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2. AB0501 Three cases of vertebral arteritis identified on FDG-PET in patients with suspected GCA at University of College London Hospital (UCLH)

Author

Moiseev, S, Tervaert, JWC, Arimura, Y, Bogdanos, D, Elena, C, Damoiseaux, J, Ferrante, M, Flores-Suarez, LF, Fritzler, M, Invernizzi, P, Jayne, D, Jennette, JC, Little, M, Mcadoo, SP, Novikov, P, Pusey, CD, Radice, A, Salama, AD, Savige, J, Segelmark, M, Shoenfeld, Y, Sinico, RA, De Sousa, MJR, Specks, U, Terrier, B, Tzioufas, A, Vermeire, S, Zhao, MH, and Bossuyt, X

Subjects
Science & Technology, Rheumatology, 1107 Immunology, cardiovascular system, 1103 Clinical Sciences, skin and connective tissue diseases, Life Sciences & Biomedicine, 1117 Public Health and Health Services, Arthritis & Rheumatology
Abstract

Background: Giant cell arteritis (GCA) may affect both cranial and extra-cranial vessels; where the latter occurs, it can be termed large-vessel GCA (LV-GCA). Large vessel involvement is common: histological evidence has been seen in 80% of autopsies of patients with known GCA, and imaging studies suggest large vessel involvement in over 80%1. LV-GCA is important to diagnose due to the risks of vascular complications such as occlusion and ischaemic stroke. The clinical diagnosis can be challenging, and the American College of Rheumatology (ACR) GCA classification criteria often underperform in cases of LV-GCA1. F-fluorodeoxyglucose positron emission tomography (FDG-PET) has been found to be useful in the detection of extra-cranial involvement to support the diagnosis of LV-GCA.2 Objectives: To appreciate the variability in presentation of cases of LV-GCA, and to further characterise a subgroup of patients with vertebral arteritis. To explore the use of FDG-PET imaging in GCA patients in addition to or in place of traditional diagnostic tools (temporal artery ultrasound / biopsy). Methods: Through evaluation of the new GCA fast-track pathway implemented at UCLH, a subgroup of patients diagnosed with vertebral arteritis was identified. The history and presentation of these patients were analysed. Results: Three patients were diagnosed with vertebral arteritis. All three were male, Caucasian and aged over 70. All were investigated for GCA due to a history of severe headache (frontal in one, occipital in one, bi-temporal in one) with associated red flag symptoms. Two had a history of jaw claudication and visual disturbances (unilateral visual loss in one, transient diplopia in the other). Both of these patients had positive temporal artery biopsies. The third patient had no ischaemic symptoms but a strong history of prominent polymyalgic features and a positive temporal artery ultrasound. Inflammatory markers were raised in two, and normal in one, of the patients. Only one had systemic symptoms (weight loss). All three proceeded to FDG-PET scans which showed vertebral arteritis and were commenced on immunosuppressive treatment. Conclusion: The cases discussed illustrate the heterogeneity of the presentation of LV-GCA, and the diagnostic challenge this poses. FDG-PET imaging is useful in confirming extra-cranial involvement and therefore guiding treatment.

Published
2020

3. Neutrophil Extracellular Traps (NETs) profiles in patients with incident SLE and lupus nephritis

Author

Bruschi, M, Bonanni, A, Petretto, A, Vaglio, A, Pratesi, F, Santucci, L, Migliorini, P, Bertelli, R, Galetti, M, Belletti, S, Cavagna, L, Moroni, G, Franceschini, F, Fredi, M, Pazzola, G, Allegri, L, Sinico, Ra, Pesce, G, Bagnasco, M, Manfredi, A, Ramirez, Ga, Ramoino, P, Bianchini, P, Puppo, F, Pupo, F, Negrini, S, Mattana, F, Emmi, G, Garibotto, G, Santoro, D, Scolari, F, Ravelli, A, Tincani, A, Cravedi, P, Volpi, S, Candiano, G, Ghiggeri, Gm, Bruschi, M, Bonanni, A, Petretto, A, Vaglio, A, Pratesi, F, Santucci, L, Migliorini, P, Bertelli, R, Galetti, M, Belletti, S, Cavagna, L, Moroni, G, Franceschini, F, Fredi, M, Pazzola, G, Allegri, L, Sinico, R, Pesce, G, Bagnasco, M, Manfredi, A, Ramirez, G, Ramoino, P, Bianchini, P, Puppo, F, Pupo, F, Negrini, S, Mattana, F, Emmi, G, Garibotto, G, Santoro, D, Scolari, F, Ravelli, A, Tincani, A, Cravedi, P, Volpi, S, Candiano, G, and Ghiggeri, G

Subjects
lupus nephritis, Systemic Lupus Erythematosus (SLE), Neutrophil Extracellular Traps (NETs)
Abstract

Objective. Neutrophil extracellular traps (NET) expose modified antigens for autoantibodies in vasculitis. Little is known about levels and removal pathways of NET in systemic lupus erythematosus (SLE), especially in lupus nephritis (LN). We determined circulating levels and defined NET removal in large subsets of patients with incident SLE (iSLE), some of whom had new-onset nephritis. Methods. Serum levels of NET (ELISA), DNase1/DNase1L3 (ELISA), and DNase activity (functional assay) were determined in 216 patients with iSLE [103 had incident LN (iLN)], in 50 patients with other primary glomerulonephritis, and in healthy controls. Ex vivo NET production by neutrophils purified from a random selection of patients was quantified as elastase/DNA release and by immunofluorescence techniques. Results. Serum NET levels were very high in iSLE/iLN compared to all groups of controls and correlated with anti-dsDNA, C3-C4, and proteinuria; iLN had the highest levels. DNase activity was decreased in iLN compared to SLE (20% had one-half DNase activity) despite similar serum levels of DNase1/DNase1L3. In these cases, pretreatment of serum with protein A restored DNase efficiency; 1 patient was homozygous for a c.289_290delAC variant of DNASE1L3. Ex vivo NET production by neutrophils purified from LN, SLE, and normal controls was similar in all cases. Conclusion. Patients with iLN have increased circulating NET and reduced DNase activity, the latter being explained by the presence of inhibitory substances in circulation and/or by rare DNase1L3 mutations. Accumulation of NET derives from a multifactorial mechanism, and is associated and may contribute to disease severity in SLE, in particular to renal lesions. (Clinical trial registration: The Zeus study was registered at ClinicalTrials.gov, study number NCT02403115).

Published
2020

4. Serum IgG2 antibody multi composition in systemic lupus erythematosus and in lupus nephritis

Author

Bruschi, M, Moroni, G, Sinico, Ra, Franceschini, F, Fredi, M, Vaglio, A, Cavagna, L, Petretto, A, Pratesi, F, Migliorini, P, Locatelli, F, Pazzola, G, Pesce, G, Bagnasco, M, Manfredi, A, Ramirez, Ga, Esposito, P, Murdaca, G, Negrini, S, Cipriani, L, Trezzi, B, Emmi, G, Cavazzana, I, Binda, V, D'Alessandro, M, Fenaroli, P, Pisani, I, Garibotto, G, Montecucco, C, Santoro, D, Scolari, F, Volpi, S, Mosca, M, Tincani, A, Candiano, G, Prunotto, M, Verrina, E, Angeletti, A, Ravelli, A, and Ghiggeri, Gm.

Published
2020

5. MALDI imaging in Fabry nephropathy: a multicenter study

Author

L'Imperio, V, Smith, A, Pisani, A, D'Armiento, M, Scollo, V, Casano, S, Sinico, R, Nebuloni, M, Tosoni, A, Pieruzzi, F, Magni, F, Pagni, F, Sinico, RA, L'Imperio, V, Smith, A, Pisani, A, D'Armiento, M, Scollo, V, Casano, S, Sinico, R, Nebuloni, M, Tosoni, A, Pieruzzi, F, Magni, F, Pagni, F, and Sinico, RA

Abstract

Background The current study evaluates the application of histology and in situ proteomics (MALDI-MSI) in Fabry nephropathy (FN), showing investigative and classification role for this coupled approach. Methods A retrospective series of 14 formalin fixed paraffin embedded (FFPE) renal biopsies with diagnosis of FN and 1 biopsy from a patient bearing a galactosidase-alpha (GLA) genetic variant of unknown significance (GVUS, c.376A>G) have been classified for clinical characteristics. Groups were compared for histological differences (following the ISGFN scoring system). Moreover, renal biopsies from these cases have been analyzed with MALDI-MSI as previously described to find proteomic signatures among different mutations and phenotypes. Results Comparison of clinical features revealed lower mean 24 h proteinuria in females (225 mg/24 h) than in males (1477.5 mg/24 h, p = 0.006). As for clinical characteristics, females significantly differed from males only for lower arterial sclerosis, with a mean value of 0.82 vs. 1.05 (p = 0.001). Proteomic analysis demonstrated specific signatures in different subgroups of FN patients. Moreover, MALDI correctly classified cases with undetermined mutation or GVUS. Conclusions The present study demonstrated the feasible application of MALDI-MSI in the analysis of FN FFPE renal biopsies, allowing the detection of putative signatures for phenotypic distinction and demonstrating genetic classification capabilities.

