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1. Surgical and oncological outcomes of hepatic resection for BCLC-B hepatocellular carcinoma: a retrospective multicenter analysis among 474 consecutive cases

Author

Di Sandro S., Centonze L., Pinotti E., Lauterio A., De Carlis R., Romano F., Gianotti L., De Carlis L., Cena T., Bagnardi V., Benuzzi L., Pezzoli I., Danieli M., Ciravegna A., Di Sandro, S, Centonze, L, Pinotti, E, Lauterio, A, De Carlis, R, Romano, F, Gianotti, L, De Carlis, L, Cena, T, Bagnardi, V, Benuzzi, L, Pezzoli, I, Danieli, M, and Ciravegna, A

Subjects
Adult, Male, medicine.medical_specialty, Carcinoma, Hepatocellular, Adolescent, Hepatocellular carcinoma, Hepatic resection, 030230 surgery, 03 medical and health sciences, Young Adult, 0302 clinical medicine, Retrospective Studie, Medicine, Effective treatment, Hepatectomy, Humans, In patient, Stage (cooking), Liver surgery, Aged, Neoplasm Staging, Retrospective Studies, Liver resection, business.industry, Incidence (epidemiology), Liver Neoplasms, Perioperative, Middle Aged, medicine.disease, Surgery, BCLC, Survival Rate, Treatment Outcome, Liver Neoplasm, 030220 oncology & carcinogenesis, Female, Neoplasm Recurrence, Local, business, Liver cancer, Human
Abstract

The Barcelona clinic liver cancer (BCLC) algorithm is the most widely accepted staging system form hepatocellular carcinoma (HCC). Liver resection is traditionally proposed to early stage HCC (BCLC-0/A), even if recent reports have shown that surgical resection could provide a safe and effective treatment also for intermediate-stage HCC (BCLC-B). In this study, we focused on surgical and oncological outcomes of hepatic resection in BLCB-B patients. Patients who received hepatic resection for early- (BCLC-0/A) or intermediate-stage (BCLC-B) HCC in two tertiary hepatobiliary centers between January 2003 and December 2016 were included in study. Four-hundred and twenty-nine patients were included in the analysis. At the time of resection, 298 patients were classified as BCLC-A/0 and 131 as BCLC-B. Despite a higher complication rate in BCLC-B group (49.6% vs 32.9%; p = 0.001), the incidence of clinically relevant complications did not differ significantly between the two groups (16.0% vs 10.1%; p = 0.079); moreover, postoperative mortality (4.6% vs 2.7%; p = 0.309) and relapse-free survival (RFS) were similar between BCLC-0/A and BCLC-B group (1-, 3-, and 5-year RFS: 74, 43, and 31% vs 59, 38, and 34%; p = 0.180). Overall survival was slightly worse in BCLC-B group (1-, 3-, and 5-year overall survival of 89, 70, and 52% vs. 77, 51, and 44%; p = 0.004). Focusing on BCLC-B group, a Child–Pugh score B (HR 2.47; p = 0.003), growing number of nodules (HR 3.04; p = 0.003), and R1 resection (HR 2.43; p = 0.005) beard a higher risk of tumor recurrence, while overall survival was negatively affected by the presence of more than two nodules (HR 3.66; p = 0.0001) and R1 resection (HR 3.06; p = 0.0001); patients presenting single-large HCC experienced a better overall survival (HR 0.53; p = 0.014) and lower recurrence-rate (HR 0.60; p = 0.046). Hepatic resection for intermediate-stage HCC shows acceptable results in terms of perioperative morbidity and mortality, with better oncological outcomes in patients with lower number of lesions despite of their size.

Published
2019

2. Clinical features and follow-up in patients with 22q11.2 deletion syndrome

Author

Cancrini, C, Puliafito, P, Digilio, M, Soresina, A, Martino, S, Rondelli, R, Consolini, R, Ruga, E, Cardinale, F, Finocchi, A, Romiti, Ml, Martire, B, Bacchetta, R, Albano, V, Carotti, A, Specchia, F, Montin, D, Cirillo, E, Cocchi, G, Trizzino, A, Bossi, G, Milanesi, O, Azzari, C, Corsello, G, Pignata, C, Aiuti, A, Pietrogrande, M, Marino, B, Ugazio, A, Plebani, A, Rossi, P, Pierani, P, Gabrielli, A, Danieli, M, De Mattia, D, Sisto, C, Dammacco, F, Ranieri, G, Pession, A, Ricci, G, Minelli, P, Lougaris, V, Badolato, R, Cattaneo, R, Airò, P, Mura, R, Cossu, F, Del Giacco, S, Manconi, P, Consarino, C, Dello Russo, A, Miniero, R, Anastasio, E, Marino, S, Russo, G, Paganelli, R, Sperlì, D, Carpino, L, Aricò, M, Gambineri, E, Lippi, F, Canessa, C, Maggi, E, Romagnani, S, Matucci, A, Vultaggio, A, Gattorno, M, Castagnola, E, Nigro, G, Presta, G, Civino, A, Buzi, F, Gambaretto, G, Fasoli, S, Salpietro, C, Gallizzi, R, Dellepiane, R, Panisi, C, Fabio, G, Carrabba, M, Roncarolo, M, Biondi, A, Vallinoto, C, Poggi, V, Menna, G, Di Nardo, R, Sottile, R, Marone, G, Spadaro, G, Carli, M, Basso, G, Putti, C, Semenzato, G, Agostini, C, D'Angelo, P, Izzi, G, Bertolini, P, Zecca, M, Marseglia, G, Maccario, R, Felici, L, Favre, C, Vecchi, V, Sacchini, P, Rinaldi, G, Livadiotti, S, Simonetti, A, Stabile, A, Duse, M, Iacobini, M, Quinti, I, Fiorilli, M, Moschese, V, Cecere, F, D'Ambrosio, A, De Zan, G, Strafella, S, Tamaro, P, Rabusin, M, Tommasini, A, Tovo, P, De Carli, M, De Carli, S, Nespoli, L, Marinoni, M, Porcellini, A, Lunardi, C, Patuzzo, G, Boner, A, Degani, D, Cancrini, C, Puliafito, P, Digilio, Mc, Soresina, A, Martino, S, Rondelli, R, Consolini, R, Ruga, Em, Cardinale, F, Finocchi, A, Romiti, Ml, Martire, B, Bacchetta, R, Albano, V, Carotti, A, Specchia, F, Montin, D, Cirillo, E, Cocchi, G, Trizzino, A, Bossi, G, Milanesi, O, Azzari, C, Corsello, G, Pignata, C, Aiuti, Alessandro, Pietrogrande, Mc, Marino, B, Ugazio, Ag, Plebani, A, Rossi, P., Cancrini, Caterina, Puliafito, Pamela, Digilio, Maria Cristina, Soresina, Annarosa, Martino, Silvana, Rondelli, Roberto, Consolini, Rita, Ruga, Ezia Maria, Cardinale, Fabio, Finocchi, Andrea, Romiti, Maria Luisa, Martire, Baldassarre, Bacchetta, Rosa, Albano, Veronica, Carotti, Adriano, Specchia, Fernando, Montin, Davide, Cirillo, Emilia, Cocchi, Guido, Trizzino, Antonino, Bossi, Grazia, Milanesi, Ornella, Azzari, Chiara, Corsello, Giovanni, Pignata, Claudio, Pietrogrande, Maria Cristina, Marino, Bruno, Ugazio, Alberto Giovanni, Plebani, Alessandro, Rossi, Paolo, Aiuti, A, Rossi, P, Pierani, P, Gabrielli, A, Danieli, Mg, De Mattia, D, Sisto, C, Dammacco, F, Ranieri, G, Pession, A, Ricci, G, Minelli, P, Lougaris, V, Badolato, R, Cattaneo, R, Airò, P, Mura, Rm, Cossu, F, Del Giacco, S, Manconi, Pe, Consarino, C, Dello Russo, Am, Miniero, R, Anastasio, E, Marino, S, Russo, G, Paganelli, R, Sperlì, D, Carpino, L, Aricò, M, Gambineri, E, Lippi, F, Canessa, C, Maggi, E, Romagnani, S, Matucci, A, Vultaggio, A, Gattorno, M, Castagnola, E, Nigro, G, Presta, G, Civino, A, Buzi, F, Gambaretto, G, Fasoli, S, Salpietro, C, Gallizzi, R, Dellepiane, Rm, Panisi, C, Fabio, G, Carrabba, M, Pietrogrande, M, Roncarolo, Mg, Biondi, A, Vallinoto, C, Poggi, V, Menna, G, Di Nardo, R, Sottile, R, Marone, G, Spadaro, G, Carli, M, Basso, G, Putti, C, Semenzato, G, Agostini, C, D'Angelo, P, Izzi, G, Bertolini, P, Zecca, M, Marseglia, G, Maccario, R, Felici, L, Favre, C, Vecchi, V, Sacchini, P, Rinaldi, G, Livadiotti, S, Simonetti, A, Stabile, A, Duse, M, Iacobini, M, Quinti, I, Fiorilli, M, Moschese, V, Cecere, F, D'Ambrosio, A, De Zan, G, Strafella, S, Tamaro, Paolo, Rabusin, M, Tommasini, A, Tovo, P, De Carli, M, De Carli, S, Nespoli, L, Marinoni, M, Porcellini, A, Lunardi, C, Patuzzo, G, Boner, A, Degani, D., Cancrini, C., Pulisfito, P., Digilio, M. C., Soresina, A., Martino, S., Rondelli, R., Consolini, R., Ruga, E. M., C. a. r. d. i. n. a. l. e., F., Finocchi, A., Romiti, M. L., Martire, B., Bacchetta, R., Albano, V., Carotti, A., Specchia, F., Montin, D., Cocchi, G., Trizzino, A., Bossi, G., Milanesi, O., Azzari, C., Corsello, G., Aiuti, A., Pietrogrande, M. C., Marino, B., Ugazio, A. G., Plebani, A., Digilio, MC, Ruga, EM, Romiti, ML, trizzino, A, Aiuti, Pietrogrande, MC, and Ugazio, AG

