin, or systemic auto-immune diseases (such as lupus).
Membranous nephropathy is among the most common causes of nephrotic syndrome among adults ages 40 to 60. Additionally, those experiencing certain types of infections, autoimmune diseases, cancers, or medications (such as non-steroidal anti-inflammatory medications) are more susceptible to being diagnosed with the condition. Although this condition is reported among children, it is relatively rare.
Signs and symptoms of membranous nephropathy may include:
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Foamy urine due to protein in the urine
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Swelling (edema) throughout the body, including legs and face
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Weight gain due to excess fluid in the body
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High blood cholesterol
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High blood pressure (hypertension)
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Blood clots (mainly associated with severe protein loss and very low levels of blood protein)
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Membranous nephropathy is usually suspected based on the medical history, clinical examination, and blood and urine tests measuring fat and protein levels. A kidney biopsy is usually required to confirm the diagnosis. A kidney biopsy is a procedure where a doctor takes a small sample of kidney tissue using a needle and studies the sample under a microscope.
Treatment of membranous nephropathy includes supportive measures such as salt and fluid restriction and medications to control blood pressure and reduce protein losses in the urine.
In moderate to severe cases of primary membranous nephropathy, immunosuppressive drugs may reduce or diminish the disease activity in the kidneys.
In cases of secondary membranous nephropathy, treatment is usually directed at the underlying cause.
Membranous nephropathy often increases the risk of blood clots, and in some cases, blood thinning medications (anti-coagulation) may be required. Your doctor will discuss this with you.
Do see your healthcare provider regularly and follow your treatment plan to prevent further damage to the kidneys and maintain kidney function. It is also essential to manage any underlying conditions contributing to the development of membranous nephropathy.
Primary membranous nephropathy may sometimes resolve on its own in mild to moderate cases. However, in many cases, treatment is needed to prevent further kidney damage and maintain kidney function. In more severe cases, the condition can lead to kidney failure, which may require a kidney transplant or dialysis. Kidney failure is defined as a significant reduction in the glomerular filtration rate (GFR), which measures the kidney's ability to filter waste products from the blood.
Not all people with membranous nephropathy will develop kidney failure. The progression of the disease and the likelihood of developing kidney failure can vary depending on several factors, which include the underlying cause of the membranous nephropathy, the severity of protein losses in the urine, and the level of kidney function.See your doctor regularly if you have membranous nephropathy to monitor your kidney function.
Your doctor will perform regular blood and urine tests that will help assess disease progression and will make adjustments to your treatment plan as required.
New treatments and therapies are being developed for kidney diseases. Ask your doctor if you want to participate in a clinical trial for membranous nephropathy. More information is available at: www.enrollmypatient.org
In the kidneys, the filters (glomeruli) are responsible for cleaning the blood by filtering out waste while keeping essential substances in our blood, such as proteins. In minimal change disease, the filters don’t function well because their structure is altered. As blood flows through the altered filters, large amounts of proteins can leak into the urine. The disease gets its name as the damage to the little filters cannot be seen under a regular microscope.
Children are most at risk for developing minimal change disease. Adults may develop minimal change disease as well, but this is less common.
The cause of minimal change disease is unclear. It may occur due to problems in the immune system, especially in children. In adults, there may be a secondary cause, such as tumors (like lymphoma), viral infections, allergies, and anti-inflammatory drug use.
Signs and symptoms of minimal change disease may include:
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Foamy urine due to protein present in urine
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Swelling (edema) throughout the body, including legs and face
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Weight gain due to excess fluid in the body
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High blood cholesterol
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Your doctor will perform blood and urine tests initially. If you are an adult, your doctor will likely recommend a kidney biopsy. A kidney biopsy is a procedure where a doctor takes a sample of kidney tissue using a needle and studies the sample under a microscope.
For both children and adults, the main treatment option is immune supression. Based on treatment response or disease recurrence, the use of each treatment option may vary. It is crucial to take these medications as prescribed and not stop them suddenly. Unfortunately, medications that suppress the immune system can often have several side effects, and often your doctor may prescribe some additional medicines to prevent some of the side effects. But in overwhelming majority of cases, the benefit of preserving the kidneys outweigh the risks associated with the medications.
