5. A 39-year-old CKD 5 patients secondary to FSGS not yet on dialysis (eGFR is 16 mls/min) was referred to you to consider him for kidney transplantation. He has proteinuria 1.5 gm/day. His serum albumin is 36 gm/L (3.6 g/dL). Native kidney biopsy is shown below (see below):
- How to prepare him for kidney transplantation?
- What is the indication of anticoagulation in this case?
- Does he need to be anticoagulated after transplantation?
- Discuss his long-term outcome with emphasis of his primary disease.
Dear All
There was a typo which I have corrected. It is secondary to FSGS, not a secondary FSGS.
It is a primary FSGS. The main objective of this case scenario anticoagulation is
not required in this case. Maybe ACE post-transplantation is required.
Post-transplantation also the patient would receive CNI. This may also help to
shut off his native kidneys.
For those colleagues who responded and treated the case as secondary FSGS, it is OK.
Dear All
Let us see how good you are
We evaluate secondary causes of FSGS by calculating BMI or infectious causes.
Because of reasonable serum albumin there is no need for anticoagulation
NO. Only in the case of recurrence of FSGS after TX with very low serum albumin.
Recurrence of FSGS in secondary or genetic forms is rare but evaluation of proteinuria after Tx should be considered.
To rule out genetic or secondary forms of FSGS, performing genetic and infectious investigations or BMI calculation is considered.
If serum albumin is less than 2 gr/dl, there will be an indication for anticoagulation in this case but, it is not necessary now.
No. But, if FSGS recur with severe hypoalbuminemia, then may be needed.
Because of the rate of FSGS recurrence after TX, this subject should be informed. Close daily monitoring for proteinuria after TX is necessary and if it happens, graft biopsy should be done. Treatment of recurrence with plasma exchange and rituximab is considered. The rate of graft failure in FSGS recurrence is high. In a patient considered for the second TX due to FSGS recurrence, the rate of recurrence reaches 80%.
For this patient with glomerular lesion secondary to FSGS, identification of probable underlying disease is essential and genetic testing can be helpful. This patient who don’t have nephrotic syndrome can be considered as undetermined cause (FSGS-UC).
Because of serum albumin level more than 2.5 gr/dl and absence of heavy proteinuria, prophylactic anticoagulation is not recommended neitherpre transplantation nor post transplantation.
Recurrence rate of primary FSGS after kidney transplantation is high and it has reported in more than 30% of patients. On the other hand, the frequency of secondary FSGS recurrence after kidney transplantation is low and depend on its etiology as well as genetic forms of FSGS. Thus, identification of underlying cause is crucial, although it is usually not obvious.
actually no indication in this patient
–
no need if level of serum protein more than 20gm/dl
1ry FSGS recure in about 33% and can progress to graft loss with 5 fold risk
How to prepare him for kidney transplantation?
What is the indication of anticoagulation in this case?
No indication for anticoagulation in this case as his serum albumin is 3.6 g/dl.
Does he need to be anticoagulated after transplantation?
No need for post transplant anticoagulation provided his medical data.
Discuss his long-term outcome with emphasis of his primary disease.
FSGS tend to recur post transplant in up to 55% of those with primary idiopathic FSGS and the recurrence is associated with high rate of graft loss (up to 5 times compared to those not having the disease )
This case scenario of patient CKD 5 with significant protinuria (1.5 gm/day) and provided biopsy shows area of segmental sclerosis in the glomerulus (FSGS),. This is quiet similar to scenario 4 (except that lower degree of protinuria and no severe hypoalbuminemia)
So,
Q1_ preparation of the patient includes exclusion of secondary causes as infection, drugs and anatomical abnormalities.
2_ exclusion of genetic causes by genetic study.
3. Control of protinuria by medical nephrectomy (ACEi and ARBS ) and may be NSAIDs
4. No need for anticoagulation, as albumin > 2 gm/dl.
5. Preemptive Plasmapheresis with or without rituximab may be needed to prevent early recurrence of 1ry FSGS, on addition start of CNI and steroids one week prior to transplantation.
Q2_ indications of anticoagulation if the patient albumin drops below 2 gm/dl which can predispose him to hypovolemia and graft hypo-perfusion or graft vessel thrombosis.
Q3_ after transplantation, no need for anticoagulation if the patient has functioning graft and no further heavy protinuria or hypoalbuminemia.
As long as serum albumin is more than 2gm/dl , the risk of thrombosis is decreased and no need for prophylactic anticoagulant
Use of ACEI after transplantation may be advised .
Q4_ high risk of recurrence is expected in 1ry FSGS.
So close monitoring with A/C ratio together with graft function is crucial.
Any increase in protinuria is considered recurrence and if increase, graft biopsy with EM examination is essential to detect early recurrence (effacement of foot processes of podocytes).
_ risk of recurrence is 30 % that increase up to 100% in re-transplantation.
– retransplantation from a living donor is contraindicated
-Genetic FSGS has no recurrence.
-2ry FSGS has minimal rate of recurrence once the etiology is treated.
How to prepare him for kidney transplantation?
Identify the underlying cause of 2nd FSGS
1-reduction in nephron mass (eg, history of reflux nephropathy, congenital absence or surgical removal of a kidney, low birth weight or premature birth)
2-Exposure to drugs and/or toxins associated with FSGS (eg, heroin, interferon, bisphosphonates [particularly pamidronate], anabolic steroids)
3-History of viral infections (eg, HIV, parvovirus B19, cytomegalovirus, Epstein-Barr virus, simian virus 40, hepatitis C)
4-Body mass index (BMI)Obesity
*Risk factors for FSGS recurrence
1-Young Age below 16 years old
2-Rapid progression to ESRD in the native kidney
3-Sever proteinuria pre transplantation.
4-Low serum albumin at diagnosis.
5-Risk of recurrence in the2nd allograft: 80%
No need to do nephrectomy because s.albumin is normal and no heavy proteinurea
What is the indication of anticoagulation in this case?
No need for anticoagulant in this case because s.albumin more than 2 gm/dL
and proteinurea less than 10 gm
and there is no history of previous thrombosis
Does he need to be anticoagulated after transplantation?
If early mobilization can achieved and normal graft function so no need for anticoagulant
Discuss his long-term outcome with emphasis of his primary disease.??
