Many people first notice focal segmental glomerulosclerosis when routine urine tests pick up protein in the urine, sometimes after puffy eyelids or swelling in the ankles and feet appears, especially later in the day. Some also see foamy urine, gain fluid-related weight, or develop high blood pressure, which can prompt a check that reveals protein loss and reduced kidney filtering. In children, the first signs of focal segmental glomerulosclerosis often show up as sudden swelling and heavy protein in the urine during a checkup after a viral illness or mild cold.
Condition
Focal segmental glomerulosclerosis
This condition has the following symptoms:
SwellingFoamy urineHigh blood pressureRapid weight gainTirednessUrination changesShortness of breathFocal segmental glomerulosclerosis is a kidney disease that causes scarring in parts of the filtering units. People with focal segmental glomerulosclerosis often notice swelling in the legs or around the eyes and foamy urine, and doctors may find high blood pressure and protein in the urine. It can affect children and adults, and the course can be slow or faster, with some people progressing to kidney failure if scarring worsens. Treatment focuses on lowering urine protein and protecting kidney function with blood pressure medicines, steroids or other immune therapies in selected cases, and salt and protein moderation. The outlook varies, but many people live long lives with careful monitoring, and dialysis or a kidney transplant may be needed if kidneys fail.
Short Overview
Symptoms
Focal segmental glomerulosclerosis often starts silently. Early symptoms of FSGS include foamy urine, swelling in the legs, ankles, or around the eyes, and sudden weight gain from fluid. Many also develop fatigue and high blood pressure as kidney function declines.
Outlook and Prognosis
Many living with focal segmental glomerulosclerosis can keep kidneys working for years with prompt treatment, blood pressure control, and regular follow-up. Some have flare‑ups or gradual decline, while others stay stable. Early symptoms of FSGS caught and managed tend to improve outcomes.
Causes and Risk Factors
Focal segmental glomerulosclerosis can be primary, or due to inherited podocyte gene changes, including APOL1 variants. Risks include HIV infection, certain drugs, obesity, reduced kidney mass, reflux, and sickle cell disease. Family history and African ancestry raise risk.
Genetic influences
Genetics play a meaningful role in focal segmental glomerulosclerosis, especially in children and people with a family history. Variants in genes affecting kidney filters (like APOL1 in some African ancestry groups) can raise risk or shape severity. Genetic testing can guide diagnosis, family counseling, and treatment choices.
Diagnosis
Doctors suspect focal segmental glomerulosclerosis when urine tests show heavy protein loss and blood tests suggest reduced kidney function. They confirm the diagnosis with a kidney biopsy. Additional workup—autoimmune, viral, medication, and genetic testing—helps identify primary versus secondary causes.
Treatment and Drugs
Treatment for focal segmental glomerulosclerosis focuses on protecting kidney function, easing symptoms, and managing triggers. Plans may include blood pressure medicines (like ACE inhibitors/ARBs), diuretics, statins, and salt restriction, plus steroid or non-steroid immunosuppressants when immune injury is suspected. Some benefit from SGLT2 inhibitors, rituximab, or plasma exchange in select patterns; kidney transplant and dialysis are options if kidney function declines.
Symptoms
Focal segmental glomerulosclerosis (FSGS) affects how your kidneys hold onto protein and move fluid, so symptoms often involve swelling and changes in urine. Early symptoms of focal segmental glomerulosclerosis can be easy to miss, and many people feel well at first. You might notice puffy ankles or eyelids, foamy urine, or a quick jump in weight from fluid. Symptoms vary from person to person and can change over time.
Swelling and puffiness: Puffiness around the eyes in the morning or swelling of the ankles by evening is common. In FSGS, this happens as fluid leaks into tissues when the kidneys lose protein. Shoes or rings may feel tighter than usual.
Foamy urine: Urine that looks unusually foamy or bubbly can signal extra protein loss. Clinicians call this proteinuria, which means protein is spilling into the urine. If the foaming is persistent, it’s worth getting checked.
Rapid weight gain: Fluid buildup can cause quick weight changes over a few days. You might see a 2–5 kg (5–10 lb) increase without eating more. This can happen in FSGS when swelling worsens.
High blood pressure: Blood pressure can rise, sometimes without obvious symptoms. Some notice headaches, nosebleeds, or blurry vision when it’s high. Keeping blood pressure controlled is important with FSGS to protect kidney function.
Fatigue and weakness: Low energy is common when the body loses protein and retains fluid. What once felt effortless can start to require more energy or focus. Rest may help, but the tiredness often lingers.
Urination changes: You may urinate less when fluid is retained, or more often at night as fluid shifts when lying down. Urine may stay foamy if protein loss continues. A healthcare professional can help sort out what’s typical aging and what warrants a closer look.
Shortness of breath: Significant fluid overload can make breathing harder, especially when lying flat or walking up stairs. If this happens suddenly or gets worse, it needs urgent medical attention. This is less common but can occur with heavy swelling.
Abdominal bloating: Fluid can collect in the belly, leading to fullness, tightness, or a stretched feeling. Clothes may fit tighter around the waist even if you haven’t eaten more. In FSGS, this often appears along with ankle swelling.
