A CF patient with progressive proteinuric renal disease: a CF-specific nodular glomerulosclerosis?

Cystic fibrosis (CF) is a multisystemic disease but without a classical disease-specific renal phenotype. A 32-year-old male patient with CF (ΔF508/ΔF508) presented with a nephrotic syndrome. Renal biopsy revealed nodular glomerulosclerosis (NGS) occurring in the absence of diabetes mellitus, amyloidosis and any other known common cause of NGS. He had a progressive decline in estimated glomerular filtration rate (eGFR) to chronic kidney disease stage V (eGFR <15 mL/min/1.73 m(2)) over a 3-year period despite optimal medical management. This is the fourth reported case of NGS in a patient with CF without diabetes and is the first to originate from a European country. This case supports the concept of a CF-related NGS.