Published
2020

6. Combined Plasmatic and Tissue Approach to Membranous Nephropathy-Proposal of a Diagnostic Algorithm Including Immunogold Labelling: Changing the Paradigm of a Serum-based Approach

Author

L'Imperio, V, Pieruzzi, F, Sinico, R, Nebuloni, M, Tosoni, A, Granata, A, Santoro, D, Capelli, I, Garozzo, M, Casano, S, Smith, A, Radice, A, Pagni, F, Sinico, RA, L'Imperio, V, Pieruzzi, F, Sinico, R, Nebuloni, M, Tosoni, A, Granata, A, Santoro, D, Capelli, I, Garozzo, M, Casano, S, Smith, A, Radice, A, Pagni, F, and Sinico, RA

Abstract

Membranous nephropathy represents the most frequent cause of nephrotic syndrome in the adult, leading to end-stage renal disease in one third of all the patients. In the last years, the discovery of circulating autoantibodies against phospholipase A2 receptor 1 (PLA2R) and thrombospondin type-1 containing 7A domain (THSD7A), shed light on the pathogenesis of idiopathic forms, being responsible for 70% and 3% of all the cases, respectively. These identifications allowed the development of serological and histologic tests to detect autoantibodies and relative targets for diagnostic and prognostic purposes. Rising evidences suggest that serum titer correlates with disease activity and response to therapy. For these reasons, for patients with nephrotic syndrome, a serum-based approach has been proposed, reserving renal biopsy only in cases with doubtful/negative serology. However, the recent introduction of useful criteria for the interpretation of PLA2R/THSD7A immunohistochemistry could lead to high values of sensitivity and specificity for the in situ detection of target antigens. The present multicentric study on a series of membranous nephropathy cases with available serum/histologic correlation will show the importance of the crosstalk among the different techniques, recovering the possible role of electron microscopy in challenging situations.

Published
2020

7. PREDICTION OF LONG-TERM EVOLUTIONARY PROFILES IN EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS (CHURG-STRAUSS) BASED ON BASELINE AND FOLLOW-UP CHARACTERISTICS

Author

Papo, M, Sinico, Ra, Teixeira, V, Urban, Ml, Mahrhold, J, Monti, S, Cassone, G, Schiavon, F, Seeliger, B, Neumann, T, Kroegel, C, Groh, M, Marvisi, C, Samson, M, Barba, T, Jayne, D, Hellmich, B, Montecucco, C, Salvarani, C, Kahn, Je, Bonnotte, B, Durel, Ca, Mouthon, L, Puechal, X, Guillevin, L, Emmi, G, Vaglio, A, and Terrier, B

Published
2019

10. Genome-wide association study of eosinophilic granulomatosis with polyangiitis reveals genomic loci stratified by ANCA status

Author

Lyons, PA, Peters, JE, Alberici, F, Liley, J, Coulson, RMR, Astle, W, Baldini, C, Bonatti, F, Cid, MC, Elding, H, Emmi, G, Epplen, J, Guillevin, L, Jayne, DRW, Jiang, T, Gunnarsson, I, Lamprecht, P, Leslie, S, Little, MA, Martorana, D, Moosig, F, Neumann, T, Ohlsson, S, Quickert, S, Ramirez, GA, Rewerska, B, Schett, G, Sinico, RA, Szczeklik, W, Tesar, V, Vukcevic, D, Akil, M, Barratt, J, Basu, N, Butterworth, AS, Bruce, I, Clarkson, M, Conlon, N, DasGupta, B, Doulton, TWR, Espigol-Frigole, G, Flossmann, O, Gabrielli, A, Gasior, J, Gregorini, G, Guida, G, Hernandez-Rodriguez, J, Hruskova, Z, Hudson, A, Knight, A, Lanyon, P, Luqmani, R, Magliano, M, Manfredi, AA, Marguerie, C, Maritati, F, Marvisi, C, McHugh, NJ, Molloy, E, Motyer, A, Mukhtyar, C, Padyukov, L, Pesci, A, Prieto-Gonzalez, S, Ramentol-Sintas, M, Reis, P, Roccatello, D, Rovere-Querini, P, Salvarani, C, Santarsia, F, Solans-Laque, R, Soranzo, N, Taylor, J, Wessels, J, Zwerina, J, Terrier, B, Watts, RA, Vaglio, A, Holle, JU, Wallace, C, Smith, KGC, Lyons, PA, Peters, JE, Alberici, F, Liley, J, Coulson, RMR, Astle, W, Baldini, C, Bonatti, F, Cid, MC, Elding, H, Emmi, G, Epplen, J, Guillevin, L, Jayne, DRW, Jiang, T, Gunnarsson, I, Lamprecht, P, Leslie, S, Little, MA, Martorana, D, Moosig, F, Neumann, T, Ohlsson, S, Quickert, S, Ramirez, GA, Rewerska, B, Schett, G, Sinico, RA, Szczeklik, W, Tesar, V, Vukcevic, D, Akil, M, Barratt, J, Basu, N, Butterworth, AS, Bruce, I, Clarkson, M, Conlon, N, DasGupta, B, Doulton, TWR, Espigol-Frigole, G, Flossmann, O, Gabrielli, A, Gasior, J, Gregorini, G, Guida, G, Hernandez-Rodriguez, J, Hruskova, Z, Hudson, A, Knight, A, Lanyon, P, Luqmani, R, Magliano, M, Manfredi, AA, Marguerie, C, Maritati, F, Marvisi, C, McHugh, NJ, Molloy, E, Motyer, A, Mukhtyar, C, Padyukov, L, Pesci, A, Prieto-Gonzalez, S, Ramentol-Sintas, M, Reis, P, Roccatello, D, Rovere-Querini, P, Salvarani, C, Santarsia, F, Solans-Laque, R, Soranzo, N, Taylor, J, Wessels, J, Zwerina, J, Terrier, B, Watts, RA, Vaglio, A, Holle, JU, Wallace, C, and Smith, KGC

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare inflammatory disease of unknown cause. 30% of patients have anti-neutrophil cytoplasmic antibodies (ANCA) specific for myeloperoxidase (MPO). Here, we describe a genome-wide association study in 676 EGPA cases and 6809 controls, that identifies 4 EGPA-associated loci through conventional case-control analysis, and 4 additional associations through a conditional false discovery rate approach. Many variants are also associated with asthma and six are associated with eosinophil count in the general population. Through Mendelian randomisation, we show that a primary tendency to eosinophilia contributes to EGPA susceptibility. Stratification by ANCA reveals that EGPA comprises two genetically and clinically distinct syndromes. MPO+ ANCA EGPA is an eosinophilic autoimmune disease sharing certain clinical features and an HLA-DQ association with MPO+ ANCA-associated vasculitis, while ANCA-negative EGPA may instead have a mucosal/barrier dysfunction origin. Four candidate genes are targets of therapies in development, supporting their exploration in EGPA.

Published
2019

11. FP165 Slowly progressive anca-associated glomerulonephritis: a multicentre study

Author

Trivioli, G, Gopaluni, S, Urban, M, Gianfreda, D, Cassia, M, Vercelloni, P, Calatroni, M, Esposito, P, Murtas, C, Alberici, F, Moroni, G, Gregorini, G, Sinico, R, Jayne, D, Vaglio, A, Urban, ML, Cassia, MA, Vercelloni, PG, Sinico, RA, Trivioli, G, Gopaluni, S, Urban, M, Gianfreda, D, Cassia, M, Vercelloni, P, Calatroni, M, Esposito, P, Murtas, C, Alberici, F, Moroni, G, Gregorini, G, Sinico, R, Jayne, D, Vaglio, A, Urban, ML, Cassia, MA, Vercelloni, PG, and Sinico, RA

Published
2019

13. Rituximab as maintenance treatment for systemic Lupus ErythematosusA multicentre observational study of 147 patients

Author

Cassia, M, Alberici, F, Jones, R, Smith, R, Casazza, G, Urban, M, Emmi, G, Moroni, G, Sinico, R, Messa, P, Hall, F, Vaglio, A, Gallieni, M, Jayne, D, Cassia, MA, Jones, RB, Smith, RM, Urban, ML, Sinico, RA, Jayne, DR, Cassia, M, Alberici, F, Jones, R, Smith, R, Casazza, G, Urban, M, Emmi, G, Moroni, G, Sinico, R, Messa, P, Hall, F, Vaglio, A, Gallieni, M, Jayne, D, Cassia, MA, Jones, RB, Smith, RM, Urban, ML, Sinico, RA, and Jayne, DR

Abstract

Objective: The efficacy of rituximab (RTX) in systemic lupus erythematosus (SLE) is a subject of debate. This study was undertaken to investigate the outcomes of RTX treatment in a European SLE cohort, with an emphasis on the role of RTX as a maintenance agent. Methods: All patients with SLE who were receiving RTX as induction therapy in 4 centers were included. Patients who received a single course of RTX and those who received RTX maintenance treatment (RMT) were followed up after treatment. Disease flares during the follow-up period were defined as an increase in disease activity and the number or dose of immunosuppressive drugs. Results: Of 147 patients, 27% experienced treatment failure at 6 months. In a multivariate analysis, a low number of previous immunosuppressive therapies (P = 0.034) and low C4 levels (P = 0.008) reduced the risk of treatment failure. Eighty patients received RMT over a median of 24.5 months during which 85 relapses, mainly musculoskeletal, were recorded (1.06 per patient). At the time of the last RTX course, 84% of the patients were in remission. Twenty-eight (35%) of 80 patients never experienced a flare during RMT and had low damage accrual. Active articular disease at the time of the first RTX administration was associated with a risk of flare during RMT (P = 0.011). After RMT, relapse-free survival was similar to that in patients receiving a single RTX course (P = 0.72). Conclusion: RMT is a potential treatment option for patients with difficult-to-treat disease. Relapses occur during RMT and are more likely in those with active articular disease at the time of the first RTX administration. Relapse risk after RMT remains high and apparently comparable to that seen after a single RTX course.