How people usually first notice
Types of Focal segmental glomerulosclerosis
Focal segmental glomerulosclerosis (FSGS) has several well-recognized variants that differ in cause and how the kidneys look under the microscope. These variants can lead to different ages of onset, response to treatment, and long-term outlook. Clinicians often describe them in these categories:
Primary (idiopathic)
Symptoms often start suddenly with swelling in the legs and foamy urine from heavy protein loss. Blood tests and scans do not reveal a clear trigger, and many respond to immune-directed treatments like steroids.
Secondary (adaptive)
This type develops as a response to another strain on the kidneys, such as obesity, reduced kidney mass, or long-standing high pressure in the filters. Treating the underlying driver and using kidney-protective medicines are the mainstays, and immune treatments usually help less.
Genetic (inherited)
Changes in podocyte genes cause the filter cells to work abnormally, leading to protein leak that often begins in childhood or young adulthood. Family history can offer clues, and genetic testing helps confirm variants of FSGS and guide treatment choices.
Collapsing variant
The filtering units collapse on themselves, and kidney function can fall quickly. People may notice rapid swelling, fatigue, and rising creatinine, and this variant often needs urgent, intensive care.
Tip lesion variant
Damage centers near the tubular opening of the filter, often with sudden heavy protein loss and swelling. This variant may respond better to steroids than some other types of FSGS.
Perihilar variant
Scarring clusters near the blood-entry side of the filter and is commonly linked with adaptive causes like larger body size or reduced nephron number. Managing the driver and controlling blood pressure and protein loss are key.
Cellular variant
There is active, inflamed-appearing injury in parts of the filter with protein leak and swelling. It can look aggressive at biopsy but may improve with timely therapy.
Did you know?
Some people with genetic forms of focal segmental glomerulosclerosis (FSGS) develop early, hard‑to‑treat protein in the urine and swelling because mutations in podocyte genes like NPHS2 or ACTN4 weaken these kidney filter cells. Variants in APOL1, more common in people of African ancestry, raise risk of rapid kidney function loss.
Causes and Risk Factors
Focal segmental glomerulosclerosis can develop on its own or as a result of other stresses on the kidneys. Secondary triggers include infections like HIV, certain medications, and conditions that overwork the kidneys such as obesity or having only one kidney. Inherited changes in genes that support the kidney filter, and APOL1 gene variants more common in people with African ancestry, can increase the chance of FSGS. Genes set the stage, but environment and lifestyle often decide how the story unfolds. Doctors also consider long-standing high blood pressure, prior kidney injury, and family history when weighing risk and planning follow-up.
Environmental and Biological Risk Factors
Environmental and biological factors can raise the chance of focal segmental glomerulosclerosis (FSGS) by putting extra stress on the kidney’s filters. Doctors often group risks into internal (biological) and external (environmental). Understanding these risks can help you plan follow-up and spot early symptoms of FSGS sooner.
Viral infections: Certain viruses, including HIV, cytomegalovirus, parvovirus B19, and COVID-19, can injure the kidney filters. This can trigger FSGS, sometimes in a fast-moving form during or soon after infection. Treating the infection reduces ongoing strain, but some scarring may remain.
Interferon medicines: Interferon-based treatments for conditions like multiple sclerosis, hepatitis, or some cancers can harm the filtering cells in the kidneys. That injury can lead to FSGS in some people. Risk often rises with higher doses or longer courses.
Bisphosphonate therapy: High-dose intravenous bisphosphonates such as pamidronate have been linked to scarring in kidney filters. The chance is greater with repeated infusions or large total doses. Kidney tests during treatment can catch early changes.
Fewer filters at birth: Being born premature or at a low birth weight can mean fewer kidney filtering units. Each unit then works harder, raising the risk of FSGS later on. This can appear years after birth.
Single kidney: Living with one working kidney, whether after surgical removal or because the other didn’t develop well, increases workload on the remaining filters. Over time, this extra pressure can contribute to scarring. Regular checkups can spot early changes in kidney function.
Urine backflow or blockage: Vesicoureteral reflux or long-standing urinary blockage can scar kidney tissue. Scarring forces the remaining healthy filters to overwork, which can lead to more damage. Early treatment of reflux or obstruction lowers future strain.
Chronic kidney scarring: Any condition that leaves lasting scars in the kidneys can start the same overwork cycle. As remaining filters take on extra flow, FSGS can develop on top of the original disease. Protecting remaining kidney function helps limit this stress.
High blood pressure: Years of poorly controlled high blood pressure can stiffen small kidney vessels and strain the filters. That mechanical stress raises the chance of secondary scarring. Tight pressure control helps limit ongoing injury.
Hyperfiltration stress: When each filter has to process more blood than usual, wear and tear builds. This biological pathway is a common route to secondary damage in the filtering mesh. Early management of the underlying cause lowers that stress.
Genetic Risk Factors
If focal segmental glomerulosclerosis (FSGS) runs in your family, inherited changes in kidney-related genes may influence who develops it and at what age. Carrying a genetic change doesn’t guarantee the condition will appear. Specific genes can affect the strength of the kidney’s filtering cells and raise the chance of scarring over time. Knowing your inherited risks can guide earlier checks if early symptoms of focal segmental glomerulosclerosis, like new swelling or foamy urine, show up.
