Long-Term Follow-Up of Two Patients with Successfully Treated Idiopathic Collapsing FSGS with Methylprednisolone Pulse

Introduction: Focal segmental glomerulosclerosis (FSGS) is one of the common causes of nephrotic syndrome in adults. Collapsing glomerulopathy (CG) is a histopathological variant of FSGS with poor response to treatment and prognosis. Here we present two cases with biopsy proven CG who achieved complete remission(CR) with methylprednisolone pulse(MP) and their long term follow up Case Description: Case 1- A 30-year-old male visited in May 2018 with anasarca, shortness of breath and oliguria. His medical and family history were not significant. On examination his blood pressure was 150/90 mm of Hg and basal pulmonary rales on both sides noted. Laboratory investigations showed in the table. Kidney biopsy was done after 4 sessions of hemodialysis which showed 25 glomeruli in light microscopy(LM) of which 3 glomeruli showed segmental collapse. Evidence of acute tubular necrosis (ATN) is present. Interstitial fibrosis and tubular atrophy (IFTA) was negligible. Immunofluorescence (IF) didn’t show any immune deposits. He was diagnosed with idiopathic CG with AKI and treated with MP of 1gm/day for 3 days followed by 1 mg/kg of prednisolone for one month, later tapered and stopped after 12 weeks. He achieved CR (table). He maintained remission during 7 years of follow up. Case 2- A 36-year-old male presented to in February 2022 with pedal oedema. His medical and family history, vitals and systemic examination were not contributory. Laboratory parameters showed in the table. Kidney biopsy showed 10 glomeruli in LM of which one showed segmental collapse. IFTA was < 5%. Mild ATN was present. He was followed for 3 years.His diagnosis and treatment response are similar to case1. Discussion: Treatment response is very poor for CG. With only oral steroids the remission rate is 0-20%. Remission rate with cyclophosphamide, calcineurin inhibitors is less than 50%. Renal survival time for CG patients is around 13 months. We are the first to use upfront MP pulse therapy in CG with AKI which resulted in achievement and maintainace of CR during long term follow up. - case 1 case 2 Laboratory parameters At presentation At 12 weeks of treatment At 84 months of follow up At presentation At 12 weeks of treatment At 36 months of follow up Serum creatinine (mg/dl) 8.1 1.0 0.9 3.5 1.2 1.1 Serum albumin (gm/dl) 1.6 3.4 3.8 2.8 3.5 3.6 Urine alumin 4+ Nil Nil 3+ Nil Nil 24 hour urine protein (mg/day) NA 250 180 4000 350 59 HBsAg, Anti HCV, HIV -I/II, Anti nuclear antibodies negative - - - - -