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11 results on '"Bottero, P."'

1. HLA–DRB4 as a genetic risk factor for Churg-Strauss syndrome

Author

Vaglio, A, Martorana, D, Maggiore, U, Grasselli, C, Zanetti, A, Garini, G, Manganelli, P, Bottero, P, Tumiati, B, SINICO, RENATO ALBERTO, Savi, M, Buzio, C, Neri, TM, Secondary, Primary Vasculitis Study Group, PESCI, ALBERTO, Vaglio, A, Martorana, D, Maggiore, U, Grasselli, C, Zanetti, A, Pesci, A, Garini, G, Manganelli, P, Bottero, P, Tumiati, B, Sinico, R, Savi, M, Buzio, C, Neri, T, Secondary, and Primary Vasculitis Study, G

Subjects
Adult, Male, Adolescent, Immunology, Human leukocyte antigen, Churg-Strauss Syndrome, Rheumatology, Risk Factors, medicine, Humans, Immunology and Allergy, Rapidly progressive glomerulonephritis, Pharmacology (medical), Risk factor, Alleles, HLA-DRB4, Aged, Anti-neutrophil cytoplasmic antibody, MED/10 - MALATTIE DELL'APPARATO RESPIRATORIO, Mononeuritis Multiplex, business.industry, HLA-DR Antigens, Systemic vasculitis, HLA, Odds ratio, Middle Aged, medicine.disease, Female, Vasculitis, business, HLA-DRB4 Chains
Abstract

OBJECTIVE: To explore the association between HLA alleles and Churg-Strauss syndrome (CSS), and to investigate the potential influence of HLA alleles on the clinical spectrum of the disease. METHODS: Low-resolution genotyping of HLA-A, HLA-B, and HLA-DR loci and genotyping of TNFA -238A/G and TNFA -308A/G single-nucleotide polymorphisms were performed in 48 consecutive CSS patients and 350 healthy controls. RESULTS: The frequency of the HLA-DRB1*07 allele was higher in the CSS patients than in controls (27.1% versus 13.3%; chi(2) = 12.64, P = 0.0003, corrected P [P(corr)] = 0.0042, odds ratio [OR] 2.42, 95% confidence interval [95% CI] 1.47-3.99). The HLA-DRB4 gene, present in subjects carrying either HLA-DRB1*04, HLA-DRB1*07, or HLA-DRB1*09 alleles, was also far more frequent in patients than in controls (38.5% versus 20.1%; chi(2) = 16.46, P = 0.000058, P(corr) = 0.000232, OR 2.49, 95% CI 1.58-3.09). Conversely, the frequency of the HLA-DRB3 gene was lower in patients than in controls (35.4% versus 50.4%; chi(2) = 7.62, P = 0.0057, P(corr) = 0.0228, OR 0.54, 95% CI 0.35-0.84). CSS has 2 major clinical subsets, antineutrophil cytoplasmic antibody (ANCA)-positive, with features of small-vessel vasculitis, and ANCA-negative, in which organ damage is mainly mediated by tissue eosinophilic infiltration; analysis of HLA-DRB4 in patients categorized by different numbers of vasculitic manifestations (purpura, alveolar hemorrhage, mononeuritis multiplex, rapidly progressive glomerulonephritis, and constitutional symptoms) showed that its frequency strongly correlated with the number of vasculitis symptoms (P for trend = 0.001). CONCLUSION: These findings indicate that HLA-DRB4 is a genetic risk factor for the development of CSS and increases the likelihood of development of vasculitic manifestations of the disease.

Published
2007
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2. Adherence issues related to sublingual immunotherapy as perceived by allergists