APOL1 high-risk: In people with African ancestry, having two APOL1 risk variants greatly raises the chance of focal segmental glomerulosclerosis. Many with these variants never develop kidney problems, and risk can differ between families.
NPHS2 (podocin): Changes in the NPHS2 gene can lead to protein loss in the urine and scarring of the kidney filter, often starting in childhood. It is usually inherited in a recessive way, meaning both parents are typically healthy carriers.
ACTN4 changes: A single altered ACTN4 gene can cause adult-onset focal segmental glomerulosclerosis in some families. Severity and age at onset can vary widely, even within the same family.
TRPC6 changes: Variants that boost TRPC6 activity can stress the kidney’s filtering cells and lead to scarring, often in the teens or adulthood. Some families show a gradual worsening over time.
INF2 changes: Changes in INF2 can cause a dominant, often adult-onset disease affecting the kidney’s filtering units. Some families also have nerve problems in the legs and feet, but this is not always present.
COL4A genes: Variants in COL4A3, COL4A4, or COL4A5 can damage the kidney’s support membrane, with blood in the urine early and focal segmental glomerulosclerosis later. Hearing or eye differences may occur in some, pointing to a collagen-related cause.
WT1 variants: Certain WT1 changes can lead to early nephrotic syndrome that does not respond to standard steroids, with FSGS features in the kidney. This pattern is most often recognized in children.
LMX1B (nails): LMX1B variants, known in nail‑patella syndrome, can weaken the kidney filter and raise the risk of focal segmental glomerulosclerosis. Clues include nail or kneecap differences in the family.
CoQ10 pathway genes: Recessive changes in genes such as COQ2, COQ6, or COQ8B (ADCK4) can cause heavy protein loss and FSGS, often in children or adolescents. A sibling may be affected if both parents carry the same change.
Mitochondrial DNA: Some mitochondrial variants passed down the maternal line can contribute to focal segmental glomerulosclerosis. These may come with other features, such as hearing loss or neurological symptoms.
Family inheritance: Patterns like autosomal dominant, recessive, X‑linked, or maternal inheritance help explain who else might be at risk for FSGS. A new (de novo) variant can also appear in a child with no prior family history.
Ancestry background: Genetic risk for focal segmental glomerulosclerosis varies by ancestry, with APOL1 risk being most relevant in people with African roots. People from other backgrounds can have gene-linked disease through many of the other genes listed here.
Lifestyle Risk Factors
Lifestyle habits don’t cause focal segmental glomerulosclerosis (FSGS), but they can meaningfully influence protein leakage, swelling, blood pressure, and how fast kidney function declines. Understanding the lifestyle risk factors for Focal segmental glomerulosclerosis can help you focus on changes that protect your kidneys. Diet and activity choices often affect proteinuria and edema, which are central to FSGS management.
High-sodium diet: Excess salt raises blood pressure and makes the kidneys retain fluid, worsening edema and protein leakage in FSGS. Cutting sodium can reduce proteinuria and help blood pressure medicines work better.
High-protein intake: Large protein loads can increase intraglomerular pressure and protein spillage in FSGS. A moderated protein intake, individualized by a clinician or dietitian, may slow kidney stress.
Obesity and weight gain: Extra weight drives hyperfiltration in remaining healthy nephrons, accelerating scarring in FSGS. Weight loss can lower proteinuria and improve blood pressure control.
Physical inactivity: Being sedentary contributes to hypertension and insulin resistance, both of which aggravate proteinuria in FSGS. Regular moderate activity can help reduce blood pressure and slow kidney decline.
Smoking: Tobacco use speeds chronic kidney disease progression and amplifies cardiovascular strain in FSGS. Quitting smoking can reduce the rate of eGFR loss and improve treatment response.
Alcohol excess: Heavy drinking raises blood pressure and can disrupt medication adherence, worsening FSGS control. Limiting alcohol supports steadier blood pressure and kidney protection.
Frequent NSAID use: Regular high-dose NSAIDs reduce renal blood flow and can increase the risk of kidney injury in FSGS. Prefer non-NSAID strategies for pain under clinician guidance.
High-sugar patterns: Diets high in sugars promote weight gain and insulin resistance, which can heighten proteinuria in FSGS. Choosing lower-glycemic, fiber-rich foods supports better metabolic and kidney outcomes.
Risk Prevention
Focal segmental glomerulosclerosis (FSGS) can’t always be prevented, but you can lower the chance of it developing from secondary causes and slow kidney damage if you’re at risk. Prevention is about lowering risk, not eliminating it completely. The same steps also help protect kidney function if you’re already living with FSGS. Work with your care team to tailor these to your health, medicines, and daily life.
Blood pressure control: Keeping blood pressure in a healthy range eases strain on the kidney filters. Many people with FSGS benefit from medicines like ACE inhibitors or ARBs to protect the kidneys.
