Ogniskowe segmentowe stwardnienie kłębuszków nerkowych
Objawy

Ogniskowe segmentowe stwardnienie kłębuszków nerkowych (FSGS) to przewlekła choroba nerek charakteryzująca się ogniskowym i segmentowym tworzeniem blizn w kłębuszkach nerkowych, prowadzącym do upośledzenia funkcji filtracyjnej. Wczesne objawy obejmują obrzęki, proteinurię (często >3,5 g/dobę), nadciśnienie tętnicze oraz piankowaty mocz. FSGS jest jedną z głównych przyczyn zespołu nerczycowego, który manifestuje się masywną proteinurią (>3,5 g/dobę), hipoalbuminemią (<3,5 g/dl), hipercholesterolemią oraz uogólnionymi obrzękami. Diagnostyka opiera się na badaniach laboratoryjnych i potwierdzeniu zmian histopatologicznych w biopsji nerki. Przebieg choroby jest zróżnicowany, a czynniki prognostyczne obejmują stopień proteinurii, wariant histologiczny (np. kolapsowy z najgorszym rokowaniem), odpowiedź na leczenie oraz obecność zwłóknienia śródmiąższowego.

Objawy ogniskowego segmentowego stwardnienia kłębuszków nerkowych (FSGS)

Ogniskowe segmentowe stwardnienie kłębuszków nerkowych (FSGS) to choroba nerek charakteryzująca się tworzeniem się blizn (stwardnień) w kłębuszkach nerkowych, które są małymi jednostkami filtracyjnymi nerek odpowiedzialnymi za usuwanie produktów przemiany materii z krwi. We wczesnych stadiach choroby blizny występują tylko w niektórych (ogniskowo) fragmentach (segmentowo) kłębuszków nerkowych, jednak z czasem mogą obejmować coraz większą część tkanki nerkowej12.

Wczesne objawy FSGS

We wczesnych stadiach FSGS często nie powoduje żadnych zauważalnych objawów. Niektórzy pacjenci mogą nie wykazywać żadnych symptomów przez dłuższy czas, a choroba może zostać wykryta przypadkowo podczas rutynowych badań lekarskich lub podczas diagnozowania innych schorzeń34. Wczesne objawy, które mogą wystąpić, obejmują:

  • Obrzęki (edema) – szczególnie widoczne wokół oczu, w nogach, kostkach i stopach, spowodowane zatrzymywaniem płynów w organizmie5
  • Przyrost masy ciała związany z zatrzymaniem płynów6
  • Piankowaty lub pienisty mocz – spowodowany obecnością nadmiernej ilości białka w moczu (proteinuria)7
  • Podwyższone ciśnienie tętnicze (nadciśnienie) – często trudne do kontrolowania za pomocą standardowych metod leczenia8

Zaawansowane objawy FSGS

Wraz z postępem choroby, objawy stają się bardziej wyraźne i mogą obejmować910:

  • Nasilone obrzęki – obejmujące twarz, ręce, nogi, stopy i brzuch
  • Zmęczenie i osłabienie
  • Utrata apetytu
  • Bóle głowy
  • Świąd skóry
  • Duszność
  • Nudności i wymioty
  • Zaburzenia snu11

Zespół nerczycowy jako objaw FSGS

FSGS jest jedną z głównych przyczyn zespołu nerczycowego, który występuje u ponad 70% pacjentów z FSGS1213. Zespół nerczycowy charakteryzuje się zestawem objawów związanych z uszkodzeniem kłębuszków nerkowych i utratą białka przez nerki:

  • Masywna proteinuria – wydalanie ponad 3,5 g białka na dobę w moczu
  • Hipoalbuminemia – niski poziom albumin we krwi (poniżej 3,5 g/dl)
  • Hipercholesterolemia – podwyższony poziom cholesterolu we krwi
  • Obrzęki – uogólnione lub zależne od ułożenia ciała
  • Zwiększona podatność na infekcje – z powodu utraty immunoglobulin z moczem14

Diagnostyka laboratoryjna FSGS

Diagnostyka FSGS opiera się na wynikach badań laboratoryjnych, które mogą wykazać charakterystyczne nieprawidłowości1516:

  • Proteinuria – obecność zwiększonej ilości białka w moczu, często w zakresie nerczycowym (powyżej 3,5 g/dobę)
  • Podwyższony poziom kreatyniny w surowicy – wskazujący na upośledzenie funkcji filtracyjnej nerek
  • Obniżony poziom albumin w surowicy krwi
  • Podwyższony poziom lipidów we krwi – hipercholesterolemia i hipertriglicerydemia
  • Mikroskopowy krwiomocz – może występować u niektórych pacjentów17

Jedynym definitywnym sposobem diagnozy FSGS jest biopsja nerki, która wykazuje charakterystyczne zmiany histopatologiczne18.

Progresja FSGS

Naturalny przebieg choroby

FSGS jest chorobą przewlekłą, a jej przebieg może być bardzo zróżnicowany. Czynniki takie jak stopień proteinurii, wstępna odpowiedź na leczenie, wariant histologiczny i współistniejące choroby wpływają na tempo progresji1920. Przebieg choroby może obejmować:

  • Stabilizację – u niektórych pacjentów choroba może pozostawać stabilna przez wiele lat
  • Remisję – u około 10% pacjentów może nastąpić spontaniczna remisja
  • Powolną progresję – stopniowe pogarszanie się funkcji nerek
  • Szybką progresję – prowadzącą do niewydolności nerek w krótkim czasie21

Czynniki wpływające na progresję

Kilka czynników ma istotny wpływ na tempo progresji FSGS2223:

  • Stopień proteinurii – pacjenci z proteinurią w zakresie nerczycowym (>3,5 g/dobę) mają ponad 50% ryzyko progresji do schyłkowej niewydolności nerek w ciągu 10 lat, podczas gdy tylko 15% pacjentów z proteinurią w zakresie nienerczcyowym rozwija niewydolność nerek w tym samym okresie
  • Odpowiedź na leczenie – pacjenci, którzy osiągają całkowitą lub częściową remisję (zmniejszenie proteinurii), mają znacznie lepsze rokowanie
  • Wariant histologiczny – wariant kolapsowy FSGS charakteryzuje się najgorszym rokowaniem i szybką progresją, podczas gdy wariant wierzchołkowy (tip lesion) ma lepsze rokowanie24
  • Czynniki demograficzne – choroba ma gorsze rokowanie u pacjentów rasy czarnej w porównaniu do pacjentów rasy białej, prawdopodobnie z powodu wariantów genu APOL125
  • Zwłóknienie śródmiąższowe – obecność zwłóknienia śródmiąższowego w biopsji nerki jest czynnikiem prognostycznym gorszego rokowania26

Tempo progresji do niewydolności nerek

U pacjentów niereagujących na leczenie średni czas od wystąpienia masywnej proteinurii do rozwoju schyłkowej niewydolności nerek (ESRD) wynosi 6-8 lat, choć obserwuje się duże różnice w przebiegu choroby2728. Tempo progresji zależy od kilku czynników:

  • U pacjentów z proteinurią w zakresie nerczycowym (>3,5 g/dobę), którzy nie osiągają remisji, około 50% rozwinie niewydolność nerek w ciągu 3-8 lat od diagnozy FSGS29
  • U pacjentów z masywną proteinurią (>10-15 g/dobę) funkcja nerek pogarsza się jeszcze szybciej (w ciągu 2-3 lat)30
  • W wariancie kolapsowym FSGS choroba charakteryzuje się ciężkim nadciśnieniem, masywną proteinurią, bardzo słabą odpowiedzią na kortykosteroidy i znacznie szybszym tempem progresji do niewydolności nerek31

Powikłania FSGS

FSGS może prowadzić do różnych powikłań zdrowotnych, które wpływają na jakość życia i rokowanie pacjentów3233:

Powikłania nerkowe

  • Przewlekła choroba nerek (PChN) – postępujące pogorszenie funkcji nerek
  • Schyłkowa niewydolność nerek (ESRD) – całkowita lub prawie całkowita utrata funkcji nerek wymagająca dializoterapii lub przeszczepu nerki34
  • Nawrót choroby po przeszczepie nerki – u 30-40% pacjentów FSGS może nawrócić w przeszczepionej nerce, czasami już w kilka dni po przeszczepie3536