However, not all patients with FSGS suffer from recurrence after kidney transplantation, and genetic and secondary FSGS have a negligible risk of recurrence but need to control on underlying causes
For primary FSGS Need cyclosporin ,high-dose methylprednisolone and rituximab in addition to immediate and intense plasmapheresis have worked well, achieving remission in more than 80% of recent recurrent cases
But there high risk of graft loss reaching 50% of cases
Reference
1-Up to date 2-Hee Gyung Kang, Il-Soo Ha, and Hae Il Cheong. Recurrence and Treatment after Renal Transplantation in Children with FSGS. BioMed Research International Volume 2016, Article ID 6832971, 7 pages http://dx.doi.org/10.1155/2016/6832971.
How to prepare him for kidney transplantation?
As the kidney biopsy shows features of perihilar variant of FSGS. As the patient has FSGS we need to have an insight into the type of FSGS responsible for the disease….Secondary FSGS will not have nephrotic range proteinuria and is secondary to obesity, hyperfiltration and other kidney diseases..Primary FSGS can be due to podocytopathy or abnormal permeability factor….If it is due to podocytopathy after transplantation the disease will not recur…If it is due to abnormal permeability factor which is only possible after clinical judgement, we have to carefully monitor the disease recurrence in the post transplant period…Daily checking urine spot protein creatinine ratio maybe useful…Patient has to be counselled about the immediate graft recurrence in 30 to 55% of the patients with primary FSGs…this requires immediate Plasmaphresis and IV rituximab based on the response…However I would like to counsel that the overall graft loss rate is lesser maybe 15 to 20% …Patients have to be initiated on ACE inhibitors for control of proteinuria..
Indication of anticoagulation in this case?
This patient has sub nephrotic range proteinuria with serum albumin of 3.6gm/dl….As per the KDIGO guidelines for membranous nephropathy anticoagulation is indicated only if serum albumin is less than 2.5gm/dl
Anticoagulation after transplant?
This patient does not need anticoagulation after transplant if there is no FSGS recurrence and serum albumin is >2.5gm/dl.If there is recurrence of FSGS after transplant and there is nephrotic syndrome then there is an indication to anticoagulate him
Long term outcome of recurrence of primary idiopathic FSGS is 10% to 48% according to various studies…reports of graft loss of 20% have been reported in the literature….Earlier onset of the disease age, previous FSGS allograft recurrence, rapidly progressive FSGS to ESRD, collapsing FSGS, mesangial hypercellularity on biopsy are risk factors for recurrence. ..Early and intensive monitoring for recurrence from the time of detection in the post transplant period is essential to avoid graft loss..
1- prepare patient for kidney transplantation
1- first of all: all preparation that which done for any recipient start from test for blood group ,immunology assessment,chemistry,virology ,images and cardiology assessment .
preparation regarding his primary disease ,FSGS as the cause of ESRD in native kidney, we do not administer any prophylactic measure to prevent recurrent FSGS,although such patient may be at high risk of recurrence, the use of preventive measure such as plasmapheresis or rituximab in these patients has been clearly shown to reduce rates of recurrence(this data from case report and small case series),large study have not confirm these finding .the risk of recurrence are:recipent with living donor ,rapid progression to ESRD,pathological charctized of the native kidney biopsy suchas,mesangial hypercelluarity and few sclerotic glommurli ,past history of graft failure due to FSGS and rapid deterioration of ckd to ESRD so in this case we need more data if the has rapid deterioration of his ckd or not so if exsisance of any risk factors make that option of prophylatic measure which are mention above.
In some experince centre TAC trough level 12-15ng/ml in combination with high dose of methylpredsolone and rituximab and intense and immdiate plasmopharesis working well achiveing remission in more than 80% of recent recurent FSGS.
also in some centre for high risk patients plasmophresis 3 to5 session pre and postrenal transplantion in addition to single dose of rituximab375mg/m2 with steroid ,CNI and MMF
for 2 weeks prior kideny transplant was showed good result.
2- no indication of anticoagulant for this case as serum albumin more than 2g/dl.
3-acorrding to serum albumin post renal transplant if less than2gm/dl can be anticoagulant.
4- the out come of recurent primary FSGS,although there is signifcant risk of graft loss,the out come of recurent FSGS have so much improved that FSGS is no longer contraindication for renal transplantation ,when plasmapheresis when started as soon as possible before circulating factor causing irrevsible damage .
in most of the studies one third of the patients has recurent. FSGS Pos transplantation and five fold time incidence of graft loss.
references
1- uptodate2022
2-. Canaud G., Delville M., Legendre C. Recurrence of focal and segmental glomerulosclerosis after transplantation. Transplantation. 2016;100(2):284–287. doi: 10.1097/TP.0000000000000902. [PubMed] [CrossRef] [Google Scholar]
How to prepare him for kidney transplantation?
▪︎The aim of preparation of this pt is to prevent recurrence of his primary disease (FSGS) post transplant. But, there is still no effective prophylaxis that can prevent FSGS recurrence [1]
ACE inhibitors
Plasmapheresis (Both rituximab and plasmapheresis have been given to prevent FSGS recurrence in many centers)
▪︎This patient can receive prophylactic and perioperative treatment with plasmapheresis and high-dose (intravenous) cyclosporine .
▪︎In recent years, therapy with rituximab has shown promising results [2].
▪︎
◇What is the indication of anticoagulation in this case? NO need for anticoagulation
Because his serum albumin is more than 2.0 to 2.5 g/dl so, there no risk of arteial and venous thromboembolism [3].
◇Does he need to be anticoagulated after transplantation?
▪︎This depends on the sevirity of hypoalbuminemia and the risk of bleeding.
▪︎I think he may need it just shortly after surgery
◇Discuss his long-term outcome with emphasis of his primary disease?
▪︎Patients with a diagnosis of FSGS had poorer graft survival compared with the general transplant population [4].
▪︎Up to 1 in 3 patients with primary FSGS will experience disease recurrence after kidney transplantation, with the risk of allograft failure 5-times the risk compared to those without disease recurrence.
{NOTE: Risk factors for disease recurrence in patient with primary FSGS includes: younger age at presentation, recipients of live-donor kidneys, non-white ethnicity, severe manifestations of disease at presentation, rapid progression to ESKD, and prior allograft failure from disease recurrence}.
____________________________________.
Ref:
[1] Alasfar S., Matar D., Montgomery R.A., et al. Rituximab and Therapeutic Plasma Exchange in Recurrent Focal Segmental Glomerulosclerosis Postkidney Transplantation. Transplantation. 2018;102:e115–e120.
