To determine the prognostic value of response to treatment in patients with focal segmental glomerulo- sclerosis. FSGS includes 10-15% of idiopathic Nephrotic syndrome in children. Bulk of evidence supports disease relationship with immune system. Unfortunately, responses to immunosuppressive drugs are not desirable and progression to end-stage renal disease is common. We analyzed 62 out of 99 cases of biopsy proven idiopathic FSGS who were followed for at least 5-years or until renal failure occurred during study. Study design was historical cohort and patients were divided into two groups: exposed (resistant to treatment) and non-exposed (responsive to treatment). Correlation between prognosis and response to treatment was statistically evaluated. P-value (0.05 and relative risk ( 1 was considered significant. In 3 out of 25 steroid responsive patients (12%) and 22 out of 37 steroid resistant patients (59.5%), disease progressed to renal failure.Disease progressed to renal failure in 2 out of 11 cyclophosphamide responsive patients (18.1%), 17 out of 23 cyclophosphamide resistant patients (74.3%), and 8 out of 14 cyclosporine resistant patients (57.1%). 2 patients who responded to cyclosporine had normal renal function at the time of the last follow up. We concluded that favorable response to steroid and cyclophosphamide treatment is a protective factor against disease progression to end stage renal disease and resistance to these drugs imply a poor prognosis. For making any definite conclusion concerning response to cyclosporine treatment and prognosis, similar studies with a larger sample are required.

Introduction. Focal segmental glomerulosclerosis (FSGS) is one of the important causes of end stage kidney disease (ESKD). We evaluated the progression risk factors of primary FSGS to chronic kidney disease (CKD) or ESKD with a predictive model including clinical and histological predictors. Methods. 201 patients with primary FSGS (59% male, mean age: 38 ±,15 years), were studied. Time-dependent Cox model and C statistics were used for the predictive model. Interaction and correlation between independent variables were estimated. Results. During 55 ±,27 months of follow-up, 82 patients (41%) developed CKD (46) or ESKD (36) patients. In adjusted model, 1 unit of higher serum creatinine (SCr) at baseline (HR = 1. 39, 95% CI: 1. 15 to 1. 70) and 1% increase in glomeruli with segmental glomerulosclerosis (SGS) (HR = 1. 03, 95% CI: 1. 02 to 1. 04) or interstitial fibrosis/tubular atrophy (IF/TA) (HR = 1. 03, 95% CI: 1. 01 to 1. 05) increased the risk of CKD/ESKD. In adjusted model, higher baseline proteinuria and collapsing variant were not associated with risk of CKD/ESKD. By adding SGS and IF/TA scores to baseline SCr in the model, discrimination by C statistics was 0. 83 (95% CI: 0. 77 to 0. 90). Median renal survival was 3. 1 years (95% CI: 2. 2 to 4. 1 years) in patients with highest risk score (baseline eGFR < 25 mL/min/1. 73m2 + IF/TA/SGS > 50%), and 8. 1 years (95% CI: 7. 7 to 8. 6 years). in those with lowest score (baseline eGFR > 75 mL/ min/1. 73m2 + IF/TA/SGS < 5%). Conclusion. In primary FSGS, higher baseline SCr, increased SGS and IF/TA, but not baseline proteinuria and collapsing pathology, were the predictors for CKD/ESKD. These findings indicated the importance of timely detection and referral in prognosis of primary FSGS.

Sarcoidosis is a multisystemic granulomatous disease of unknown etiology. Renal involvement in sarcoidosis patients is occurred, but the incidence and prevalence is uncertain. The most common renal involvement of systemic sarcoidosis is nephrocalcinosis and interstitial nephritis. After sarcoidosis was diagnosed in a 31-year-old male patient, we performed a renal biopsy because of nephrotic range proteinuria and renal dysfunction. The collapsing variant of focal segmental glomerulosclerosis (FSGS) secondary to sarcoidosis was diagnosed by kidney biopsy.

Background and Aim: Rituximab is a novel therapy that can help patients with steroid-dependent or resistant nephrotic syndrome. The aim of this study was to evaluate the efficacy of rituximab in children with corticosteroid-dependent and resistant nephrotic syndrome and to determine the factors associated with its efficacy. Methods: In this study, 40 children with corticosteroid-dependent or resistant nephrotic syndrome who were treated with rituximab in Dr. Sheikh Hospital, Mashhad, between 2014 and 2018 were enrolled. Patients with a history of hematuria, severe urinary tract infection, or secondary nephrotic syndrome were excluded. Results: The mean age of patients was 11. 9 ±,5. 04 years, and 55% were female. The most common underlying pathology of nephrotic syndrome was focal segmental glomerulonephritis (FSGS) (42. 5%) followed by membranoproliferative glomerulonephritis (MPGN) and minimal change disease (MCD). Most of the participants (62. 5%) were steroid-dependent and the rest (27. 5%) were steroid resistant. Only 10% of the patients showed complications following rituximab administration and 57. 5% went into complete remission. A negative family history and steroid-dependent nephrotic syndrome were significantly associated with a better treatment response. Moreover, patients with steroid-resistant nephrotic syndrome were more likely to have a positive family history, while factors associated with steroid response included underlying pathology, gender, and family history. Conclusion: Rituximab can cause remission in more than half of the patients with steroid-resistant or dependent nephrotic syndrome. Moreover, the only factors that reduce response to rituximab are a history of corticosteroid resistance and a positive family history of nephrotic syndrome.