Published
2019

14. MALDI-MSI Pilot Study Highlights Glomerular Deposits of Macrophage Migration Inhibitory Factor (MIF) as a Possible Indicator of Response to Therapy in Membranous Nephropathy

Author

L'Imperio, V, Smith, A, Ajello, E, Piga, I, Stella, M, Denti, V, Tettamanti, S, Sinico, R, Pieruzzi, F, Garozzo, M, Vischini, G, Nebuloni, M, Pagni, F, Magni, F, L'Imperio V, Smith A, Ajello E, Piga I, Stella M, Denti V, Tettamanti S, Sinico RA, Pieruzzi F, Garozzo M, Vischini G, Nebuloni M, Pagni F, Magni F., L'Imperio, V, Smith, A, Ajello, E, Piga, I, Stella, M, Denti, V, Tettamanti, S, Sinico, R, Pieruzzi, F, Garozzo, M, Vischini, G, Nebuloni, M, Pagni, F, Magni, F, L'Imperio V, Smith A, Ajello E, Piga I, Stella M, Denti V, Tettamanti S, Sinico RA, Pieruzzi F, Garozzo M, Vischini G, Nebuloni M, Pagni F, and Magni F.

Abstract

Membranous Nephropathy (MN) is the most frequent cause of nephrotic syndrome in adults and the disease course is characterised by the "rule of third", with one third of patients experiencing complete remission and the remaining experiencing relapses or progression of the disease. Additionally, the therapeutic approach is not standardised, leading to further heterogeneity in terms of response and outcome. In this pilot study, MALDI-MSI analysis was performed on renal biopsies (n = 13) obtained from two homogeneous groups of patients which differentially responded to the immunosuppressive treatments (Ponticelli regimen). A signal at m/z 1303 displayed the greatest discriminatory power when comparing the two groups and was observed to be of higher intensity in the glomeruli of the non-responding patients. The corresponding tryptic peptide was identified as macrophage migration inhibitory factor (MIF). Despite much effort being made in recent years to understand the pathogenesis of MN, a biomarker able to predict the outcome of these patients following therapeutic treatment is still lacking. Here, we highlight a protein (MIF), verified by immunohistochemistry, which can differentiate between these MN patients and could be a valuable starting point for a further study focused on verifying its predictive role in therapy response. This article is protected by copyright. All rights reserved

Published
2019

15. Milder clinical presentation of lupus nephritis and improved renal survival during the last 50 years: a multicentric study

Author

Gatto, M, MORONI, GABRIELLA, Raffiotta, F, VERCELLONI, PAOLO GILLES, Quaglini, S, Sacchi, L, Gainfreda, D, Vaglio, A, Sinico, RA, Doria, A, Gatto, M, Moroni, G, Raffiotta, F, Vercelloni, P, Quaglini, S, Sacchi, L, Gainfreda, D, Vaglio, A, Sinico, R, and Doria, A

Subjects
lupus nephritis, outcome, clinical presentation
Abstract

Background Lupus nephritis (LN) presentation changed over time following earlier diagnosis and treatment. Objectives To evaluate changes in LN clinical and histological presentation in the last 5 decades. Methods This is a retrospective multicentric study on prospectively collected data in four Italian hospital centres. Patients diagnosed between 1970 and 2016 were recruited provided they had a biopsy-proven LN that was retrospectively reclassified according to the ISN/RPS classification criteria. Follow-up was subdivided into three periods (P) based on the year of LN diagnosis: P1:1970–1895; P2:1986–2000; P3:2001–2016. Predictors of patient and renal survival were investigated by univariate and multivariate analysis; survival curves were compared by log-rank test. Clinical pictures at presentation included isolated urinary abnormalities, nephrotic syndrome, nephritic syndrome, rapidly progressive renal failure. Outcome at last observation was defined as complete renal remission or partial renal remission, or poor renal outcome, including chronic kidney disease (CKD) or end stage renal disease (ESRD). Results 499 patients were included (85.6% females) with a median follow-up of 10.6 years (IQR 4–18). We observed an increase in both age at diagnosis of LN (P1 28.4±10.4; P2 29±11.5; P3 34.4±13.3 years) and disease duration before LN diagnosis (P1 1.3±1.3; P2 2.6±4.5; P3 4.6±6.3 years) from 1970 to 2016 (p

Published
2018

16. Caractéristiques au diagnostic et profils évolutifs des patients atteints de granulomatose éosinophilique avec polyangéite (Churg-Strauss) : données d’une étude rétrospective collaborative européenne

Author

Papo, M, Emmi, G, Schiavon, F, Groh, M, Samson, M, Kahn, J, Sinico, R, Puéchal, X, Mouthon, L, Guillevin, L, Vaglio, A, Terrier, B, Kahn, JE, Sinico, RA, Papo, M, Emmi, G, Schiavon, F, Groh, M, Samson, M, Kahn, J, Sinico, R, Puéchal, X, Mouthon, L, Guillevin, L, Vaglio, A, Terrier, B, Kahn, JE, and Sinico, RA

Published
2018

17. Caractéristiques de l’atteinte rénale au cours de la granulomatose éosinophilique avec polyangéite : étude rétrospective multicentrique portant sur 63 patients avec histologie rénale

Author

Durel, C, Sinico, R, Teixeira, V, Jayne, D, Belenfant, X, Marchand-Adam, S, Pugnet, G, Gaultier, J, Le Gallou, T, Beauport, D, Karras, A, Durel, CA, Sinico, RA, Beauport, DT, Durel, C, Sinico, R, Teixeira, V, Jayne, D, Belenfant, X, Marchand-Adam, S, Pugnet, G, Gaultier, J, Le Gallou, T, Beauport, D, Karras, A, Durel, CA, Sinico, RA, and Beauport, DT

Published
2018

18. Efficacité des traitements utilisés chez les patients suivis pour une granulomatose éosinophilique avec polyangéite (Churg-Strauss) en fonction du profil évolutif de la maladie : données d’une étude rétrospective collaborative européenne

Author

Papo, M, Emmi, G, Schiavon, F, Groh, M, Kahn, J, Sinico, R, Samson, M, Puéchal, X, Mouthon, L, Guillevin, L, Vaglio, A, Terrier, B, Kahn, JE, Sinico, RA, Papo, M, Emmi, G, Schiavon, F, Groh, M, Kahn, J, Sinico, R, Samson, M, Puéchal, X, Mouthon, L, Guillevin, L, Vaglio, A, Terrier, B, Kahn, JE, and Sinico, RA

Published
2018

19. Routine immunohistochemical staining in membranous nephropathy: in situ detection of phospholipase A2 receptor and thrombospondin type 1 containing 7A domain

Author

L'Imperio, V, Pieruzzi, F, Sinico, R, Nebuloni, M, Granata, A, Smith, A, Radice, A, Pagni, F, Sinico, RA, L'Imperio, V, Pieruzzi, F, Sinico, R, Nebuloni, M, Granata, A, Smith, A, Radice, A, Pagni, F, and Sinico, RA