Powikłania pozanerkowe

  • Nadciśnienie tętnicze – często trudne do kontrolowania
  • Hipercholesterolemia – zwiększająca ryzyko chorób sercowo-naczyniowych
  • Zakrzepica – zwiększone ryzyko zakrzepów z powodu utraty antytrombiny III z moczem
  • Niedoczynność tarczycy – z powodu utraty hormonów tarczycy przez nerki37
  • Zwiększona podatność na infekcje – z powodu utraty immunoglobulin
  • Niedokrwistość – związana z przewlekłą chorobą nerek38

Rokowanie w FSGS

Rokowanie w FSGS jest zróżnicowane i zależy od wielu czynników3940:

Odpowiedź na leczenie

Osiągnięcie remisji, czy to spontanicznej czy indukowanej leczeniem, wiąże się z korzystnym rokowaniem nerkowym41:

  • Całkowita remisja (proteinuria <300 mg/dobę) lub częściowa remisja (proteinuria <3,5 g/dobę) wiąże się z 80% przeżyciem nerek w ciągu 10 lat
  • U pacjentów niereagujących na leczenie przeżycie nerek wynosi około 50% w ciągu 10 lat42

Warianty histologiczne

Różne warianty histologiczne FSGS wiążą się z różnym rokowaniem43:

  • Wariant wierzchołkowy (tip lesion) – najlepsze rokowanie, większa szansa na osiągnięcie remisji
  • Wariant nieokreślony (NOS) – pośrednie rokowanie
  • Wariant kolapsowy – najgorsze rokowanie, szybka progresja do niewydolności nerek44

Czynniki demograficzne i kliniczne

  • Wiek – choroba ma często łagodniejszy przebieg u dzieci niż u dorosłych45
  • Rasa – gorsze rokowanie u pacjentów rasy czarnej
  • Poziom proteinurii – wyższy poziom proteinurii wiąże się z gorszym rokowaniem
  • Funkcja nerek w momencie diagnozy – gorsza funkcja nerek na początku choroby wiąże się z szybszą progresją
  • Zwłóknienie śródmiąższowe w biopsji – silny czynnik prognostyczny gorszego rokowania46

Jakość życia pacjentów z FSGS

FSGS może znacząco wpływać na jakość życia pacjentów w różnych obszarach4748:

Wpływ fizyczny

  • Zmęczenie i brak energii – utrudniające wykonywanie codziennych czynności
  • Obrzęki – powodujące dyskomfort i utrudniające poruszanie się
  • Ból i dyskomfort – związane z obrzękami i innymi objawami
  • Problemy ze snem – spowodowane objawami fizycznymi i psychologicznymi

Wpływ psychologiczny

  • Depresja i lęk – związane z przewlekłą chorobą i niepewnością co do przyszłości
  • Poczucie beznadziejności – szczególnie u pacjentów niereagujących na leczenie
  • Stygmatyzacja – związana ze zmianami w wyglądzie (obrzęki, przyrost masy ciała)
  • Stres – związany z leczeniem i koniecznością regularnych wizyt lekarskich49

Wpływ społeczny

  • Ograniczenia w aktywności zawodowej – związane z objawami i koniecznością leczenia
  • Ograniczenia w życiu towarzyskim – spowodowane zmęczeniem, obrzękami i innymi objawami
  • Obciążenie finansowe – związane z kosztami leczenia i potencjalną niezdolnością do pracy
  • Zależność od innych – w przypadku zaawansowanej choroby i leczenia nerkozastępczego50

Osoby z FSGS muszą być regularnie monitorowane przez specjalistów, aby ocenić funkcję nerek i dostosować leczenie. Mimo że FSGS jest chorobą przewlekłą i nie można naprawić uszkodzonych kłębuszków nerkowych, odpowiednie leczenie może spowolnić progresję choroby i poprawić jakość życia pacjentów5152.

Kolejne rozdziały

Zapraszamy do dalszego czytania naszego leksykonu.

Wybierz kolejny rozdział z menu poniżej, aby otworzyć nową podstronę kompedium wiedzy i uzyskać szczegółowe informację o leku, substancji lub chorobie.