[2] Michael Rudnicki. FSGS Recurrence in Adults after Renal Transplantation
https://doi.org/10.1155/2016/3295618
[3] Judit Gordon-Cappitelli and Michael J. Choi.Prophylactic Anticoagulation in Adult Patients with Nephrotic Syndrome
CJASN January 2020, 15 (1) 123-125; DOI: https://doi.org/10.2215/CJN.05250419
[4] Anna Francis, Peter Trnka, and Steven J. McTaggart Long-Term Outcome of Kidney Transplantation in Recipients with Focal Segmental Glomerulosclerosis
https://doi.org/10.2215/CJN.03060316
How to prepare him for kidney transplantation?
Patient with known history FSGS should be investigated for the primary cause of injury. In patients with primary FSGS as the cause of ESKD in the native kidneys, prophylactic measures are not administered to prevent recurrent FSGS. Although such patients may be at high risk of recurrence, the use of preventive measures (such as plasmapheresis or rituximab in these patients has not been clearly shown to reduce rates of recurrence. The maintenance immunosuppression given in the post-transplantation period will prevent in a part the recurrence of FSGS in the allograft.
What is the indication of anticoagulation in this case?
In this case the patient does not have indication for prophylactic or therapeutic anticoagulation
Discuss his long-term outcome with emphasis of his primary disease.
Long term outcomes and risk of recurrence depends on the primary disease. So patients with primary FSGS has high risk of recurrence reaching 50% after first transplant, and up to 80% in the second transplant. Patients who developed ESRD due to secondary causes have to control their underlying comorbid conditions such as DM, hypertension, obesity or undergo treatment and cure of causative viral illness and recovery from substance addiction (eg. cocaine).\
Along with immunosuppression trough level post transplantation, patient should be monitored for proteinuria frequently. The presence of proteinuria should prompt further evaluation with kidney biopsy.
The LM shows Perihilar variant of focal segmental glomerulosclerosis with Hyaline deposition and sclerosis at the vascular pole of the glomerulus.
For preparation of renal transplantation
-optimal blood pressure (BP) control and antiportienuric measures using ACEIs or ARBs
-lipid-lowering agents to control hyperlipidemia as statins
– Steroid therapy is only considered for patients with idiopathic FSGS associated with nephrotic syndrome and our patient here as nephritic range protienuria
the prognosis in nephritic FSGS patients is determined by their response to prednisone and other immunosuppressives. Complete remission is protein excretion of less than 200-300 mg/d, and partial response is excretion of 200-3500 mg/d, or a greater than 50% reduction in baseline proteinuria.
-Plasmapharesis and Rituximab can be given although there is no effective prophylaxis that can prevent FSGS recurrence but can allow remission in the event of recurrence
Since there is a direct proportion relation between the declining serum albumin level and thrombotic risk and in this case his serum albumin is normal and proteinuria is 1.5 g /dl non nephrotic range so he doesnot need anticoagulation
The serum albumin threshold below which to start anticoagulation is not clearly established, although most studies mentioned that the level is between 20 and 30 g/l
According to follow up after transplantation if FSGS recurred with heavy nephrotic range proteinuria also considering the HAS BLED score for bleeding risk at that time it will be decided accordingly ;on the other hand if no recurrence occurred and graft is functioning well ,anticoagulation wont be needed
Primary idiopathic FSGS recurs in 30%–50% of patients following kidney transplantation (KT), with other studies using stricter clinical and diagnostic definitions of FSGS putting the incidence of recurrence disease at 11%.
Recurrence risk is related to older age at primary disease onset, native kidney nephrectomies, white race, and lower BMI at transplant.
Reference
-Kurian, Sunil M.et al .UNOS/OPTN Data-guided Assessment of Focal Segmental Glomerulosclerosis After Kidney Transplantation and Evaluation of Immunosuppressive Protocols in a Steroid-free Center, Transplantation Direct: September 2021 – Volume 7 – Issue 9 – p e738
-Uffing A et al.Recurrence of FSGS after Kidney Transplantation in Adults.CJASN February 2020, 15 (2) 247-256
-Naciri Bennani, Hamza et al. “Kidney Transplantation for Focal Segmental Glomerulosclerosis: Can We Prevent Its Recurrence? Personal Experience and Literature Review.” Journal of clinical medicine vol. 11,1 93. 24 Dec. 2021.
-Lin R, McDonald G, Jolly T, Batten A, Chacko B. A Systematic Review of Prophylactic Anticoagulation in Nephrotic Syndrome. Kidney Int Rep. 2019;5(4):435-447.
How to prepare him for kidney transplantation?
The current image showing most probably the perihilar variant of FSGS,can not judge is it primary or secondary without detailed history,Autoimmune profile,Electron microscopy examination of the renal biopsy sample.
in general the primary form FSGS is the frequent form of post transplant recurrence.
FSGS histological variants making difference in steroid response and prognosis to ESRD (the tip lesion has been considered the most responsive to steroid therapy while the collapsing variant has been thought to be steroid-resistant and associated with a more aggressive clinical course.)
So preparation for kidney transplantation In the current case with low level proteinuria and normal level of serum albumin ,no need for anticoagulation.
Plasmapharesis is needed perioperative +/- Rituximab
What is the indication of anticoagulation in this case?
Anticoagulation is indicated postoperatively as DVT prophylaxis and once the patient is mobilizing ,it will be no need for it.
Discuss his long-term outcome with emphasis of his primary disease.
Primary FSGS has a high frequent rate of recurrence which can happened after a few hours of transplantation with aggressive proteinuria due to presence of soluble factors.
CNI as maintenance of immunosuppression has a great role as anti proteinuria measures .In addition of adding ACE/ARBS if stable graft function with strict follow up.
Long term outcome of the patient with respect to primary disease
The slide shows collapsing variant of FSGS.
The risk of recurrence is similar to classic FSGS, but if collapsing FSGS recurs after kidney transplant, then there is possibility of severe vascular abnormalities, higher serum creatinine levels, and higher degree of graft failure compared with non-collapsing variants of FSGS.
Multimodal treatment could resolve the proteinuria in this patient and allow space for full or partial remission.
