Renal involvement in cystic fibrosis: diseases spectrum and clinical relevance.
OBJECTIVE
Clinically relevant kidney involvement is uncommonly described in adult patients with cystic fibrosis (CF). We sought to report on a series of patients with CF and kidney biopsy-documented renal involvement.


METHODS
A retrospective study was undertaken in two referral centers for adult patients with CF in Paris, France. Patients who had undergone a biopsy of native kidneys between 1992 and 2008 were identified, and their medical records were reviewed.


RESULTS
We identified 13 adult patients with CF and renal disease. Proteinuria was present in all but two cases and was associated with progressive renal impairment in four patients (median serum creatinine 85 micromol/L; range 53 to 144 micromol/L). Renal biopsy disclosed a heterogeneous spectrum of nephropathies including AA amyloidosis (n = 3), diabetic glomerulopathy (n = 3), FSGS (n = 2), minimal-change disease (n = 1), postinfectious glomerulonephritis (n = 1), IgA nephropathy related to Henoch-Schönlein purpura (n = 1), membranous nephropathy (n = 1), and chronic interstitial nephropathy (n = 1). Chronic renal failure occurred in five patients, and one patient reached ESRD.


CONCLUSIONS
Although rare, clinically significant renal disease may arise in young adult patients with CF. Given the wide spectrum of diseases that may be encountered, definite diagnosis by kidney biopsy is mandatory to optimize clinical treatment of these complex patients, particularly in the perspective of organ transplantation.
Yahiaoui
Yasmina
Y
Department of Nephrology, Hôpital du Kremlin Bicêtre, 78 rue du Général Leclerc 94275 Le Kremlin-Bicetre, France. yyasmina@free.fr
Jablonski
Mathieu
M
Hubert
Dominique
D
Mosnier-Pudar
Helen
H
Noël
Laure-Hélène
LH
Stern
Marc
M
Grenet
Dominique
D
Grünfeld
Jean-Pierre
JP
Chauveau
Dominique
D
Fakhouri
Fadi
F
D000328:Adult; D000686:Amyloidosis / Q000453:epidemiology / Q000473:pathology; D001706:Biopsy; D003550:Cystic Fibrosis / Q000453:epidemiology*; D003928:Diabetic Nephropathies / Q000453:epidemiology / Q000473:pathology; D005260:Female; D005500:Follow-Up Studies; D005921:Glomerulonephritis / Q000453:epidemiology / Q000473:pathology; D005922:Glomerulonephritis, IGA / Q000453:epidemiology / Q000473:pathology; D006801:Humans; D015994:Incidence; D007668:Kidney / Q000473:pathology*; D007674:Kidney Diseases / Q000453:epidemiology* / Q000473:pathology*; D007676:Kidney Failure, Chronic / Q000453:epidemiology / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009402:Nephrosis, Lipoid / Q000453:epidemiology / Q000473:pathology; D010297:Paris / Q000453:epidemiology; D012189:Retrospective Studies; D055815:Young Adult