Treat protein leakage: If urine tests show protein, medicines that reduce protein loss can slow FSGS damage. Your doctor may adjust doses over time to keep protein levels as low as possible.
Regular screening: Simple urine and blood tests can spot kidney stress early, especially if you have diabetes, obesity, or a family history of FSGS. Screenings and check-ups are part of prevention too.
Know early signs: Noticing early symptoms of focal segmental glomerulosclerosis—like leg or ankle swelling and foamy urine—can prompt faster testing. Early care may prevent further scarring.
Healthy weight: Carrying excess weight can raise pressure inside the kidneys and trigger secondary FSGS. Gradual weight loss through diet and movement can lower that strain.
Diabetes management: Keeping glucose in target range protects tiny kidney blood vessels. Regular A1C checks and medication adjustments can reduce FSGS risk tied to diabetes.
Limit salt: Too much salt can drive up blood pressure and fluid buildup, stressing the kidneys. Cooking more at home and choosing lower-sodium options can make a noticeable difference.
Avoid kidney-harming drugs: Frequent use of NSAID painkillers (like ibuprofen or naproxen) and certain supplements can injure kidneys. Ask your doctor or pharmacist before using over‑the‑counter pain relievers or new supplements if you have FSGS risk.
Stay active: Regular physical activity helps control blood pressure, weight, and blood sugar, all key to preventing secondary FSGS. Aim for steady movement most days in ways that fit your body.
Control cholesterol: High cholesterol can worsen kidney scarring over time. Diet changes and, when needed, statins may lower this risk in FSGS.
Prevent infections: Vaccines and prompt treatment of infections reduce inflammation that can stress kidneys. This is especially important if you take immune‑affecting medicines.
Avoid risky steroids: Anabolic steroids or non‑prescribed hormones can harm kidneys and have been linked to FSGS. Skip performance enhancers and discuss any hormone use with your doctor.
APOL1 and ancestry: People of West African ancestry with strong family history may discuss APOL1 testing and earlier monitoring. Tailored screening can catch changes before they progress.
Medication review: Some prescription drugs can affect kidneys; a periodic review helps balance benefits and risks. Never stop a medicine without medical advice—ask about safer alternatives if needed.
Hydration habits: Steady, moderate fluid intake supports kidney function unless your doctor advises limits. Avoid frequent dehydration from extreme dieting or strenuous activity without fluids.
How effective is prevention?
Focal segmental glomerulosclerosis (FSGS) includes several types, so prevention depends on the cause. When FSGS is secondary to risks like obesity, uncontrolled HIV, heroin use, or reflux/obstruction, tackling those factors can meaningfully lower risk or slow damage. If it’s genetic or primary (immune-mediated), true prevention isn’t possible, but early detection, tight blood pressure and protein control, and prompt treatment can reduce complications. For many, consistent care helps preserve kidney function longer, though it can’t guarantee avoiding progression.
Transmission
Focal segmental glomerulosclerosis (FSGS) is not an infection and cannot be spread from person to person. In some people, it runs in families because of changes in genes that affect the kidney’s filtering cells; these can be passed down in a dominant or recessive way, and sometimes arise as a new change even when no one else in the family is affected. How Focal segmental glomerulosclerosis is inherited varies by the gene involved, so a genetics professional can explain your family’s pattern. Some people carry risk variants—more common in those with recent African ancestry—that raise the chance of FSGS but do not by themselves cause disease; other health or environmental factors may be needed. FSGS can also develop from non-genetic causes like long-standing kidney strain, certain infections, or medicines; in these situations the underlying illness may be contagious, but the kidney scarring itself is not.
When to test your genes
Consider genetic testing if FSGS appeared at a young age, runs in your family, resists standard treatments, or recurs after a kidney transplant. Testing can clarify the cause, guide medication choices, and shape donor selection and transplant planning. Ask a nephrologist or genetic counselor about targeted panels for podocyte and complement pathway genes.
Diagnosis
When focal segmental glomerulosclerosis (FSGS) affects the kidneys, daily life clues often come first—puffiness around the eyes or ankles, foamy urine, or feeling unusually tired. These changes usually lead to lab checks of kidney function and protein in the urine, and then to more focused tests. Doctors usually begin with a detailed history, exam, and urine tests before deciding on imaging or a kidney biopsy. Below is how FSGS is diagnosed in most clinics, and what to expect during the diagnosis of FSGS.
History and exam: Your clinician asks about swelling, urine changes, recent infections, medicines, and other health conditions. They look for ankle or eyelid puffiness and check blood pressure to spot signs of kidney strain.
Urine tests: A urine dipstick and lab measurements check for high protein and blood. An albumin-to-creatinine ratio or a 24‑hour urine collection estimates how much protein you’re losing each day.
Blood tests: Creatinine and estimated GFR show how well your kidneys filter waste. Albumin, cholesterol, and electrolytes help gauge the effects of protein loss and overall kidney health.
Blood pressure check: Many people with FSGS have high blood pressure, which can worsen kidney injury. Regular measurements help guide treatment decisions and track response to therapy.
Rule out other causes: Tests for diabetes, autoimmune conditions, and infections like hepatitis or HIV help exclude more common reasons for protein loss. Reviewing medications and exposures can also uncover a secondary cause.