References :
Preparing the patient for transplant
Indication of Anticoagulation
Need for Anticoagulation after transplant
References
-Primary FSGS ; Preparation
All is about proper Counseling, early diagnosis and treatment of recurrence ;
1.Age; more in young
2.Race ; more in white
3.Rapidly progressive disease to ESRD in < 3 years
4.BMI ; more in low BMI
5.Nephrectomy
6.Mesengial proliferation in the native biopsy
7.Previous failed allograft due to recurrent FSGS
8.Living-donor transplantation
Management of recurrent FSGS must be disscuss with the patient including cost implications and the possible duration of treatment in case of recurrence ;
-Anti-coagulation is not indicated
-Long-term outcomes as indicated above
He can proceed for transplant as he has proteinuria of 1.5 gm only with albumin of 36 so doesn’t require anticoagulation.He can be kept on ACEi if feasible and best would be if he has no proteinuria before transplant.
He should be explained that recurrence rate is quite high in primary FSGS and usually happens in first three months and in case of recurrence the literature says that around 40% of patients will lose there graft within five years however that should not stop someone going for transplant.
Post transplant surveillance
We should know prior to transplant that what is native kidney proteinuria as it will help making decision post transplant. Usually we expect native proteinuria to subside within one month post transplant.
To help detect early recurrence posttransplantation, all patients with primary FSGS as the cause of ESKD in the native kidneys should be screened for proteinuria. We obtain a random or spot urine protein-to-creatinine ratio on the first postoperative day, the day of scheduled hospital discharge, weekly for four weeks, and then monthly for one year after transplantation . If the ratio is >0.5 g/g, we obtain a 24-hour protein collection to confirm the result obtained with the random urine protein-to-creatinine ratio.
Threshold for graft biopsy should be low as not to miss early recurrence
1- ACEI or ARBS
2-can be transplanted as serum albumin is normal
3- no anticoagulation due to no nephrotic range proteinuria and serum albumin is normal
5- no need for anticoagulation post transplant
6- as regard recurrence and long term outcome :
30-70% of the primary cases can recur early or late post transplant, genetic type has low rate of recurrence, FSGS recur mostly late secondary to reduced renal mass.
Risk factors for recurrence of FSGS :
1- Young age
2- High BMI
3- White race
4- Rapid progression to ESRD in native kidney
5- Pre transplant severe proteinuria
6- Low serum albumin at the time of diagnosis
7- 80% risk of recurrence in second transplant
Prevention and treatment of recurrent FSGS:
1- Pre transplant plasmapheresis and rituximab is of benefit to prevent recurrence and also used in treatment of recurrence
2- Protocol biopsy is important in early detection.
How to prepare him for kidney transplantation?
This perihillar focal segmental glomeruli sclerosis ( primary FSGS) but should be role out secondary causes
Plasmapharesis plus rituximab 3 sessions before kidney transplant and post transplant
extensive immunosuppressive agents ( steroid and calcinurine inhibitors and MFF
antiprotinuric agent
What is the indication of anticoagulation in this case?
No indication because serum albumin normal
Does he need to be anticoagulated after transplantation?
I think no need for long time; just temporary post operation
Discuss his long-term outcome with emphasis of his primary disease.
with adherence to immunosuppressive agents the graft outcome is good
References
Audrey et al: Recurrence of FSGS after kidney transplant in adults; CJASN. org volume 15 february, 2020 (tango study 30)
1. This is perihilar FSGS which can be found in primary and secondary types.
So we should exclude causes of secondary type.
2. His serum albumin is normal, no indicatin for anticoagulant.
3. I do not think he need anticoagulnt post transplant , but just shortly after surgery.
He need diet advise
ACEi
Statins
Immunosuppression after transplantation
Prepare him for kidney transplantation:
The patient is considered pre emptive renal transplant candidate of primary FSGS aetiology.
It would be wise to counsel the patient about the high early possibility of recurrence preceeding any other steps.
Preparation for renal transplantation shall be as routine regarding laboratory (renal functions, correction of anaemia if existed to avoid hazards of blood transfusion and desensitization, knowing his baseline level of proteinuria, virology screening, anticoagulation profile, tissue typing, cross match by flow cytometry and CDC, calculated PRA according to need based on the transplant centre schedule) and other procedures like imaging techniques according to transplant centre protocols.
This case in my opinion would need good induction especially by Rituximab to achieve the best control for primary FSGS, with triple immunosuppressive regimen based on tacrolimus, MMF and corticosteroids.
Related donors are not recommended for such cases.
No indication of anticoagulation in this case with normal serum albumin, no history of DVT or thromboembolic events or cardiac diseases suggesting the need for anticoagulation.
In my opinion, no need for anticoagulation post transplantation except if operative events occurred mandating the use of anticoagulation.
Long-term outcome with emphasis of his primary disease:
Idiopathic FSGS recurs post-transplant in one third of cases and is associated with a five-fold higher risk of graft loss. Response to treatment is associated with significantly better outcomes.
However there are predictors for recurrence as proteinuria, native kidney nephrectomy, race, BMI and age.
Plasmapheresis and rituximab were the most frequent treatment for recurrence.
Partial or complete remission occurred in 57% of patients and was associated with better graft survival.
Reference:
CJASN February 2020, 15 (2) 247-256; DOI: https://doi.org/10.2215/CJN.08970719
Findings
The slide shown features of FSGS – perihilar variant and at the vascular pole there is hyaline deposition and sclerosis. FSGS is a common cause of ESRD and common finding in idiopathic nephrotic syndrome. Not all cases will have recurrence of FSGS after renal transplant. So the pathological finding of FSGS is not an indication of postponement of transplant. Recurrence rates can be as high as 60%. No biomarker has been validated yet to predict recurrence. Monitoring for proteinuria is recommended.
How to prepare him for kidney transplant.
3-5 sessions of Plasmaphresis pre and immediately post transplant will remove the circulating factors. Also intense immune suppression with methryprednisolone or rituximab before transplant with standard immunosuppression is necessary.
What is the indication of anticoagulation in this case??
Not indicated as serum albumin is normal.
Does he need anticoagulation after transplant
No as serum albumin is normal
Long term outcome with emphasis on primary disease.
Recurrent FSGS can resolve and may not cause harm to the graft in some cases or can lead to graft damage and loss . In aggressively treated cases , the long term outcome is good and those with poor response to treatment , graft loss can be five folds.
Audrey Uffing et al. Recurrence of FSGS after Kidney Transplantation in Adults. CJASN February 2020, 15 (2) 247-256.
** How to prepare him for kidney transplantation?
The biopsy showed perihilar variant of focal segmental glomerulosclerosis (FSGS). Hyaline deposition and sclerosis occur at the vascular pole of the glomerulus.