  1. 09.04.2026
  2. www.leksykon.com.pl

Materiały źródłowe

  • #1 Focal segmental glomerulosclerosis (FSGS) – Symptoms and causes – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/fsgs/symptoms-causes/syc-20354693
    Focal segmental glomerulosclerosis (FSGS) results from scar tissue that develops in the glomeruli. Glomeruli are tiny structures within the kidney that filter waste products from the blood to create urine. A healthy glomerulus is shown on the left. When scar tissue develops in a glomerulus, kidney function gets worse (shown right). […] FSGS is a serious condition that can lead to kidney failure, which can only be treated with dialysis or kidney transplant. Treatment options for FSGS depend on the type you have. […] Symptoms of focal segmental glomerulosclerosis (FSGS) might include: Swelling, called edema, in the legs and ankles, around the eyes and in other body parts. Weight gain from fluid buildup. Foamy urine from protein buildup, called proteinuria. […] Focal segmental glomerulosclerosis (FSGS) may lead to other health concerns, also called complications, including: Nephrotic syndrome. This kidney condition causes the body to pass too much protein in the urine. Nephrotic syndrome raises the risk of other health conditions, such as blood clots and high blood pressure. Kidney failure. Damage to the kidneys that can’t be fixed causes the kidneys to stop working. The only treatments for kidney failure are dialysis or kidney transplant.
  • #2 Focal Segmental Glomerulosclerosis: Symptoms & Treatment
    https://my.clevelandclinic.org/health/diseases/21149-focal-segmental-glomerulosclerosis-fsgs
    Focal segmental glomerulosclerosis (FSGS) is a rare disease that affects the filters in your kidneys. When these filters are scarred, they cant remove waste from your blood, which can lead to kidney damage and failure. Treatment for FSGS manages symptoms and prevents the disease from worsening. […] FSGS happens when small parts or sections of these tiny filters scar or harden (sclerosis). Of the damaged glomeruli, only some show scarring. When this scarring happens, its hard for your kidneys to act like a strainer. This can lead to kidney damage and, potentially, kidney failure. Treatment depends on what type of FSGS you have. […] FSGS doesnt always cause symptoms you might notice on your own. Healthcare providers often find signs or symptoms during a routine exam or when testing for other medical conditions. Some of these signs and symptoms include: Edema (swelling in your arms, legs or face). High cholesterol. Lower-than-average amounts of albumin (a protein in your blood). Proteinuria (abnormally high levels of protein in your pee). Sudden weight gain caused by extra fluids in your body. High blood pressure (hypertension).
  • #3 Focal segmental glomerulosclerosis (FSGS) – Symptoms, causes, treatment | National Kidney Foundation
    https://www.kidney.org/kidney-topics/focal-segmental-glomerulosclerosis-fsgs
    What are the signs and symptoms of FSGS? Early stages may not cause any symptoms. You may only see some signs on your own, while others may be found by your healthcare provider. Signs and symptoms of FSGS include: Swelling in body parts like your legs, ankles and around your eyes (called edema) […] FSGS can cause nephrotic syndrome. Nephrotic Syndrome: A set of symptoms that happen together and affect your kidneys. These include: Swelling in body parts like your legs, ankles, or around your eyes (edema) […] If the condition is advanced, the symptoms may be like those of kidney failure. People may report fatigue, a poor appetite, headache, itchy skin, shortness of breath and/or nausea. […] You should talk with your doctor about your condition because the progression of the disease depends on many factors. FSGS is a chronic disease, because the scarred glomeruli cannot be repaired. Treatment can slow the process of kidney disease. Everyone is different in how they respond to treatment. Over time, some patients with FSGS gradually get worse until they reach kidney failure. If this occurs, they will need a kidney transplant or dialysis to stay alive. Some people respond well to treatment and may live with the disease for many years while being monitored for any signs of change.
  • #4 Focal Segmental Glomerulosclerosis: Symptoms & Treatment
    https://my.clevelandclinic.org/health/diseases/21149-focal-segmental-glomerulosclerosis-fsgs
    Focal segmental glomerulosclerosis (FSGS) is a rare disease that affects the filters in your kidneys. When these filters are scarred, they cant remove waste from your blood, which can lead to kidney damage and failure. Treatment for FSGS manages symptoms and prevents the disease from worsening. […] FSGS happens when small parts or sections of these tiny filters scar or harden (sclerosis). Of the damaged glomeruli, only some show scarring. When this scarring happens, its hard for your kidneys to act like a strainer. This can lead to kidney damage and, potentially, kidney failure. Treatment depends on what type of FSGS you have. […] FSGS doesnt always cause symptoms you might notice on your own. Healthcare providers often find signs or symptoms during a routine exam or when testing for other medical conditions. Some of these signs and symptoms include: Edema (swelling in your arms, legs or face). High cholesterol. Lower-than-average amounts of albumin (a protein in your blood). Proteinuria (abnormally high levels of protein in your pee). Sudden weight gain caused by extra fluids in your body. High blood pressure (hypertension).
  • #5 Focal segmental glomerulosclerosis (FSGS) – Symptoms and causes – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/fsgs/symptoms-causes/syc-20354693
    Focal segmental glomerulosclerosis (FSGS) results from scar tissue that develops in the glomeruli. Glomeruli are tiny structures within the kidney that filter waste products from the blood to create urine. A healthy glomerulus is shown on the left. When scar tissue develops in a glomerulus, kidney function gets worse (shown right). […] FSGS is a serious condition that can lead to kidney failure, which can only be treated with dialysis or kidney transplant. Treatment options for FSGS depend on the type you have. […] Symptoms of focal segmental glomerulosclerosis (FSGS) might include: Swelling, called edema, in the legs and ankles, around the eyes and in other body parts. Weight gain from fluid buildup. Foamy urine from protein buildup, called proteinuria. […] Focal segmental glomerulosclerosis (FSGS) may lead to other health concerns, also called complications, including: Nephrotic syndrome. This kidney condition causes the body to pass too much protein in the urine. Nephrotic syndrome raises the risk of other health conditions, such as blood clots and high blood pressure. Kidney failure. Damage to the kidneys that can’t be fixed causes the kidneys to stop working. The only treatments for kidney failure are dialysis or kidney transplant.
  • #6 Focal segmental glomerulosclerosis (FSGS)
    https://www.kidneyfund.org/all-about-kidneys/other-kidney-diseases/focal-segmental-glomerulosclerosis-fsgs
    Focal segmental glomerulosclerosis (FSGS) is a rare type of kidney disease that causes scarring in some parts of the kidney filters, known as glomeruli. FSGS can make it hard for your kidneys to filter waste, which can lead to kidney failure, also called end-stage renal disease (ESRD) or end-stage kidney disease (ESKD). […] In early stages of FSGS, you may not notice any symptoms. As FSGS gets worse, you may start to notice symptoms like swelling in your legs or weight gain. Other symptoms will only be found by your doctor through tests, such as: Protein in your urine (pee), called proteinuria, Low levels of protein in your blood, Too much fat in your blood (high cholesterol). […] FSGS can lead to nephrotic syndrome. Nephrotic syndrome is a group of symptoms that may happen together, which can mean that your kidneys are not working as well as they should. Signs and symptoms of nephrotic syndrome include: Severe swelling around eyes and in your ankles and feet, Foamy urine from protein in the urine, Weight gain from fluid retention, Fatigue.
  • #7 Focal segmental glomerulosclerosis (FSGS): symptoms and treatments
    https://www.kidneyresearchuk.org/conditions-symptoms/focal-and-segmental-glomerulosclerosis/
    Some people may have no visible symptoms and may only discover that they have higher than normal amounts of protein in their urine (proteinuria) after a routine urine test. In some cases, levels of protein loss and fluid retention can be so high that it causes puffiness (oedema) around the eyes, abdomen, feet and legs, and weight gain – nephrotic syndrome. Urine can also appear foamy. If kidney failure is already well advanced symptoms could include tiredness and lack of energy, itching and muscle cramps. Some people with FSGS may experience: High blood pressure – which needs to be controlled to protect the kidneys from further damage and to reduce the risk of heart disease and stroke. High cholesterol levels – which may need to be controlled with a special diet and cholesterol lowering drugs. Low thyroid hormone levels – due to loss of thyroid hormone via the kidneys. This can be controlled by thyroid hormone supplements.
  • #8 Focal Segmental Glomerulosclerosis (FSGS) | UNC Kidney Center
    https://unckidneycenter.org/kidneyhealthlibrary/glomerular-disease/focal-segmental-glomerulosclerosis-fsgs/
    Many people with FSGS have no symptoms at all. The most common complaint is edema, or swelling, especially in the legs. Often patients find that their shoes no longer fit, or that they have suddenly put on weight. High blood pressure, or hypertension, is also a very common finding. The hypertension in people with FSGS can often be very difficult to treat. […] FSGS can also cause abnormal results in two different routine laboratory tests ordered by your doctor. The first is a test of kidney function called creatinine. It is measured by taking a blood sample. Everyone has a certain amount of a substance called creatinine floating in his or her blood. This substance is always being produced by healthy muscles, but since the kidneys constantly filter it out the level of creatinine stays low. When the filters become damaged, the level of creatinine left in the blood goes up. A higher level of creatinine alerts the doctor that the kidneys aren’t filtering as well as they should.
  • #9 Focal segmental glomerulosclerosis (FSGS) – Symptoms, causes, treatment | National Kidney Foundation
    https://www.kidney.org/kidney-topics/focal-segmental-glomerulosclerosis-fsgs