Kidney ultrasound: Imaging checks kidney size and structure and looks for blockage or scarring. Normal images don’t rule out FSGS, but they help guide next steps.
Kidney biopsy: A small needle sample from the kidney is examined under a microscope to confirm FSGS and its pattern. Special stains, immunofluorescence, and electron microscopy help distinguish FSGS from other kidney diseases.
Repeat measurements: Protein in the urine can fluctuate, so confirming persistent high levels over time is important. This helps ensure treatment is based on a stable, accurate picture.
Genetic testing: Some conditions have a genetic link, meaning they can run in families. Testing may be considered if FSGS starts in childhood, recurs after transplant, or there’s a strong family history.
Specialist referral: In some cases, specialist referral is the logical next step. A nephrologist can coordinate testing and interpret biopsy findings to tailor treatment.
Stages of Focal segmental glomerulosclerosis
Focal segmental glomerulosclerosis does not have defined progression stages. It’s a pattern of kidney scarring that can behave differently from person to person—sometimes staying fairly stable, sometimes coming in relapses, and sometimes gradually worsening—so doctors track protein loss in the urine and kidney function rather than a fixed set of stages. Different tests may be suggested to help confirm the diagnosis and gauge severity, including urine protein checks, blood creatinine to estimate eGFR, and blood pressure measurements. Early symptoms of focal segmental glomerulosclerosis can be subtle—ankle swelling, foamy urine, or weight gain from fluid—and doctors often confirm with a kidney biopsy and then monitor with periodic urine protein-to-creatinine ratios and eGFR trends.
Did you know about genetic testing?
Did you know genetic testing can clarify why focal segmental glomerulosclerosis (FSGS) happened and whether it’s likely to run in your family? Finding a genetic cause can steer treatment choices, avoid medications that may not help, guide screening of relatives, and inform kidney transplant planning by estimating the risk of recurrence. It can also connect you with clinical trials and support earlier, kidney‑protective care like blood pressure control and targeted monitoring.
Outlook and Prognosis
Many people ask, “What does this mean for my future?”, and with focal segmental glomerulosclerosis (FSGS) the answer depends on the type, how quickly kidney function changes, and how you respond to treatment. Some people respond well to medicines that calm kidney inflammation and protein loss, while others have more stubborn disease that keeps protein high in the urine and slowly wears down kidney function. Everyone’s journey looks a little different. If kidney scarring progresses despite treatment, chronic kidney disease can develop over years, and a portion of people will eventually need dialysis or a kidney transplant.
Looking at the long-term picture can be helpful. Doctors call this the prognosis—a medical word for likely outcomes. In general, people with lower protein in the urine after starting treatment tend to have a better outlook, and those with persistent heavy protein are at higher risk for kidney failure over time. Some inherited forms and certain high-risk variants (for example, APOL1 risk alleles) can drive a faster course, while secondary FSGS from another cause may improve if the trigger is treated. Early symptoms of FSGS, like swelling in the legs or foamy urine, aren’t a reliable guide to severity, so regular labs and blood pressure checks matter more than how you feel day to day. When kidney failure occurs, survival depends on timely dialysis or transplant; after transplant, FSGS can return in the new kidney in some people, but treatments given around the time of surgery can lower that risk.
With ongoing care, many people maintain stable kidney function for years. Keeping blood pressure well controlled, reducing protein in the urine with the right medicines, managing cholesterol, and treating the underlying cause when possible all improve the long-term outlook. Talk with your doctor about what your personal outlook might look like. They can review your response to therapy, lab trends, and any genetic findings to help you plan next steps and monitor for early warning signs of change.
Long Term Effects
Focal segmental glomerulosclerosis (FSGS) can have a long arc, ranging from stable kidney function to gradual decline over years. Long-term effects vary widely, and two people with the same diagnosis can have very different paths. Many remember that early symptoms of focal segmental glomerulosclerosis were subtle—puffy ankles or foamy urine—while the bigger impacts emerged slowly. Outlook depends on how well protein loss is controlled, blood pressure is managed, and whether scarring continues to progress.
Kidney function decline: Some people stay stable for years, while others develop chronic kidney disease that can lead to kidney failure. Doctors often describe these as long-term effects or chronic outcomes.
Ongoing protein loss: Protein in the urine may persist, even when you feel otherwise well. This can strain the kidneys over time and signal ongoing damage.
Swelling and fluid: Puffiness in the legs, ankles, or around the eyes can continue or come and go. For many, this can mean tighter shoes by evening or needing to elevate the legs after activity.
High blood pressure: Blood pressure often rises as kidney function changes. Keeping it controlled is key to slowing further kidney damage.
Heart risk higher: Extra strain on the heart and blood vessels can build over the years. Cholesterol changes and high blood pressure together raise long-term cardiovascular risk.
Blood clots: Some people face a higher chance of clots in the legs or lungs. This risk tends to be higher when protein loss is severe.
Infection risk: Losing protective proteins in the urine can make infections more likely. You may notice more frequent sinus, skin, or respiratory infections during active phases.