Abstract

Background: Membranous nephropathy (MN) can be idiopathic (iMN) or manifest as a result of systemic underlying conditions as a secondary epiphenomenon. For the prognostic and predictive consequences of this discrimination, the routine use of reliable markers is crucial. This large MN series aimed to evaluate the routine and standardized immunohistochemical (IHC) employment of a panel of 3 biomarkers—phospholipase A2 receptor (PLA2R), thrombospondin type-1 domain-containing 7A (THSD7A), and immunoglobulin (Ig)G4—in the differential diagnosis of MN forms, contributing to the validation of the technique and the correct interpretation of reproducible patterns of reactivity. Methods: We classified 95 patients with a biopsy proven diagnosis of MN as primary (n = 72) or secondary (n = 23) cases based on clinical data. After performing an IHC assay directed against PLA2R, THSD7A and IgG4 antigens, samples were interpreted by three different nephropathologists to assess the positivity/negativity of the staining according to new interpretation criteria. Results: Useful interpretation criteria were introduced to exclude false positive patterns of reactivity and to identify only true granular membranous or mesangial deposits in MN. The IHC directed against PLA2R resulted positive in 51 iMN cases and negative in 21, while 4/23 secondary forms were considered positive. Based on these data the technique showed a sensitivity of 71% and specificity of 83%. On the other hand, the IHC analysis for IgG4 resulted positive in 44 cases of iMN and negative in 28 cases, while only 4/23 secondary forms were positive (same cases positive to PLA2R). Finally, THSD7A was found to be positive only in 1 case, which was negative to PLA2R and IgG4. The combination of the results allowed a classification of the series into two major groups: “double-positive” (PLA2R+/IgG4+/THSD7A−) and “triple-negative” (PLA2R−/IgG4−/THSD7A−) cases. Conclusions: Based on these data, the diagnostic performance of the t

Published
2018

20. Anticoagulant-related nephropathy: a pathological note

Author

L'Imperio, V, Guarnieri, A, Pieruzzi, F, Sinico, R, Pagni, F, Sinico, RA, L'Imperio, V, Guarnieri, A, Pieruzzi, F, Sinico, R, Pagni, F, and Sinico, RA

Abstract

The wide employment of oral anticoagulants and the introduction of new anticoagulant agents highlight disparate kind of toxicities that can affect many different organ systems. Renal toxicity by oral anticoagulants is a well-known entity characterized by hematuria and the worsening of renal function associated with uncontrolled INR values. Although it is mainly a clinical diagnosis, renal biopsy may help especially in challenging cases when multiple comorbidities and underlying renal conditions exist. The mechanism of the anticoagulant-induced damage is still debated and special tissue stains (such as Perls’) could help in detecting the direct tubular toxicity induced by chronic glomerular bleeding. The employment of a diagnostic clinic-pathological flow-chart can help in the prompt detection and full characterization of these cases, improving the management of the patient.

Published
2018

21. ANCA-associated vasculitis in childhood: Recent advances

Author

Calatroni, M, Oliva, E, Gianfreda, D, Gregorini, G, Allinovi, M, Ramirez, G, Bozzolo, E, Monti, S, Bracaglia, C, Marucci, G, Bodria, M, Sinico, R, Pieruzzi, F, Moroni, G, Pastore, S, Emmi, G, Esposito, P, Catanoso, M, Barbano, G, Bonanni, A, Vaglio, A, Sinico, RA, Pieruzzi, FUEG, Vaglio, A., Calatroni, M, Oliva, E, Gianfreda, D, Gregorini, G, Allinovi, M, Ramirez, G, Bozzolo, E, Monti, S, Bracaglia, C, Marucci, G, Bodria, M, Sinico, R, Pieruzzi, F, Moroni, G, Pastore, S, Emmi, G, Esposito, P, Catanoso, M, Barbano, G, Bonanni, A, Vaglio, A, Sinico, RA, Pieruzzi, FUEG, and Vaglio, A.

Abstract

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides are rare systemic diseases that usually occur in adulthood. They comprise granulomatosis with polyangiitis (GPA, Wegener's), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome). Their clinical presentation is often heterogeneous, with frequent involvement of the respiratory tract, the kidney, the skin and the joints. ANCA-associated vasculitis is rare in childhood but North-American and European cohort studies performed during the last decade have clarified their phenotype, patterns of renal involvement and their prognostic implications, and outcome. Herein, we review the main clinical and therapeutic aspects of childhood-onset ANCA-associated vasculitis, and provide preliminary data on demographic characteristics and organ manifestations of an Italian multicentre cohort.

Published
2017

22. [OP.4B.05] MIR-30A-3P EXPRESSION IN THE HEARTS OF DIABETIC RATS

Author

Castoldi, G, Di Gioia, C, Roma, F, Carletti, R, Sinico, R, Stella, A, Zerbini, G, Perseghin, G, ROMA, FRANCESCA, Sinico, RA, Castoldi, G, Di Gioia, C, Roma, F, Carletti, R, Sinico, R, Stella, A, Zerbini, G, Perseghin, G, ROMA, FRANCESCA, and Sinico, RA

Published
2017

23. Revisiting the systemic vasculitis in eosinophilic granulomatosis with polyangiitis (Churg-Strauss). A study of 157 patients by the Groupe d'Etudes et de Recherche sur les Maladies Orphelines Pulmonaires and the European Respiratory Society Taskforce on eosinophilic granulomatosis with polyangiitis (Churg-Strauss)

Author

Cottin, V, Bel, E, Bottero, P, Dalhoff, K, Humbert, M, Lazor, R, Sinico, R, Sivasothy, P, Wechsler, M, Groh, M, Marchand Adam, S, Khouatra, C, Wallaert, B, Taillé, C, Delaval, P, Cadranel, J, Bonniaud, P, Prévot, G, Hirschi, S, Gondouin, A, Dunogué, B, Chatté, G, Briault, C, Pagnoux, C, Jayne, D, Guillevin, L, Cordier, J, Sinico, Ra, Cordier, J., Cottin, V, Bel, E, Bottero, P, Dalhoff, K, Humbert, M, Lazor, R, Sinico, R, Sivasothy, P, Wechsler, M, Groh, M, Marchand Adam, S, Khouatra, C, Wallaert, B, Taillé, C, Delaval, P, Cadranel, J, Bonniaud, P, Prévot, G, Hirschi, S, Gondouin, A, Dunogué, B, Chatté, G, Briault, C, Pagnoux, C, Jayne, D, Guillevin, L, Cordier, J, Sinico, Ra, and Cordier, J.

Abstract

Objective: To guide nosology and classification of patients with eosinophilic granulomatosis with polyangiitis (EGPA) based on phenotype and presence or absence of ANCA. Methods: Organ manifestations and ANCA status were retrospectively analyzed based on the presence or not of predefined definite vasculitis features or surrogates of vasculitis in patients asthma, eosinophilia, and at least one systemic organ manifestation attributable to systemic disease. Results: The study population included 157 patients (mean age 49.4. ±. 14.1), with a follow-up of 7.4. ±. 6.4. years. Patients with ANCA (31%) more frequently had weight loss, myalgias, arthralgias, biopsy-proven vasculitis, glomerulonephritis on biopsy, hematuria, leukocytoclastic capillaritis and/or eosinophilic infiltration of arterial wall on biopsy, and other renal disease. A total of 41% of patients had definite vasculitis manifestations (37%) or strong surrogates of vasculitis (4%), of whom only 53% had ANCA. Mononeuritis multiplex was associated with systemic vasculitis (p. =0.005) and with the presence of ANCA (p. <. 0.001). Overall, 59% of patients had . polyangiitis as defined by definite vasculitis, strong surrogate of vasculitis, mononeuritis multiplex, and/or ANCA with at least one systemic manifestation other than ENT or respiratory. Patients with . polyangiitis had more systemic manifestations including arthralgias (p. =0.02) and renal disease (p. =0.024), had higher peripheral eosinophilia (p. =0.027), and a trend towards less myocarditis (p. =0.057). Using predefined criteria of vasculitis and surrogates of vasculitis, ANCA alone were found to be insufficient to categorise patients with vasculitis features. Conclusion: We suggest a revised nomenclature and definition for EGPA and a new proposed entity referred to as hypereosinophilic asthma with systemic (non vasculitic) manifestations

Published
2017

24. Pulse versus daily oral cyclophosphamide for induction of remission in ANCA-associated vasculitis: long-term follow-up

Author

Harper, L1, Morgan, Md, Walsh, M, Hoglund, P, Westman, K, Flossmann, O, Tesar, V, Vanhille, P, de Groot, K, Luqmani, R, Flores Suarez LF, Watts, R, Pusey, C, Bruchfeld, A, Rasmussen, N, Blockmans, D, Savage, Co, Jayne, D, EUVAS i. n. v. e. s. t. i. g. a. t. o. r. s. Abramowicz, D, Wissing, M, Madhoun, P, Stolear, J, Ekstrand, A, Chabova, V, Rychlik, I, Bataille, P, Puechal, X, Leblau, J, Esnault, Vl, Gross, Wl, Weidner, S, Rupprecht, H, Schneider, M, Specker, C, Schmitt, W, van der Woude, F, Vischedyck, M, Feighery, C, Garibotto, Giacomo, Sinico, Ra, Santostefano, M, Dadoniene, J, Hagen, Ec, Oudejans, I, Verburgh, C, Ballarin, J, Mirapeix, E, Valles, M, Pettersson, E, Eriksson, P, Selga, D, Segelmark, M, Sterner, G, Lundberg, I, Svenungsson, E, Chizzolini, C, Adu, D, de Souza, R., and Chizzolini, Carlo