    What are the signs and symptoms of FSGS? Early stages may not cause any symptoms. You may only see some signs on your own, while others may be found by your healthcare provider. Signs and symptoms of FSGS include: Swelling in body parts like your legs, ankles and around your eyes (called edema) […] FSGS can cause nephrotic syndrome. Nephrotic Syndrome: A set of symptoms that happen together and affect your kidneys. These include: Swelling in body parts like your legs, ankles, or around your eyes (edema) […] If the condition is advanced, the symptoms may be like those of kidney failure. People may report fatigue, a poor appetite, headache, itchy skin, shortness of breath and/or nausea. […] You should talk with your doctor about your condition because the progression of the disease depends on many factors. FSGS is a chronic disease, because the scarred glomeruli cannot be repaired. Treatment can slow the process of kidney disease. Everyone is different in how they respond to treatment. Over time, some patients with FSGS gradually get worse until they reach kidney failure. If this occurs, they will need a kidney transplant or dialysis to stay alive. Some people respond well to treatment and may live with the disease for many years while being monitored for any signs of change.
  • #10 Focal Segmental Glomerulosclerosis: Symptoms & Treatment
    https://my.clevelandclinic.org/health/diseases/21149-focal-segmental-glomerulosclerosis-fsgs
    FSGS can lead to high blood pressure and high cholesterol. In some cases, FSGS can lead to kidney failure. This condition can be life-threatening and requires emergency medical treatment. Seek immediate care if you have FSGS and experience any of these symptoms of kidney failure: Unmanaged high blood pressure. Anemia. Nausea, vomiting or loss of appetite. Swelling in your arms, legs or face. […] Treatment for FSGS depends on the type and cause, your age and whether you have other health conditions. The goal of treatment is to manage your symptoms to help you maintain a good quality of life and slow scarring so that it doesnt lead to kidney failure. […] FSGS is a chronic disease that cant be undone or reversed. This means treatment can only slow the progression of kidney disease, not cure you from the disease.
  • #11
    https://link.springer.com/article/10.1007/s12325-023-02651-6
    Focal segmental glomerulosclerosis (FSGS) is a leading cause of kidney disease and can progress to end stage kidney disease (ESKD). […] Salient sign/symptom/side-effect domains included swelling/puffiness (edema), pain/aches/discomfort, fatigue, weight changes, skin problems, respiratory problems, and sleep problems. […] Patients report impairment in aspects of daily life and emotional wellbeing. […] Symptoms such as swelling, fatigue, and nausea limited patients energy and caused pain, which made it difficult to complete household chores. […] Patients reported that FSGS significantly impacted their daily lives. […] The overall experience of living with FSGS could also cause feelings of depression, hopelessness, and sadness, often related to swelling and weight gain, or because FSGS symptoms or treatment side effects prevented them from doing the things they wanted to do.
  • #12 Focal Segmental Glomerulosclerosis – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/sites/books/NBK532272/
    Focal segmental glomerulosclerosis (FSGS) is histologically characterized by segmental scarring that affects only a portion of the glomerulus and involves some, but not all, glomeruli in a biopsy sample. […] Patients with FSGS may present with clinical features of nephrotic or nephritic syndrome or renal insufficiency. Nephrotic syndrome is the most common clinical manifestation of FSGS, occurring in over 70% of patients. This condition is characterized by generalized or dependent edema, fatigue, and appetite loss. Hypertension is also a common feature and can be severe, with diastolic blood pressure exceeding 120 mm Hg. […] The most common clinical manifestation of FSGS, observed in over 70% of patients, is nephrotic syndrome, characterized by generalized or dependent edema, fatigue, and loss of appetite. Hypertension is also common and can be severe, with diastolic blood pressure exceeding 120 mm Hg, particularly in patients of Afro-Caribbean origin.
  • #13 FSGS – RKD – About the Disease & Find Resources
    https://travere.com/our-science/therapeutic-areas/focal-segmental-glomerulosclerosis-fsgs/
    FSGS is often an aggressive, progressive condition. As more podocytes become damaged or lost, proteinuria can reach a nephrotic range (3 grams or more per day). More than 70% of newly diagnosed adult patients with primary FSGS have proteinuria in the nephrotic range. […] Further disease progression can cause nephrotic syndrome. Nephrotic syndrome refers to a group of symptoms associated with kidney damage including, proteinuria of ≥3.5 g/d, low levels of blood albumin (≤3.5 g/dL), with or without edema. […] In FSGS, proteinuria is a standard measure of disease activity, and a predictor and contributor to disease progression.
  • #14 Focal segmental glomerulosclerosis – Wikipedia
    https://en.wikipedia.org/wiki/Focal_segmental_glomerulosclerosis
    Focal segmental glomerulosclerosis (FSGS) is a histopathologic finding of scarring (sclerosis) of glomeruli and damage to renal podocytes. This process damages the filtration function of the kidney, resulting in protein presence in the urine due to protein loss. FSGS is a leading cause of excess protein loss nephrotic syndrome in children and adults in the US. Signs and symptoms include proteinuria and edema. Kidney failure is a common long-term complication of the disease. […] The most common symptoms are a result of abnormal loss of protein from the glomerulus of the kidney, and include: Frothy urine (due to excess protein), Excess water retention (pitting edema, due to loss of serum albumin), Susceptibility to infection (due to loss of serum antibodies). Common signs are also due to loss of blood proteins by the glomerulus of the kidney, including: Protein in the urine (often in the nephrotic syndrome-range of 3.5 g/day), Low serum albumin (3.5 g/dl), Low serum antibodies, High serum cholesterol (compensatory by the liver to compensate for low serum oncotic pressure), Fatty casts in the urine (secondary to hypercholesterolemia).
  • #15 Focal Segmental Glomerulosclerosis (FSGS) | UNC Kidney Center
    https://unckidneycenter.org/kidneyhealthlibrary/glomerular-disease/focal-segmental-glomerulosclerosis-fsgs/
    Many people with FSGS have no symptoms at all. The most common complaint is edema, or swelling, especially in the legs. Often patients find that their shoes no longer fit, or that they have suddenly put on weight. High blood pressure, or hypertension, is also a very common finding. The hypertension in people with FSGS can often be very difficult to treat. […] FSGS can also cause abnormal results in two different routine laboratory tests ordered by your doctor. The first is a test of kidney function called creatinine. It is measured by taking a blood sample. Everyone has a certain amount of a substance called creatinine floating in his or her blood. This substance is always being produced by healthy muscles, but since the kidneys constantly filter it out the level of creatinine stays low. When the filters become damaged, the level of creatinine left in the blood goes up. A higher level of creatinine alerts the doctor that the kidneys aren’t filtering as well as they should.
  • #16 Focal Segmental Glomerulosclerosis (FSGS) | UNC Kidney Center
    https://unckidneycenter.org/kidneyhealthlibrary/glomerular-disease/focal-segmental-glomerulosclerosis-fsgs/
    The second test is for protein in your urine. Urine normally contains only a very small amount of protein. It is just too big to pass through the filters. But when the filters become scarred or damaged, they can leak protein into the urine. If a doctor finds too much protein in your urine, it usually means something is wrong with the filters. Some people with a large amount of protein in their urine say that their urine looks foamy, like bubbles built up in dishwater. […] None of the above symptoms, or even all of them together, is specific for FSGS. If you or your doctor are concerned about FSGS, the only way to know for sure is to have a kidney biopsy. […] If not treated, most patients with FSGS will eventually develop complete renal failure and require dialysis or a kidney transplant to survive. Even with treatment, many patients will still eventually require dialysis. How long this will take varies widely (2-20 years), and is difficult to predict. The appearance of your kidney biopsy under the microscope may give your doctor some idea of the time frame. Specific forms of FSGS have been associated with different rates of progression and responsiveness to therapy.
  • #17 Focal Segmental Glomerulosclerosis Clinical Presentation: History, Physical Examination
    https://emedicine.medscape.com/article/245915-clinical
    Patients with focal segmental glomerulosclerosis (FSGS) typically present with signs and symptoms of nephrotic syndrome. In children, this usually begins with facial swelling, followed by swelling of the entire body. Adults can present with dependent edema. Typically, edema develops over a few weeks, but the onset may be abrupt, with weight gain of 15-20 lb or more. Frequently, the onset of edema follows a recent upper respiratory tract infection. Foamy urine may be noted. Fatigue and loss of appetite are common. […] The most common clinical presenting feature, found in more than 70% of patients, is nephrotic syndrome, which is characterized by generalized or dependent edema, massive proteinuria, hypoalbuminemia, and hyperlipidemia. […] Less than a third of patients with FSGS present with nonnephrotic proteinuria along with microscopic hematuria and hypertension.
  • #18 Focal Segmental Glomerulosclerosis (FSGS) | UNC Kidney Center
    https://unckidneycenter.org/kidneyhealthlibrary/glomerular-disease/focal-segmental-glomerulosclerosis-fsgs/
