@article {10563, title = {Outcome of childhood-onset full-house nephropathy.}, journal = {Nephrol Dial Transplant}, volume = {32}, year = {2017}, month = {2017 Jul 01}, pages = {1194-1204}, abstract = {

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.

}, keywords = {Adolescent, Age of Onset, Child, Disease Progression, Female, Glomerular Filtration Rate, Glomerulonephritis, Humans, Kidney Diseases, Lupus Erythematosus, Systemic, Lupus Nephritis, Male, Proteinuria, Recurrence, Remission Induction, Retrospective Studies, Risk Factors, Survival Rate}, issn = {1460-2385}, doi = {10.1093/ndt/gfw230}, author = {Ruggiero, Barbara and Vivarelli, Marina and Gianviti, Alessandra and Pecoraro, Carmine and Peruzzi, Licia and Benetti, Elisa and Ventura, Giovanna and Pennesi, Marco and Murer, Luisa and Coppo, Rosanna and Emma, Francesco} } @article {7745, title = {Orofacial granulomatosis in children: think about Crohn{\textquoteright}s disease.}, journal = {Dig Liver Dis}, volume = {47}, year = {2015}, month = {2015 Apr}, pages = {338-41}, abstract = {

BACKGROUND: The term orofacial granulomatosis is conventionally used to describe patients with granulomatous lesions affecting the orofacial tissues, in absence of intestinal lesions. Lip swelling and facial swelling are the most common clinical signs. Despite the fact that histologically it is not distinguishable from Crohn{\textquoteright}s disease, and that both diseases have a chronic/recurrent course, the relationship between orofacial granulomatosis and Crohn{\textquoteright}s disease is still debated.

METHODS: Herein we present five cases of orofacial granulomatosis.

RESULTS: All patients presented concomitant Crohn{\textquoteright}s disease, supporting the hypothesis that orofacial granulomatosis and Crohn{\textquoteright}s disease may be one single disease. Thalidomide was effective in inducing remission of oral and intestinal symptoms in all five cases and could be considered a valid treatment opportunity for these patients.

CONCLUSIONS: Orofacial granulomatosis and Crohn{\textquoteright}s disease may be part of the same disease; both may respond to thalidomide.

}, keywords = {Adolescent, Biopsy, Child, Colonoscopy, Crohn Disease, Diagnosis, Differential, Female, Granulomatosis, Orofacial, Humans, Immunosuppressive Agents, Male, Thalidomide}, issn = {1878-3562}, doi = {10.1016/j.dld.2014.12.012}, author = {Lazzerini, Marzia and Martelossi, Stefano and Cont, Gabriele and Bersanini, Chiara and Ventura, Giovanna and Fontana, Massimo and Zuin, Giovanna and Ventura, Alessandro and Taddio, Andrea} }