Subjects
Male, Pediatrics, 22q11.2 deletion, Delayed Diagnosis, Time Factors, Chromosomes, Human, Pair 22, Developmental Disabilities, digeorge syndrome, Sex Factor, Severity of Illness Index, Retrospective Studie, DiGeorge syndrome, Early Diagnosi, Age Factor, Prospective Studies, Neonatal hypocalcemia, Prospective cohort study, Child, medicine.diagnostic_test, Delayed Diagnosi, Primary immune disorders, Age Factors, del 22q, MIM, Abnormalities, Multiple, Adolescent, Adult, Child, Preschool, DiGeorge Syndrome, Early Diagnosis, Female, Follow-Up Studies, Genetic Testing, Humans, Infant, Infant, Newborn, Monitoring, Physiologic, Retrospective Studies, Risk Assessment, Sex Factors, Young Adult, Disease Progression, Cohort, Abnormalities, Multiple, Pediatrics, Perinatology and Child Health, Human, medicine.medical_specialty, Time Factor, Monitoring, Developmental Disabilitie, Italian Association of Pediatric Haematology and Oncology, Context (language use), Chromosomes, Follow-Up Studie, Severity of illness, medicine, 22q11DS, 22q11.2 deletion syndrome, AIEOP, Mendelian Inheritance in Man, Preschool, Physiologic, Genetic testing, Settore MED/38 - Pediatria Generale e Specialistica, business.industry, Retrospective cohort study, medicine.disease, Newborn, Prospective Studie, Pair 22, business
Abstract

Objective To investigate the clinical manifestations at diagnosis and during follow-up in patients with 22q11.2 deletion syndrome to better define the natural history of the disease. Study design A retrospective and prospective multicenter study was conducted with 228 patients in the context of the Italian Network for Primary Immunodeficiencies. Clinical diagnosis was confirmed by cytogenetic or molecular analysis. Results The cohort consisted of 112 males and 116 females; median age at diagnosis was 4 months (range 0 to 36 years 10 months). The diagnosis was made before 2 years of age in 71% of patients, predominantly related to the presence of heart anomalies and neonatal hypocalcemia. In patients diagnosed after 2 years of age, clinical features such as speech and language impairment, developmental delay, minor cardiac defects, recurrent infections, and facial features were the main elements leading to diagnosis. During follow-up (available for 172 patients), the frequency of autoimmune manifestations ( P = .015) and speech disorders ( P = .002) increased. After a median follow-up of 43 months, the survival probability was 0.92 at 15 years from diagnosis. Conclusions Our data show a delay in the diagnosis of 22q11.2 deletion syndrome with noncardiac symptoms. This study provides guidelines for pediatricians and specialists for early identification of cases that can be confirmed by genetic testing, which would permit the provision of appropriate clinical management.

Published
2014

3. Immunosuppressive therapy in lupus nephritis: the Euro-Lupus Nephritis Trial, a randomized trial of low-dose versus high-dose intravenous cyclophosphamide

Author

Houssiau, F, Vasconcelos, C, D'Cruz, D, Sebastiani, G, De Ramon Garrido, E, Danieli, M, Abramovicz, D, Blockmans, D, Mathieu, A, Direskeneli, H, Galeazzi, M, Gül, A, Levy, Y, Petera, P, Popovic, R, Petrovic, R, Cattaneo, R, Font, J, Depresseux, G, Cosyns, J, Cervera, R., SINICO, RENATO ALBERTO, Houssiau, F, Vasconcelos, C, D'Cruz, D, Sebastiani, G, De Ramon Garrido, E, Danieli, M, Abramovicz, D, Blockmans, D, Mathieu, A, Direskeneli, H, Galeazzi, M, Gül, A, Levy, Y, Petera, P, Popovic, R, Petrovic, R, Sinico, R, Cattaneo, R, Font, J, Depresseux, G, Cosyns, J, and Cervera, R

Subjects
Adult, Male, Kidney Function Test, Adolescent, Dose-Response Relationship, Drug, Immunology, Remission Induction, Injections, Intravenou, Lupus Nephriti, Middle Aged, Kidney Function Tests, Lupus Nephritis, Immunosuppressive Agent, Rheumatology, Azathioprine, Injections, Intravenous, Humans, Female, Single-Blind Method, Treatment Failure, Cyclophosphamide, Immunosuppressive Agents, Human, Aged
Abstract

Objective. Glomerulonephritis is a severe manifestation of systemic lupus erythematosus (SLE) that is usually treated with an extended course of intravenous (IV) cyclophosphamide (CYC). Given the side effects of this regimen, we evaluated the efficacy and the toxicity of a course of low-dose IV CYC prescribed as a remission-inducing treatment, followed by azathioprine (AZA) as a remission-maintaining treatment. Methods. In this multicenter, prospective clinical trial (the Euro-Lupus Nephritis Trial [ELNT]), we randomly assigned 90 SLE patients with proliferative glomerulonephritis to a high-dose IV CYC regimen (6 monthly pulses and 2 quarterly pulses; doses increased according to the white blood cell count nadir) or a low-dose IV CYC regimen (6 fortnightly pulses at a fixed dose of 500 mg), each of which was followed by AZA. Intent-to-treat analyses were performed. Results. Followup continued for a median of 41.3 months in the low-dose group and 41 months in the high-dose group. Sixteen percent of those in the low-dose group and 20% of those in the high-dose group experienced treatment failure (not statistically significant by Kaplan-Meier analysis). Levels of serum creatinine, albumin, C3, 24-hour urinary protein, and the disease activity scores significantly improved in both groups during the first year of followup. Renal remission was achieved in 71% of the low-dose group and 54% of the high-dose group (not statistically significant). Renal flares were noted in 27% of the low-dose group and 29% of the high-dose group. Although episodes of severe infection were more than twice as frequent in the high-dose group, the difference was not statistically significant. Conclusion. The data from the ELNT indicate that in European SLE patients with proliferative lupus nephritis, a remission-inducing regimen of low-dose IV CYC (cumulative dose 3 gm) followed by AZA achieves clinical results comparable to those obtained with a high-dose regimen

Published
2002

4. Five-year follow-up of 165 Italian patients with undifferentiated connective tissue diseases

Author

Danieli, M. G., Fraticelli, P., Franceschini, F., Cattaneo, R., Farsi, A., Passaleva, A., Pietrogrande, M., Invernizzi, F., Vanoli, M., Scorza, R., Sabbadini, M. G., Gerli, R., Corvetta, A., Farina, G., Salsano, Felice, Priori, R., Valesini, Guido, Danieli, G., and Sep, Oct

Subjects
Adult, Male, Vasculitis, Adolescent, Anti-Inflammatory Agents, Raynaud Disease, Middle Aged, Autoimmune Diseases, Treatment Outcome, Italy, Antibodies, Antinuclear, Disease Progression, Humans, Female, Steroids, Prospective Studies, Connective Tissue Diseases, undifferentiated ctd, connective tissue diseases, autoimmune diseases, outcome, connective tissue diseases (ctds), Aged, Follow-Up Studies
Abstract

To study those conditions with a proven or hypothesised immunologic pathogenesis and denominated under a working definition of undifferentiated connective tissue diseases (UCTD).A multicentre prospective study was organised involving 10 tertiary referral centers of internal medicine in Italy, with the aim of describing the natural history of UCTD and the prevalence of its different clinical and immunological manifestations.After a five-year follow-up period, data on 165 patients were available for analysis. UCTDs occur mainly in females in their fourth decade of life. Articular and mucocutaneous features and Raynaud's phenomenon represent the most common findings. Nevertheless, we also detected a relatively high incidence of permanent major organ damage. Regarding the immunologic parameters, we documented some conflicting results in the correlation between serologic abnormalities and clinical features. In 10 patients UCTD evolved to a major disease, generally systemic lupus erythematosus or Sjögren's syndrome.A low rate of evolution to a defined autoimmune disease, the limited use of steroid or immunosuppressive therapy, and a favourable course in the majority of cases are the main characteristics of patients with UCTDs.

Published
1999

5. Differential risk of non-Hodgkin's lymphoma in Italian patients with primary Sjogren's syndrome

Author

Valesini, G., Priori, R., Bavoillot, D., Osborn, J., Danieli, M. G., Nicoletta Del Papa, Gerli, R., Pietrogrande, M., Sabbadini, M. G., Silvestris, F., and Valsecchi, L.