Subjects
Male, Time Factors, Administration, Oral, Kidney, law.invention, Cyclophosphamide/administration & dosage, Randomized controlled trial, law, Risk Factors, Secondary Prevention, Immunology and Allergy, ddc:616, Aged, 80 and over, ANCA, Remission Induction, Middle Aged, Female, Vasculitis, Immunosuppressive Agents, medicine.drug, Systemic vasculitis, Adult, medicine.medical_specialty, Cyclophosphamide, Adolescent, Kidney/physiology, Immunology, General Biochemistry, Genetics and Molecular Biology, Antibodies, Antineutrophil Cytoplasmic, Young Adult, Rheumatology, Internal medicine, medicine, Kidney Function, Humans, Adverse effect, Immunosuppressive Agents/administration & dosage, Aged, Retrospective Studies, business.industry, Vasculitis/drug therapy/immunology/mortality, Antibodies, Antineutrophil Cytoplasmic/immunology, Retrospective cohort study, medicine.disease, Surgery, Regimen, Pulse Therapy, Drug, business, Follow-Up Studies
Abstract

INTRODUCTION: The previously reported randomised controlled trial of a consensus regimen of pulse cyclophosphamide suggested that it was as effective as a daily oral (DO) cyclophosphamide for remission induction of antineutrophil cytoplasm autoantibodies-associated systemic vasculitis when both were combined with the same glucocorticoid protocol (CYCLOPS study (Randomised trial of daily oral versus pulse Cyclophosphamide as therapy for ANCA-associated Systemic Vasculitis published de groot K, harper L et al Ann Int Med 2009)). The study had limited power to detect a difference in relapse. This study describes the long-term outcomes of patients in the CYCLOPS study. METHODS: Long-term outcomes were ascertained retrospectively from 148 patients previously recruited to the CYCLOPS Trial. Data on survival, relapse, immunosuppressive treatment, cancer incidence, bone fractures, thromboembolic disease and cardiovascular morbidity were collected from physician records retrospectively. All patients were analysed according to the group to which they were randomised. RESULTS: Median duration of follow-up was 4.3 years (IQR, 2.95-5.44 years). There was no difference in survival between the two limbs (p=0.92). Fifteen (20.8%) DO and 30 (39.5%) pulse patients had at least one relapse. The risk of relapse was significantly lower in the DO limb than the pulse limb (HR=0.50, 95% CI 0.26 to 0.93; p=0.029). Despite the increased risk of relapse in pulse-treated patients, there was no difference in renal function at study end (p=0.82). There were no differences in adverse events between the treatment limbs. DISCUSSION: Pulse cyclophosphamide is associated with a higher relapse risk than DO cyclophosphamide. However, this is not associated with increased mortality or long-term morbidity. Although the study was retrospective, data was returned in 90% of patients from the original trial.

Published
2016

25. Eosinophilic granulomatosis with polyangiitis (churg-straus syndrome)

Author

Dammacco, F, Ribatti, D, Vacca, A, Sinico, R, Bottero, P, Sinico, RA, Dammacco, F, Ribatti, D, Vacca, A, Sinico, R, Bottero, P, and Sinico, RA

Abstract

Eosinophilic Granulomatosis with Polyangiitis (EGPA) is defined as an eosinophil-rich and granulomatous inflammation involving the respiratory tract, and necrotizing vasculitis affecting small to medium-sized vessels, and associated with asthma and eosinophilia. It is usually classified among the so-called antineutrophil antibody (ANCA)-associated systemic vasculitides (AASVs) because of its clinical and pathologic features that overlap with those of the other AASVs. However, recent studies on large cohorts of patients have found that ANCA, usually P-ANCA/MPO-ANCA, were present in less than 40 % of patients. Moreover, ANCA status was shown to segregate with clinical phenotype. Preliminary results suggest that ANCA-positive and ANCA-negative patients also might have a different genetic background. Corticosteroids remain the cornerstone of the initial treatment of EGPA. The addition of cyclophosphamide is indicated in patients with poor-prognosis factors or in patients without poor-prognosis factors that relapse early. How long should maintenance therapy be continued remains to be established. However, the vast majority of patients require long-term corticosteroids treatment to control asthma.

Published
2016

26. Anti-M-Type Phospholipase A2 Receptor (PLA2R) Antibodies: Diagnostic Performance and Clinical Significance in Idiopathic Membranous Nephropathy (IMN)

Author

Mezzina, N., Brunini, F., Trezzi, B., Gallieni, M., D Amico, M., Stellato, T., Santoro, D., Gian Marco Ghiggeri, Radice, A., Sinico, Ra, Mezzina, N, Brunini, F, Trezzi, B, Gallieni, M, D'Amico, M, Stellato, T, Santoro, D, Ghiggeri, G, Radice, A, and Sinico, R

Subjects
membranous glomerulonephritis, anti-PLA2r antibody, diagnostic performance
Published
2014

28. Risk for chronic kidney disease in the general population italian reports for the the world kiney days 2007-2009

Author

Galassi, A, Brancaccio, D, Andreucci, Ve, Andreucci, M, Caglioti, A, Faga, T, Mazzitello, G, Antonelli, A, Carlini, A, Giusti, R, Rosati, A, Apperti, V, Auricchio, Mr, Avella, F, Barbato, A, Romano, P, Barzaghi, B, Bedani, Pl, Bellinghieri, G, Costantino, G, Bernardi, A, Bolasco, P, Ferrara, R, Pani, A, Bonomini, M, Busnach, G, Conte, F, Limido, A, Messa, Pg, Sinico, Ra, Spotti, D, Cadinu, F, Campieri, C, Capuano, M, Terribile, M, Cardone, F, Paglia, S, Castellino, P, Di Landro, D, Fatuzzo, P, Liuzzo, G, Sicurezza, E, Casu, Md, Centrone, E, Ciofani, A, Cossu, M, D'Amaro, E, D'Apice, L, Dal Canton, A, Fasoli, G, De Ferrari, G, Cappelli, Gianni, Gusmano, R, De Simone, W, Delgado, G, Di Iorio, B, Di Luca, M, Di Natale, E, Farfaglia, P, Cantú, P, Feriozzi, S, Galeotti, P, Fiorini, F, Frascà, Gm, Gallucci, M, Buongiorno, E, Gesualdo, L, Giannattasio, M, Detomaso, F, Giannetto, M, Gianni, S, Giliberti, A, Grassi, C, Lupi, Gp, Imperiali, P, Juliano, P, Li Vecchi, M, Lucenti, T, Maffucci, G, Anelli, Am, Manno, M, Schena, Fp, Mazzaferro, S, Migliorati, M, Morrone, L, Mura, M, Petrarulo, F, Pizzarelli, F, Ricciardi, B, Riccobene, G, Rondanini, V, Russo, D, Sasdelli, M, Mura, C, Scarpino, L, Selvi, S, Tarchini, R, Tedesco, A, Timio, F, Traversari, L, Venditti, G, Viganò, S, Locatelli, F., Galassi, A., Andreucci, M, Caglioti, A., Faga, T., Mazzitello, G., Antonelli, A., Carlini, A., Giusti, R., Rosati, A., Apperti, V., Auricchio, M. R., Avella, F., Barbato, A., Romano, P., Barzaghi, B., Bedani, P. L., Bellinghieri, G., Costantino, G., Bernardi, A., Bolasco, P., Ferrara, R., Pani, A., Bonomini, M., Brancaccio, D., Busnach, G., Conte, F., Limido, A., Messa, P. G., Sinico, R. A., Spotti, D., Cadinu, F., Campieri, C., Capuano, M., Terribile, M., Cardone, F., Paglia, S., Castellino, P., Di Landro, D., Fatuzzo, P., Liuzzo, G., Sicurezza, E., Casu, M. D., Centrone, E., Ciofani, A., Cossu, M., D'Amaro, E., D'Apice, L., Dal Canton, A., Fasoli, G., De Ferrari, G., Cappelli, G., Gusmano, R., De Simone, W., Delgado, G., Di Iorio, B., Di Luca, M., Di Natale, E., Farfaglia, P., Cantù, P., Feriozzi, S., Galeotti, P., Fiorini, F., Frascà, G. M., Gallucci, M., Buongiorno, E., Gesualdo, L., Giannattasio, M., Detomaso, F., Giannetto, M., Gianni, S., Giliberti, A., Grassi, C., Lupi, G. P., Imperiali, P., Juliano, P., Li Vecchi, M., Lucenti, T., Maffucci, G., Anelli, A. M., Manno, M., Schena, F. P., Mazzaferro, S., Migliorati, M., Morrone, L., Mura, M., Petrarulo, F., Pizzarelli, F., Ricciardi, B., Riccobene, G., Rondanini, V., Russo, Domenico, Sasdelli, M., Mura, C., Scarpino, L., Selvi, S., Tarchini, R., Tedesco, A., Timio, M., Traversari, L., Venditti, G., Viganò, S., Locatelli, F., Galassi, A, Caglioti, A, Faga, T, Mazzitello, G, Antonelli, A, Carlini, A, Giusti, R, Rosati, A, Apperti, V, Auricchio, M, Avella, F, Barbato, A, Romano, P, Barzaghi, B, Bedani, P, Bellinghieri, G, Costantino, G, Bernardi, A, Bolasco, P, Ferrara, R, Pani, A, Bonomini, M, Brancaccio, D, Busnach, G, Conte, F, Limido, A, Messa, P, Sinico, R, Spotti, D, Cadinu, F, Campieri, C, Capuano, M, Terribile, M, Cardone, F, Paglia, S, Castellino, P, Di Landro, D, Fatuzzo, P, Liuzzo, G, Sicurezza, E, Casu, M, Centrone, E, Ciofani, A, Cossu, M, D'Amaro, E, D'Apice, L, Dal Canton, A, Fasoli, G, De Ferrari, G, Cappelli, G, Gusmano, R, De Simone, W, Delgado, G, Di Iorio, B, Di Luca, M, Di Natale, E, Farfaglia, P, Cantù, P, Feriozzi, S, Galeotti, P, Fiorini, F, Frascà, G, Gallucci, M, Buongiorno, E, Gesualdo, L, Giannattasio, M, Detomaso, F, Giannetto, M, Gianni, S, Giliberti, A, Grassi, C, Lupi, G, Imperiali, P, Juliano, P, Li Vecchi, M, Lucenti, T, Maffucci, G, Anelli, A, Manno, M, Schena, F, Mazzaferro, S, Migliorati, M, Morrone, L, Mura, M, Petrarulo, F, Pizzarelli, F, Ricciardi, B, Riccobene, G, Rondanini, V, Russo, D, Sasdelli, M, Mura, C, Scarpino, L, Selvi, S, Tarchini, R, Tedesco, A, Timio, M, Traversari, L, Venditti, G, Viganò, S, and Locatelli, F