This variant is also may be due to a secondary form of FSGS, occurring as an adaptive response to other injuries resulting in loss of functioning nephrons, such as in obesity-related kidney disease.
FSGS have a particularly high risk of recurrence.
In order to eliminate the circulating factors, preemptiveplasmapheresis/immunoadsorption may be considered: 3–5 sessions prior to transplantation followed by 3–5 sessions immediately at post-transplantation. An additional single dose of rituximab (375 mg/m2), plus immunosuppression with corticosteroids, CNI and MMF for 2 weeks prior to kidney transplantation, has been shown to prevent recurrence.(1)
** What is the indication of anticoagulation in this case?
No indication of anticoagulat, because of normal serum albmuin level.
**Does he need to be anticoagulated after transplantation?
I think he does not need for his normal serum albmuin
**Discuss his long-term outcome with emphasis of his primary disease.
Recurrent FSGS can lead to early graft failure. In addition, it can spontaneously resolve without treatment.
Schachter et al. reported that, recurrent FSGS increased the occurrence of death or ESKD from 12 to 27 %.
Similarly, in a review of 2,687 adolescents with a kidney transplant enrolled in the North American Pediatric Renal Transplant Collaborative Study (NAPRTCS) database between 1987 and 2001, of whom 338 had primary FSGS, recurrent FSGS accounted for 15 % of all graft failures 17 % in recipients of living related donor kidneys and 14 % in recipients of deceased donor kidneys . Recurrent FSGS resulted in a loss of the expected living-donor graft survival advantage
In contrast, Sener et al. found no adverse effect of recurrent disease on allograft survival in patients with FSGS. A similar finding was also reported by Jungraithmayr in a series of 86 pediatric patients
Thus, although the short-term course may be impacted by recurrent FSGS, this complication should be treated aggressively because it is not uniformly associated with adverse long-term outcomes.(2)
References
(1) Cochat P, Fargue S, Mestrallet G, et al. Disease recurrence in paediatric renal transplantation. Pediatr Nephrol. 2009;24(11):2097–2108. [PMC free article] [PubMed] [Google Scholar]
(2) Schachter ME, Monahan M, Radhakrishnan J, Crew J, Pollak M, Ratner L, Valeri AM, Stokes MB, et al.Appel GB (2010) Recurrent focal segmental glomerulosclerosis in the renal allograft: single center experience in the era of modern immunosuppression. Clin Nephrol 74(3):173–181
CAS Article PubMed Google Scholar
The kidney biopsy picture reveals a perihilar variety of FSGS with hyaline deposition and sclerosis at the vascular pole.
As mentioned in the clinical scenario, it is a case of secondary FSGS (the responsible condition has not been mentioned). The clinical clues to a secondary form of FSGS include a nephrotic or subnephrotic range proteinuria without nephrotic syndrome ( as seen in this case with subnephrotic proteinuria and absence of hypoalbuminemia). (1) Such patients, on biopsy usually show a perihilar variety with glomerulomegaly. (2)
The patient should be evaluated for the primary reason of this secondary FSGS. The various causes of secondary FSGS include:
1) Viral infections: HIV, CMV, Parvovirus B19, HCV, EBV, SARS COV-2
2) Drug-induced: Direct acting anti-viral therapy, Heroin, Lithium, Interferon, NSAIDs, mTOR inhibitors, Calcineurin inhibitors, Anabolic steroids, anthracyclins
3) Reduced nephron number: Reflux nephropathy, sickle cell disease, renal dysplasia
4) Normal nephron number: obesity related, primary glomerular disease, systemic diseases like diabetes and hypetension.
The detailed history should be taken and the primary reason of this secondary FSGS should be managed. For example, treatment of viral infections, discontinuation of the offending drugs.
The indications for guidelines for anticoagulation in nephrotic syndrome have been proposed especially for membranous nephropathy. (1) It includes use of warfarin or low molecular weight heparin in patients with serum albumin less than 2-2.5 g/dl having high risk of venous thromboembolism.
Patients with serum albumin 3-3.2 g/dl with high arterial thromboembolic risk should be given aspirin as prophylaxis. (1)
These can be extrapolated to any cause of nephrotic syndrome.
So, in the index case, there is no need of anticoagulation.
Post-transplantation, the patient needs to be under surveillance. In the event of patient developing nephrotic syndrome, he will be requiring anticoagulation as per the criteria. There is no indication to put him on routine anticoagulation post-operatively.
The primary reason of the secondary FSGS should will provide a clue towards the prognosis. The patient and the family members should be counselled regarding the risk of recurrence.
Secondary FSGS usually does not recur if the primary cause of FSGS no longer remains after the kidney transplant. (3) The risk of recurrence of secondary FSGS is lower, but we need to remain vigilant and proteinuria post-transplant should be closely monitored.
References:
1) Rovin BH, Adler SG, Barratt J, Bridoux F, Burdge KA, Chan TM, Cook HT, Fervenza FC, Gibson KL, Glassock RJ, Jayne DRW, Jha V, Liew A, Liu ZH, Mejía-Vilet JM, Nester CM, Radhakrishnan J, Rave EM, Reich HN, Ronco P, Sanders JF, Sethi S, Suzuki Y, Tang SCW, Tesar V, Vivarelli M, Wetzels JFM, Lytvyn L, Craig JC, Tunnicliffe DJ, Howell M, Tonelli MA, Cheung M, Earley A, Floege J. Executive summary of the KDIGO 2021 Guideline for the Management of Glomerular Diseases. Kidney Int. 2021 Oct;100(4):753-779. doi: 10.1016/j.kint.2021.05.015. PMID: 34556300.
2) De Vriese AS, Sethi S, Nath KA, Glassock RJ, Fervenza FC. Differentiating Primary, Genetic, and Secondary FSGS in Adults: A Clinicopathologic Approach. J Am Soc Nephrol. 2018 Mar;29(3):759-774. doi: 10.1681/ASN.2017090958. Epub 2018 Jan 10. PMID: 29321142; PMCID: PMC5827609.
3) Kang HG, Ha IS, Cheong HI. Recurrence and Treatment after Renal Transplantation in Children with FSGS. Biomed Res Int. 2016;2016:6832971. doi: 10.1155/2016/6832971. Epub 2016 Apr 24. PMID: 27213154; PMCID: PMC4860214.
Thank you
The histological pattern of FSGS ( TIP ,collapsing , perihilar , NOS) it will not have impact on the rate of recurrence, only the degree of clinical proteinuria with sever nephrotic syndrome and failure to achieve remission with treatment is the clue for high rate of recurrence and in this patient his proteinuria of 1.5gm with normal serum albumin likely he have secondary FSGS with low rate of recurrence post transplantation as per report from TANGO group .
