In FSGS with Nephrotic Syndrome (NS) 23 patients (61%) of 38 with functional outcome are in remission with very long follow up (from 60 to 331 months) after treatment with Steroids alone or in combination with Cyclophosphamide. 

It would be interesting to assess whether in 11 patients with follow up from 5 to 27.6 years some parameters are associated with less severe disease favouring very long term remission.

Three parameters were considered [age<vs≥35  yrs, normal or high blood pressure (BP0 and BP1 ) baseline 24hP <vs≥ 6.5] to verify whether they assess lower disease severity  favouring remission.

Age≥ vs <35 yrs and BP1 va BP0 are characterized by very significant differences of age, baseline and last  eGFR, TID score and AH score. The patients with baseline 24hP < vs ≥6.5) were significantly different only for proteinuric parameters (TUP/C, IgG/C, Alb/C, α1m/C).

In all remission patients the  last value of  24hP is not significantly different according to the considered parameters and is independent from the baseline values  eGFR and TUP/C and duration of follow up

In the last years several studies evaluated which is the most favourable prognostic factor for long-term stable renal function and remission of proteinuria in Focal Segmental Glomerulosclerosis (FSGS) with nephrotic syndrome (NS) (1-15). In observational studies proteinuric biomarkers based on high molecular weight proteins excretion such as IgG and α2-macroglobulin showed  a high predictive value for remission in patients treated with steroids and Cyclophosphamide. The functional outcomes in all patients treated with Steroids alone or in combination with Cyclophosphamide include remission, persistent NS (PNS) with normal renal function (NRF) or chronic renal failure (CRF), and progression to ESRD. The interesting aim of this study is to assess if the very long follow up of  patients with remission (159±77, 60-331) may be dependent on clinical, functional, histologic and proteinuric markers  associated with less severe disease favouring a very long follow up of remission

The patients cohort  included in the study was not selected.  The patients attending  the Nephrology and  Dialysis Unit of San Carlo Borromeo Hospital, Milan, Italy, between January 1992 and April 2006  with renal biopsy diagnosis of Focal Segmental Glomerulosclerosis (FSGS) were 46; at baseline 6 patients have   persistent non-nephrotic proteinuria (<3>

Proteinuria was measured in 24 hour urine collection and second morning urine sample by the Coomassie blue method (modified with sodium-dodecyl-sulphate) and expressed as 24/hour proteinuria and protein creatinine/ratio (mg urinary protein/g urinary creatinine). Serum and urinary creatinine were measured enzymatically and expressed in mg/dL. Serum albumin and IgG and urinary IgG, α2-macroglobulin (α2m), Albumin and α1-microglobulin (α1m) were measured by immunonephelometry; urinary proteins were expressed as urinary protein/creatinine ratio (IgG/C, α2m/C, Alb/C, α1m/C).  Estimated glomerular filtration rate (eGFR) was measured by the Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI) formula (16). Three types of renal lesion that are markers of disease severity in any type of GN were evaluated: percentage of glomeruli with global glomerulosclerosis (GGS%); extent of tubulo-interstitial damage (TID) evaluated semi-quantitatively by a score: tubular atrophy, interstitial fibrosis and inflammatory cell infiltration graded 0, 1 or 2 if absent, focal or diffuse (TID global score: 0-6) and extent of Arteriolar Hyalinosis (AH) evaluated semiquantitatively by a score: 0, 1, 2, 3 if absent,  focal, diffuse, diffuse with lumen reduction, respectively (AH global score 0-4). 

Continuous variables are expressed as means±SD. Categorical variables are expressed as the number of patients (%). The differences of mean were determined by t-test; categorical variables by the chi-square test. All statistical analyses were performed using Stata 15.1 (StataCorp LP, TX, USA). Two-sided p<0>

In 38 patients with NS and functional outcome  23 patients (61%) developed remission: the remission patients with age <35 xss=removed xss=removed xss=removed xss=removed xss=removed xss=removed p =0.04), p =0.001) p=0.017); p =0.18); xss=removed xss=removed p=0.85); p=0.0001), p=0.04), p=0.0002) p=0.006) p=0.001);  xss=removed>

 In the 23 patients with remission the follow up  is rather long (from 60  to 331 months); it would be interesting to assess whether the patients with very long follow (27.6 years) were characterized by clinical, functional, histologic and proteinuric markers less severe than in  patients with lower follow up. The last value of 24hP is not significantly different according to the baseline markers of severity of disease (age, normal or high blood pressure, baseline 24hP) (Table 3). Thus no one factor has been identified  as associated  with the duration of follow up. Few patients after remission show recurrence of NS that was still responsive to the first treatment. It may be suggested that the patients characterized by markers associated with more severe disease (age, blood pressure, baseline 24hP)  should be treated with higher dosage and  longer duration of the treatment that developed the first remission. 

Table 1.Clinical, functional, histologic and proteinuric parameters in patients with Focal Segmental Glomerulosclerosis (FSGS) with different outcome. 

Table 2. Clinical, functional, histologic and proteimuric parameters in remission patients according to basel. 24hP, normal or high blood pressure (BP0 and BP1), age