Author

Scurati, S., Frati, F., Passalacqua, G., Puccinelli, P., Hilaire, C., Incorvaia, C., D Avino, G., Comi, R., Lo Schiavo, M., Pezzuto, F., Montera, C., Pio, A., Teresa Ielpo, M., Cellini, F., Vicentini, L., Pecorari, R., Aresu, T., Capra, L., Benedictis, E., Bombi, C., Zauli, D., Vanzi, A., Alberto Paltrinieri, C., Bondioli, A., Paletta, I., Ventura, D., Mei, F., Paolini, F., Colangelo, C., Cavallucci, E., Cucinelli, F., Tinari, R., Ermini, G., Beltrami, V., Novembre, E., Begliomini, C., Marchese, E., Solito, E., Ammannati, V., Molino, G., Galli, E., Baldassini, M., Di Michele, L., Calvani, M., Gidaro, M., Venuti, A., Li Bianchi, E., Benassi, F., Pocobelli, D., Zangari, P., Rocco, M. G., Lo Vecchio, A., Pingitore, G., Grimaldi, O., Schiavino, D., Perrone, N., Antonietta Frieri, M., Di Rienzo, V., Tripodi, S., Scarpa, A., Tomsic, M., Bonaguro, R., Enrico Senna, G., Sirena, A., Turatello, F., Crescioli, S., Favero, E., Billeri, L., Chieco Bianchi, F., Gemignani, C., Zanforlin, M., Angiola Crivellaro, M., Hendrick, B., Maltauro, A., Masieri, S., Elisabetta Conte, M., Fama, M., Pozzan, M., Bonadonna, P., Casanova, S., Vallerani, E., Schiappoli, M., Borghesan, F., Giro, G., Casotto, S., Berardino, L., Zanoni, G., Ariano, R., Aquilina, R., Pellegrino, R., Marsico, P., Del Giudice, A., Narzisi, G., Tomaselli, V., Fornaca, G., Favro, M., Loperfido, B., Gallo, C., Buffoni, S., Gani, F., Raviolo, P., Faggionato, S., Truffelli, T., Vivalda, L., Albano, M., Enzo Rossi, R., Lattuada, G., Bona, F., Quaglio, L., Chiesa, A., Trapani, M., Seminara, R., Cucchi, B., Oderda, S., Borio, G., Galeasso, G., Garbaccio, P., Marco, A., Marengo, F., Cadario, G., Manzoni, S., Vinay, C., Curcio, A., Silvestri, A., Peduto, A., Riario-Sforza, G. G., Maria Forgnone, A., Barocelli, P., Tartaglia, N., Feyles, G., Giacone, A., Ricca, V., Guida, G., Nebiolo, F., Bommarito, L., Heffler, E., Vietti, F., Galimberti, M., Savi, E., Pappacoda, A., Bottero, P., Porcu, S., Felice, G., Berra, D., Francesca Spina, M., Pravettoni, V., Calamari, A. M., Varin, E., Iemoli, E., Lietti, D., Ghiglioni, D., Alessandro Fiocchi, Tosi, A., Poppa, M., Caviglia, A., Restuccia, M., Russello, M., Alciato, P., Manzotti, G., Ranghino, E., Luraschi, G., Rapetti, A., Rivolta, F., Allegri, F., Terracciano, L., Agostinis, F., Paolo Piras, P., Ronchi, G., Gaspardini, G., Caria, V., Tolu, F., Fantasia, D., Carta, P., Moraschini, A., Quilleri, R., Santelli, A., Prandini, P., Del Giudice, G., Apollonio, A., Bonazza, L., Teresa Franzini, M., Branchi, S., Zanca, M., Rinaldi, S., Catelli, L., Zanoletti, T., Cosentino, C., Della Torre, F., Cremonte, L., Musazzi, D., Suli, C., Rivolta, L., Ottolenghi, A., Marino, G., Sterza, G., Sambugaro, R., Orlandini, A., Minale, P., Voltolini, S., Bignardi, D., Omodeo, P., Tiri, A., Milani, S., Ronchi, B., Licardi, G., Bruni, P., Scibilia, J., Schroeder, J., Crosti, F., Maltagliati, A., Alesina, M. R., Mosca, M., Leone, G., Napolitano, G., Di Gruttola, G., Scala, G., Mascio, S., Valente, A., Marchetiello, I., Catello, R., Gazulli, A., Del Prete, A., Varricchio, A. M., Carbone, A., Forestieri, A., Stillitano, M., Leonetti, L., Tirroni, E., Castellano, F., Abbagnara, F., Romano, F., Levanti, C., Cilia, M., Longo, R., Ferrari, A., Merenda, R., Di Ponti, A., Guercio, E., Surace, L., Ammendola, G., Tansella, F., Peccarisi, L., Stragapede, L., Minenna, M., Granato, M., Fuiano, N., Pannofino, A., Ciuffreda, S., Giannotta, A., Morero, G., D Oronzio, L., Taddeo, G., Nettis, E., Cinquepalmi, G., Lamanna, C., Mastrandrea, F., Minelli, M., Salamino, F., Muratore, L., Latorre, F., Quarta, C., Ventura, M., D Ippolito, G., Giannoccaro, F., Dambra, P., Pinto, L., Triggiani, M., Munno, G., Manfredi, G., Lonero, G., Damiano, V., Errico, G., Di Leo, E., Manzari, F., Spagna, V., Arsieni, A., Matarrese, A., Mazzarella, G., Scarcia, G., Scarano, R., Ferrannini, A., Pastore, A., Maionchi, P., Filannino, L., Tria, M., Giuliano, G., Damiani, E., Scichilone, N., Marchese, M., Lucania, A., Marino, M., Strazzeri, L., Tumminello, S., Vitale, G. I., Gulotta, S., Gragotto, G., Zambito, M., Greco, D., Valenti, G., Licitra, G., Cannata, E., Filpi, R., Contraffatto, M., Sichili, S., Randazzo, S., Scarantino, G., Lo Porto, B., Pavone, F., Di Bartolo, C., Paternò, A., Rapisarda, F., Laudani, E., Leonardi, S., Padua, V., Cabibbo, G., Marino