Subjects
Proteinuria, Time Factors, Italy, Risk Factors, Nephrology, Chronic Disease, Diabetic Nephropathies/etiology, Hypertension, Humans, Diabetic Nephropathies, Kidney Diseases
Published
2010

29. International recommendations for the assessment of autoantibodies to cellular antigens referred to as anti-nuclear antibodies

Author

Agmon-Levin, N, Damoiseaux, J, Kallenberg, C, Sack, U, Witte, Torsten, Herold, M, Bossuyt, X, Musset, L, Cervera, R, Plaza-Lopez, A, Diaz, C., Souza, MJ, Radice, A, Eriksson, C, Hultgren, O, Viander, M, Kamashta, M, Regenass, Stephan, Andrade, LE, Wiik, Anna, Tincani, A, Rönnelid, J, Bloch, DB, Fritzler, MJ, Chan, EK, Garcia De La Torre, I, Konstantinov, K, Lahita, R, Wilson, M, Vainio, O, Fabien, N, Sinico, RA, Meroni, P, Shoenfeld, Y, Mascart, Françoise, Agmon-Levin, N, Damoiseaux, J, Kallenberg, C, Sack, U, Witte, Torsten, Herold, M, Bossuyt, X, Musset, L, Cervera, R, Plaza-Lopez, A, Diaz, C., Souza, MJ, Radice, A, Eriksson, C, Hultgren, O, Viander, M, Kamashta, M, Regenass, Stephan, Andrade, LE, Wiik, Anna, Tincani, A, Rönnelid, J, Bloch, DB, Fritzler, MJ, Chan, EK, Garcia De La Torre, I, Konstantinov, K, Lahita, R, Wilson, M, Vainio, O, Fabien, N, Sinico, RA, Meroni, P, Shoenfeld, Y, and Mascart, Françoise

Abstract

info:eu-repo/semantics/published

Published
2014

31. PULSE VERSUS DAILY ORAL CYCLOPHOSPHAMIDE FOR INDUCTION OF REMISSION IN ANTINEUTROPHIL CYTOPLASMIC ANTIBODY-ASSOCIATED VASCULITIS: A RANDOMIZED TRIAL

Author

de Groot, K, Harper, L, Jayne, Dr, Flores Suarez LF, Gregorini, G, Gross, Wl, Luqmani, R, Pusey, Cd, Rasmussen, N, Sinico, Ra, Tesar, V, Vanhille, P, Westman, K, Savage, Co, EUVAS Collaborators Abramowicz, D, Wissing, M, Madhoun, P, Blockmans, D, Stolear, J, Ekstrand, A, Chabova, V, Teasr, V, Rychlik, I, Bataille, P, Puechal, X, Leblau, J, Esnault, Vl, Weidner, S, Rupprecht, H, Schneider, M, Specker, C, Schmitt, W, van der Woude, F, Vischedyck, M, Feighery, C, Garibotto, Giacomo, Santostefano, M, Dadoniene, J, Flores Suarez, L, Hagen, Ec, Oudejans, I, Verburgh, C, Ballarin, J, Mirapeix, E, Valles, M, Bruchfeld, A, Pettersson, E, Eriksson, P, Selga, D, Segelmark, M, Lundberg, I, Svenungsson, E, Chizzolini, C, Adu, D, Savage, C, de Souza, R, and Watts, R.

Published
2009

32. Antiprothrombin antibodies: a comparative analysis of home made and commercial methods. A collaborative study of the 'Forum Interdisciplinare per la Ricerca nelle Malattie Autoimmuni (FIRMA)'

Author

Tincani, A, Morozzi, G, Afeltra, A, Alessandri, Cristiano, Allegri, F, Bistoni, O, Bizzaro, N, Caccavo, D, Galeazzi, M, Gerli, R, Giovannelli, L, Longobardo, G, Lotzniker, M, Malacarne, F, Migliorini, P, Parodi, A, Pregnolato, F, Radice, A, Riccieri, Valeria, Ruffelli, M, Sinico, Ra, Tozzoli, R, Villalta, D, Marcolongo, R, and Meroni, P.

Published
2007

33. Antiprothrombin antibodies: a comparative analysis of homemade and commercial methods. A collaborative study by the Forum Interdisciplinare per la Ricerca nelle Malattie Autoimmuni (FIRMA)

Author

Tincani, Angela, Morozzi, G, Afeltra, A, Alessandri, C, Allegri, F, Bistoni, O, Bizzaro, N, Caccavo, D, Galeazzi, M, Gerli, R, Giovannelli, L, Longobardo, G, Lotzniker, M, Malacarne, F, Migliorini, P, Parodi, A, Pregnolato, F, Radice, A, Riccieri, V, Ruffelli, M, Sinico, Ra, Tozzoli, R, Villalta, D, Marcolongo, R, Meroni, P, Forum, and INTERDISCIPLINARE PER LA RICERCA NELLE MALATTIE AUTOIMMUNI FIRMA

Published
2007

34. European Working Party on Systemic Lupus Erythematosus. 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, Roberto, Cauli, A, CHWALINSKA SADOWSKA, H, Contu, L, Cosyns, Jp, Danieli, Mg, Dcruz, D, Depresseux, G, Direskeneli, H, Domènech, I, Espinosa, G, FERNÁNDEZ NEBRO, A, Ferrara, Gb, Font, J, Frutos, Ma, Galeazzi, M, GARCÌA CARRASCO, M, GARCÍA IGLESIAS MF, GARCÍA TOBARUELA, A, George, J, Gil, A, GONZÁLEZ SANTOS, P, Grana, M, Gül, A, Haga, Hj, DE HARO LIGER, M, Houssiau, F, Hughes, Gr, Ingelmo, M, JEDRYKA GÓRAL, A, Khamashta, Ma, Lavilla, P, Levi, Y, LÓPEZ DULPA, 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, Jc, Pintado, V, DE PITA, O, Popovic, R, Pucci, G, Puddu, P, DE RAMÓN, E, RAMOS CASALS, M, RODRÍGUEZ 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, Angela, Tokgöz, G, URBANO MÁRQUEZ, A, Vasconcelos, C, Vázquez, Jj, Veronesi, J, Vianna, J, and Vivancos, J.

Published
2006

35. 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

36. 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

37. European Working Party on Systemic Lupus Erythematosus. Lessons from the 'Euro-Lupus Cohort'

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, Roberto, 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, Galeazzi, M, GARCÍA CARRASCO, M, GARCÍA IGLESIAS MF, GARCÍA TOBARUELA, A, George, J, Gil, A, GONZÁLEZ SANTOS, P, Grana, M, Gül, A, Haga, Hj, DE 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, Morozzi, G, Nicolopoulou, N, Papasteriades, C, Passiu, G, Perelló, I, Petera, P, Petrovic, R, Piette, Jc, 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, Gd, Sherer, Y, Shoenfeld, Y, Simpatico, A, Sinico, Ra, Smolen, J, Tincani, Angela, Tokgöz, G, URBANO MÁRQUEZ, A, Vasconcelos, C, Vázquez, Jj, Veronesi, M, Vianni, J, and Vivancos, J.