If you fail to respond to the initial therapy, your doctor will discuss alternative treatment options with you.
Other treatments that are used in addition to medications that suppress the immune system may be used to manage the symptoms of the disease. These typically include:
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Medications that reduce blood pressure and reduce protein leakage.
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Diuretics (water pills) that are used to reduce swelling throughout the body.
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Cholesterol-lowering pills if your cholesterol level is very high.
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You may be advised to reduce your water and salt intake to reduce swelling.
Regular follow-up with your doctor is critical to ensure that you are responding well to treatment and not developing side effects.
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Kidney failure is rare, with minimal change in disease. Most patients respond well to first-line treatment. Relapses can occur over the long term, usually treated with a different medication. If you have many relapses, your doctor may discuss alternate therapies.
New treatments and therapies are being developed for kidney disease. Ask your doctor if you want to participate in a clinical trial for minimal change disease. More information is available at: www.enrollmypatient.org
Nephrotic syndrome is defined by abnormal loss of protein through the kidneys into the urine (greater than 3.5 grams per day in adults and greater than 50 mg/kg per day in children), leading to low protein levels (albumin, in particular) in the blood. Low albumin in the blood leads to fluid retention and swelling in some areas of the body, high cholesterol levels in the blood, increased risk for blood clots, and increased risk for infections. Some main causes of nephrotic syndrome include:
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Minimal change disease: This is the most common cause of nephrotic syndrome in young children.
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Focal segmental glomerulosclerosis (FSGS): Caused by scarring of the kidney’s glomeruli, either immune-mediated, due to genetic causes, or due to several factors combined. This is a common cause of nephrotic syndrome in adults. Several conditions can result in this particular disease.
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Membranous nephropathy: This occurs due to the thickening and damage of certain parts of the glomeruli.
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Children and adults of all ages can be affected by nephrotic syndrome. However, some groups may be at higher risk depending on the underlying cause of nephrotic syndrome.
Signs and symptoms of nephrotic syndrome may include:
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Foamy urine due to protein present in urine
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Swelling (edema) throughout the body, including legs and face
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Weight gain due to excess fluid in the body
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Tiredness
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Loss of appetite
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High cholesterol levels
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High blood pressure
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Anemia
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Increased risk for infections
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Increased risk for blood clots
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Many symptoms are attributed to the loss of proteins through the kidneys.
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If your history and clinical examination suggest nephrotic syndrome, your doctor will arrange for blood and urine tests. Blood tests assess your kidney function, the level of protein (albumin, in particular) in your blood, your cholesterol levels, and possibly a panel of tests to check for any potential causes leading to the condition. Urine tests are done to check for protein losses.
Sometimes your doctor may require a 24-hour urine collection to quantify the amount of protein lost in the urine accurately. This can also be checked on a single urine sample. Your doctor may arrange an ultrasound scan of your kidneys. Usually, a kidney biopsy is required to confirm the diagnosis.
The treatment of nephrotic syndrome depends on the underlying cause. Management will vary among the adult and pediatric populations.
Treatment also varies depending on symptoms and the extent of kidney damage. Blood pressure medications can help lower blood pressure and reduce protein loss. Diuretics can be used to reduce swelling. Statins can be prescribed to lower cholesterol. Blood thinners may be used if patients develop blood clotting disorders. Finally, patients must be updated on their vaccinations due to the increased risk of infections. In some patients, treating the underlying cause can lead to remission.
The likelihood of developing kidney failure with nephrotic syndrome depends on the underlying cause. Patients with minimal change pathology have an excellent prognosis, and kidney failure is rare. Patients with focal segmental glomerulosclerosis have a poor prognosis. 40-60% develop end-stage kidney disease (ESKD) in 10-20 years. Of patients with membranous nephropathy, ~30-40% developed ESKD in ten years. If kidney failure does develop, then dialysis or a kidney transplant will be needed.
New treatments and therapies are being developed for kidney diseases. Ask your doctor if you want to participate in a clinical trial for Nephrotic Syndrome. More information is available at: www.enrollmypatient.org