Subjects
Adult, Aged, 80 and over, Male, Incidence, Lymphoma, Non-Hodgkin, Middle Aged, Sjogren's Syndrome, Italy, Risk Factors, Splenomegaly, Prevalence, Humans, Female, Lymph Nodes, Age of Onset, Aged, Autoantibodies
Abstract

To assess the incidence of non-Hodgkin's lymphoma (NHL) and estimate the relative risk (RR) of developing lymphoproliferative complications in a large population of Italian patients with Sjögren's syndrome (SS) and to ascertain if any difference exists between the north and centre-south of Italy.Differential relative risks of NHL were obtained by comparing the number of observed cases with cases identified on the basis of age-sex-time specific incidence rates extracted from regional cancer registries.Among the 331 patients with SS studied, 9 cases of NHL occurred, while no lymphoid malignancy appeared in patients with overlapping connective tissue disease (secondary SS) or in males with primary SS. As the number of NHL cases identified on the basis of the rate in the cancer registries would have been 0.27, the RR is 33.3 (p0.001). The incidence rate of NHL in Italian patients with SS is about 5.1/1000 person-years. 5.4/1000 per year in the north of the country and 4.8/1000 per year in the centre-south. The relative risks are, respectively, 34.7 and 32.5.Italian patients with primary SS have increased risk of developing NHL. In this group, the absence of a significant difference between the north and the centre-south of Italy contrasts with the higher incidence of NHL in the general population of northern regions and strengthens the direct connection between primary SS and NHL.

6. Risk factors for Sjögren's syndrome: A case-control study

Author

Priori, R., Emanuela Medda, Conti, F., Cassarà, E. A. M., Sabbadini, M. G., Antonioli, C. M., Gerli, R., Danieli, M. G., Giacomelli, R., Pietrogrande, M., Valesini, G., and Stazi, M. A.

Subjects
Adult, Male, rheumatoidarthritis, smoking, Autoimmune Diseases, autoimmune-diseases, Odds Ratio, Humans, risk factors, Life Style, Reproductive History, Aged, primary sjogrens syndrome, transfusions, sjögren's syndrome, Middle Aged, Health Surveys, Pedigree, familial aggregation, Logistic Models, Sjogren's Syndrome, Case-Control Studies, Female, epidemiology, pregnancy
Abstract

The aim of this study was to investigate potential risk factors for Sjögren's syndrome (SS) by means of a multi-centre case-control study, focusing in particular on familial and environmental risk factors. 140 female SS patients and 109 female controls with orthopaedic problems were consecutively enrolled in seven university hospitals in Italy.Information regarding the patient's lifestyle, her medical, menstrual and pregnancy history, and any family history of autoimmune diseases (AD) was obtained through a detailed structured questionnaire. The odds ratio (OR) and 95% confidence interval (95%CI) were calculated using unconditional logistic regression, adjusting for age and family size. The probability of first-degree relatives developing an autoimmune disease was also investigated.A positive family history of AD was significantly associated with SS. Subjects with a first-degree relative (FDR) with AD showed a seven-fold increase in the risk for SS compared to controls (OR=7.4, 95%CI 2.8-20.1); the strength of this association increased with the number of relatives affected. Similarly, the FDR of SS patients had a higher risk of AD in comparison to subjects without FDR affected by SS. Women with one or more pregnancies had an increased risk of SS (OR=2.1, 95%CI 1.0-4.3).This study suggests that a family history of AD is associated with SS.

7. The Italian Registry for Primary Immunodeficiencies (Italian Primary Immunodeficiency Network; IPINet): Twenty Years of Experience (1999–2019)

Author

A G Ugazio, Giovanna Russo, Maria Giovanna Danieli, Alberto Tommasini, Maria Cristina Pietrogrande, Raffaele Badolato, Andrea Pession, Carlo Agostini, Fabio Cardinale, Eleonora Gambineri, Baldassare Martire, Adele Civino, Manuela Baronio, Marco Gattorno, Marco Zecca, Viviana Moschese, Chiara Azzari, Alessio Benvenuto, Marzia Duse, Antonino Trizzino, Luisa Gazzurelli, Isabella Quinti, Vassilios Lougaris, Andrea Matucci, Giuseppe Spadaro, Claudio Lunardi, Angelo Vacca, Roberto Rondelli, Maria Caterina Putti, Luciana Chessa, Giovanna Fabio, Andrea Biondi, Fausto Cossu, Roberto Paganelli, Paolo Rossi, Rita Consolini, Alessandro Aiuti, Gian Luigi Marseglia, Luigi Carpino, Caterina Cancrini, Maddalena Marinoni, Silvana Martino, Claudio Pignata, Annarosa Soresina, Patrizia Bertolini, Alessandro Plebani, Lougaris, V., Pession, A., Baronio, M., Soresina, A., Rondelli, R., Gazzurelli, L., Benvenuto, A., Martino, S., Gattorno, M., Biondi, A., Zecca, M., Marinoni, M., Fabio, G., Aiuti, A., Marseglia, G., Putti, M. C., Agostini, C., Lunardi, C., Tommasini, A., Bertolini, P., Gambineri, E., Consolini, R., Matucci, A., Azzari, C., Danieli, M. G., Paganelli, R., Duse, M., Cancrini, C., Moschese, V., Chessa, L., Spadaro, G., Civino, A., Vacca, A., Cardinale, F., Martire, B., Carpino, L., Trizzino, A., Russo, G., Cossu, F., Badolato, R., Pietrogrande, M. C., Quinti, I., Rossi, P., Ugazio, A., Pignata, C., Plebani, A., Lougaris, V, Pession, A, Baronio, M, Soresina, A, Rondelli, R, Gazzurelli, L, Benvenuto, A, Martino, S, Gattorno, M, Biondi, A, Zecca, M, Marinoni, M, Fabio, G, Aiuti, A, Marseglia, G, Putti, M, Agostini, C, Lunardi, C, Tommasini, A, Bertolini, P, Gambineri, E, Consolini, R, Matucci, A, Azzari, C, Danieli, M, Paganelli, R, Duse, M, Cancrini, C, Moschese, V, Chessa, L, Spadaro, G, Civino, A, Vacca, A, Cardinale, F, Martire, B, Carpino, L, Trizzino, A, Russo, G, Cossu, F, Badolato, R, Pietrogrande, M, Quinti, I, Rossi, P, Ugazio, A, Pignata, C, and Plebani, A

Subjects
Adult, Male, medicine.medical_specialty, Pediatrics, Primary immunodeficiencies, Adolescent, Databases, Factual, Primary Immunodeficiency Diseases, Immunology, Age at diagnosis, History, 21st Century, Combined immunodeficiencies, Young Adult, Medical microbiology, patient registry, Epidemiology, medicine, Prevalence, Immunology and Allergy, Humans, Registries, Geography, Medical, Child, Adult patients, business.industry, Infant, Newborn, Infant, History, 20th Century, medicine.disease, Prognosis, Settore MED/38, Natural history, Italy, Child, Preschool, Population Surveillance, Cohort, Primary immunodeficiency, Original Article, Female, business
Abstract

Primary immunodeficiencies (PIDs) are heterogeneous disorders, characterized by variable clinical and immunological features. National PID registries offer useful insights on the epidemiology, diagnosis, and natural history of these disorders. In 1999, the Italian network for primary immunodeficiencies (IPINet) was established. We report on data collected from the IPINet registry after 20 years of activity. A total of 3352 pediatric and adult patients affected with PIDs are registered in the database. In Italy, a regional distribution trend of PID diagnosis was observed. Based on the updated IUIS classification of 2019, PID distribution in Italy showed that predominantly antibody deficiencies account for the majority of cases (63%), followed by combined immunodeficiencies with associated or syndromic features (22.5%). The overall age at diagnosis was younger for male patients. The minimal prevalence of PIDs in Italy resulted in 5.1 per 100.000 habitants. Mortality was similar to other European registries (4.2%). Immunoglobulin replacement treatment was prescribed to less than one third of the patient cohort. Collectively, this is the first comprehensive description of the PID epidemiology in Italy.