Introduction. Focal segmental glomerulosclerosis is one of the causes of ESRD that can recur in the transplant kidney (TX). Proteinuria may recur within hours after the graft is received whereas overt sclerosis is not evident until weeks or months later. Extensive foot process effacement is sometimes the earliest sign of recurrence of FSGS. However, segmentally sclerotic glomeruli in transplant kidney could also be seen secondarily due to other causes like hypertension, chronic allograft nephropathy, chronic transplant glomerulopathy, and calcineurin inhibitor toxicity. Otherwise, it could also be considered as de novo FSGS. In this study, regarding the importance of differential diagnosis of recurrent primary FSGS (Rec FSGS) from secondary FSGS (Sec FSGS) due to the possible recurrence of FSGS in next transplant, we tried to find out the diagnostic criteria of Rec vs Sec FSGS based on clinical findings at presentation, time of diagnosis after transplant, and light microscopic and EM findings. We also studied the spectrum of different morphologic variants of FSGS in transplant kidney based on new Columbia classification. Methods. All Tx biopsies with segmental sclerosis and/ or extensive foot process effacement (FPE) and negative IF between 1995 and 2006 were reviewed excluding cases diagnosed as Tx glomerulopathy. All slides, reports, EM phothomirograph, clinical history, and follow-up reports were reviewed. Light microscopic findings were classified by the Colombia schema. Findings of Sec FSGS such as CNI toxicity, expanded lamina rara interna of GBM, and limited FPE were assessed and cases were classified as primary vs Sec FSGS. Results. Forty-two patients (29 males, 13 females) met entry criteria. Average age was 37±13.8 years (range, 11 to 56 years). Four patients were children (< 18 years old). Twenty cases (48%) were African American and 13 (31%) were Caucasian. Twenty-three (55%) had nephritic proteinuria at the time of biopsy. Biopsy interval ranged from 4 days to 8 years after Tx. Twenty-three (54%) cases were classified as Rec FSGS, 15 (35%) as Sec FSGS, and 4 (10%) as likely de novo FSGS. Ten (54%) cases showed only extensive FPE, 4 (17%) cellular (CELL), 4 (17%) collapsing (COLL), and 4 (17%) not otherwise specified (NOS) lesions. In cases classified as likely Sec FSGS, NOS lesion was the most common morphologic variant, in 6 (40%), followed by 3 (20%) COLL, 2 (13%) CELL, and 3 (20%) with only FPE. Rec FSGS was the most common in early biopsies (85% of all FSGS cases in first 6 months). In contrast, 13 (65%) biopsies at >2years showed Sec FSGS. Nearly all patients, whether Rec or Sec FSGS, lost their kidney during the following months to years. Conclusion. Early time of recurrence and extensive FPE were characteristic of Rec FSGS. NOS variant is more common in Sec, whereas extensive FPE alone is the most common finding in Rec FSGS. COLL, related to CNI toxicity, and CELL lesion can be seen in both Rec and Sec FSGS. We conclude that integrated analysis of LM, EM, and clinical data help to differentiate varying etiologies of sclerotic lesions in the Tx.

Objectives: The present study aimed to examine the expression of CD44 and claudin-1 markers using immunohistochemical methods to differentiate patients with minimal change disease (MCD) from those afflicted with focal segmental glomerulosclerosis (FSGS). Materials and Methods: In this descriptive-analytical study, twenty patients with definite FSGS, twenty patients with FSGS/MCD spectrum, and seven patients with definite MCD were randomly chosen from Imam Reza hospital affiliated with Tabriz University of Medical Sciences. All patients underwent renal biopsy, and then the presence of the immune complex was examined in the obtained samples using immunofluorescence staining. Some renal specimens were paraffin-embedded for the evaluation of the expression levels of CD44 and claudin-1 utilizing the immunohistochemistry method. Results: Among the twenty samples obtained from patients with definite FSGS, 13 samples (65%) were double-positive for CD44 and claudin-1, 3 specimens (15%) were positive only for claudin-1, and 4 samples (20%) were double-negative for CD44 and claudin-1. The percentages of renal specimens expressing claudin-1 in patients with definite FSGS, FSGS/MCD, and MCD were 80%, 85%, and 0%, respectively. Finally, the percentages of renal samples expressing CD44 in patients with definite FSGS, FSGS/ MCD, and MCD were 65%, 10%, and 0%, respectively. Conclusions: The results of the present research indicated that the rate of CD44-positive specimens was higher in patients with FSGS while the percentage of claudin-1-positive samples was more frequent in MCD patients compared with FSGS patients.