Outcome of childhood-onset full-house nephropathy.
BACKGROUND
Patients with full-house nephropathy (FHN) present renal lesions that are indistinguishable from those of lupus nephritis (LN) but lack the systemic features necessary to meet diagnostic criteria for systemic lupus erithematosus (SLE). Some have been reported to develop a delayed SLE with time. The clinical outcome of children having FHN without SLE has never been reported.


METHODS
Children with biopsy-proven FHN were selected after excluding SLE cases by the absence of America College of Rheumatology criteria. The proportion of patients with complete (proteinuria <0.5 g/day) or partial remission (proteinuria ≤50% from baseline), relapse (estimated glomerular filtration rate <25% and/or proteinuria ≥50% from baseline) and progression to Stage III chronic kidney disease (CKD) was described according to age and gender groups with the Kaplan-Meier curve and compared with the Log-rank test. Entity of treatment was summarized by a score at induction (0-6 months) and maintenance (6-18 months). Cox-regression model was performed to test predictors of remission, relapse and progression to CKD.


RESULTS
Among 42 patients (28 pre-pubertal) who met the inclusion criteria, 39 (92.9%) achieved partial and 32 (76.2%) complete remission of nephropathy over 2.78 and 7.51 months of follow-up. At 10 years, the probability of progressing to CKD was 4.8%. Of those achieving remission, 18% had a renal flare mainly within 4 years after remission. Pre-pubertal males achieved complete remission more frequently than other patients but often relapsed; pre-pubertal females were treated more aggressively. Cox-regression analysis did not find independent predictors of remission or relapse.


CONCLUSIONS
The outcome of the patients with FHN we investigated was encouraging. Recurrences are limited to the first 4 years following diagnosis, allowing progressive withdrawal of immunosuppression in patients achieving remission. Evaluation of risk factors for adverse outcome is necessary especially in pre-pubertal children.
IRCCS - Istituto di Ricerche Farmacologiche Mario Negri, Clinical Reasearch Center for Rare Diseases 'Aldo e Cele Daccò', Bergamo, Italy.
Barbara
B
Ruggiero
Division of Nephrology and Dialysis, Children's Hospital Bambino Gesù, IRCCS, Rome, Italy.
Marina
M
Vivarelli
Division of Nephrology and Dialysis, Children's Hospital Bambino Gesù, IRCCS, Rome, Italy.
Alessandra
A
Gianviti
Unit of Nephrology and Dialysis, Santobono Hospital, Naples, Italy.
Carmine
C
Pecoraro
Division of Nephrology, Regina Margherita Children's Hospital, Turin, Italy.
Licia
L
Peruzzi
Pediatric Nephrology, Dialysis and Transplant Unit, Department of Woman and Child Health, Azienda Ospedaliera-University of Padova, Padua, Italy.
Elisa
E
Benetti
Department of Pediatrics, Institute for Maternal and Child Health, IRCCS Burlo Garofolo, Trieste, Italy.
Giovanna
G
Ventura
Department of Pediatrics, Institute for Maternal and Child Health, IRCCS Burlo Garofolo, Trieste, Italy.
Marco
M
Pennesi
Pediatric Nephrology, Dialysis and Transplant Unit, Department of Woman and Child Health, Azienda Ospedaliera-University of Padova, Padua, Italy.
Luisa
L
Murer
Division of Nephrology, Regina Margherita Children's Hospital, Turin, Italy.
Rosanna
R
Coppo
Division of Nephrology and Dialysis, Children's Hospital Bambino Gesù, IRCCS, Rome, Italy.
Francesco
F
Emma
D000293:Adolescent; D017668:Age of Onset; D002648:Child; D018450:Disease Progression; D005260:Female; D005919:Glomerular Filtration Rate; D005921:Glomerulonephritis / Q000175:diagnosis / Q000209:etiology / Q000401:mortality*; D006801:Humans; D007674:Kidney Diseases / Q000175:diagnosis / Q000209:etiology / Q000401:mortality*; D008180:Lupus Erythematosus, Systemic / Q000150:complications*; D008181:Lupus Nephritis / Q000175:diagnosis / Q000209:etiology / Q000401:mortality*; D008297:Male; D011507:Proteinuria / Q000473:pathology; D012008:Recurrence; D012074:Remission Induction; D012189:Retrospective Studies; D012307:Risk Factors; D015996:Survival Rate
children; full-house nephropathy; lupus-like nephritis; rare diseases; seronegative lupus nephritis