Cholesterol changes: Cholesterol and triglycerides can stay elevated. These changes may need long-term monitoring and treatment to protect the heart.
Bone health strain: Ongoing protein loss can affect vitamin D and calcium balance. Over time, this may weaken bones and raise fracture risk.
Transplant recurrence: In some, FSGS can return after a kidney transplant. Doctors may track these changes over years to see patterns and respond early.
Childhood growth impact: In children, ongoing kidney issues and treatments can slow growth and delay puberty. Growth usually improves when kidney health is steadier.
How is it to live with Focal segmental glomerulosclerosis?
Living with focal segmental glomerulosclerosis can feel unpredictable: energy may run low, ankles or eyelids can swell by day’s end, and bathroom visits become a quiet barometer of how the kidneys are coping. Daily life often involves medicines, reduced salt and protein where advised, regular lab checks, and planning around fatigue or flare days, but many still work, study, and stay active with thoughtful pacing. Family and friends may take on more—driving to appointments, helping with meals, noticing swelling—yet they also become key partners in staying on track and celebrating stable stretches. With a clear plan, open communication with your care team, and support at home or in community groups, people often find a steady rhythm even when the condition itself ebbs and flows.
Treatment and Drugs
Treatment for focal segmental glomerulosclerosis (FSGS) aims to protect kidney function, reduce protein loss in urine, and manage swelling and blood pressure. Doctors often start with medicines that lower pressure inside the kidney filters and reduce protein in the urine, such as ACE inhibitors or ARBs, along with diuretics for fluid buildup, cholesterol-lowering drugs, and salt restriction; if nephrotic-range protein is present, blood thinners may be considered based on risk. When the immune system is involved, steroids are commonly used, and some people may receive additional immune-suppressing drugs like calcineurin inhibitors or mycophenolate—A doctor may adjust your dose to balance benefits and side effects. If FSGS is caused by another condition (like obesity, certain infections, medications, or a genetic change), treating that cause is key, and genetic testing can guide choices when suspected. For advanced disease, supportive care, dialysis, or kidney transplant may be discussed, and because FSGS can return after transplant, close monitoring and early treatment are essential.
Non-Drug Treatment
Focal segmental glomerulosclerosis (FSGS) care often includes practical steps that support your kidneys day to day, reduce swelling, and protect heart health. Alongside medicines, non-drug therapies can make a meaningful difference over time. If you notice early symptoms of focal segmental glomerulosclerosis, like new ankle swelling or foamy urine, these steps may help slow strain on your kidneys. Your care team can tailor them to your stage of kidney disease and personal goals.
Lower sodium: Aim for less than 2,000 mg sodium per day (about 5 g of salt). This helps control swelling and blood pressure.
Balanced protein: Avoid very high-protein diets that can strain kidneys. A common target is about 0.8 g per kg body weight per day (roughly 0.36 g per lb), guided by a dietitian.
Edema care: Elevate legs when sitting and consider compression socks if your clinician says they’re appropriate. These steps can reduce ankle and lower-leg swelling and discomfort.
Fluid guidance: Follow your clinician’s advice on fluids, which may stay normal or be modestly limited if swelling is significant. Spreading drinks through the day can help you feel less puffy.
Home blood pressure: Check blood pressure at home and keep a simple log to share at visits. Steady readings help your team fine-tune care and protect kidney function.
Heart-healthy eating: Emphasize vegetables, fruits, whole grains, lean proteins, and unsalted foods. Limiting ultra-processed foods and added sugars supports blood pressure and weight.
Physical activity: Aim for regular moderate exercise, like brisk walking 150 minutes per week (about 30 minutes, 5 days). Activity helps control blood pressure, weight, and energy.
Weight management: Gradual, sustainable weight loss can ease pressure on kidneys if you live with excess weight. Small changes in portions and movement often add up.
Avoid kidney stressors: Skip NSAIDs like ibuprofen unless your clinician says otherwise, and be cautious with herbal supplements. Stay well hydrated during illness, and ask about contrast dye before scans.
Vaccinations: Stay current with flu, COVID-19, and clinician-recommended shots such as pneumococcal and hepatitis B. This lowers infection risk when protein loss may weaken defenses.
Smoking cessation: Quitting tobacco improves blood flow to the kidneys and heart. Nicotine replacement or counseling can boost your chances of success.
Sleep and stress: Aim for regular sleep and use stress-reduction practices like breathing exercises or gentle yoga. Lower stress may help blood pressure and day-to-day coping.
Skin and clot care: Treat skin gently, moisturize, and report redness or pain in the legs promptly, as nephrotic states can raise clot risk. Your clinician can advise on warning signs to watch for.
Regular monitoring: Keep lab appointments and urine checks to track protein levels and kidney function. Early changes on tests often guide timely adjustments to your plan.
Care coordination: See a renal dietitian and nephrology nurse if available for practical, personalized advice. Family members often play a role in supporting new routines.
Did you know that drugs are influenced by genes?
Genes can affect how your body processes FSGS medicines—changing how strongly steroids work, how calcineurin inhibitors (like tacrolimus) are tolerated, or how quickly side effects appear. In some people, variants such as APOL1 also guide drug choice and expected response.
