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Focal segmental glomerulosclerosis in desquamative interstitial pneumonia
K J Sheth1, H E Leichter, G Kishaba
1Department of Pediatrics, Children's Hospital of Wisconsin, Medical College of Wisconsin, Milwaukee.
Abstract:
Renal involvement in desquamative interstitial pneumonitis (DIP) manifesting as chronic renal failure has been reported only once. An uncommon disorder in children, DIP has been associated with a variety of systemic disorders and has an immune-mediated pathogenesis. A 16-year-old Black male was diagnosed to have DIP on lung biopsy at the age of 10 months. He was first noted to have proteinuria at age 5 which progressed to nephrotic syndrome by age 13 when the laboratory tests showed elevated IgG, normal serum complement, increased circulating immune complexes and absent anti-GBM antibodies. A percutaneous renal biopsy specimen performed at age 13 revealed focal segmental glomerulosclerosis. Despite prednisone treatment of 2 mg/kg/day for 12 weeks, renal failure progressed requiring hemodialysis. Pulmonary functions, although reduced, remained stable.
Insights
Desquamative interstitial pneumonitis (DIP) rarely affects the kidneys, leading to chronic renal failure. This case highlights a pediatric DIP patient who developed focal segmental glomerulosclerosis and required hemodialysis.
Area of Science:
- Pediatric Nephrology
- Pulmonology
- Immunology
Background:
- Desquamative interstitial pneumonitis (DIP) is a rare lung disorder with immune-mediated pathogenesis.
- Renal involvement in DIP is exceptionally uncommon, with only one prior report of chronic renal failure.
Observation:
- A pediatric patient diagnosed with DIP at 10 months old developed proteinuria at age 5.
- Proteinuria progressed to nephrotic syndrome by age 13, with elevated IgG and immune complexes.
- Renal biopsy revealed focal segmental glomerulosclerosis despite treatment.
Findings:
- The patient experienced progressive renal failure requiring hemodialysis.
- Pulmonary function, though reduced, remained stable throughout the observation period.
- Laboratory findings indicated an immune-mediated process without anti-GBM antibodies.
Implications:
- This case underscores the potential for severe renal complications in pediatric DIP.
- Further research into the immune mechanisms linking DIP and renal disease is warranted.
- Highlights the importance of monitoring renal function in children diagnosed with DIP.