Guzzardi, G., Deluca, F., Agozzino, C., Pettinato, R., Ghini, M., Scurati S., Frati F., Passalacqua G., Puccinelli P., Hilaire C., Incorvaia C., D'Avino G., Comi R., Lo Schiavo M., Pezzuto F., Montera C., Pio A., Teresa Ielpo M., Cellini F., Vicentini L., Pecorari R., Aresu T., Capra L., De Benedictis E., Bombi C., Zauli D., Vanzi A., Alberto Paltrinieri C., Bondioli A., Paletta I., Ventura D., Mei F., Paolini F., Colangelo C., Cavallucci E., Cucinelli F., Tinari R., Ermini G., Beltrami V., Novembre E., Begliomini C., Marchese E., Solito E., Ammannati V., Molino G., Galli E., Baldassini M., Di Michele L., Calvani M., Gidaro M., Venuti A., Li Bianchi E., Benassi F., Pocobelli D., Zangari P., De Rocco M.G., Lo Vecchio A., Pingitore G., Grimaldi O., Schiavino D., Perrone N., Antonietta Frieri M., Di Rienzo V., Tripodi S., Scarpa A., Tomsic M., Bonaguro R., Enrico Senna G., Sirena A., Turatello F., Crescioli S., Favero E., Billeri L., Chieco Bianchi F., Gemignani C., Zanforlin M., Angiola Crivellaro M., Hendrick B., Maltauro A., Masieri S., Elisabetta Conte M., Fama M., Pozzan M., Bonadonna P., Casanova S., Vallerani E., Schiappoli M., Borghesan F., Giro G., Casotto S., Berardino L., Zanoni G., Ariano R., Aquilina R., Pellegrino R., Marsico P., Del Giudice A., Narzisi G., Tomaselli V., Fornaca G., Favro M., Loperfido B., Gallo C., Buffoni S., Gani F., Raviolo P., Faggionato S., Truffelli T., Vivalda L., Albano M., Enzo Rossi R., Lattuada G., Bona F., Quaglio L., Chiesa A., Trapani M., Seminara R., Cucchi B., Oderda S., Borio G., Galeasso G., Garbaccio P., De Marco A., Marengo F., Cadario G., Manzoni S., Vinay C., Curcio A., Silvestri A., Peduto A., Riario-Sforza G.G., Maria Forgnone A., Barocelli P., Tartaglia N., Feyles G., Giacone A., Ricca V., Guida G., Nebiolo F., Bommarito L., Heffler E., Vietti F., Galimberti M., Savi E., Pappacoda A., Bottero P., Porcu S., Felice G., Berra D., Francesca Spina M., Pravettoni V., Calamari A.M., Varin E., Iemoli E., Lietti D., Ghiglioni D., Fiocchi A., Tosi A., Poppa M., Caviglia A., Restuccia M., Russello M., Alciato P., Manzotti G., Ranghino E., Luraschi G., Rapetti A., Rivolta F., Allegri F., Terracciano L., Agostinis F., Paolo Piras P., Ronchi G., Gaspardini G., Caria V., Tolu F., Fantasia D., Carta P., Moraschini A., Quilleri R., Santelli A., Prandini P., Del Giudice G., Apollonio A., Bonazza L., Teresa Franzini M., Branchi S., Zanca M., Rinaldi S., Catelli L., Zanoletti T., Cosentino C., Della Torre F., Cremonte L., Musazzi D., Suli C., Rivolta L., Ottolenghi A., Marino G., Sterza G., Sambugaro R., Orlandini A., Minale P., Voltolini S., Bignardi D., Omodeo P., Tiri A., Milani S., Ronchi B., Licardi G., Bruni P., Scibilia J., Schroeder J., Crosti F., Maltagliati A., Alesina M.R., Mosca M., Leone G., Napolitano G., Di Gruttola G., Scala G., Mascio S., Valente A., Marchetiello I., Catello R., Gazulli A., Del Prete A., Varricchio A.M., Carbone A., Forestieri A., Stillitano M., Leonetti L., Tirroni E., Castellano F., Abbagnara F., Romano F., Levanti C., Cilia M., Longo R., Ferrari A., Merenda R., Di Ponti A., Guercio E., Surace L., Ammendola G., Tansella F., Peccarisi L., Stragapede L., Minenna M., Granato M., Fuiano N., Pannofino A., Ciuffreda S., Giannotta A., Morero G., D'Oronzio L., Taddeo G., Nettis E., Cinquepalmi G., Lamanna C., Mastrandrea F., Minelli M., Salamino F., Muratore L., Latorre F., Quarta C., Ventura M., D'Ippolito G., Giannoccaro F., Dambra P., Pinto L., Triggiani M., Munno G., Manfredi G., Lonero G., Damiano V., Errico G., Di Leo E., Manzari F., Spagna V., Arsieni A., Matarrese A., Mazzarella G., Scarcia G., Scarano R., Ferrannini A., Pastore A., Maionchi P., Filannino L., Tria M., Giuliano G., Damiani E., Scichilone N., Marchese M., Lucania A., Marino M., Strazzeri L., Tumminello S., Vitale G.I., Gulotta S., Gragotto G., Zambito M., Greco D., Valenti G., Licitra G., Cannata E., Filpi R., Contraffatto M., Sichili S., Randazzo S., Scarantino G., Lo Porto B., Pavone F., Di Bartolo C., Paterno A., Rapisarda F., Laudani E., Leonardi S., Padua V., Cabibbo G., Marino Guzzardi G., Deluca F., Agozzino C., Pettinato R., Ghini M., Scurati S, Frati F, Passalacqua G, Puccinelli P, Hilaire C, Incorvaia I, D'Avino G, Comi R, Lo Schiavio M, Pezzuto F, Montera C, Pio A, Ielpo MT, Cellini F, Vicentini L, Pecorari R, Aresu T, Capra L, De Benedictis E, Bombi C, Zauli D, and et al