Thank you
Standard preparation , Ace or ARB to control proteinuria. if donor available preemptive
transplant.
What is the indication of anticoagulation in this case?
There is no indication of anticoagulant.
No unless for postoperative prophylaxis.
secondary FSGS has no risk of recurrence posttransplant.
Thank you
The patient is 39 yrs old with preemptive CKD ,due to secondary FSGS, with proteinuria , normal albumin& biopsy showed segmental sclerosis.
-No need for native nephrectomy as he has normal albumin .
– Anticoagulant is not needed.
The patient can go for a kidney transplant with stander preparation.
No indication for anticoagulant, serum albumin within normal
No need for it unless indicated.
Secondary FSGS is less aggressive & low rate of recurrence post-transplant & better prognosis so no need for preoperative plasmapheresis & rituximab ( as this is used to decrease recurrence in primary FSCG )
Thank you
How to prepare him for kidney transplantation?
This is the classical picture of secondary FSGS showing perihilar variant which usually occurs in obesity, hypertension, reflux nephropathy should be identified and addressed. Other features suggestive of secondary FSGS in this case include sub-nephrotic proteinuria and normal albumin levels.Also risk of recurrence post transplantation should be told to the patient. Although no prophylactic role of plasmapheresis and Rituximab before transplantation for reducing proteinuria but sometimes needed if very heavy proteinuria and no other therapy available.
What is the indication of anticoagulation in this case?
Anticoagulation can only be considered if confirmed thrombosis i.e., DVT or Pulmonary embolism or any evidence of thrombophilia i.e., protein C and S deficiency or APLA.
Does he need to be anticoagulated after transplantation?
He will need anticoagulation after transplantation if serum albumin is <20-25g/l and any of following like proteinuria>10g/day, BMI>35kg/m2,genetic disposition for thromboembolism or recent surgery or prolonged immobilization.
Discuss his long-term outcome with emphasis of his primary disease.
Recurrence in secondary FSGS is less and prognosis depends on the cause i.e.,hyperfiltration and obesity has indolent course while ESRD develops due to viral infection.
REFERENCE:
1-Richard A. Lafayette Facing the Vexing Problem of Recurrent FSGS after Kidney Transplantation. Clin J Am Soc Nephrol 2020 Feb 7; 15(2): 171–173
Thank you
How to prepare him for kidney transplantation?
The clinical manifestation like sub-nephrotic range proteinuria and accepted serum albumin is mainly correlated with secondary FSGS which have many causes (HIV , Heroin, decreased renal mass tissue as with single kidney , reflux nephropathy ,obesity and DM).
So better to treat associated condition before transplantation as HIV etc.
As mentioned he is a case of secondary FSGS which as per L/M showing per-hilar segmental sclerosis with larger glomeruli that indicative of the compensatory hyper filtration which takes place in these patients but to differentiate need E/M which reveled diffuse foot process effacement on primary , whereas secondary FSGS usually does not.
What is the indication of anticoagulation in this case?
There is no indication for anticoagulation in such cases with serum albumin above 2.5 gm(1).
Does he need to be anticoagulated after transplantation?
With these albumin and proteinuria level , no need.
So it will depend on post-operative condition.
Discuss his long-term outcome with emphasis of his primary disease.?
Risk or recurrence is low specially with treating secondary causes of FSGS.
Reference:
1-KDIGO 2021 CLINICAL PRACTICE GUIDELINE FOR THE MANAGEMENT OF GLOMERULAR DISEASES.
Thank you
How to prepare him for kidney transplantation?
This patient has proteinuria with good albumin level. Kidney biopsy showed perihilar hyalinosis and sclerosis involving the majority glomeruli and with segmental sclerosis. GBM is thickened and some glomerulomegaly noted. Elector microscopy will determine diffuse foot process effacement without identifiable cause in primary Fsgs and segmental Foot process effacement or diffuse foot process effacement with identifiable cause will be labelled as secondary Fsgs. Clinical scenario with perihilar FSGS indicates this patient has secondary Fsgs.
What is the indication of anticoagulation in this case?
No indication for anticoagulation as indication for anticoagulation will be Hypoalbuminemia below 25 g/L or 20 g/L depending on bromocresol green, or purple requires anticoagulation.
Does he need to be anticoagulated after transplantation?
No indication for anticoagulation post-transplant.
Discuss his long-term outcome with emphasis of his primary disease.
References:
Thank you
The LM picture with PAS stain shows the typical NOS variant of FSGS, again FSGS is histological pattern of kidney injury mainly characterized by glomerular sclerosis and hyalinosis, could be primary or due to different causes like genetics, familial, drugs and toxins, infectious causes, obesity small nephron mass adaptive hyperfiltration, reflux, SCD, the given proteinuria level and serum albumin its more in favor the secondary type of FSGS. Which is usually less aggressive with better prognosis and lower rate of recurrence compared to primary (idiopathic FSGS). however still i would like to ask for IF and EM to get complete picture of the case as still primary autoimmune disease with Focal segmental scarring can’t be ruled out and the EM histology will further help us with the type of deposits and the pattern of foot process effacement (focal VS Diffuse) also need more history about possible secondary causes like history of viral infection, parvo virus, HIV, CMV, or reflux nephropathy ,SCD, drug history, BMI ,family history , apol1 gene screen in African black race.
He needs proper counselling about the chance of recurrence of the disease after transplantation which is less in secondary FSGS including genetic or inherited types less rate of recurrence compared to primary FSGS, which is more aggressive and associated with higher rate of early relapse post transplantation due to generalized podocytes injury.
Primary nephrotic syndrome in general associated with increased thrombosis (venous, arterial thrombosis), The incidence of TE in NS in the range of 3–44%, due to the urinary loss of essential prothrombotic proteins in the urine like antithrombine111 , fibrinogen , protein s so the risk of VTE reported more with membranous nephropathy, followed by FSGS, MCD, both level of Proteinuria (heavy proteinuria > 3.5gm and lower serum albumin levels < 20gm/dl associated with the increased tendency to VTE (5).
In one study they found that the main predictors for increased the risk of VTE in nephrotic syndrome is the ratio between urinary proteinuria and the serum albumin while degree of GFR decline with other medical comorbid like DM, HTN, Smoking, previous thrombosis, atherosclerosis are the main factors for increased the arterial thrombosis (ATE) (4). In his case he does not need anticoagulation in both pre and post transplantation period provided he does not have any from above mentioned risk factors.
