Focal segmental glomerulosclerosis in desquamative interstitial pneumonia.

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.