Subjects
medicine.medical_specialty, Pathology, genetic structures, efficacy, Alternative medicine, Medicine (miscellaneous), Adherence, Cost, Efficacy, Side effects, Sublingual immunotherapy, Settore MED/10 - Malattie Dell'Apparato Respiratorio, sublingual immunotherapy, ALLERGEN, cost, medicine, Subcutaneous immunotherapy, Sublingual immunotherapy, adherence, Clinical efficacy, Intensive care medicine, Pharmacology, Toxicology and Pharmaceutics (miscellaneous), sublingual immunoterapy, Original Research, Asthma, AEROALLERGENS, side effects, business.industry, Health Policy, medicine.disease, Slit, eye diseases, Clinical trial, Patient Preference and Adherence, immunotherapy, sense organs, Allergists, ADHERENCE TO TREATMENT, business, Social Sciences (miscellaneous)
Abstract

Silvia Scurati1, Franco Frati1, Gianni Passalacqua2, Paola Puccinelli1, Cecile Hilaire1, Cristoforo Incorvaia3, Italian Study Group on SLIT Compliance 1Scientific and Medical Department, Stallergenes, Milan, Italy; 2Allergy and Respiratory Diseases, Department of Internal Medicine, Genoa; 3Allergy/Pulmonary Rehabilitation, ICP Hospital, Milan, ItalyObjectives: Sublingual immunotherapy (SLIT) is a viable alternative to subcutaneous immunotherapy to treat allergic rhinitis and asthma, and is widely used in clinical practice in many European countries. The clinical efficacy of SLIT has been established in a number of clinical trials and meta-analyses. However, because SLIT is self-administered by patients without medical supervision, the degree of patient adherence with treatment is still a concern. The objective of this study was to evaluate the perception by allergists of issues related to SLIT adherence.Methods: We performed a questionnaire-based survey of 296 Italian allergists, based on the adherence issues known from previous studies. The perception of importance of each item was assessed by a VAS scale ranging from 0 to 10.Results: Patient perception of clinical efficacy was considered the most important factor (ranked 1 by 54% of allergists), followed by the possibility of reimbursement (ranked 1 by 34%), and by the absence of side effects (ranked 1 by 21%). Patient education, regular follow-up, and ease of use of SLIT were ranked first by less than 20% of allergists.Conclusion: These findings indicate that clinical efficacy, cost, and side effects are perceived as the major issues influencing patient adherence to SLIT, and that further improvement of adherence is likely to be achieved by improving the patient information provided by prescribers.Keywords: adherence, sublingual immunotherapy, efficacy, cost, side effects

