[Case of a focal segmental glomerulosclerosis collapsing variant associated with a hypertrophic column of Bertin]

Area of Science:

• Nephrology
• Pathology
• Internal Medicine

Background:

• Focal segmental glomerulosclerosis (FSGS) collapsing variant is a severe form of kidney disease.
• It is characterized by poor response to standard treatments and a grave prognosis.
• Early diagnosis and intervention are crucial for managing FSGS collapsing variant.

Observation:

• A 59-year-old Japanese male presented with anasarca, weight gain, and hypertension.
• Initial investigations revealed elevated serum creatinine and proteinuria, with imaging suggesting a hypertrophic column of Bertin.
• Renal biopsy confirmed focal segmental glomerulosclerosis collapsing variant with podocyte abnormalities.

Findings:

• The patient's condition initially worsened, with increased serum creatinine and decreased albumin.
• Following treatment with steroid pulse therapy and LDL apheresis, serum creatinine decreased to 1.1 mg/dL and urinary protein to 2.5 g/gCr.
• This combined therapy demonstrated significant efficacy in improving renal function and reducing proteinuria.

Implications:

• Combined steroid pulse and LDL apheresis therapy can be effective in managing focal segmental glomerulosclerosis collapsing variant.
• This approach offers a potential therapeutic strategy for patients with this aggressive kidney disease.
• Further research is warranted to explore the long-term efficacy and broader applicability of this treatment combination.