    The second test is for protein in your urine. Urine normally contains only a very small amount of protein. It is just too big to pass through the filters. But when the filters become scarred or damaged, they can leak protein into the urine. If a doctor finds too much protein in your urine, it usually means something is wrong with the filters. Some people with a large amount of protein in their urine say that their urine looks foamy, like bubbles built up in dishwater. […] None of the above symptoms, or even all of them together, is specific for FSGS. If you or your doctor are concerned about FSGS, the only way to know for sure is to have a kidney biopsy. […] If not treated, most patients with FSGS will eventually develop complete renal failure and require dialysis or a kidney transplant to survive. Even with treatment, many patients will still eventually require dialysis. How long this will take varies widely (2-20 years), and is difficult to predict. The appearance of your kidney biopsy under the microscope may give your doctor some idea of the time frame. Specific forms of FSGS have been associated with different rates of progression and responsiveness to therapy.
  • #19 Focal Segmental Glomerulosclerosis – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/sites/books/NBK532272/
    Approximately 50% of patients with persistent nephrotic-range proteinuria progress to ESRD within 3 to 8 years of an FSGS diagnosis. […] The natural history of FSGS varies significantly. It is characterized by edema, proteinuria that is often refractory to steroids or other immunosuppressive treatments, worsening hypertension, and progressively declining renal function. In cases that are unresponsive to treatment, the average time between the onset of gross proteinuria and progression to ESRD is 6 to 8 years, although variations may exist in the course of the disease. Achieving remission, whether spontaneous or induced, is associated with favorable renal outcomes. […] The prognosis is significantly worse in the Black population compared to White patients, likely due to APOL1 gene variants. The collapsing form of FSGS is characterized by severe hypertension, a poor response to corticosteroids, massive proteinuria, and a much faster progression to ESRD.
  • #20 Focal Segmental Glomerulosclerosis: Practice Essentials, Pathophysiology, Etiology
    https://emedicine.medscape.com/article/245915-overview
    Focal segmental glomerulosclerosis (FSGS) is one of the most common causes of primary glomerular disease in adults. FSGS causes asymptomatic proteinuria or nephrotic syndrome with or without kidney insufficiency. Generally, FSGS results in progressive kidney injury; it accounts for 2.3% of all cases of end-stage kidney disease (ESKD), and is the leading glomerular cause of ESKD. The most common clinical presenting feature of FSGS (70% of patients) is nephrotic syndrome, characterized by generalized or dependent edema, along with fatigue and loss of appetite. However, some patients may be asymptomatic. The natural history of FSGS varies a great deal. A typical course runs from edema that is difficult to manage, to proteinuria refractory to corticosteroids and other immunosuppressive agents, to worsening hypertension and progressive loss of kidney function. In patients who do not respond to therapy, the average time from the onset of gross proteinuria to ESKD is 6-8 years, although wide variations in the time course occur. One of the key factors that determines renal survival is the persistence and degree of proteinuria. In patients who are unresponsive to therapy and who continue to have massive proteinuria of greater than 10 g/d, most develop ESKD within 5 years. The prognosis is much worse in black patients compared with white patients. In the collapsing form of FSGS, the disease is marked by severe hypertension, more massive proteinuria, a very poor response to corticosteroids, and a much faster rate of progression to ESKD. The presence of interstitial fibrosis on an initial kidney biopsy specimen is a uniform predictor of poor renal prognosis. The level of proteinuria greatly influences the outcome in FSGS. In patients with non-nephritic proteinuria, kidney function remains stable and fewer than 15% progress to ESKD in 10 years. More than 50% of patients with persistent nephritic syndrome develop ESKD in 10 years. In those with massive proteinuria greater than 10-15 g/day, kidney function deteriorates even more rapidly (over 2-3 years).
  • #21 Focal Segmental Glomerulosclerosis – Genitourinary Disorders – Merck Manual Professional Edition
    https://www.merckmanuals.com/professional/genitourinary-disorders/glomerular-disorders/focal-segmental-glomerulosclerosis
    Focal segmental glomerulosclerosis is scattered (segmental) mesangial sclerosis that begins in some but not all (focal) glomeruli and eventually involves all glomeruli. It manifests mainly in adolescents but also in young and middle-aged adults. Patients have insidious onset of proteinuria, mild hematuria, hypertension, and azotemia. […] Patients with focal segmental glomerulosclerosis (FSGS) commonly present with heavy proteinuria, hypertension, kidney dysfunction, edema, or a combination. Sometimes the only sign is asymptomatic proteinuria that is not in the nephrotic range (3 g/day). Microscopic hematuria is occasionally present. […] Prognosis is poor. Spontaneous remissions occur in 10% of patients. Kidney failure occurs in about half of patients within 8 years; in some, particularly those with significant tubulointerstitial fibrosis, kidney failure occurs much more rapidly despite treatment. The disorder is more rapidly progressive in adults than in children.
  • #22 Focal segmental glomerulosclerosis – Wikipedia
    https://en.wikipedia.org/wiki/Focal_segmental_glomerulosclerosis
    The majority of untreated cases of FSGS will progress to end-stage kidney disease. Important prognostic factors include the degree of proteinuria and initial response to therapy. Patients with nephrotic-range (3.5 g/day) proteinuria have over a 50% rate of progression to end-stage kidney disease at 10 years. Only 15% of patients with sub-nephrotic ranges of proteinuria progress to end-stage renal failure at 10 years. Initial response to therapy also dictates long-term outcomes. Those defined as having a „complete response” typically manifest a proteinuria of 300 mg/day; those with a „partial response” manifest a sub-nephrotic range of proteinuria, 3.5 g/day. Either complete or partial response is associated with 80% kidney survival at 10 years, compared with about 50% among non-responsive patients.
  • #23 Focal Segmental Glomerulosclerosis: Practice Essentials, Pathophysiology, Etiology
    https://emedicine.medscape.com/article/245915-overview
    Focal segmental glomerulosclerosis (FSGS) is one of the most common causes of primary glomerular disease in adults. FSGS causes asymptomatic proteinuria or nephrotic syndrome with or without kidney insufficiency. Generally, FSGS results in progressive kidney injury; it accounts for 2.3% of all cases of end-stage kidney disease (ESKD), and is the leading glomerular cause of ESKD. The most common clinical presenting feature of FSGS (70% of patients) is nephrotic syndrome, characterized by generalized or dependent edema, along with fatigue and loss of appetite. However, some patients may be asymptomatic. The natural history of FSGS varies a great deal. A typical course runs from edema that is difficult to manage, to proteinuria refractory to corticosteroids and other immunosuppressive agents, to worsening hypertension and progressive loss of kidney function. In patients who do not respond to therapy, the average time from the onset of gross proteinuria to ESKD is 6-8 years, although wide variations in the time course occur. One of the key factors that determines renal survival is the persistence and degree of proteinuria. In patients who are unresponsive to therapy and who continue to have massive proteinuria of greater than 10 g/d, most develop ESKD within 5 years. The prognosis is much worse in black patients compared with white patients. In the collapsing form of FSGS, the disease is marked by severe hypertension, more massive proteinuria, a very poor response to corticosteroids, and a much faster rate of progression to ESKD. The presence of interstitial fibrosis on an initial kidney biopsy specimen is a uniform predictor of poor renal prognosis. The level of proteinuria greatly influences the outcome in FSGS. In patients with non-nephritic proteinuria, kidney function remains stable and fewer than 15% progress to ESKD in 10 years. More than 50% of patients with persistent nephritic syndrome develop ESKD in 10 years. In those with massive proteinuria greater than 10-15 g/day, kidney function deteriorates even more rapidly (over 2-3 years).
  • #24 Focal Segmental Glomerulosclerosis – Genitourinary Disorders – Merck Manual Professional Edition
    https://www.merckmanuals.com/professional/genitourinary-disorders/glomerular-disorders/focal-segmental-glomerulosclerosis
    The presence of segmental sclerosis consistently at the glomerular pole where the tubule originates (tip lesion) may portend a more favorable response to corticosteroid therapy. Another variant, in which the capillary walls are wrinkled or collapsed (collapsing FSGS, which is typical in association with IV drug abuse or HIV infection), suggests more severe disease and rapid progression to kidney failure. […] FSGS may recur after kidney transplantation; proteinuria sometimes returns within hours of transplantation. Of patients whose transplant was for kidney failure caused by FSGS, up to 40% lose their graft due to recurrent FSGS within 5 years; risk is highest in younger patients, patients who are not Black, patients who develop kidney failure 3 years after disease onset, patients with mesangial proliferation, and patients with repeat transplants when the diagnosis before the first transplant was primary FSGS.
  • #25 Focal Segmental Glomerulosclerosis – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/sites/books/NBK532272/
    Approximately 50% of patients with persistent nephrotic-range proteinuria progress to ESRD within 3 to 8 years of an FSGS diagnosis. […] The natural history of FSGS varies significantly. It is characterized by edema, proteinuria that is often refractory to steroids or other immunosuppressive treatments, worsening hypertension, and progressively declining renal function. In cases that are unresponsive to treatment, the average time between the onset of gross proteinuria and progression to ESRD is 6 to 8 years, although variations may exist in the course of the disease. Achieving remission, whether spontaneous or induced, is associated with favorable renal outcomes. […] The prognosis is significantly worse in the Black population compared to White patients, likely due to APOL1 gene variants. The collapsing form of FSGS is characterized by severe hypertension, a poor response to corticosteroids, massive proteinuria, and a much faster progression to ESRD.
  • #26 Focal Segmental Glomerulosclerosis: Practice Essentials, Pathophysiology, Etiology
    https://emedicine.medscape.com/article/245915-overview