Published
2010

3. 162. Cluster analysis to explore clinical subclassification of eosinophilic granulomatosis with polyangiitis (churg–strauss)

Author

C. Kroegel, Andrea Gioffredi, Julian Grosskreutz, Federica Maritati, Gina Gregorini, Alfred Mahr, Matthieu Resche-Rigon, David Saadoun, Wojciech Szczeklik, Bernhard Hellmich, Paolo Fraticelli, Barbara Sokołowska, Frank Moosig, Carla Maldini, Phillip Bremer, Thomas Neumann, Augusto Vaglio, Giuseppe Guida, Renato Alberto Sinico, Paolo Bottero, Alberto Pesci, Jochen Zwerina, Giacomo Emmi, Emma Rubenstein, Rubenstein, E, Maldini, C, Vaglio, A, Bottero, P, Bremer, P, Emmi, G, Fraticelli, P, Gioffredi, A, Gregorini, G, Grosskreutz, J, Guida, G, Hellmich, B, Kroegel, C, Maritati, F, Pesci, A, Resche-Rigon, M, Saadoun, D, Sinico, R, Sokolowska, B, Szczeklik, W, Zwerina, J, Moosig, F, Neumann, T, and Mahr, A

Subjects
medicine.medical_specialty, EGPA, cluster analysis, Rheumatology, business.industry, Eosinophilic, medicine, Pharmacology (medical), Disease cluster, Granulomatosis with polyangiitis, medicine.disease, business, Dermatology, Churg strauss
Published
2019

4. Churg–Strauss angiitis

Author

Paolo Bottero, Renato Alberto Sinico, Sinico, R, and Bottero, P

Subjects
Male, medicine.medical_specialty, Churg-Strauss Syndrome, urologic and male genital diseases, Gastroenterology, Antibodies, Antineutrophil Cytoplasmic, Immunosuppressive Agent, Glucocorticoid, Rheumatology, Maintenance therapy, immune system diseases, Internal medicine, Eosinophilia, medicine, Humans, cardiovascular diseases, skin and connective tissue diseases, Cyclophosphamide, Glucocorticoids, Pathological, Asthma, business.industry, Middle Aged, medicine.disease, allergic granulomatous angiiti, microscopic polyangiiti, respiratory tract diseases, Purpura, vasculiti, Wegener's granulomatosi, anti-neutrophil cytoplasmic antibodies (ANCAs), Immunology, Drug Therapy, Combination, Female, medicine.symptom, Microscopic polyangiitis, Vasculitis, business, Immunosuppressive Agents, Human
Abstract

Churg-Strauss angiitis or syndrome (CSA) is defined as an eosinophil-rich and granulomatous inflammation involving the respiratory tract, and necrotising vasculitis affecting small- to medium-sized vessels, and is associated with asthma and eosinophilia. It is usually classified among the so-called anti-neutrophil antibody (ANCA)-associated systemic vasculitides (AASVs) because of its clinical and pathological features that overlap with those of the other AASVs. However, two recent studies on large cohorts of patients have found that ANCAs, usually P-ANCAs/MPO-ANCAs, were present in only 38% of patients. Moreover, the ANCA status was shown to segregate with clinical phenotype. ANCA-positive patients were significantly more likely to have disease manifestations associated with small-vessel vasculitis, including necrotising glomerulonephritis, mononeuritis and purpura, whereas ANCA-negative cases were significantly more likely to have cardiac and lung involvement. Vasculitis was documented less frequently in histological specimens from ANCA-negative patients in comparison with ANCA-positive ones. These findings have led to postulate the predominance of distinct pathogenetic mechanisms in the two subsets of patients: an ANCA-mediated process in ANCA-positive patients and tissue infiltration by eosinophils with subsequent release of toxic product in ANCA-negative cases. 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 CSA. The addition of cyclophosphamide is indicated in treatment of patients with poor-prognosis factors or in patients without poor-prognosis factors but those that are prone to relapses. The length of the maintenance therapy remains to be established. However, the vast majority of patients require long-term corticosteroids treatment to control asthma. © 2009 Elsevier Ltd. All rights reserved

Published
2009
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5. Eosinophilic granulomatosis with polyangiitis (churg-straus syndrome)

Author

Paolo Bottero, Renato Alberto Sinico, Dammacco, F, Ribatti, D, Vacca, A, Sinico, R, and Bottero, P