Pharmacological Treatments
Medicines for focal segmental glomerulosclerosis (FSGS) aim to lower protein in the urine, calm overactive immune signals, reduce swelling, and protect kidney function over time. Not everyone responds to the same medication in the same way. High-dose steroids are often tried first, with other immune therapies used if FSGS relapses or doesn’t improve; steroid-resistant FSGS treatment options typically include calcineurin inhibitors and, in select cases, rituximab. Blood pressure, cholesterol, and fluid control drugs also play a key role in protecting the kidneys for the long term.
Corticosteroids (prednisone): Helps quiet immune-driven injury in the kidney’s filters and reduce protein leakage. Courses often last months with a slow taper, and doctors monitor blood sugar, bone health, mood, and infection risk.
Calcineurin inhibitors: Cyclosporine or tacrolimus can help when steroids don’t work or cause frequent relapses. Levels are checked with blood tests, and kidney function and blood pressure need close monitoring.
Rituximab: An infusion used in some steroid-dependent or steroid-resistant cases, and sometimes for recurrent FSGS after transplant. It can reduce protein in the urine and help maintain remission, but increases infection risk and may cause infusion reactions.
ACE inhibitors/ARBs: Lisinopril or losartan lower pressure inside kidney filters and cut protein in the urine. They help protect kidney function long term, but potassium and creatinine need checking and they’re avoided in pregnancy.
SGLT2 inhibitors: Dapagliflozin or empagliflozin can reduce protein leakage and slow kidney decline, even in people without diabetes. Risks include genital yeast infections and dehydration, and they’re adjusted based on kidney function.
Diuretics: Medicines like furosemide or torsemide help move extra fluid to ease leg swelling and shortness of breath. Doses are tailored to symptoms, with checks on sodium and potassium to prevent cramps or dizziness.
Statins: Atorvastatin or rosuvastatin treat high cholesterol that often comes with nephrotic-range protein loss. They lower cardiovascular risk, with monitoring for muscle aches or changes in liver tests.
Mycophenolate mofetil: An immune-suppressing option when steroids aren’t tolerated or effective. It may reduce relapses, but requires blood test monitoring and reliable contraception due to pregnancy risks.
ACTH gel (repository corticotropin): An injection sometimes used when other therapies fail or aren’t tolerated. It can lower proteinuria in select cases, with side effects similar to steroids such as weight gain, mood changes, and high blood pressure.
Genetic Influences
In some families, FSGS appears in siblings or across generations, pointing to a genetic factor. It’s natural to ask whether family history plays a role. Certain gene changes that affect the kidney’s filtering cells (podocytes) can lead to familial FSGS, and a variant in a gene called APOL1 raises risk in people with African or Caribbean ancestry; even so, many who carry these changes never develop kidney disease. Some inherited types show up in childhood and may be harder to treat, while others begin in adulthood—so age of onset and severity can vary widely, even within one family. Many cases of focal segmental glomerulosclerosis are not inherited and relate to other causes, such as immune or medication triggers. Genetic testing and counseling may be suggested if focal segmental glomerulosclerosis starts early, is difficult to treat, or if several relatives are affected, and results can inform care decisions and transplant planning. While testing can’t forecast the early symptoms of FSGS or predict the exact course, it can clarify personal risk and what it may mean for close relatives.
How genes can cause diseases
Humans have more than 20 000 genes, each carrying out one or a few specific functiosn in the body. One gene instructs the body to digest lactose from milk, another tells the body how to build strong bones and another prevents the bodies cells to begin lultiplying uncontrollably and develop into cancer. As all of these genes combined are the building instructions for our body, a defect in one of these genes can have severe health consequences.
Through decades of genetic research, we know the genetic code of any healthy/functional human gene. We have also identified, that in certain positions on a gene, some individuals may have a different genetic letter from the one you have. We call this hotspots “Genetic Variations” or “Variants” in short. In many cases, studies have been able to show, that having the genetic Letter “G” in the position makes you healthy, but heaving the Letter “A” in the same position disrupts the gene function and causes a disease. Genopedia allows you to view these variants in genes and summarizes all that we know from scientific research, which genetic letters (Genotype) have good or bad consequences on your health or on your traits.
Pharmacogenetics — how genetics influence drug effects
Genes can influence both how focal segmental glomerulosclerosis (FSGS) behaves and how medicines work in your body. When testing shows a single-gene cause affecting the kidney’s filters, FSGS usually doesn’t respond to steroids or other immune-suppressing drugs, so care often focuses on kidney-protective steps, blood pressure control, and planning for transplant if needed. Primary (non-genetic) FSGS is more likely to respond to medicines like steroids or calcineurin inhibitors; by contrast, genetic FSGS rarely comes back after a kidney transplant, while primary FSGS has a higher risk of recurrence, which shapes how doctors prepare and monitor. Alongside your medical history and biopsy findings, genetic testing can help match the treatment plan to the type of FSGS you have and may spare you from medicines unlikely to help.