Published
2020

8. Initial presenting manifestations in 16,486 patients with inborn errors of immunity include infections and noninfectious manifestations

Author

Tim Niehues, Catherine Waruiru, Conleth Feighery, Uwe Schauer, Virginie Courteille, Kai Lehmberg, Ingo Müller, I. Esteves, Henner Morbach, Michael Borte, Patrick Hundsdoerfer, Klaus Schwarz, Ewelina Gowin, Alessandro Aiuti, Andreas Holbro, Federica Barzaghi, João Farela Neves, Dagmar Graf, Hannah Tamary, Veneta Milenova, Benedikt Boetticher, Eleonora Gambineri, Vera Goda, Alia Eldash, Jan-Christian Wasmuth, Fabio Candotti, Svetlana O. Sharapova, Markus Metzler, Juergen Brunner, Anna Hilfanova, Brindusa Ruxandra Capilna, Pere Soler-Palacín, Arnau Antolí, Horst von Bernuth, Vassilios Lougaris, Maria Carrabba, Bernd H. Belohradsky, Julian Thalhammer, Nathalie de Vergnes, Peter Olbrich, Peter Kopač, Leif G. Hanitsch, Alexandra Nieters, Filomeen Haerynck, Juliana Gabzdilova, Sezin Aydemir, Rabab El Hawary, Patrick F.K. Yong, Maria Giovanna Danieli, Alberto Tommasini, Sandra Steinmann, Ulrich Baumann, Figen Dogu, Elisabeth Förster-Waldl, Carolina Marasco, Donato Amodio, Lorenzo Lodi, Xavier Solanich, Caterina Cancrini, Brigita Sitkauskiene, Torsten Witte, Clementina Vanessa, Nima Rezaei, Jean-Christophe Goffard, Kirsten Wittke, Emmanouil Liatsis, Helen Baxendale, Susana L. Silva, Bodo Grimbacher, Henrike Ritterbusch, Evangelia Farmaki, Safa Meshaal, Sujal Ghosh, Larysa Kostyuchenko, David Edgar, Simone Cesaro, R Zeuner, Nerea Salmón Rodríguez, Isabella Quinti, Stephan Ehl, Pauline Brosselin, Joerg C. Henes, Pilar Llobet Agulló, Rosa Maria Dellepiane, Andrea Meinhardt, Marina Kojić, Georgios Sogkas, Stephan Borte, Catharina Schuetz, Suheyla Ocak, Karin Marschall, Lukas M. Gasteiger, Stefan Raffac, Sofia Tantou, Sadia Noorani, Matthaios Speletas, Philippe Randrianomenjanahary, Ursula Holzer, Ayca Kiykim, Johannes G. Liese, Angelo Vacca, Gisela Fecker, Ekrem Unal, Koen J. van Aerde, Alba Parra-Martínez, Kaan Boztug, Sophie Stiehler, Sybille Landwehr-Kenzel, Claudio Pignata, Jennifer Neubert, Janine Reichenbach, Shahnaz Parvin, Sarah Goddard, Andrea Schroll, Dirk Holzinger, Asghar Aghamohammadi, Hassan Abolhassani, Johannes Trück, Estela Paz-Artal, Shereen M. Reda, Anna Shcherbina, Maria Raptaki, Jaroslava Orosova, Beata Wolska-Kuśnierz, Tessa Kerre, Gerrit Ahrenstorf, Ben Zion Garty, Dirk Foell, Benjamin Becker, Ulrike F. Demel, Androniki Kapousouzi, Abraham Rutgers, Klaus Warnatz, Gemma Rocamora Blanch, Stephan Rusch, Luis M. Allende, Dalia Abd Elaziz, Safa Baris, Jorisvan Montfrans, Dominik T. Schneider, Raphael Scheible, Juana Gil-Herrera, Gerhard Kindle, Annarosa Soresina, Giovanna Fabio, Uwe Wintergerst, Emilia Faria, Maria Fasshauer, Silvia Ricci, Aisha Elmarsafy, Barbara Pietrucha, Carsten Speckmann, Nizar Mahlaoui, Ulrich Heininger, Isabelle Meyts, Matthew Buckland, Efimia Papadopoulou-Alataki, Robin Kobbe, A Herwadkar, Sebastian F. N. Bode, Ali Sobh, László Maródi, Baldassarre Martire, Chiara Azzari, Maximilian Heeg, Katja Masjosthusmann, Michael H. Albert, Matteo Chinello, Juan Luis Santos-Pérez, Aarnoud Huissoon, Tanya I. Coulter, Hendrik Schulze-Koops, Norbert Graf, Radwa Alkady, Jolanta Bernatoniene, Seraina Prader, Alenka Gagro, Joachim Roesler, Taco W. Kuijpers, Ewa Więsik-Szewczyk, Maria Elena Maccari, Conrad Ferdinand Lippert, Miriam González-Amores, Johannes Dirks, Daniel E Pleguezuelo, Christof M. Kramm, Anders Fasth, Volker Schuster, Olov Ekwall, Nikolaus Rieber, Javier Carbone, Petra Kaiser-Labusch, Diana Ernst, Lucia Augusta Baselli, Luis Ignacio Gonzalez-Granado, Maria Kanariou, Stefanie S. V. Henriet, Sigune Goldacker, Kerstin Felgentreff, Oana Joean, Fine Roosens, Fabian Hauck, Eva C. Schwaneck, Milos Jesenak, Manfred Hoenig, Lenka Kapustova, Christoph Boesecke, Alain Fischer, Sara Pereira da Silva, Julia Körholz, Ansgar Schulz, Carolynne Schwarze-Zander, Mikko Seppänen, Nermeen Galal, Nora Naumann-Bartsch, Tomaz Garcez, Peter Ciznar, Klara M. Posfay-Barbe, Zelimir Pavle Eric, Reinhold E. Schmidt, Hermann J. Girschick, Sabine Heine, Anika-Kerstin Biegner, Annick A. J. M. van de Ven, Stefan Schreiber, J. Merlijn van den Berg, Nurit Assia Batzir, Alexandra Jablonka, Kim Stol, Gregor Dückers, Antonios G.A. Kolios, Ioannis Kakkas, Christian Klemann, Marina N. Guseva, Sofia Grigoriadou, Elif Karakoc-Aydiner, Antonio Marzollo, Peter D. Arkwright, Urs C. Steiner, Sara Sebnem Kilic, Romina Dieli-Crimi, Gergely Kriván, Monika Sparber-Sauer, Marco Cazzaniga, Fulvio Porta, Paraskevi Maggina, Tomas Milota, Robbert G. M. Bredius, Martine Pergent, Klaus Tenbrock, Jana Pachlopnik Schmid, Florentia Dimitriou, Cathal Laurence Steele, Helen Bourne, Anna Bobcakova, Gerd Horneff, Judith Potjewijd, Marc Schmalzing, Tobias Ankermann, Paul Ryan, Oksana Boyarchuk, Necil Kutukculer, Carl Friedrich Classen, Zita Chovancová, Moira Thomas, Cinzia Milito, Michaela Bitzenhofer-Grüber, Faranaz Atschekzei, Eva Hlaváčková, Viviana Moschese, Julie Smet, Hans-Hartmut Peter, Carla Teixeira, Sabine M El-Helou, Suzanne de Kruijf Bazen, Helmut Wittkowski, Donate Jakoby, Marina Garcia-Prat, Esther de Vries, Richard Herriot, Sven Kracker, Alessandro Plebani, Lisa Göschl, Laura Hora Marques, Anna Sediva, Jiri Litzman, Mark M. Gompels, Renate Krüger, Şefika İlknur Kökçü Karadağ, Nadine Binder, Anna Szaflarska, Peter Jandus, Lisa Ibberson, Johann Greil, Ulf Schulze-Sturm, Mehtap Sirin, Aydan Ikinciogullari, Edyta Heropolitańska-Pliszka, Michael E. Weiss, Alla Skapenko, Lukas Wisgrill, Hana Alachkar, Uta Behrends, Silvia Sánchez-Ramón, Maria N. Hatzistilianou, Otilia Petrovicova, Darko Richter, Zoreh Nademi, Jürgen K. Rockstroh, Sohilla Lotfy, Markus G. Seidel, Timothy Ronan Leahy, Audra Blažienė, Translational Immunology Groningen (TRIGR), Paediatric Infectious Diseases / Rheumatology / Immunology, AII - Inflammatory diseases, ARD - Amsterdam Reproduction and Development, University of Zurich, Ehl, Stephan, Thalhammer, J., Kindle, G., Nieters, A., Rusch, S., Seppanen, M. R. J., Fischer, A., Grimbacher, B., Edgar, D., Buckland, M., Mahlaoui, N., Ehl, S., Boztug, K., Brunner, J., Demel, U. F., Forster-Waldl, E., Gasteiger, L. M., Goschl, L., Kojic, M., Schroll, A., Seidel, M. G., Wintergerst, U., Wisgrill, L., Sharapova, S. O., Goffard, J. -C., Kerre, T., Meyts, I., Roosens, F., Smet, J., Haerynck, F., Eric, Z. P., Milenova, V., Gagro, A., Richter, D., Chovancova, Z., Hlavackova, E., Litzman, J., Milota, T., Sediva, A., Elaziz, D. A., Alkady, R. S., El Sayed El Hawary, R., Eldash, A. S., Galal, N., Lotfy, S., Meshaal, S. S., Reda, S. M., Sobh, A., Elmarsafy, A., Brosselin, P., Courteille, V., De Vergnes, N., Kracker, S., Pergent, M., Randrianomenjanahary, P., Ahrenstorf, G., Albert, M. H., Ankermann, T., Atschekzei, F., Baumann, U., Becker, B. C., Behrends, U., Belohradsky, B. H., Biegner, A. -K., Binder, N., Bode, S. F. N., Boesecke, C., Boetticher, B., Borte, M., Borte, S., Classen, C. F., Dirks, J., Duckers, G., El-Helou, S., Ernst, D., Fasshauer, M., Fecker, G., Felgentreff, K., Foell, D., Ghosh, S., Girschick, H. J., Goldacker, S., Graf, N., Graf, D., Greil, J., Hanitsch, L. G., Hauck, F., Heeg, M., Heine, S. I., Henes, J. C., Hoenig, M., Holzer, U., Holzinger, D., Horneff, G., Hundsdoerfer, P., Jablonka, A., Jakoby, D., Joean, O., Kaiser-Labusch, P., Klemann, C., Kobbe, R., Korholz, J., Kramm, C. M., Kruger, R., Landwehr-Kenzel, S., Lehmberg, K., Liese, J. G., Lippert, C. F., Maccari, M. E., Masjosthusmann, K., Meinhardt, A., Metzler, M., Morbach, H., Muller, I., Naumann-Bartsch, N., Neubert, J., Niehues, T., Peter, H. -H., Rieber, N., Ritterbusch, H., Rockstroh, J. K., Roesler, J., Schauer, U., Scheible, R., Schmalzing, M., Schmidt, R. E., Schneider, D. T., Schreiber, S., Schuetz, C., Schulz, A., Schulze-Koops, H., Schulze-Sturm, U., Schuster, V., Schwaneck, E. C., Schwarz, K., Schwarze-Zander, C., Sirin, M., Skapenko, A., Sogkas, G., Sparber-Sauer, M., Speckmann, C., Steinmann, S., Stiehler, S., Tenbrock, K., von Bernuth, H., Warnatz, K., Wasmuth, J. -C., Weiss, M., Witte, T., Wittke, K., Wittkowski, H., Zeuner, R. A., Farmaki, E., Hatzistilianou, M. N., Kakkas, I., Kanariou, M. G., Kapousouzi, A., Liatsis, E., Maggina, P., Papadopoulou-Alataki, E., Raptaki, M., Speletas, M., Tantou, S., Goda, V., Krivan, G., Marodi, L., Abolhassani, H., Aghamohammadi, A., Rezaei, N., Feighery, C., Leahy, T. R., Ryan, P., Batzir, N. A., Garty, B. Z., Tamary, H., Aiuti, A., Amodio, D., Azzari, C., Barzaghi, F., Baselli, L. A., Cancrini, C., Carrabba, M., Cazzaniga, M., Cesaro, S., Chinello, M., Danieli, M. G., Dellepiane, R. M., Fabio, G., Gambineri, E., Lodi, L., Lougaris, V., Marasco, C., Martire, B., Marzollo, A., Milito, C., Moschese, V., Pignata, C., Plebani, A., Porta, F., Quinti, I., Ricci, S., Soresina, A., Tommasini, A., Vacca, A., Vanessa, C., Blaziene, A., Sitkauskiene, B., Gowin, E., Heropolitanska-Pliszka, E., Pietrucha, B., Szaflarska, A., Wiesik-Szewczyk, E., Wolska-Kusnierz, B., Esteves, I., Faria, E., Marques, L. H., Neves, J. F., Silva, S. L., Teixeira, C., Pereira da Silva, S., Capilna, B. R., Guseva, M. N., Shcherbina, A., Bobcakova, A., Ciznar, P., Gabzdilova, J., Jesenak, M., Kapustova, L., Orosova, J., Petrovicova, O., Raffac, S., Kopac, P., Allende, L. M., Antoli, A., Blanch, G. R., Carbone, J., Dieli-Crimi, R., Garcia-Prat, M., Gil-Herrera, J., Gonzalez-Granado, L. I., Agullo, P. L., Olbrich, P., Parra-Martinez, A., Paz-Artal, E., Pleguezuelo, D. E., Rodriguez, N. S., Sanchez-Ramon, S., Santos-Perez, J. L., Solanich, X., Soler-Palacin, P., Gonzalez-Amores, M., Ekwall, O., Fasth, A., Bitzenhofer-Gruber, M., Candotti, F., Dimitriou, F., Heininger, U., Holbro, A., Jandus, P., Kolios, A. G. A., Marschall, K., Schmid, J. P., Posfay-Barbe, K. M., Prader, S., Reichenbach, J., Steiner, U. C., Truck, J., Bredius, R. G., de Kruijf- Bazen, S., de Vries, E., Henriet, S. S. V., Kuijpers, T. W., Potjewijd, J., Rutgers, A., Stol, K., van Aerde, K. J., Van den Berg, J. M., van de Ven, A. A. J. M., Montfrans, J., Aydemir, S., Baris, S., Dogu, F., Ikinciogullari, A., Karakoc-Aydiner, E., Kilic, S. S., Kiykim, A., Kokcu Karadag, S. I., Kutukculer, N., Ocak, S., Unal, E., Boyarchuk, O., Hilfanova, A., Kostyuchenko, L. V., Alachkar, H., Arkwright, P. D., Baxendale, H. E., Bernatoniene, J., Coulter, T. I., Garcez, T., Goddard, S., Gompels, M. M., Grigoriadou, S., Herriot, R., Herwadkar, A., Huissoon, A., Ibberson, L., Nademi, Z., Noorani, S., Parvin, S., Steele, C. L., Thomas, M., Waruiru, C., Yong, P. F. K., and Bourne, H.