Subjects
medicine.medical_specialty, Cyclophosphamide, business.industry, medicine.disease, Dermatology, respiratory tract diseases, Maintenance therapy, immune system diseases, Necrotizing Vasculitis, Eosinophilic, medicine, Eosinophilia, Churg-Strauss syndrome, Eosinophilic granulomatosis with polyangiitis (EGPA), Asthma, Eosinophilia, Anti-neutrophil cytoplasmic antibody (ANCA), Vasculitis, cardiovascular diseases, medicine.symptom, skin and connective tissue diseases, Vasculitis, business, Granulomatosis with polyangiitis, medicine.drug, Asthma
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 anti-neutrophil 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

6. Renal Involvement in Churg-Strauss Syndrome

Author

Cinzia Tosoni, Carlo Buzio, Stefano Monti, Gaia Giammarresi, Umberto Maggiore, Alberto Pesci, Renato Alberto Sinico, Giovanni Garini, Gina Gregorini, Paolo Bottero, Lucafrancesco Di Toma, Filomena Vecchio, Bruno Tumiati, Ettore Sabadini, Genesio Balestrieri, Sinico, R, Di Toma, L, Maggiore, U, Tosoni, C, Bottero, P, Sabadini, E, Giammarresi, G, Tumiati, B, Gregorini, G, Pesci, A, Monti, S, Balestrieri, G, Garini, G, Vecchio, F, and Buzio, C

Subjects
Adult, Male, Nephrology, medicine.medical_specialty, Pathology, Adolescent, Interstitial nephritis, Churg Strauss syndrome, systemic vasculitis, Churg-Strauss Syndrome, urologic and male genital diseases, Gastroenterology, Antibodies, Antineutrophil Cytoplasmic, Nephropathy, Internal medicine, Prevalence, medicine, Humans, Obstructive uropathy, Aged, Retrospective Studies, Anti-neutrophil cytoplasmic antibody, Aged, 80 and over, MED/10 - MALATTIE DELL'APPARATO RESPIRATORIO, medicine.diagnostic_test, business.industry, Middle Aged, Prognosis, medicine.disease, Female, Kidney Diseases, Renal biopsy, business, Systemic vasculitis, Kidney disease
Abstract

BACKGROUND: Churg-Strauss syndrome (CSS) is a rare disorder characterized by asthma, eosinophilia, and systemic vasculitis. Renal involvement is not regarded as a prominent feature, and its prevalence and severity vary widely in published reports that usually refer to small series of selected patients. METHODS: We examined the prevalence, clinicopathologic features, and prognosis of renal disease in 116 patients with CSS. RESULTS: There were 48 men and 68 women with a mean age of 51.9 years (range, 18 to 86 years). Signs of renal abnormalities were present in 31 patients (26.7%). Rapidly progressive renal insufficiency was documented in 16 patients (13.8%); urinary abnormalities, 14 patients (12.1%); and chronic renal impairment, 1 patient. There were 3 additional cases of obstructive uropathy. Sixteen patients underwent renal biopsy, which showed necrotizing crescentic glomerulonephritis in 11 patients. Other diagnoses were eosinophilic interstitial nephritis, mesangial glomerulonephritis, and focal sclerosis. Antineutrophil cytoplasmic antibody (ANCA) was positive in 21 of 28 patients (75.0%) with nephropathy versus 19 of 74 patients without (25.7%; P < 0.001). In particular, all patients with necrotizing crescentic glomerulonephritis were ANCA positive. After a median follow-up of 4.5 years, 10 patients died (5 patients with nephropathy) and 7 patients developed mild chronic renal insufficiency. Five-year mortality rates were 11.7% (95% confidence interval, 3.9 to 33.3) in patients with nephropathy and 2.7% (95% confidence interval, 0.7 to 10.7) in those without (P = 0.10). CONCLUSION: Renal abnormalities are present in about one quarter of patients with CSS. The prevailing picture is ANCA-associated necrotizing crescentic glomerulonephritis; however, other forms of nephropathy also may occur. Outcome and long-term follow-up usually are good.