    Focal segmental glomerulosclerosis (FSGS) is one of the most common causes of primary glomerular disease in adults. FSGS causes asymptomatic proteinuria or nephrotic syndrome with or without kidney insufficiency. Generally, FSGS results in progressive kidney injury; it accounts for 2.3% of all cases of end-stage kidney disease (ESKD), and is the leading glomerular cause of ESKD. The most common clinical presenting feature of FSGS (70% of patients) is nephrotic syndrome, characterized by generalized or dependent edema, along with fatigue and loss of appetite. However, some patients may be asymptomatic. The natural history of FSGS varies a great deal. A typical course runs from edema that is difficult to manage, to proteinuria refractory to corticosteroids and other immunosuppressive agents, to worsening hypertension and progressive loss of kidney function. In patients who do not respond to therapy, the average time from the onset of gross proteinuria to ESKD is 6-8 years, although wide variations in the time course occur. One of the key factors that determines renal survival is the persistence and degree of proteinuria. In patients who are unresponsive to therapy and who continue to have massive proteinuria of greater than 10 g/d, most develop ESKD within 5 years. The prognosis is much worse in black patients compared with white patients. In the collapsing form of FSGS, the disease is marked by severe hypertension, more massive proteinuria, a very poor response to corticosteroids, and a much faster rate of progression to ESKD. The presence of interstitial fibrosis on an initial kidney biopsy specimen is a uniform predictor of poor renal prognosis. The level of proteinuria greatly influences the outcome in FSGS. In patients with non-nephritic proteinuria, kidney function remains stable and fewer than 15% progress to ESKD in 10 years. More than 50% of patients with persistent nephritic syndrome develop ESKD in 10 years. In those with massive proteinuria greater than 10-15 g/day, kidney function deteriorates even more rapidly (over 2-3 years).
  • #27 Focal Segmental Glomerulosclerosis: Practice Essentials, Pathophysiology, Etiology
    https://emedicine.medscape.com/article/245915-overview
    Focal segmental glomerulosclerosis (FSGS) is one of the most common causes of primary glomerular disease in adults. FSGS causes asymptomatic proteinuria or nephrotic syndrome with or without kidney insufficiency. Generally, FSGS results in progressive kidney injury; it accounts for 2.3% of all cases of end-stage kidney disease (ESKD), and is the leading glomerular cause of ESKD. The most common clinical presenting feature of FSGS (70% of patients) is nephrotic syndrome, characterized by generalized or dependent edema, along with fatigue and loss of appetite. However, some patients may be asymptomatic. The natural history of FSGS varies a great deal. A typical course runs from edema that is difficult to manage, to proteinuria refractory to corticosteroids and other immunosuppressive agents, to worsening hypertension and progressive loss of kidney function. In patients who do not respond to therapy, the average time from the onset of gross proteinuria to ESKD is 6-8 years, although wide variations in the time course occur. One of the key factors that determines renal survival is the persistence and degree of proteinuria. In patients who are unresponsive to therapy and who continue to have massive proteinuria of greater than 10 g/d, most develop ESKD within 5 years. The prognosis is much worse in black patients compared with white patients. In the collapsing form of FSGS, the disease is marked by severe hypertension, more massive proteinuria, a very poor response to corticosteroids, and a much faster rate of progression to ESKD. The presence of interstitial fibrosis on an initial kidney biopsy specimen is a uniform predictor of poor renal prognosis. The level of proteinuria greatly influences the outcome in FSGS. In patients with non-nephritic proteinuria, kidney function remains stable and fewer than 15% progress to ESKD in 10 years. More than 50% of patients with persistent nephritic syndrome develop ESKD in 10 years. In those with massive proteinuria greater than 10-15 g/day, kidney function deteriorates even more rapidly (over 2-3 years).
  • #28 Focal Segmental Glomerulosclerosis: Practice Essentials, Pathophysiology, Etiology
    https://emedicine.medscape.com/article/245915-overview
    The collapsing form of FSGS is marked by severe hypertension, more massive proteinuria, a very poor response to corticosteroids, and a much faster rate of progression to end-stage kidney disease (ESKD). A typical course runs from edema that is difficult to manage, to proteinuria that is refractory to corticosteroids and other immunosuppressive agents, to worsening hypertension and a progressive loss of kidney function. In nonresponders, the average time from the onset of proteinuria to ESKD is 6-8 years, although wide variations in the time course occur. The presence of interstitial fibrosis on an initial kidney biopsy specimen is a uniform predictor of poor renal prognosis. Blacks have a worse prognosis than Whites.
  • #29 Focal Segmental Glomerulosclerosis – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/sites/books/NBK532272/
    Approximately 50% of patients with persistent nephrotic-range proteinuria progress to ESRD within 3 to 8 years of an FSGS diagnosis. […] The natural history of FSGS varies significantly. It is characterized by edema, proteinuria that is often refractory to steroids or other immunosuppressive treatments, worsening hypertension, and progressively declining renal function. In cases that are unresponsive to treatment, the average time between the onset of gross proteinuria and progression to ESRD is 6 to 8 years, although variations may exist in the course of the disease. Achieving remission, whether spontaneous or induced, is associated with favorable renal outcomes. […] The prognosis is significantly worse in the Black population compared to White patients, likely due to APOL1 gene variants. The collapsing form of FSGS is characterized by severe hypertension, a poor response to corticosteroids, massive proteinuria, and a much faster progression to ESRD.
  • #30 Focal Segmental Glomerulosclerosis: Practice Essentials, Pathophysiology, Etiology
    https://emedicine.medscape.com/article/245915-overview
    Focal segmental glomerulosclerosis (FSGS) is one of the most common causes of primary glomerular disease in adults. FSGS causes asymptomatic proteinuria or nephrotic syndrome with or without kidney insufficiency. Generally, FSGS results in progressive kidney injury; it accounts for 2.3% of all cases of end-stage kidney disease (ESKD), and is the leading glomerular cause of ESKD. The most common clinical presenting feature of FSGS (70% of patients) is nephrotic syndrome, characterized by generalized or dependent edema, along with fatigue and loss of appetite. However, some patients may be asymptomatic. The natural history of FSGS varies a great deal. A typical course runs from edema that is difficult to manage, to proteinuria refractory to corticosteroids and other immunosuppressive agents, to worsening hypertension and progressive loss of kidney function. In patients who do not respond to therapy, the average time from the onset of gross proteinuria to ESKD is 6-8 years, although wide variations in the time course occur. One of the key factors that determines renal survival is the persistence and degree of proteinuria. In patients who are unresponsive to therapy and who continue to have massive proteinuria of greater than 10 g/d, most develop ESKD within 5 years. The prognosis is much worse in black patients compared with white patients. In the collapsing form of FSGS, the disease is marked by severe hypertension, more massive proteinuria, a very poor response to corticosteroids, and a much faster rate of progression to ESKD. The presence of interstitial fibrosis on an initial kidney biopsy specimen is a uniform predictor of poor renal prognosis. The level of proteinuria greatly influences the outcome in FSGS. In patients with non-nephritic proteinuria, kidney function remains stable and fewer than 15% progress to ESKD in 10 years. More than 50% of patients with persistent nephritic syndrome develop ESKD in 10 years. In those with massive proteinuria greater than 10-15 g/day, kidney function deteriorates even more rapidly (over 2-3 years).
  • #31 Focal Segmental Glomerulosclerosis: Practice Essentials, Pathophysiology, Etiology
    https://emedicine.medscape.com/article/245915-overview
    The collapsing form of FSGS is marked by severe hypertension, more massive proteinuria, a very poor response to corticosteroids, and a much faster rate of progression to end-stage kidney disease (ESKD). A typical course runs from edema that is difficult to manage, to proteinuria that is refractory to corticosteroids and other immunosuppressive agents, to worsening hypertension and a progressive loss of kidney function. In nonresponders, the average time from the onset of proteinuria to ESKD is 6-8 years, although wide variations in the time course occur. The presence of interstitial fibrosis on an initial kidney biopsy specimen is a uniform predictor of poor renal prognosis. Blacks have a worse prognosis than Whites.
  • #32 Focal segmental glomerulosclerosis (FSGS) – Symptoms and causes – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/fsgs/symptoms-causes/syc-20354693
    Focal segmental glomerulosclerosis (FSGS) results from scar tissue that develops in the glomeruli. Glomeruli are tiny structures within the kidney that filter waste products from the blood to create urine. A healthy glomerulus is shown on the left. When scar tissue develops in a glomerulus, kidney function gets worse (shown right). […] FSGS is a serious condition that can lead to kidney failure, which can only be treated with dialysis or kidney transplant. Treatment options for FSGS depend on the type you have. […] Symptoms of focal segmental glomerulosclerosis (FSGS) might include: Swelling, called edema, in the legs and ankles, around the eyes and in other body parts. Weight gain from fluid buildup. Foamy urine from protein buildup, called proteinuria. […] Focal segmental glomerulosclerosis (FSGS) may lead to other health concerns, also called complications, including: Nephrotic syndrome. This kidney condition causes the body to pass too much protein in the urine. Nephrotic syndrome raises the risk of other health conditions, such as blood clots and high blood pressure. Kidney failure. Damage to the kidneys that can’t be fixed causes the kidneys to stop working. The only treatments for kidney failure are dialysis or kidney transplant.