Subjects
0301 basic medicine, Male, Pediatrics, syndromic, Sex Factor, Disease, registry, medicine.disease_cause, Cohort Studies, 0302 clinical medicine, Primary Immunodeficiency Disease, inborn error of immunity, Immunology and Allergy, warning signs, Age Factor, Registries, Family history, presenting symptom, Child, Primary immunodeficiency, Granuloma, autoimmune, immune dysregulation, inflammatory, Adult, Autoimmune Diseases, Female, Humans, Infections, Lymphoproliferative Disorders, Middle Aged, Primary Immunodeficiency Diseases, Sex Factors, Age Factors, 10177 Dermatology Clinic, Infections/epidemiology, 3. Good health, Settore MED/02, Warning signs, Lymphoproliferative Disorder, 2723 Immunology and Allergy, Infection, Human, medicine.medical_specialty, Immunology, 610 Medicine & health, Malignancy, primary immunodeficiency, Autoimmune Disease, 03 medical and health sciences, Immunity, Autoimmune Diseases/epidemiology, medicine, 2403 Immunology, business.industry, warning sign, Common variable immunodeficiency, Granuloma/epidemiology, Immune dysregulation, medicine.disease, Primary Immunodeficiency Diseases/epidemiology, 030104 developmental biology, Lymphoproliferative Disorders/epidemiology, Cohort Studie, business, 030215 immunology
Abstract

BACKGROUND: Inborn errors of immunity (IEI) are rare diseases, which makes diagnosis a challenge. A better description of the initial presenting manifestations should improve awareness and avoid diagnostic delay. Although increased infection susceptibility is a well-known initial IEI manifestation, less is known about the frequency of other presenting manifestations.OBJECTIVE: We sought to analyze age-related initial presenting manifestations of IEI including different IEI disease cohorts.METHODS: We analyzed data on 16,486 patients of the European Society for Immunodeficiencies Registry. Patients with autoinflammatory diseases were excluded because of the limited number registered.RESULTS: Overall, 68% of patients initially presented with infections only, 9% with immune dysregulation only, and 9% with a combination of both. Syndromic features were the presenting feature in 12%, 4% had laboratory abnormalities only, 1.5% were diagnosed because of family history only, and 0.8% presented with malignancy. Two-third of patients with IEI presented before the age of 6 years, but a quarter of patients developed initial symptoms only as adults. Immune dysregulation was most frequently recognized as an initial IEI manifestation between age 6 and 25 years, with male predominance until age 10 years, shifting to female predominance after age 40 years. Infections were most prevalent as a first manifestation in patients presenting after age 30 years.CONCLUSIONS: An exclusive focus on infection-centered warning signs would have missed around 25% of patients with IEI who initially present with other manifestations.

Published
2021

9. Single Hepatocellular Carcinoma approached by curative-intent treatment: A propensity score analysis comparing radiofrequency ablation and liver resection

Author

Maria Danieli, Marc Najjar, S. Di Sandro, Laura Benuzzi, Andrea Lauterio, Leonardo Centonze, Vincenzo Bagnardi, Antonio Rampoldi, R. De Carlis, I. Pezzoli, L. De Carlis, Francesca Botta, Di Sandro, S, Benuzzi, L, Lauterio, A, Botta, F, De Carlis, R, Najjar, M, Centonze, L, Danieli, M, Pezzoli, I, Rampoldi, A, Bagnardi, V, and De Carlis, L

Subjects
Male, medicine.medical_specialty, Cirrhosis, Carcinoma, Hepatocellular, Radiofrequency ablation, Hepatocellular carcinoma, Urology, 030230 surgery, law.invention, Resection, 03 medical and health sciences, 0302 clinical medicine, Postoperative Complications, law, medicine, Hepatectomy, Humans, Laparoscopy, Propensity Score, Aged, Curative intent, medicine.diagnostic_test, Liver resection, business.industry, Liver Neoplasms, General Medicine, Middle Aged, medicine.disease, Survival Rate, Safety profile, surgical procedures, operative, Oncology, 030220 oncology & carcinogenesis, Propensity score matching, Catheter Ablation, Surgery, Female, business, therapeutics
Abstract