The histologic sub types of FSGS in native kidney does not change the risk rate of recurrence Also, one the clinical challenges are the time of recurrence more than 3 months will be very difficult to differentiate primary VS denovo or, secondary FSGS. Usually, early recurrence in < 3 months with average rate of 1.5 month more in favor the diagnosis of Primary FSGS.
Donors from the African black race should be tested for APLO1 genotype, and if found positive, such donors should be avoided, due to the increased risk of post transplant de-novo collapsing FSGS with progressive graft loss .
References:
1-Uffing A, Pérez-Sáez MJ, Mazzali M, et al. Recurrence of FSGS after Kidney Transplantation in Adults. Clin J Am Soc Nephrol. 2020;15(2):247-256.
2-Kidney transplantation in adult: Focal segmental glomerulosclerosis in the transplanted kidney. Up to date medicine 2022.
3-Naciri Bennani H, Elimby L, Terrec F, et al. Kidney Transplantation for Focal Segmental Glomerulosclerosis: Can We Prevent Its Recurrence? Personal Experience and Literature Review. J Clin Med. 2021;11(1):93. Published 2021 Dec 24. doi:10.3390/jcm11010093
4- Mahmoodi BK, ten Kate MK, Waanders F, Veeger NJ, Brouwer JL, Vogt L, Navis G, van der Meer J: High absolute risks and predictors of venous and arterial thromboembolic events in patients with nephrotic syndrome: Results from a large retrospective cohort study. Circulation 117: 224–230, 2008.
5- Kelddal S, Nykjær KM, Gregersen JW, Birn H. Prophylactic anticoagulation in nephrotic syndrome prevents thromboembolic complications. BMC Nephrol. 2019;20(1):139. Published 2019 Apr 25.
Thank you
In preparation for his kidney transplant, what steps should we take to make him ready,
There is a considerable chance of getting FSGS again after a transplant; this risk increases with time.
I will study the patient’s medical history, do a physical examination on the patient, and request laboratory tests in order to search for any possible secondary causes that should be corrected before the transplantation in order to prevent recurrence after the transplantation.
Some instances of secondary causes include the following: The parvovirus, HIV infection, drugs(heroin, bisphosphonates), and being overweight.
Both preemptive therapeutic plasma exchanges and rituximab are examples of medications that might be utilized before a transplant in the case of primary FSGS.
post-transplant anticoagulation may be required if:
previous graft loss due to vascular thrombosis.
Pro-coagulant state(for Ex, antiphospholipid syndrome, protein C or S deficiency
Metallic heart valve.
with the previous history, the patient does not have a high risk for thrombosis.
Discuss the long-term effects of his illness, with special attention to his underlying condition.
Recurrent glomerular disease is often a late complication after transplantation. The only exception to this rule is primary focal segmental glomerulosclerosis (FSGS), which may occur at any time. The main FSGS recurrence rate ranges from 20 to 50 per cent, and the incidence of graft loss due to recurrence is 50 per cent.
Factors that increase the likelihood of relapse – Several risk factors for the recurrence of FSGS after transplantation have been reported. These risk factors include a younger age of disease onset, rapid progression of the initial disease, being White, prior native kidney nephrectomy, lower body mass index (BMI) at transplantation, and history of recurrence in a prior allograft. Other risk factors include having a lower body mass index (BMI) at the time of transplantation. In the United States, white kidney transplant patients who received organs from African-American donors may be at an especially elevated risk of experiencing a recurrence. On the other side, receivers of African descent seem to represent a group with a decreased risk, since recurrence is less common in this population.
In most cases, the presence of an FSGS history in one’s family indicates a lesser chance of recurrence. On the other hand, the particular mutation could have a role in determining how likely the familial FSGS is to reoccur.
Thank you. Also if serum albumin is below 20 gm/L.
It is important to differentiate between primary, genetic and secondary FSGS,
Clinical features of secondary FSGS include absent nephrotic syndrome at disease onset with slow deterioration of kidney functions and presence of clear cause as infection (HIV), drugs (heroin, lithium, anabolic steroids), reduced kidney mass, longstanding DM or morbid obesity.
kidney biopsy of secondary FSGS commonly shows mild effacement of foot process and glomerular hypertrophy
Anticoagulation is not indicated as serum albumin> 2g/dl
No need for anticoagulation as long as there is no hypoalbuminemia or increased risk of thromboembolism
The risk of recurrence of secondary FSGS is low,
The primary disease should be controlled as weight control in obese patients, blood pressure control.
De Vriese AS, Sethi S, Nath KA, Glassock RJ, Fervenza FC. Differentiating primary, genetic, and secondary FSGS in adults: a clinicopathologic approach. Journal of the American Society of Nephrology. 2018 Mar 1;29(3):759-74.
Uffing A, Pérez-Sáez MJ, Mazzali M, Manfro RC, Bauer AC, de Sottomaior Drumond F, O’shaughnessy MM, Cheng XS, Chin KK, Ventura CG, Agena F. Recurrence of FSGS after kidney transplantation in adults. Clinical Journal of the American Society of Nephrology. 2020 Feb 7;15(2):247-56.
Thank you
How to prepare him for kidney transplantation?
What is the indication of anticoagulation in this case?
Does he need to be anticoagulated after transplantation?
Discuss his long-term outcome with emphasis of his primary disease.
Thank you
How to prepare him for kidney transplantation?
The image shows a perihilar variant of FSGS. It usually indicates a secondary cause of FSGS, e.g. obesity, reflux nephropathy, hypertension, & sickle cell disease. However, it can also occur in primary FSGS.
Primary, or idiopathic, FSGS is a diagnosis of exclusion, & is characterized by early & often severe recurrence of proteinuria after transplantation.
Therefore, before transplantation, I would look for & address any possible secondary causes & treat accordingly, for example weight reduction, optimizing his diabetes management, & treating HIV infection.
If diagnosis of primary, or idiopathic, FSGS is entertained, the recipient should be well counseled & informed about the risk of recurrence & realistic expected outcomes.
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What is the indication of anticoagulation in this case?
No
Anticoagulation is not indicated; his serum albumin is normal, the proteinuria is not massive, and, more importantly, no any significant risk factors are mentioned in the history.
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Does he need to be anticoagulated after transplantation?