  • #33 Focal Segmental Glomerulosclerosis: Symptoms & Treatment
    https://my.clevelandclinic.org/health/diseases/21149-focal-segmental-glomerulosclerosis-fsgs
    FSGS can lead to high blood pressure and high cholesterol. In some cases, FSGS can lead to kidney failure. This condition can be life-threatening and requires emergency medical treatment. Seek immediate care if you have FSGS and experience any of these symptoms of kidney failure: Unmanaged high blood pressure. Anemia. Nausea, vomiting or loss of appetite. Swelling in your arms, legs or face. […] Treatment for FSGS depends on the type and cause, your age and whether you have other health conditions. The goal of treatment is to manage your symptoms to help you maintain a good quality of life and slow scarring so that it doesnt lead to kidney failure. […] FSGS is a chronic disease that cant be undone or reversed. This means treatment can only slow the progression of kidney disease, not cure you from the disease.
  • #34 Focal segmental glomerulosclerosis (FSGS)
    https://www.kidneyfund.org/all-about-kidneys/other-kidney-diseases/focal-segmental-glomerulosclerosis-fsgs
    FSGS is a long-term (chronic) condition that cannot be reversed. Scarring can make it hard for your kidneys to filter out waste from your body. While treatments can help slow its progress, some people with FSGS eventually develop kidney failure. If this happens, you will need a kidney transplant or dialysis to live. […] With secondary FSGS, kidney function may get worse over time, and protein levels in the urine can increase. […] Genetic FSGS can appear early in life with more severe symptoms or show up later with milder symptoms. […] The goal of treatment is to stop proteinuria, the leaking of protein into your urine. For primary FSGS, treatment usually includes: Medicines called immunosuppressants that will stop your body from attacking your kidneys. […] For all forms of FSGS, doctors recommend: Blood pressure medicines to lower the amount of protein lost and to control your blood pressure. […] Plasma exchange is a safe procedure but may have some side effects during or after the procedure that include low calcium levels, low body temperature, and low blood pressure.
  • #35 Focal Segmental Glomerulosclerosis – Genitourinary Disorders – Merck Manual Professional Edition
    https://www.merckmanuals.com/professional/genitourinary-disorders/glomerular-disorders/focal-segmental-glomerulosclerosis
    The presence of segmental sclerosis consistently at the glomerular pole where the tubule originates (tip lesion) may portend a more favorable response to corticosteroid therapy. Another variant, in which the capillary walls are wrinkled or collapsed (collapsing FSGS, which is typical in association with IV drug abuse or HIV infection), suggests more severe disease and rapid progression to kidney failure. […] FSGS may recur after kidney transplantation; proteinuria sometimes returns within hours of transplantation. Of patients whose transplant was for kidney failure caused by FSGS, up to 40% lose their graft due to recurrent FSGS within 5 years; risk is highest in younger patients, patients who are not Black, patients who develop kidney failure 3 years after disease onset, patients with mesangial proliferation, and patients with repeat transplants when the diagnosis before the first transplant was primary FSGS.
  • #36 About FSGS :: ZyVersa Therapeutics, Inc.
    https://www.zyversa.com/pipeline/about-fsgs
    70% of FSGS patients present with nephrotic syndrome. […] FSGS Returns in 30%-40% of patients after kidney transplant. […] There is a significant unmet need for effective FSGS-specific treatments that can delay disease progression, prevent end-stage renal disease, and improve patients’ quality of life.
  • #37 Focal segmental glomerulosclerosis (FSGS): symptoms and treatments
    https://www.kidneyresearchuk.org/conditions-symptoms/focal-and-segmental-glomerulosclerosis/
    Some people may have no visible symptoms and may only discover that they have higher than normal amounts of protein in their urine (proteinuria) after a routine urine test. In some cases, levels of protein loss and fluid retention can be so high that it causes puffiness (oedema) around the eyes, abdomen, feet and legs, and weight gain – nephrotic syndrome. Urine can also appear foamy. If kidney failure is already well advanced symptoms could include tiredness and lack of energy, itching and muscle cramps. Some people with FSGS may experience: High blood pressure – which needs to be controlled to protect the kidneys from further damage and to reduce the risk of heart disease and stroke. High cholesterol levels – which may need to be controlled with a special diet and cholesterol lowering drugs. Low thyroid hormone levels – due to loss of thyroid hormone via the kidneys. This can be controlled by thyroid hormone supplements.
  • #38 Focal Segmental Glomerulosclerosis: Symptoms & Treatment
    https://my.clevelandclinic.org/health/diseases/21149-focal-segmental-glomerulosclerosis-fsgs
    FSGS can lead to high blood pressure and high cholesterol. In some cases, FSGS can lead to kidney failure. This condition can be life-threatening and requires emergency medical treatment. Seek immediate care if you have FSGS and experience any of these symptoms of kidney failure: Unmanaged high blood pressure. Anemia. Nausea, vomiting or loss of appetite. Swelling in your arms, legs or face. […] Treatment for FSGS depends on the type and cause, your age and whether you have other health conditions. The goal of treatment is to manage your symptoms to help you maintain a good quality of life and slow scarring so that it doesnt lead to kidney failure. […] FSGS is a chronic disease that cant be undone or reversed. This means treatment can only slow the progression of kidney disease, not cure you from the disease.
  • #39 Focal segmental glomerulosclerosis – Symptoms, diagnosis and treatment | BMJ Best Practice
    https://bestpractice.bmj.com/topics/en-gb/939
    Focal segmental glomerulosclerosis is caused by an injury to podocytes in the renal glomeruli. […] May be asymptomatic or present with nephrotic syndrome. […] It manifests initially with proteinuria, which progresses to nephrotic syndrome and ultimately to end-stage renal failure. […] Prognosis is extremely variable and depends on the levels of proteinuria and plasma creatinine, the morphological subtype, the degree of renal fibrosis, and the response to therapy.
  • #40 Focal segmental glomerulosclerosis – Overview of Information and Clinical Research
    https://clinicaltrials.eu/disease/focal-segmental-glomerulosclerosis-2/
    Focal Segmental Glomerulosclerosis (FSGS) is a chronic kidney condition characterized by scarring in the glomeruli, the tiny filtering units within the kidneys. This scarring can lead to proteinuria, where excess protein is lost in the urine, and in severe cases, it can progress to nephrotic syndrome or kidney failure. […] The prognosis for individuals diagnosed with Focal Segmental Glomerulosclerosis (FSGS) can vary significantly. While some patients experience spontaneous remission, others may face a more challenging journey. Studies indicate that spontaneous remissions occur in about 10% of patients. For those who do not achieve remission, the disease can progress, with approximately 50% of patients developing renal failure within 10 years. […] Living with FSGS requires ongoing management and monitoring. While the disease is chronic and the scarred glomeruli cannot be repaired, treatment can slow the progression of kidney disease. Patients may live with the disease for many years, and the key is to respond well to treatment and be vigilant for any signs of change. For those who do not respond to treatment, the disease may progress to kidney failure, necessitating dialysis or a kidney transplant.
  • #41 Focal Segmental Glomerulosclerosis – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/sites/books/NBK532272/
    Approximately 50% of patients with persistent nephrotic-range proteinuria progress to ESRD within 3 to 8 years of an FSGS diagnosis. […] The natural history of FSGS varies significantly. It is characterized by edema, proteinuria that is often refractory to steroids or other immunosuppressive treatments, worsening hypertension, and progressively declining renal function. In cases that are unresponsive to treatment, the average time between the onset of gross proteinuria and progression to ESRD is 6 to 8 years, although variations may exist in the course of the disease. Achieving remission, whether spontaneous or induced, is associated with favorable renal outcomes. […] The prognosis is significantly worse in the Black population compared to White patients, likely due to APOL1 gene variants. The collapsing form of FSGS is characterized by severe hypertension, a poor response to corticosteroids, massive proteinuria, and a much faster progression to ESRD.
  • #42 Focal segmental glomerulosclerosis – Wikipedia
    https://en.wikipedia.org/wiki/Focal_segmental_glomerulosclerosis
    The majority of untreated cases of FSGS will progress to end-stage kidney disease. Important prognostic factors include the degree of proteinuria and initial response to therapy. Patients with nephrotic-range (3.5 g/day) proteinuria have over a 50% rate of progression to end-stage kidney disease at 10 years. Only 15% of patients with sub-nephrotic ranges of proteinuria progress to end-stage renal failure at 10 years. Initial response to therapy also dictates long-term outcomes. Those defined as having a „complete response” typically manifest a proteinuria of 300 mg/day; those with a „partial response” manifest a sub-nephrotic range of proteinuria, 3.5 g/day. Either complete or partial response is associated with 80% kidney survival at 10 years, compared with about 50% among non-responsive patients.
  • #43 Pathology Outlines – Focal segmental glomerulosclerosis-general
    https://www.pathologyoutlines.com/topic/kidneyfsgs.html
    Focal segmental glomerulosclerosis (FSGS) is a glomerular disease characterized by segmental and focal sclerosis of the tuft in the absence of underlying immune complex mediated disease. Patients generally present with nephrotic or subnephrotic proteinuria. Tip variant FSGS typically presents with sudden onset nephrotic syndrome with anasarca and weight gain. In secondary FSGS, patients tend to have subnephrotic range proteinuria at presentation in the absence of edema, hypoproteinemia or hypoalbuminemia. Approximately 30 – 60% of patients will inexorably progress to end stage kidney disease (ESKD) over a 5 – 10 year period of observation. It can recur in 20 – 25% of patients who receive a kidney transplant. Tip variant exhibits a better outcome in terms of achieving remission, while the collapsing variant is the worst one. Prognosis of collapsing FSGS is much worse in the Black population compared to the White population.