Introduction: Patients with a single small Hepatocellular Carcinoma (HCC) may be definitively treated by Radiofrequency ablation (RFA) with a very low rate of peri-operative morbidity. However, results are still controversial comparing RFA to Liver Resection (LR). Methods: All consecutive patients treated by RFA or LR for a single untreated small HCC on liver cirrhosis between January 2006-December 2016 were enrolled. Patients were matched 1:1 basing on: age, MELD-score, platelet count, nodule's diameter, HCV status, α-fetoprotein level, and Albumin-Bilirubin score. First analysis compered LR to RFA. Second analysis compared Laparoscopic LR (LLR) to RFA. Results: Of 484 patients with single small HCC, 91 patients were selected for each group after a 1:1 propensity score matching (PS-M). The 5-years OS was 70% and 60% respectively for LR and RFA group (P = 0.666). The 5-year RFS was 36% and 21% respectively for LR and RFA group (P < 0.001). Patients treated by LR had a significantly longer hospital stay and higher complications rate. Comparing 50 cases of LLR and 50 of RFA, the 5-years OS was 79% and 56% respectively for LLR and RFA group (P = 0.22). The 5-year RFS was 54% and 19% respectively for LR and RFA group (P < 0.001). Post-operative complications were not significantly different. Conclusions: LLR confers similar peri-operative complications rate compared to RFA. LLR should be considered as a first-line approach for the treatment of a single small HCC as it combines the effectiveness of open LR and the safety profile of RFA

Published
2019
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10. Minor laparoscopic liver resection for Hepatocellular Carcinoma is safer than minor open resection, especially for less compensated cirrhotic patients: Propensity score analysis

Author

Enrico Pinotti, Luciano De Carlis, Maria Danieli, Vincenzo Buscemi, Riccardo De Carlis, Vincenzo Bagnardi, Andrea Lauterio, Stefano Di Sandro, Marc Najjar, Laura Benuzzi, Di Sandro, S, Bagnardi, V, Najjar, M, Buscemi, V, Lauterio, A, De Carlis, R, Danieli, M, Pinotti, E, Benuzzi, L, and De Carlis, L

Subjects
Liver Cirrhosis, Male, medicine.medical_specialty, Carcinoma, Hepatocellular, Cirrhosis, Hepatocellular carcinoma, Prognosi, Liver Cirrhosi, medicine.medical_treatment, 030230 surgery, Follow-Up Studie, 03 medical and health sciences, Postoperative Complications, 0302 clinical medicine, Retrospective Studie, Propensity score matching, Hepatectomy, Humans, Medicine, Prospective Studies, Propensity Score, Prospective cohort study, Survival rate, Aged, Retrospective Studies, business.industry, Liver Neoplasms, Retrospective cohort study, Perioperative, Laparoscopic liver resection, Length of Stay, Prognosis, medicine.disease, Surgery, Survival Rate, Prospective Studie, Oncology, Liver Neoplasm, 030220 oncology & carcinogenesis, Female, Laparoscopy, Postoperative Complication, business, Complication, Human, Follow-Up Studies
Abstract

Background Laparoscopic liver resection (LLR) has gained significant popularity over the last 10 years. First experiences of LLR compared to open liver resection (OLR) reported a similar survival and a better safety profile for LLR. Materials and methods This is a retrospective analysis of prospectively collected data of all consecutive patients treated by liver resection for HCC on liver cirrhosis between January 2005 and March 2017. The choice of procedure (LLR vs OLR) was generally based on tumor localization, history of previous upper abdominal surgery and patient's preference. The type of resection and indication for surgery were unrelated to the adopted technique. Based on pre-operative variables and confirmed cirrhosis, a 1:1 propensity score matching (PSM) model was developed to compare outcomes of LLR and OLR in patients with HCC. Outcomes of interest included morbidity, mortality and long-term cure potential. Results After-PSM, the LLR group demonstrated better perioperative results including: lower complication rate (50.7% in OLR vs 29.3% in LLR, p = 0.0035), significantly lower intra-operative blood loss (200 ml in OLR vs 150 ml in LLR, p = 0.007) and shorter hospital length of stay (median 9 days in OLR vs 7 days in LLR, p = 0.0018). Moreover there was no significant difference between the two groups in 3-year survival (76%, CI: 60%–86% in LLR vs 68%, CI: 55%–79% in OLR, p = 0.32) or recurrence-free survival rates (44%, CI: 28%–58%, vs 44%, CI: 31%–57%, p = 0.94). Conclusions Minor LLR appeared significantly safer compared to minor OLR for HCC. LLR was associated with fewer post-operative complication, lower operative blood loss and a shorter hospital stay along with similar survival and recurrence-free survival rates

Published
2018

11. The 10-year follow-up data of the Euro-Lupus Nephritis Trial comparing low-dose and high-dose intravenous cyclophosphamide

Author

David D'Cruz, Daniel Abramovicz, Yair Levy, Gian Domenico Sebastiani, Frédéric Houssiau, Alberto Cauli, Rajko Popovic, Mauro Galeazzi, Geneviève Depresseux, Roberto Cattaneo, Peter Petera, Haner Direskeneli, Ricard Cervera, Carlos Vasconcelos, Radmila Petrovic, E. De Ramon Garrido, Ahmet Gül, Maria Giovanna Danieli, J Font, J-P Cosyns, Renato Alberto Sinico, Daniel Engelbert Blockmans, Houssiau, F, Vasconcelos, C, D'Cruz, D, Sebastiani, G, De Ramon Garrido, E, Danieli, M, Abramovicz, D, Blockmans, D, Cauli, A, Direskeneli, H, Galeazzi, M, Gül, A, Levy, Y, Petera, P, Popovic, R, Petrovic, R, Sinico, R, Cattaneo, R, Font, J, Depresseux, G, Cosyns, J, and Cervera, R

Subjects
Adult, Male, medicine.medical_specialty, Adolescent, Cyclophosphamide, Immunology, Lupus nephritis, Urology, Renal function, Injections, Intravenou, Kidney Function Tests, General Biochemistry, Genetics and Molecular Biology, Immunosuppressive Agent, Young Adult, chemistry.chemical_compound, Rheumatology, Azathioprine, medicine, Humans, Immunology and Allergy, Creatinine, Kidney Function Test, Biochemistry, Genetics and Molecular Biology (all), Proteinuria, Dose-Response Relationship, Drug, business.industry, Lupus Nephriti, Middle Aged, medicine.disease, Lupus Nephritis, Surgery, Regimen, Treatment Outcome, chemistry, Epidemiologic Method, Injections, Intravenous, Drug Therapy, Combination, Female, medicine.symptom, Epidemiologic Methods, business, Nephritis, Immunosuppressive Agents, Human, medicine.drug, Kidney disease
Abstract

Objective:To update the follow-up of the Euro-Lupus Nephritis Trial (ELNT), a randomised prospective trial comparing low-dose (LD) and high-dose (HD) intravenous (IV) cyclophosphamide (CY) followed by azathioprine (AZA) as treatment for proliferative lupus nephritis.Patients and methods:Data for survival and kidney function were prospectively collected during a 10-year period for the 90 patients randomised in the ELNT, except in 6 lost to follow-up.Results:Death, sustained doubling of serum creatinine and end-stage renal disease rates did not differ between the LD and HD group (5/44 (11%) vs 2/46 (4%), 6/44 (14%) vs 5/46 (11%) and 2/44 (5%) vs 4/46 (9%), respectively) nor did mean serum creatinine, 24 h proteinuria and damage score at last follow-up. Most patients in both groups were still treated with glucocorticoids, other immunosuppressant agents and blood pressure lowering drugs. After 10 years of follow-up, the positive predictive value for a good outcome of an early drop in proteinuria in response to initial immunosuppressive therapy was confirmed.Conclusion:The data confirm that a LD IVCY regimen followed by AZA—the “Euro-Lupus regimen”—achieves good clinical results in the very long term.

Published
2009
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12. Association tests with systemic lupus erythematosus (SLE) of IL10 markers indicate a direct involvement of a CA repeat in the 5′ regulatory region

Author

Nadia Barizzone, Raffaella Scorza, M G Danieli, M. Cappelli, S D' Alfonso, Patricia Momigliano-Richiardi, Marta Mellai, M Lanceni, Mara Giordano, Maria Grazia Sabbadini, Maurizio Marchini, P Rovere, D'Alfonso, S, Giordano, M, Mellai, M, Lanceni, M, Barizzone, N, Marchini, M, Scorza, R, Danieli, M, Cappelli, M, ROVERE QUERINI, Patrizia, Sabbadini, M, and Momigliano Richiardi, P.

Subjects
Genetic Markers, Male, Association test, Genotype, 5' Flanking Region, Immunology, Ca repeat, Single-nucleotide polymorphism, Biology, Regulatory region, Gene Frequency, immune system diseases, Genetics, Humans, Lupus Erythematosus, Systemic, Dna pools, skin and connective tissue diseases, Genetics (clinical), Chi-Square Distribution, Interleukin-10, Interleukin 10, Genetic Techniques, Haplotypes, Female, Microsatellite Repeats
Abstract

Many lines of evidence suggest that IL10 is a strong candidate gene for systemic lupus erythematosus (SLE) susceptibility. In our previously reported study an allele (IL10.G-140bp) of the microsatellite IL10.G located at position -1100 was significantly increased in Italian SLE patients in comparison with controls. Starting from this observation, we tested if sequence variations in the vicinity of IL10.G were more strongly associated with SLE. We performed a comprehensive association study including 26 SNPs (of which four were newly identified in the present study by DHPLC analysis) spanning 8.5 Kb of the 5' flanking and the transcribed region of the IL10 gene. The association study was performed by the DNA pool method on an extended panel of Italian patients (205) and controls (631). Haplotypic associations were studied by individual typing of seven selected markers surrounding IL10.G. Gene, genotype and haplotype frequencies were not significantly different in patients and controls. Thus the IL10.G microsatellite remains to date the only IL10 marker associated with SLE in our population. A meta-analysis of all published results indicates a possible direct role of the IL10.G repeat number in SLE susceptibility.