No, for the same reasons mentioned in the answer of the above question.
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Discuss his long-term outcome with emphasis of his primary disease:
Biopsy-proven primary FSGS carries a 32% chance of recurrence after transplantation.
FSGS recurrence had negative impact on graft survival, there is nearly 5-fold increase in the risk of graft loss.
However, successfully treated recipients with partial or complete remission have outcomes similar to those with no recurrent disease.
The risk factors for recurrence include:
– Older age
– White race
– United States transplants
– Lower BMI
– Prior nephrectomy
– Prior transplant recurrence
Treatment with PP resulted in complete or partial remission in 57% of recurrent cases.
Adding rituximab to PP gave similar or even slightly inferior outcomes (47% response).
Women & younger recipients were more likely to experience remission.
Genetic & secondary FSGS have a very minimal risk of recurrence.
So, a pathologic diagnosis of FSGS should not, in itself, exclude kidney transplantation.
Close monitoring of proteinuria with prompt & intensive treatment for recurrence is necessary.
Reference
1. Richard A. Lafayette Facing the Vexing Problem of Recurrent FSGS after
Kidney Transplantation. Clin J Am Soc Nephrol 2020 Feb 7; 15(2): 171–173.
doi: 10.2215/CJN.14841219
2. Hee Gyung Kang, Il-Soo Ha, and Hae Il Cheong Recurrence and Treatment
after Renal Transplantation in Children with FSGS. Biomed Res Int;
2016: 6832971.Published online 2016 Apr 24. doi: 10.1155/2016/6832971
Excellent
However, I disagree with many of the risk factors of recurrence you mentioned. Can you review it again?
The risk factors, I have mentioned here, were actually seen in recurrence of idiopathic, rather than secondary, FSGS, which is the case in this scenario.
I think this is the usual presentation of secondary FSGS ( low level of proteinuria, normal serum albumin level), kidney biopsy shows perihilar form of FSGS which is the common form of FSGS, so we need to look for the primary disease that causes secondary FSGS and if it is a controllable disease, then we can go safely for transplantation.
There is no indication of anticoagulation in this case as the serum albumin level is above 2.5 g/dl.
He will not need it.
The risk of recurrence of secondary FSGS post-transplantation is low especially if the primary cause is corrected such as obesity, and medications.
Thanks, but it could be primary FSGS
Yes, it could be
He is not nephrotic, with non nephrotic range proteinuria,consistant with secondary FSGF.
There is no indication for anticoagulation in this case context, as the nephrotic syndrome with massive hypoalbuminemia below 2 gm/dl os the main indication for anticoagulation.
Similarly being non nephrotic is against the assumption of being genetic or idiopathic FSGS. As idiopathic and genetic FSGS are typically presented with full blown nephrotic syndrom.
However, I would request for EM study to confirm the partial Podocytopathy related to secondary FSGS.
There is no risk of recurrence of secondary FSGS, however its indicated ti look for the possible underlying etiology of secondary FSGS and treat accordingly like obesity.
In conclusion its low risk case and can proceed safly for kidney transplantation.
I would look at Immune flouresence study as well to find out about the primary renal disease that caused the glomerular disease initially, and then secondary FSGS, like IgA glomerular disease with secondary FSGS
Thanks, Wael
The patient has risk factors for recurrence of FSGS. He’s young, we don’t know the speed of disease progression, he already has pre-transplant proteinuria (not severe), and normal albumin levels at diagnosis. We do not have information about the genotype, as APOL1 is supposed to have a reduced risk of recurrence.
With the data we have, there would be a risk of recurrence of the underlying disease after transplantation, and one should be aware of the risks. Plasmapheresis and rituximab can be used to decrease the risk of recurrence as well as for treatment. After transplantation, the patient must adhere to the biopsy protocol.
There is no indication of anticoagulation pre or post-transplantation (normal levels of albumina and proteinuria in low levels).
The patient needs to be aware of the risk of recurrence of the underlying disease and of possible therapies that can considerably decrease their immunity and increase the risk of adverse events, mainly related to infections derived from the use of rituximab and the immunosuppression to be performed.
Thanks, Filipe
Will you wait for recurrence to happen then treat or you give a prophylactic treatment as you suggested?
I believe The risk is too high to wait in this scenario.
During counciling of this patient it is important to know the type of FSGS ( primary, secondary or genetic), because primary FSGS associated with high rate of recurrence.
Also family history & genetic study are an important in determining the risk of recurrence. Differentiation between primary & secondary types can be done by clinical & histological features( secondary type usually presented with sub-nephrotic range proteinuria, & slowly progression to ESRD.
Risk factors can predict recurrence:
(a) recurrence on previous transplant
(b) young age at diagnosis
(c) rapid progression to ESRD
(d) albuminuria <2.5g/dl
(e) race ( black & Hispanc).
The photo above shows one glomerulus (PAS stain) with NOS variant of FSGS.
There is no indication of anticoagulation pre or post transplantation.
FSGS had the poorer transplant outcome than other causes of ESRD due to high recurrence rate, which can occur hours- days post transplantation. Most cases of recurrence occur during first 2 years post transplantation, & live kidney donor offer better 5 years survival than deceased donor. The variant has not predict the recurrence.
References:
Thanks, Ban
A very clear answer. I agree with the risk of recurrence you mentioned and your treatment plan.
Biopsy shows perihilar FSGS, and presentation of this case goes with a secondary or genetic form of FSGS
Secondary FSGS occurs due to hyperfiltration (obesity, DM, sickle cell anemia, single kidney), toxins (heroin, IFN, anabolic steroids), viral infection (HIV) or previous injury from any form of glomerulonephritis
The cause of secondary FSGS should be known as some causes may constitute a contraindication to transplantation such as drug addiction, and other factors may need treatment before transplantation like HIV.
No clear indication of anticoagulation in this case as serum albumin is > 3 mg/dl, anticoagulation may be indicated if there is compelling indication like AF, associated DVT, APS
No need for anticoagulation after transplantation except if recurrence occurs and serum albumin decreases and meets the criteria mentioned in scenario 4
Patients should be informed about the risk of recurrence which will differ according to the cause of secondary FSGS, but generally, it is uncommon especially if the cause of secondary FSGS is known. A family history of FSGS is considered a lower risk factor for recurrence.
Very good.
The biopsy shows starting thin starting fibrous crescent which could be a clue to a secondary cause so to exclude other initial pathologies IF would be informative.
Thanks, Sherif Excellent as usual