  • #44 Focal Segmental Glomerulosclerosis: Practice Essentials, Pathophysiology, Etiology
    https://emedicine.medscape.com/article/245915-overview
    The collapsing form of FSGS is marked by severe hypertension, more massive proteinuria, a very poor response to corticosteroids, and a much faster rate of progression to end-stage kidney disease (ESKD). A typical course runs from edema that is difficult to manage, to proteinuria that is refractory to corticosteroids and other immunosuppressive agents, to worsening hypertension and a progressive loss of kidney function. In nonresponders, the average time from the onset of proteinuria to ESKD is 6-8 years, although wide variations in the time course occur. The presence of interstitial fibrosis on an initial kidney biopsy specimen is a uniform predictor of poor renal prognosis. Blacks have a worse prognosis than Whites.
  • #45 Focal Segmental Glomerulosclerosis – Genitourinary Disorders – Merck Manual Professional Edition
    https://www.merckmanuals.com/professional/genitourinary-disorders/glomerular-disorders/focal-segmental-glomerulosclerosis
    Focal segmental glomerulosclerosis is scattered (segmental) mesangial sclerosis that begins in some but not all (focal) glomeruli and eventually involves all glomeruli. It manifests mainly in adolescents but also in young and middle-aged adults. Patients have insidious onset of proteinuria, mild hematuria, hypertension, and azotemia. […] Patients with focal segmental glomerulosclerosis (FSGS) commonly present with heavy proteinuria, hypertension, kidney dysfunction, edema, or a combination. Sometimes the only sign is asymptomatic proteinuria that is not in the nephrotic range (3 g/day). Microscopic hematuria is occasionally present. […] Prognosis is poor. Spontaneous remissions occur in 10% of patients. Kidney failure occurs in about half of patients within 8 years; in some, particularly those with significant tubulointerstitial fibrosis, kidney failure occurs much more rapidly despite treatment. The disorder is more rapidly progressive in adults than in children.
  • #46 Focal Segmental Glomerulosclerosis: Practice Essentials, Pathophysiology, Etiology
    https://emedicine.medscape.com/article/245915-overview
    Focal segmental glomerulosclerosis (FSGS) is one of the most common causes of primary glomerular disease in adults. FSGS causes asymptomatic proteinuria or nephrotic syndrome with or without kidney insufficiency. Generally, FSGS results in progressive kidney injury; it accounts for 2.3% of all cases of end-stage kidney disease (ESKD), and is the leading glomerular cause of ESKD. The most common clinical presenting feature of FSGS (70% of patients) is nephrotic syndrome, characterized by generalized or dependent edema, along with fatigue and loss of appetite. However, some patients may be asymptomatic. The natural history of FSGS varies a great deal. A typical course runs from edema that is difficult to manage, to proteinuria refractory to corticosteroids and other immunosuppressive agents, to worsening hypertension and progressive loss of kidney function. In patients who do not respond to therapy, the average time from the onset of gross proteinuria to ESKD is 6-8 years, although wide variations in the time course occur. One of the key factors that determines renal survival is the persistence and degree of proteinuria. In patients who are unresponsive to therapy and who continue to have massive proteinuria of greater than 10 g/d, most develop ESKD within 5 years. The prognosis is much worse in black patients compared with white patients. In the collapsing form of FSGS, the disease is marked by severe hypertension, more massive proteinuria, a very poor response to corticosteroids, and a much faster rate of progression to ESKD. The presence of interstitial fibrosis on an initial kidney biopsy specimen is a uniform predictor of poor renal prognosis. The level of proteinuria greatly influences the outcome in FSGS. In patients with non-nephritic proteinuria, kidney function remains stable and fewer than 15% progress to ESKD in 10 years. More than 50% of patients with persistent nephritic syndrome develop ESKD in 10 years. In those with massive proteinuria greater than 10-15 g/day, kidney function deteriorates even more rapidly (over 2-3 years).
  • #47
    https://link.springer.com/article/10.1007/s12325-023-02651-6
    Focal segmental glomerulosclerosis (FSGS) is a leading cause of kidney disease and can progress to end stage kidney disease (ESKD). […] Salient sign/symptom/side-effect domains included swelling/puffiness (edema), pain/aches/discomfort, fatigue, weight changes, skin problems, respiratory problems, and sleep problems. […] Patients report impairment in aspects of daily life and emotional wellbeing. […] Symptoms such as swelling, fatigue, and nausea limited patients energy and caused pain, which made it difficult to complete household chores. […] Patients reported that FSGS significantly impacted their daily lives. […] The overall experience of living with FSGS could also cause feelings of depression, hopelessness, and sadness, often related to swelling and weight gain, or because FSGS symptoms or treatment side effects prevented them from doing the things they wanted to do.
  • #48
    https://link.springer.com/article/10.1007/s12325-023-02651-6
    The conceptual model highlights the complexity of the lived patient experience of FSGS. […] This review identified swelling (edema) and fatigue as particularly bothersome symptoms of FSGS, associated with pain, weight changes, skin problems, and a driver of stigmatization and emotional and psychological impairment. […] Further qualitative research can allow refinement and confirmation of the conceptual model. […] The insights gained from this study can be used to ensure that drug developers and other researchers can measure what matters to patients by informing selection and/or development of relevant COAs for inclusion in future clinical research.
  • #49
    https://link.springer.com/article/10.1007/s12325-023-02651-6
    The conceptual model highlights the complexity of the lived patient experience of FSGS. […] This review identified swelling (edema) and fatigue as particularly bothersome symptoms of FSGS, associated with pain, weight changes, skin problems, and a driver of stigmatization and emotional and psychological impairment. […] Further qualitative research can allow refinement and confirmation of the conceptual model. […] The insights gained from this study can be used to ensure that drug developers and other researchers can measure what matters to patients by informing selection and/or development of relevant COAs for inclusion in future clinical research.
  • #50
    https://www.kidney.org.uk/focal-and-segmental-glomerulosclerosis
    High blood pressure may develop. This damages the kidneys and puts a strain on the heart and the rest of the circulation. Therefore high blood pressure should be treated vigorously. […] As noted above, kidney failure may sometimes occur. If so, it develops slowly, so you need not be concerned about a sudden change in your condition overnight. […] The condition itself does not cause any specific symptoms or pain. Fluid retention or kidney failure may affect day-to-day life. Most patients with this disease, however, lead normal lives and go work, have children and so on.
  • #51 Focal segmental glomerulosclerosis (FSGS) – Symptoms, causes, treatment | National Kidney Foundation
    https://www.kidney.org/kidney-topics/focal-segmental-glomerulosclerosis-fsgs
    What are the signs and symptoms of FSGS? Early stages may not cause any symptoms. You may only see some signs on your own, while others may be found by your healthcare provider. Signs and symptoms of FSGS include: Swelling in body parts like your legs, ankles and around your eyes (called edema) […] FSGS can cause nephrotic syndrome. Nephrotic Syndrome: A set of symptoms that happen together and affect your kidneys. These include: Swelling in body parts like your legs, ankles, or around your eyes (edema) […] If the condition is advanced, the symptoms may be like those of kidney failure. People may report fatigue, a poor appetite, headache, itchy skin, shortness of breath and/or nausea. […] You should talk with your doctor about your condition because the progression of the disease depends on many factors. FSGS is a chronic disease, because the scarred glomeruli cannot be repaired. Treatment can slow the process of kidney disease. Everyone is different in how they respond to treatment. Over time, some patients with FSGS gradually get worse until they reach kidney failure. If this occurs, they will need a kidney transplant or dialysis to stay alive. Some people respond well to treatment and may live with the disease for many years while being monitored for any signs of change.
  • #52 Focal segmental glomerulosclerosis – Overview of Information and Clinical Research
    https://clinicaltrials.eu/disease/focal-segmental-glomerulosclerosis-2/
    Focal Segmental Glomerulosclerosis (FSGS) is a chronic kidney condition characterized by scarring in the glomeruli, the tiny filtering units within the kidneys. This scarring can lead to proteinuria, where excess protein is lost in the urine, and in severe cases, it can progress to nephrotic syndrome or kidney failure. […] The prognosis for individuals diagnosed with Focal Segmental Glomerulosclerosis (FSGS) can vary significantly. While some patients experience spontaneous remission, others may face a more challenging journey. Studies indicate that spontaneous remissions occur in about 10% of patients. For those who do not achieve remission, the disease can progress, with approximately 50% of patients developing renal failure within 10 years. […] Living with FSGS requires ongoing management and monitoring. While the disease is chronic and the scarred glomeruli cannot be repaired, treatment can slow the progression of kidney disease. Patients may live with the disease for many years, and the key is to respond well to treatment and be vigilant for any signs of change. For those who do not respond to treatment, the disease may progress to kidney failure, necessitating dialysis or a kidney transplant.