Published
2002

38. 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

39. Hepatitis C virus genotype in patients with essential mixed cryoglobulinaemia

Author

G. Arrigo, Sinico Ra, Giuseppe D'Amico, G Portera, M Fasola, J. Zhou, Renoldi P, A. Gibelli, A. Fornasieri, M.L. Ribero, Sinico, R, Ribero, M, Fornasieri, A, Renoldi, P, Zhou, J, Fasola, M, Portera, G, Arrigo, G, Gibelli, A, and D'Amico, G

Subjects
Adult, Male, Genotype, Hepacivirus, Molecular Sequence Data, Enzyme-Linked Immunosorbent Assay, Viremia, Liver disease, Retrospective Studie, medicine, Humans, Retrospective Studies, Aged, Hepaciviru, Base Sequence, biology, business.industry, Case-control study, virus diseases, General Medicine, Hepatitis C, Hepatitis C Antibodies, Middle Aged, medicine.disease, biology.organism_classification, Cryoglobulinemia, digestive system diseases, Case-Control Studies, Immunology, biology.protein, RNA, Viral, Female, Antibody, Case-Control Studie, Hepatitis C Antibodie, business, Human
Abstract

We studied 54 patients with essential mixed cryoglobulinaemia (EMC), (23 males, 31 females) mean age 61 years (range 28-77). Forty-one (76%) had type II cryoglobulinaemia and 13 (24%) type III. Antibodies to HCV were detectable by second-generation ELISA in 49 patients (91%) with confirmed or indeterminate RIBA results. HCV RNA was detected by RT PCR using 5' UTR nested primers; HCV genotypes 1a, 1b, 2 and 3a were identified by genotype-specific core-region nested primers. All patients (49) with antibodies to HCV in their serum were HCV-RNA positive; 27 (55.1%) had HCV subtype 1b and 21 (42.8%) type 2. In one patient the HCV genotype could not be determined. The genotype distribution was not different from that found in patients with chronic hepatitis C without cryoglobulinaemia. However, the presence of HCV subtype 1b correlated significantly with signs of chronic hepatitis and presence of peripheral neuropathy. Severity of disease tended to be worse in patients infected with HCV subtype 1b, but this was mainly due to liver disease. HCV genotypes may influence the clinical expression and, in particular, the severity of liver involvement in patients with EMC. Extent and severity of EMC disease in general may also be affected by the different HCV genotypes. These findings may have therapeutical implications, since the different HCV genotypes respond differently to interferon treatment.

Published
1995

40. NOMENCLATURE OF SYSTEMIC VASCULITIDES - PROPOSAL OF AN INTERNATIONAL CONSENSUS CONFERENCE

Author

JENNETTE, JC, FALK, RJ, ANDRASSY, K, BACON, PA, CHURG, J, GROSS, WL, HAGEN, EC, HOFFMAN, GS, HUNDER, GG, KALLENBERG, CGM, MCCLUSKEY, RT, SINICO, RA, REES, AJ, VANES, LA, WALDHERR, R, WIIK, A, Faculteit Medische Wetenschappen/UMCG, and Translational Immunology Groningen (TRIGR)

Subjects
RHEUMATOLOGY 1990 CRITERIA, DISEASES, CLASSIFICATION, ARTERITIS
Published
1994

41. European consensus statement on the terminology used in the management of lupus glomerulonephritis

Author

Gordon, C, primary, Jayne, D, additional, Pusey, C, additional, Adu, D, additional, Amoura, Z, additional, Aringer, M, additional, Ballerin, J, additional, Cervera, R, additional, Calvo-Alén, J, additional, Chizzolini, C, additional, Dayer, JM, additional, Doria, A, additional, Ferrario, F, additional, Floege, J, additional, Guillevin, L, additional, Haubitz, M, additional, Hiepe, F, additional, Houssiau, F, additional, Lesavre, P, additional, Lightstone, L, additional, Meroni, PL, additional, Meyer, O, additional, Moulin, B, additional, O’Reilly, K, additional, Praga, M, additional, Schulze-Koops, H, additional, Sinico, RA, additional, Smith, KGC, additional, Tincani, A, additional, Vasconcelos, C, additional, and Hughes, G, additional

Published
2009
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42. Pulse versus daily oral cyclophosphamide for induction of remission in antineutrophil cytoplasmic antibody-associated vasculitis: a randomized trial.

Author

de Groot K, Harper L, Jayne DR, Flores Suarez LF, Gregorini G, Gross WL, Luqmani R, Pusey CD, Rasmussen N, Sinico RA, Tesar V, Vanhille P, Westman K, Savage CO, EUVAS (European Vasculitis Study Group), de Groot, Kirsten, Harper, Lorraine, Jayne, David R W, Flores Suarez, Luis Felipe, and Gregorini, Gina

Abstract

Background: Current therapies for antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis are limited by toxicity.Objective: To compare pulse cyclophosphamide with daily oral cyclophosphamide for induction of remission.Design: Randomized, controlled trial. Random assignments were computer-generated; allocation was concealed by faxing centralized treatment assignment to providers at the time of enrollment. Patients, investigators, and assessors of outcomes were not blinded to assignment.Setting: 42 centers in 12 European countries.Patients: 149 patients who had newly diagnosed generalized ANCA-associated vasculitis with renal involvement but not immediately life-threatening disease.Intervention: Pulse cyclophosphamide, 15 mg/kg every 2 to 3 weeks (76 patients), or daily oral cyclophosphamide, 2 mg/kg per day (73 patients), plus prednisolone.Measurement: Time to remission (primary outcome); change in renal function, adverse events, and cumulative dose of cyclophosphamide (secondary outcomes).Results: Groups did not differ in time to remission (hazard ratio, 1.098 [95% CI, 0.78 to 1.55]; P = 0.59) or proportion of patients who achieved remission at 9 months (88.1% vs. 87.7%). Thirteen patients in the pulse group and 6 in the daily oral group achieved remission by 9 months and subsequently had relapse. Absolute cumulative cyclophosphamide dose in the daily oral group was greater than that in the pulse group (15.9 g [interquartile range, 11 to 22.5 g] vs. 8.2 g [interquartile range, 5.95 to 10.55 g]; P < 0.001). The pulse group had a lower rate of leukopenia (hazard ratio, 0.41 [CI, 0.23 to 0.71]).Limitations: The study was not powered to detect a difference in relapse rates between the 2 groups. Duration of follow-up was limited.Conclusion: The pulse cyclophosphamide regimen induced remission of ANCA-associated vasculitis as well as the daily oral regimen at a reduced cumulative cyclophosphamide dose and caused fewer cases of leukopenia.Primary Funding Source: The European Union. [ABSTRACT FROM AUTHOR]

Published
2009
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43. Prevalence and clinical significance of antineutrophil cytoplasmic antibodies in Churg-Strauss syndrome.

Author

Sinico RA, Di Toma L, Maggiore U, Bottero P, Radice A, Tosoni C, Grassolli C, Pavone L, Gregorini G, Monti S, Frassi M, Vecchio F, Corace C, Venegoni E, and Buzio C

Abstract

OBJECTIVE: Churg-Strauss syndrome (CSS) is classified among the so-called antineutrophil cytoplasmic antibody-associated systemic vasculitides (AASVs) because of its clinicopathologic features that overlap with the other AASVs. However, while antineutrophil cytoplasmic antibodies (ANCAs) are consistently found in 75-95% of patients with Wegener's granulomatosis or microscopic polyangiitis, their prevalence in CSS varies widely and their clinical significance remains uncertain. We undertook this study to examine the prevalence and antigen specificity of ANCAs in a large cohort of patients with CSS. Moreover, we evaluated the relationship between ANCA positivity and clinicopathologic features. METHODS: Immunofluorescence and enzyme-linked immunosorbent assay were used to determine the presence or absence of ANCAs in 93 consecutive patients at the time of diagnosis. The main clinical and pathologic data, obtained by retrospective analysis, were correlated with ANCA status. RESULTS: ANCAs were present by immunofluorescence in 35 of 93 patients (37.6%). A perinuclear ANCA (pANCA) pattern was found in 26 of 35 patients (74.3%), with specificity for myeloperoxidase (MPO) in 24 patients, while a cytoplasmic ANCA pattern, with specificity for proteinase 3, was found in 3 of 35 patients (8.6%). Atypical patterns were found in 6 of 30 patients with anti-MPO antibodies (20.0%). ANCA positivity was associated with higher prevalences of renal disease (51.4% versus 12.1%; P < 0.001) and pulmonary hemorrhage (20.0% versus 0.0%; P = 0.001) and, to a lesser extent, with other organ system manifestations (purpura and mononeuritis multiplex), but with lower frequencies of lung disease (34.3% versus 60.3%; P = 0.019) and heart disease (5.7% versus 22.4%; P = 0.042). CONCLUSION: ANCAs are present in approximately 40% of patients with CSS. A pANCA pattern with specificity for MPO is found in most ANCA-positive patients. ANCA positivity is mainly associated with glomerular and alveolar capillaritis. [ABSTRACT FROM AUTHOR]