Published
2006
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7. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management

Author

David Jayne, Romain Lazor, Jean-François Cordier, Ulrich Specks, Marc Humbert, Klaus Dalhoff, Wolfgang L. Gross, Vincent Cottin, Peter A. Merkel, Matthieu Groh, Augusto Vaglio, Alfred Mahr, Paolo Bottero, Renato Alberto Sinico, Bertrand Dunogué, J. Charles Jennette, Loïc Guillevin, Christian Pagnoux, Michael E. Wechsler, Julia U Holle, Luc Mouthon, Chiara Baldini, Elisabeth H. Bel, Groh, M, Pagnoux, C, Baldini, C, Bel, E, Bottero, P, Cottin, V, Dalhoff, K, Dunogué, B, Gross, W, Holle, J, Humbert, M, Jayne, D, Jennette, J, Lazor, R, Mahr, A, Merkel, P, Mouthon, L, Sinico, R, Specks, U, Vaglio, A, Wechsler, M, Cordier, J, Guillevin, L, AII - Amsterdam institute for Infection and Immunity, and Pulmonology

Subjects
United State, medicine.medical_specialty, Consensus, Advisory Committees, Churg-Strauss syndrome, MEDLINE, Consensu, Recommendations, Immunosuppressive Agent, Glucocorticoid, Eosinophilic, Eosinophilia, Internal Medicine, Medicine, Humans, Churg–Strauss syndrome, Disease management (health), Glucocorticoids, Churg strauss, Pulmonologists, Advisory Committee, business.industry, Task force, Eosinophilic granulomatosis with polyangiitis, Medicine (all), Granulomatosis with Polyangiitis, Disease Management, Evidence-based medicine, Recommendation, medicine.disease, United States, Asthma, Surgery, Europe, Anti-neutrophil cytoplasm antibody-associated vasculitis, Anti-neutrophil cytoplasm antibody-associated vasculiti, Family medicine, Eosinophilic granulomatosis with polyangiiti, Granulomatosis with Polyangiiti, business, Granulomatosis with polyangiitis, Rituximab, Churg-Strauss Syndrome, Immunosuppressive Agents, Human
Abstract

Objective To develop disease-specific recommendations for the diagnosis and management of eosinophilic granulomatosis with polyangiitis (Churg–Strauss syndrome) (EGPA). Methods The EGPA Consensus Task Force experts comprised 8 pulmonologists, 6 internists, 4 rheumatologists, 3 nephrologists, 1 pathologist and 1 allergist from 5 European countries and the USA. Using a modified Delphi process, a list of 40 questions was elaborated by 2 members and sent to all participants prior to the meeting. Concurrently, an extensive literature search was undertaken with publications assigned with a level of evidence according to accepted criteria. Drafts of the recommendations were circulated for review to all members until final consensus was reached. Results Twenty-two recommendations concerning the diagnosis, initial evaluation, treatment and monitoring of EGPA patients were established. The relevant published information on EGPA, antineutrophil-cytoplasm antibody-associated vasculitides, hypereosinophilic syndromes and eosinophilic asthma supporting these recommendations was also reviewed. Discussion These recommendations aim to give physicians tools for effective and individual management of EGPA patients, and to provide guidance for further targeted research.

Published
2015

8. Can HLA-DRB4 Help to Identify Asthmatic Patients at Risk of Churg-Strauss Syndrome?

Author

R. A. Sinico, F. Vecchio, F. Ierna, I. Cuppari, P. Bottero, M. Bonini, F. Motta, Bottero, P, Motta, F, Bonini, M, Vecchio, F, Ierna, F, Cuppari, I, and Sinico, R

Subjects
Pediatrics, medicine.medical_specialty, Article Subject, business.industry, Churg-Strauss syndrome, Asthma, HLA, ANCA, medicine.disease, Gastroenterology, Pulmonary function testing, Prednisone, Internal medicine, Cohort, otorhinolaryngologic diseases, Medicine, Eosinophilia, Eosinophilic vasculitis, medicine.symptom, business, Sinusitis, HLA-DRB4, Asthma, medicine.drug, Research Article
Abstract

HLA-DRB4 gene is associated with Churg-Strauss syndrome (CSS), a systemic eosinophilic vasculitis with a prodromal phase characterized by severe asthma, eosinophilia, nasal polyposis, and sinusitis. Aim of this study was to evaluate if the presence of HLA-DRB4 in asthmatic patients is associated with a clinical picture resembling that of the prodromal phase of CSS. HLA-DRB1 was determined in a cohort of 159 asthmatic patients and its frequency was compared with that of 1808 blood donors. HLA-DRB4 presence/absence was correlated with clinical features, including sinusitis, nasal polyposis, eosinophils, antiasthmatic drugs, asthma severity, and pulmonary function tests. HLA-DRB4 gene was associated with severe persistent asthma before treatment (P<0.02), near fatal or severe hypoxemic asthma (P<0.01), sinusitis (P<0.01), nasal polyposis (P<0.01), number of patients with eosinophils >1000/μl: (P<0.05), need of beclomethasone >1000–2000 μg/daily (P<0.001), use of a third controller (P<0.05), and oral prednisone (P<0.02). HLA-DRB4 gene is associated in asthmatic patients with a clinical picture characterized by asthma severity, sinusitis, nasal polyposis, and eosinophilia closely resembling that of the prodromal phase of CSS and might be useful to suspect corticosteroids-masked cases of CSS.

