Sustained Remission Following Methylprednisolone Pulse Therapy in Patients with Idiopathic Collapsing Glomerulopathy and Acute Kidney Injury.

Dear Editor,

We report two adult patients with idiopathic collapsing glomerulopathy (CG) and acute kidney injury (AKI) who achieved sustained remission following methylprednisolone pulse (MP) therapy.

Case 1: A 28-year-old male presented in May 2018 with anasarca, dyspnea, and oligoanuria. Laboratory evaluation showed anemia (hemoglobin-8.7 g/dL), severe hypoalbuminemia (1.8 g/dL), serum creatinine 8.2 mg/dL, and metabolic acidosis. Urinalysis revealed 4+ proteinuria; urine protein-creatinine ratio was 2 g/g. Serological tests for ANA, HBsAg, anti-HCV, and HIV were negative. He required four sessions of hemodialysis. Kidney biopsy demonstrated segmental glomerular collapse with podocyte hypertrophy in 3 glomeruli [Figure 1a, b], severe acute tubular necrosis (ATN), and mild interstitial fibrosis and tubular atrophy (IFTA), with negative immunofluorescence (IF). He received MP 1 g/day for three days, followed by oral prednisolone (1 mg/kg/day). Complete remission was achieved, steroids were tapered over 24 weeks, and he remained relapse-free during 7 years of follow-up.

Close

Figure 1:

(a) Periodic acid Schiff-stained section 400x, showing a collapsed glomerular tuft with overlying podocyte hypertrophy and hyperplasia (blue arrow) and surrounding acute tubular necrosis (black arrow), (b) Masson trichrome-stained section 400x, demonstrating segmental glomerular collapse (black arrow) with prominent podocyte proliferation (red arrow), (c) Hematoxylin & eosin-stained section 400x, highlighting a collapsed glomerulus with circumferential podocyte hyperplasia (arrow), and (d) Jones methenamine silver-stained section 400x, showing collapse of the glomerular capillary tuft (red arrow) with hypertrophied podocytes (yellow arrow).

Export to PPT

Case 2: A 36-year-old male presented in February 2022 with pedal edema and weakness. Investigations showed anemia (hemoglobin-11 g/dL), hypoalbuminemia (2.9 g/dL), serum creatinine 3.2 mg/dL, and 24-h proteinuria of 5.7 g. Serology was negative for ANA, HBsAg, anti-HCV, and HIV. Kidney biopsy revealed focal segmental collapse with podocyte hypertrophy in one glomerulus [Figure 1c, d], mild ATN, minimal IFTA, and negative IF. He was treated with MP pulse therapy followed by oral prednisolone tapered over 24 weeks, achieving complete remission without relapse over 3 years of follow-up.

CG is a histopathological variant of focal segmental glomerulosclerosis. In general, the treatment response to CG is very poor. With oral steroids and other immunosuppressants, the remission rate is 0%-65%.¹ The ESRD rate and renal survival time with the above medication is 30%-100% and 13-23 months, respectively.¹ AKI in the CG is rarely reported, and the outcome is decimal.² The data on the use of MP in the management of CG is limited.³

The above cases suggest that early MP pulse therapy may result in sustained remission in adult-onset idiopathic CG presenting with AKI.