Published
2005
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47. Anti-C1q Autoantibodies in Lupus Nephritis: Prevalence and Clinical Significance

Author

Bruna Bollini, Girolamo Arrigo, Renato Alberto Sinico, Caterina Corace, Antonella Radice, Masami Ikehata, Maurizio Li Vecchi, Gaia Giammarresi, Sinico, R, Radice, A, Ikehata, M, Giammarresi, G, Corace, C, Arrigo, G, Bollini, B, Li Vecchi, M, SINICO RA, RADICE A, MASAMI I, GIAMMARRESI G, CORACE C, ARRIGO G, BOLLINI B, and LI VECCHI M

Subjects
Biopsy, SLE, Lupus nephritis, Enzyme-Linked Immunosorbent Assay, Systemic lupus erythematosu, Anti-DNA antibodie, Severity of Illness Index, General Biochemistry, Genetics and Molecular Biology, Follow-Up Studie, Nephropathy, Cohort Studies, History and Philosophy of Science, immune system diseases, Autoimmune disease, Prevalence, medicine, Humans, Lupus Erythematosus, Systemic, Connective Tissue Diseases, Glomerulonephriti, skin and connective tissue diseases, Renal flare, Connective Tissue Disease, Autoantibodies, Biochemistry, Genetics and Molecular Biology (all), Systemic lupus erythematosus, business.industry, Lupus nephriti, Complement C1q, General Neuroscience, Autoantibody, Glomerulonephritis, Biomarker, medicine.disease, Lupus Nephritis, Autoantibodie, Antibodies, Anti-Idiotypic, Italy, Antibodies, Antinuclear, Immunology, Anti-C1q antibodie, Cohort Studie, business, Nephritis, Biomarkers, Follow-Up Studies, Human, Anti-SSA/Ro autoantibodies
Abstract

Recently, anti-C1q autoantibodies have been proposed as a useful marker in systemic lupus erythematosus (SLE) since their occurrence correlates with renal involvement and, possibly, with nephritic activity. We aimed to evaluate the prevalence of anti-C1q antibodies in patients with SLE, with and without renal involvement, and to correlate these markers' presence and levels with the activity of the disease and nephropathy. We studied 61 patients with SLE, 40 of whom had biopsy-proven lupus nephritis; 35 patients with other connective tissue diseases; and 54 healthy controls. In addition, 18 lupus nephritis patients were followed up during the disease time course. Anti-C1q antibodies were measured using "homemade" ELISA with high salt concentration (1 M sodium chloride). High anti-C1q antibody titers (> 55 AU) were present in 27 of 61 (44%) SLE patients and in 4% and 0% of normal blood donors and pathologic controls, respectively. Anti-C1q antibodies were found in 60% of patients with lupus nephritis compared with only 14% of SLE patients without nephropathy (P < 0.05). Moreover, patients who were positive for anti-C1q antibodies had a higher European Consensus Lupus Activity Measurement (ECLAM) score (4.35 vs. 2.2); 89% of patients with active lupus nephritis showed high titers of anti-C1q antibodies compared with 0% of patients with inactive nephritis. Anti-C1q and anti-dsDNA antibodies agreed in 79% of cases. Our results confirm that anti-C1q antibodies are present in a significant percentage of SLE patients, and that their presence and levels correlate with disease activity-in particular, during renal flare-ups. © 2005 New York Academy of Sciences

Published
2005

48. Otorhinolaryngological manifestationsin granulomatosis with polyangiitis (Wegener's)

Author

Ulrich Specks, Mario Bussi, Pier Luigi Meroni, Roberto Teggi, Renato Alberto Sinico, Matteo Trimarchi, Trimarchi, Matteo, Sinico, Ra, Teggi, R, Bussi, Mario, Specks, U, Meroni, Pl, Trimarchi, M, Sinico, R, Bussi, M, and Meroni, P

Subjects
Pathology, medicine.medical_specialty, Lymphoma, Septal perforation, Immunology, Paranasal Sinuse, Sarcoidosi, Nose, Granulomatosis with polyangiiti, Diagnosis, Differential, Cocaine, Paranasal Sinuses, otorhinolaryngologic diseases, medicine, Humans, Immunology and Allergy, Fungal sinusiti, Otitis media, Anti-neutrophil cytoplasmic antibody, business.industry, ANCA, Granulomatosis with Polyangiitis, medicine.disease, Subglottic stenosi, Fungal sinusitis, medicine.anatomical_structure, Paranasal sinuses, Wegener's granulomatosi, Sarcoidosis, Differential diagnosis, Vasculitis, Granulomatosis with polyangiitis, business, Crusting, Human
Abstract

Granulomatosis with polyangiitis (Wegener's, GPA) is an uncommon disease of unknown etiology classically involves the ELK triad of the ear, nose, throat (E), lungs (L) and kidneys (K) with necrotizing granulomatous inflammation and vasculitis. Most of the initial symptoms begin in the head and neck region with a wide spectrum of involvement of any site ranging from the nasal septum, paranasal sinuses, oral mucosa, larynx and even the external, middle and internal ear. Diagnosis may be delayed because the onset is heterogeneous and sometimes limited to one organ. The pathologic findings of a characteristic inflammatory reaction pattern, and the serum findings of elevated antineutrophil cytoplasmic antibodies can help to establish the diagnosis. The differentiation from other conditions that mimic GPA such as lymphoma and infections is of critical importance to initiate appropriate treatment. Treatment of the underlying disease is medical with the use of immunosuppressive agents and will not be reviewed here. This review focuses on the otorhinolaryngologic manifestation and complication of GPA as well as their surgical management and specifies the role of the otorhinolaryngologist as an integral member of the multidisciplinary care team for patients with GPA. © 2012 Elsevier B.V.

Published
2013

49. Cocaine-induced midline destructive lesions - an autoimmune disease?

Author

Mario Bussi, Ulrich Specks, Pier Luigi Meroni, Matteo Trimarchi, Renato Alberto Sinico, Trimarchi, M, Bussi, M, Sinico, R, Meroni, P, Specks, U, Trimarchi, Matteo, Bussi, Mario, Sinico, Ra, and Specks, U.

Subjects
Pathology, medicine.medical_specialty, Immunology, Perforation (oil well), Autoimmune Disease, Autoimmune Diseases, Diagnosis, Differential, Cocaine-Related Disorders, Cocaine, Nose Diseases, Nasal septum, Immunology and Allergy, Medicine, Humans, Nose, Anti-neutrophil cytoplasmic antibody, Nose Disease, business.industry, medicine.disease, medicine.anatomical_structure, Cocaine-Related Disorder, Nasal administration, Differential diagnosis, business, Granulomatosis with polyangiitis, Human, Respiratory tract
Abstract

In Europe it is estimated that around 13. million of adults (15-64. years) have used cocaine at least once in their lifetime. The most frequently used route of administration for the drug is intranasal inhalation, or "snorting", and thus the adverse effects of cocaine on the nasal tract are very common. Habitual nasal insufflations of cocaine may cause mucosal lesions, and if cocaine use becomes chronic and compulsive, progressive damage of the mucosa and perichondrium leads to ischemic necrosis of septal cartilage and perforation of the nasal septum. Occasionally, cocaine-induced lesions cause extensive destruction of the osteocartilaginous structures of nose, sinuses and palate that can mimic other diseases such as tumors, infections, and immunological diseases. Thorough diagnostic workup, including endoscopic, radiologic, histopathologic and serologic testing is imperative to arrive at the proper diagnosis and to initiate appropriate local and systemic treatment. Positive antineutrophil cytoplasmic antibody (ANCA) test results may be found in an unexpectedly large proportion of patients with CIMDL. In several instances their lesions are clinically indistinguishable from granulomatosis with polyangiitis (Wegener's) limited to the upper respiratory tract. CIMDL seem to be the result of a necrotizing inflammatory tissue response triggered by cocaine abuse in a subset of patients predisposed to produce ANCA, particularly those reacting with HNE. The presence of these HNE-ANCA seems to promote or define the disease phenotype. CIMDL do not respond well to immunosuppressive therapy. Only the consistent removal of persistent stimuli of autoantibody production (cocaine, bacterial superinfections) can halt the disease process, prevent the progression of the lesions and promise success of surgical repair procedures. © 2012 Elsevier B.V.

Published
2012

50. 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 - Lessons from long-term followup of patients in the Euro-Lupus Nephritis Trial

Author

DİRESKENELİ, RAFİ HANER, Houssiau, FA, Vasconcelos, C, D'Cruz, D, Sebastiani, GD, Garrido, ED, 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, R, Font, J, Depresseux, GV, Cosyns, JP, and Cervera, R

Subjects
PREDNISONE, CYCLOPHOSPHAMIDE, COMBINATION, PULSE METHYLPREDNISOLONE, MYCOPHENOLATE-MOFETIL, DISEASE
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

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