Genes can also affect how you process certain drugs used for FSGS. If tacrolimus is part of treatment or given after transplant, common differences in a liver-enzyme gene can change how much drug you need; some centers use genotyping to help choose the starting dose and reach a safe level sooner. If azathioprine is considered, variants in enzyme genes that break down this drug can raise the risk of low white blood cells, so pre-treatment testing is often used to reduce side effects. In people of African ancestry, high-risk forms of a gene called APOL1 are linked with more aggressive FSGS and may influence response to therapy, and these results are also considered when evaluating living kidney donors.
Interactions with other diseases
Living with focal segmental glomerulosclerosis (FSGS) often means watching how other health issues affect your kidneys day to day. High blood pressure and diabetes can speed kidney damage, while conditions like HIV, hepatitis B, obesity, and sickle cell disease can trigger or worsen certain types of FSGS. Because swelling in the legs, shortness of breath, and tiredness can mimic early symptoms of focal segmental glomerulosclerosis, heart or liver problems may be confused with kidney trouble and delay the right care. People with FSGS also face higher risks tied to the nephrotic state—high cholesterol, blood clots, and infections—which adds to overall heart and vascular risk if another illness is present. Some cancer drugs, immune therapies, and pain relievers can strain the kidneys or interact with FSGS treatments, so plans often need careful coordination between specialists. Ask if any medications for one condition might interfere with treatment for another.
Special life conditions
Pregnancy with focal segmental glomerulosclerosis (FSGS) needs close, shared care between kidney and obstetric teams. Blood pressure can rise, swelling may worsen, and protein in the urine often increases; some will see temporary declines in kidney function. Doctors may suggest closer monitoring during pregnancy and the weeks after birth, with careful choices about safe blood pressure medicines and whether to adjust steroids or other treatments.
Children with FSGS can have puffiness around the eyes in the morning, frothy urine, and slowed growth if protein loss is heavy. Kidney biopsies and response to medicines can differ by age, and some children experience relapses tied to infections or missed doses, so school and sports plans may need flexibility. Teens may also need support with adherence and body-image concerns from swelling.
Older adults living with FSGS often have other health conditions, like diabetes or heart disease, that shape treatment choices and side effects. Medication doses may be adjusted more conservatively, and fall risk from diuretics or blood pressure drops is watched closely. For many, this can mean balancing kidney protection with maintaining energy and independence.
Active athletes can stay engaged, but intense training may need tweaks if swelling, fatigue, or blood pressure issues flare. Hydration, salt intake, and safe use of anti-inflammatory pain relievers deserve special attention because some pain medicines can strain the kidneys. It helps to look ahead and prepare for tournaments, travel, or heat exposure with your care team’s guidance.
History
Throughout history, people have described swelling of the legs, puffy eyelids, and unusually foamy urine—signs we now recognize as heavy protein loss from the kidneys. Long before kidney biopsies, families and healers noticed that some relatives seemed prone to these episodes after illness or pregnancy, while others recovered and then relapsed years later. Early physicians grouped these stories under “dropsy” or “nephrosis,” because the exact cause inside the kidney wasn’t clear.
From early theories to modern research, the story of focal segmental glomerulosclerosis (FSGS) took shape as tools improved. In the mid-20th century, the introduction of the kidney biopsy and light microscopy let clinicians see scarring in only parts of some filters—the “focal” and “segmental” pattern that defines FSGS. Electron microscopy added finer detail, showing injury to the delicate foot processes that help keep protein in the bloodstream. Initially understood only through symptoms, later the structural pattern on biopsy distinguished FSGS from other causes of nephrotic syndrome, like minimal change disease.
With each decade, descriptions became more precise. Pathologists outlined subtypes based on how the scarring looked and where it appeared in the kidney filter. Around the same time, doctors noticed that FSGS could appear after certain viral infections, medication exposures, or severe stress on the kidneys, such as very low birth weight or reduced kidney mass. Reports also documented that FSGS can recur after a kidney transplant, highlighting that in some people circulating factors in the blood may drive the condition.
Advances in genetics reshaped understanding again. In the late 1990s and 2000s, researchers identified changes in genes that act like dimmer switches for podocyte function—the specialized cells that line the kidney filters. Variants in genes such as NPHS2 (podocin), ACTN4, TRPC6, INF2, and APOL1 (risk variants common in people with West African ancestry) helped explain why FSGS can run in families, start in childhood for some, or respond differently to treatment for others. These discoveries did not replace biopsy, but they added another layer: FSGS describes a pattern of scarring that can arise from different root causes.
In recent decades, awareness has grown that FSGS is not a single disease but a final common pathway. This shift matters for care. It explains why two people with the same biopsy pattern may have different early symptoms of FSGS, different triggers, and different responses to therapy. It also guided clinical trials toward more tailored approaches—separating presumed immune-related FSGS from genetic or secondary forms—while improving supportive care like blood pressure control and kidney-protective medications.
Looking back helps explain today’s approach: combine careful history, lab tests, and biopsy findings with targeted genetic testing when appropriate. Over time, the way the condition has been understood has changed, yet those early observations about swelling and protein loss still anchor how clinicians recognize FSGS in everyday practice.