Published
2002
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13. Central nervous system involvement in systemic lupus erythematosus patients without overt neuropsychiatric manifestations

Author

L Ferrario, Paolo Fraticelli, Angela Tincani, Orsola Gambini, Claudio Rugarli, D Croce, MP Domeneghetti, L Vanzulli, Massimo Piccirilli, C Messa, R Quartesan, G. Danieli, U Salvolini, C Piccini, Alessandro Padovani, Alberto Pupi, E Emmi, M. Vanoli, Luca Massacesi, Alessandro Farsi, Marco Bartolini, Marco Candela, Roberto Gerli, M De Cristoforis, Maria Grazia Sabbadini, Maria Giovanna Danieli, Gian Paolo Anzola, Ferruccio Fazio, A. Passaleva, Roberto Gasparotti, L Origgi, Raffaella Scorza, Enrica Bozzolo, Angelo A. Manfredi, Roberto Cattaneo, Arturo Campana, Sabbadini, Mg, Manfredi, ANGELO ANDREA M. A., Bozzolo, E, Ferrario, L, Rugarli, C, Scorza, R, Origgi, L, Vanoli, M, Gambini, O, Vanzulli, L, Croce, D, Campana, A, Messa, C, Fazio, F, Tincani, A, Anzola, G, Cattaneo, R, Padovani, A, Gasparotti, R, Gerli, R, Quartesan, R, Piccirilli, M, Farsi, A, Emmi, E, Domeneghetti, M, Piccini, C, Massacesi, L, Pupi, A, De Cristoforis, M, Danieli, M, Candela, M, Fraticelli, P, Bartolini, M, Salvolini, U, Danieli, G, Passaleva, A., Sabbadini, M, Manfredi, A, Messa, M, and Passaleva, A

Subjects
Adult, Male, Pediatrics, medicine.medical_specialty, Pathology, Central nervous system, 030204 cardiovascular system & hematology, Single-photon emission computed tomography, never-NPSLE, SPECT, 03 medical and health sciences, 0302 clinical medicine, systemic lupus erythematosus, Rheumatology, Neuroimaging, medicine, Humans, Lupus Erythematosus, Systemic, Tomography, Emission-Computed, Single-Photon, 030203 arthritis & rheumatology, Brain Diseases, Systemic lupus erythematosus, Lupus erythematosus, medicine.diagnostic_test, business.industry, Mental Disorders, Neuropsychology, Magnetic resonance imaging, Middle Aged, central nervous system, medicine.disease, Magnetic Resonance Imaging, medicine.anatomical_structure, Cerebrovascular Circulation, Female, Cognition Disorders, Tomography, X-Ray Computed, business, Neurocognitive
Abstract

Objective: To verify whether features of CNS involvement can be detected in SLE patients without overt neuropsychiatric manifestations.Methods: 114 SLE patients who had never received a diagnosis of neuropsychiatric lupus (never NPSLE) were studied and compared to 65 SLE patients with known neuropsychiatric involvement (NPSLE). The study relied on evaluation of neurocognitive functions by means of a battery of neuropsychological tests, on psychiatric and neuropsychological assessments and on neuroimaging studies (computed tomography, magnetic resonance, single photon emission computed tomography (SPECT)).Results: Clinical features, including disease duration/activity and pharmacological therapy, of never-NPSLE and NPSLE patients were similar. Short-term and long-term memory, visuo-spatial and verbal information processing were similarly compromised in never-NPSLE and in NPSLE patients; only attention was significantly more compromised in NPSLE patients. Psychiatric morbidity was higher than expected in never-NPSLE patients, although less than in the control neuropsychiatric group. Ischemic lesions, multiple small high intensity lesions and cortical atrophy, detected by CT and MR scans, as well as abnormal SPECT were also frequently detected in never NPSLE patients. Interestingly, left parietal and occipital area hypoperfusion by SPECT was significantly more frequent in the patients with impaired visuo-spatial intelligence and short-term memory.Conclusions: Most abnormalities detected by available diagnostic tools and characteristics of neuropsychiatric SLE are also present in non-symptomatic patients. They may derive from an unexpected widespread involvement of the CNS and are not per se sufficient, in the absence of clinical manifestations, for a diagnosis of neuropsychiatric SLE.

Published
1999
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14. Systemic lupus erythematosus in Europe at the change of the millennium: lessons from the 'Euro-Lupus Project'

Author

Cervera, R, ABARCA-COSTALAGO, M, Abramovicz, D, Allegri, F, Annunziata, P, Aydintug, Ao, Bacarelli, Mr, Bellisai, F, Bernardino, I, BIERNAT-KALUZA, E, Blockmans, D, Boki, K, Bracci, L, Campanella, V, Camps, Mt, Carcassi, C, Cattaneo, R, Cauli, A, CHWALINSKA-SADOWSKA, H, Contu, L, Cosyns, Jp, Danieli, Mg, Dcruz, D, Depresseux, G, Direskeneli, H, Domenech, I, Espinosa, G, FERNANDEZ-NEBRO, A, Ferrara, Gb, Font, J, Frutos, Ma, Galeazzi, M., GARCIA-CARRASCO, M, GARCIA IGLESIAS MF, GARCIA-TOBARUELA, A, George, J, Gil, A, GONZALEZ-SANTOS, P, Grana, M, Gul, A, Haga, Hj, DE HARO-LIGER, M, Houssiau, F, Hughes, Gr, Ingelmo, M, JEDRYKA-GORAL, A, Khamashta, Ma, Lavilla, P, Levi, Y, LOPEZ-DULPA, M, LOPEZ-SOTO, A, Maldykowa, H, Marcolongo, R, Mathieu, A, Morozzi, G, Nicolopoulou, N, Papasteriades, C, Passiu, G, Perello, I, Petera, P, Petrovic, R, Piette, Jc, Pintado, V, DE PITA, O, Popovic, R, Pucci, G, Puddu, P, DE RAMON, E, RAMOS-CASALS, M, RODRIGUEZ-ANDREU, J, RUIZ-IRASTORZA, G, SANCHEZ-LORA, J, Sanna, G, Scorza, R, Sebastiani, Gd, Sherer, Y, Shoenfeld, Y, Simpatico, A, Sinico, Ra, Smolen, J, Tincani, A, Tokgoz, G, URBANO-MARQUEZ, A, Vasconcelos, C, Vazquez, Jj, Veronesi, J, Vianna, J, Vivancos, J, UCL - MD/MINT - Département de médecine interne, UCL - (SLuc) Service de rhumatologie, UCL - MD/MNOP - Département de morphologie normale et pathologique, UCL - (SLuc) Service d'anatomie pathologique, Cervera, R, Abarca Costalago, M, Abramovicz, D, Allegri, F, Annunziata, P, Aydintug, A, Bacarelli, M, Bellisai, F, Bernardino, I, Biernat Kaluza, E, Blockmans, D, Boki, K, Bracci, L, Campanella, V, Camps, M, Carcassi, C, Cattaneo, R, Cauli, A, Chwalinska Sadowska, H, Contu, L, Cosyns, J, Danieli, M, D́cruz, D, Depresseux, G, Direskeneli, H, Domènech, I, Espinosa, G, Fernández Nebro, A, Ferrara, G, Font, J, Frutos, M, Galeazzi, M, García Carrasco, M, García Iglesias, M, García Tobaruela, A, George, J, Gil, A, González Santos, P, Grana, M, Gül, A, Haga, H, de Haro Liger, M, Houssiau, F, Hughes, G, Ingelmo, M, Jedryka Góral, A, Khamashta, M, Lavilla, P, Levi, Y, López Dupla, M, López Soto, A, Maldykowa, H, Marcolongo, R, Mathieu, A, Morozzi, G, Nicolopoulou, N, Papasteriades, C, Passiu, G, Perelló, I, Petera, P, Petrovic, R, Piette, J, Pintado, V, de Pita, O, Popovic, R, Pucci, G, Puddu, P, de Ramón, E, Ramos Casals, M, Rodríguez Andreu, J, Ruiz Irastorza, G, Sánchez Lora, J, Sanna, G, Scorza, R, Sebastiani, G, Sherer, Y, Shoenfeld, Y, Simpatico, A, Sinico, R, Smolen, J, Tincani, A, Tokgöz, G, Urbano Márquez, A, Vasconcelos, C, Vázquez, J, Veronesi, M, Vianna, J, and Vivancos, J

Subjects
Male, medicine.medical_specialty, Prognosi, Epidemiology, Immunology, Systemic lupus erythematosu, Disease, Prognostic factors, Autoimmune Disease, Follow-Up Studie, Autoimmune Diseases, Cohort Studies, Systemic lupus erythematosus, immune system diseases, medicine, Immunology and Allergy, Humans, Lupus Erythematosus, Systemic, Prospective Studies, Age of Onset, Mortality, Prospective cohort study, skin and connective tissue diseases, Survival rate, Prognostic factor, business.industry, medicine.disease, Prognosis, Europe, Survival Rate, Prospective Studie, Family medicine, Antibodies, Antinuclear, Cohort, Female, Cohort Studie, Age of onset, Morbidity, business, Human, Cohort study, Follow-Up Studies
Abstract

The "Euro-Lupus Cohort" is composed by 1000 patients with systemic lupus erythematosus (SLE) that have been followed prospectively since 1991. These patients have been gathered by a European consortium-the "Euro-Lupus Project Group". This consortium was originated as part of the network promoted by the "European Working Party on SLE", a working group created in 1990 in order to promote research in Europe on the different problems related to this disease. The "Euro-Lupus Cohort" provides an updated information on the SLE morbidity and mortality characteristics in the present decade as well as defines several clinical and immunological prognostic factors. © 2005 Elsevier B.V. All rights reserved.

Published
2006

15. Early response to immunosuppressive therapy predicts good renal outcome in lupus nephritis: Lessons from long-term followup of patients in the euro-lupus nephritis trial

Author

Houssiau, Fa, Vasconcelos, C, D'Cruz, D, Sebastiani, Gd, DE RAMON GARRIDO, E, Danieli, Mg, Abramovicz, D, Blockmans, D, Mathieu, A, Direskeneli, H, Galeazzi, M, Gul, A, Levy, Y, Petera, P, Popovic, R, Petrovic, R, Sinico, Ra, Cattaneo, Roberto, Font, J, Depresseux, G, Cosyns, Jp, Cervera, R., Houssiau, F, Vasconcelos, C, D'Cruz, D, Sebastiani, G, De Ramon Garrido, E, Danieli, M, Abramovicz, D, Blockmans, D, Mathieu, A, Direskeneli, H, Galeazzi, M, Gül, A, Levy, Y, Petera, P, Popovic, R, Petrovic, R, Sinico, R, Cattaneo, R, Font, J, Depresseux, G, Cosyns, J, and Cervera, R

Subjects
Adult, Male, Time Factors, Adolescent, Time Factor, Immunology, Injections, Intravenou, Kidney Function Tests, Follow-Up Studie, Immunosuppressive Agent, Rheumatology, Azathioprine, Humans, Cyclophosphamide, Kidney Function Test, Dose-Response Relationship, Drug, Lupus Nephriti, Lupus Nephritis, Survival Rate, Proteinuria, Treatment Outcome, Injections, Intravenous, Female, Immunosuppressive Agents, Follow-Up Studies, Human
Abstract

Arthritis Rheum. 2004 Dec;50(12):3934-40. Early response to immunosuppressive therapy predicts good renal outcome in lupus nephritis: lessons from long-term followup of patients in the Euro-Lupus Nephritis Trial. Houssiau FA, Vasconcelos C, D'Cruz D, Sebastiani GD, de Ramon Garrido E, Danieli MG, Abramovicz D, Blockmans D, Mathieu A, Direskeneli H, Galeazzi M, Gül A, Levy Y, Petera P, Popovic R, Petrovic R, Sinico RA, Cattaneo R, Font J, Depresseux G, Cosyns JP, Cervera R. Université Catholique de Louvain, Brussels, Belgium. houssiau@ruma.ucl.ac.be Abstract OBJECTIVE: In the Euro-Lupus Nephritis Trial (ELNT), 90 patients with lupus nephritis were randomly assigned to a high-dose intravenous cyclophosphamide (IV CYC) regimen (6 monthly pulses and 2 quarterly pulses with escalating doses) or a low-dose IV CYC regimen (6 pulses of 500 mg given at intervals of 2 weeks), each of which was followed by azathioprine (AZA). After a median followup of 41 months, a difference in efficacy between the 2 regimens was not observed. The present analysis was undertaken to extend the followup and to identify prognostic factors. METHODS: Renal function was prospectively assessed quarterly in all 90 patients except 5 who were lost to followup. Survival curves were derived using the Kaplan-Meier method. RESULTS: After a median followup of 73 months, there was no significant difference in the cumulative probability of end-stage renal disease or doubling of the serum creatinine level in patients who received the low-dose IV CYC regimen versus those who received the high-dose regimen. At long-term followup, 18 patients (8 receiving low-dose and 10 receiving high-dose treatment) had developed permanent renal impairment and were classified as having poor long-term renal outcome. We demonstrated by multivariate analysis that early response to therapy at 6 months (defined as a decrease in serum creatinine level and proteinuria

Published
2004

16. Lessons from the 'Euro-Lupus Cohort'

Author

Cervera, R., Abarca Costalago, M., Abramovicz, D., Allegri, F., PASQUALE ANNUNZIATA, Aydintug, Ao, Bacarelli, Mr, Bellisai, F., Bernardino, I., Biernat Kaluza, E., Blockmans, D., Boki, K., LUISA BRACCI, Campanella, V., Camps, Mt, Carcassi, C., Cattaneo, R., Cauli, A., Chwalinska Sadowska, H., Contu, L., Cosyns, Jp, Danieli, Mg, D Cruz, D., Depresseux, G., Direskeneli, H., Domènech, I., Espinosa, G., Fernández Nebro, A., Ferrara, Gb, Font, J., Frutos, Ma, MAURO GALEAZZI, García Carrasco, M., GARCÍA IGLESIAS MF, García Tobaruela, A., George, J., Gil, A., González Santos, P., Grana, M., Gül, A., Haga, Hj, Haro Liger, M., Houssiau, F., Hughes, Gr, Ingelmo, M., Jedryka Góral, A., Khamashta, Ma, Lavilla, P., Levi, Y., López Dupla, M., López Soto, A., Maldykowa, H., Marcolongo, R., Mathieu, A., GABRIELLA MOROZZI, Nicolopoulou, N., Papasteriades, C., Passiu, G., Perelló, I., Petera, P., Petrovic, R., Piette, Jc, Pintado, V., Pita, O., Popovic, R., Pucci, G., Puddu, P., Ramón, E., Ramos Casals, M., Rodríguez Andreu, J., Ruiz Irastroza, G., Sánchez Lora, J., Sanna, G., Scorza, R., Sebastini, Gd, Sherer, Y., Shoenfeld, Y., Simpatico, A., Sinico, Ra, Smolen, J., Tincani, A., Tokgöz, G., Urbano Márquez, A., Vasconcelos, C., Vázquez, Jj, Veronesi, M., Vianni, J., Vivancos, J., EUROPEAN WORKING PARTY ON SYSTEMIC LUPUS ERYTHEMATOSUS, Cervera, R, Abarca Costalago, M, Abramovicz, D, Allegri, F, Annunziata, P, Aydintug, A, Bacarelli, M, Bellisai, F, Bernardino, I, Biernat Kaluza, E, Blockmans, D, Boki, K, Bracci, L, Campanella, V, Camps, M, Carcassi, C, Cattaneo, R, Cauli, A, Chwalinska Sadowska, H, Contu, L, Cosyns, J, Danieli, M, D'Cruz, D, Depresseux, G, Direskeneli, H, Domènech, I, Espinosa, G, Fernández Nebro, A, Ferrara, G, Font, J, Frutos, M, Galeazzi, M, García Carrasco, M, García Iglesias, M, García Tobaruela, A, George, J, Gil, A, González Santos, P, Grana, M, Gül, A, Haga, H, de Haro Liger, M, Houssiau, F, Hughes, G, Ingelmo, M, Jedryka Góral, A, Khamashta, M, Lavilla, P, Levi, Y, López Dupla, M, López Soto, A, Maldykowa, H, Marcolongo, R, Mathieu, A, Morozzi, G, Nicolopoulou, N, Papasteriades, C, Passiu, G, Perelló, I, Petera, P, Petrovic, R, Piette, J, Pintado, V, de Pita, O, Popovic, R, Pucci, G, Puddu, P, de Ramón, E, Ramos Casals, M, Rodríguez Andreu, J, Ruiz Irastroza, G, Sánchez Lora, J, Sanna, G, Scorza, R, Sebastini, G, Sherer, Y, Shoenfeld, Y, Simpatico, A, Sinico, R, Smolen, J, Tincani, A, Tokgöz, G, Urbano Márquez, A, Vasconcelos, C, Vázquez, J, Veronesi, M, Vianni, J, and Vivancos, J

Subjects
Adult, Male, Adolescent, Prognosi, Middle Aged, Prognosis, Cohort Studies, Europe, Survival Rate, Prospective Studie, Antibodies, Antinuclear, Humans, Lupus Erythematosus, Systemic, Female, Prospective Studies, Cohort Studie, Age of Onset, Human
Abstract

The "Euro-Lupus Cohort" is composed by 1,000 patients with systemic lupus erythematosus (SLE) that have been followed prospectively since 1991. These patients have been gathered by a European consortium - the "Euro-Lupus Project Group". This consortium was originated as part of the network promoted by the "European Working Party on SLE", a working group created in 1990 in order to promote research in Europe on the different problems related to this disease. The "Euro-Lupus Cohort" provides an updated information on the SLE morbidity and mortality characteristics in the present decade as well as defines several clinical and immunological prognostic factors.

Published
2002

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