Published
2014

9. L5. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss)

Author

Romain Lazor, Marc Humbert, Jean-François Cordier, Pasupathy Sivasothy, Loïc Guillevin, Klaus Dalhoff, Vincent Cottin, Renato Alberto Sinico, Paolo Bottero, Michael E. Wechsler, Elisabeth H. Bel, AII - Amsterdam institute for Infection and Immunity, Pulmonology, Cordier, J, Cottin, V, Guillevin, L, Bel, E, Bottero, P, Dalhoff, K, Humbert, M, Lazor, R, Sinico, R, Sivasothy, P, and Wechsler, M

Subjects
Pathology, medicine.medical_specialty, Prognosi, Adrenal cortex hormones, Churg-strauss syndrome, Eosinophil, Churg-Strauss Syndrome, Adrenal Cortex Hormone, Diagnosis, Differential, Immunosuppressive Agent, Adrenal Cortex Hormones, Terminology as Topic, Hypereosinophilic Syndrome, Eosinophilic, Humans, Medicine, Churg strauss, business.industry, Hypereosinophilic syndrome, Medicine (all), General Medicine, Prognosis, medicine.disease, Eosinophils, Differential diagnosis, business, Granulomatosis with polyangiitis, Immunosuppressive Agents, Human
Published
2013

10. The common allergens in the Churg-Strauss syndrome

Author

G. M. Patruno, A. Grittini, M. Bonini, B. Colombo, P. Bottero, R. A. Sinico, F. Vecchio, Bottero, P, Bonini, M, Vecchio, F, Grittini, A, Patruno, G, Colombo, B, and Sinico, R

Subjects
Adult, Male, medicine.medical_specialty, Allergy, Vasculiti, Exacerbation, Immunology, Eosinophil, Churg-Strauss Syndrome, medicine.disease_cause, Immunoglobulin E, Gastroenterology, Allergen, Retrospective Studie, Immunopathology, Internal medicine, otorhinolaryngologic diseases, medicine, Respiratory Hypersensitivity, Immunology and Allergy, Eosinophilia, Humans, Asthma, Aged, Retrospective Studies, biology, business.industry, Respiratory disease, Allergens, Middle Aged, medicine.disease, respiratory tract diseases, Eosinophils, surgical procedures, operative, biology.protein, Female, IgE, medicine.symptom, business, Human
Abstract

Background: The asthmatic-prodromal phase of Churg–Strauss syndrome (CSS) is usually considered allergic, but data about the involved allergens are scarce. The aim of our work was to examine the prevalence of allergy in a group of CSS patients and in two control groups of persistent asthmatic subjects selected for eosinophilia >10% [first control group patients (CGP1)] and eosinophils

Published
2007

11. Prevalence and clinical significance of antineutrophil cytoplasmic antibodies in Churg-Strauss syndrome

Author

Umberto Maggiore, Caterina Corace, Stefano Monti, Gina Gregorini, Renato Alberto Sinico, Laura Pavone, Carlo Buzio, Antonella Radice, Filomena Vecchio, Lucafrancesco Di Toma, Emanuela Venegoni, Chiara Grasselli, Cinzia Tosoni, Paolo Bottero, Micol Frassi, Sinico, R, Di Toma, L, Maggiore, U, Bottero, P, Radice, A, Tosoni, C, Grasselli, C, Pavone, L, Gregorini, G, Monti, S, Frassi, M, Vecchio, F, Corace, C, Venegoni, E, and Buzio, C

Subjects
Lung Diseases, Pathology, Kidney Disease, Fluorescent Antibody Technique, Churg-Strauss Syndrome, urologic and male genital diseases, Lung Disease, Cohort Studies, Immunosuppressive Agent, Glucocorticoid, immune system diseases, Proteinase 3, Antibody Specificity, Retrospective Studie, Immunology and Allergy, Pharmacology (medical), skin and connective tissue diseases, Cell Nucleu, Aged, 80 and over, biology, medicine.diagnostic_test, Panca, Mononeuritis Multiplex, Middle Aged, Kidney Diseases, Drug Therapy, Combination, Microscopic polyangiitis, Immunosuppressive Agents, Human, Adult, medicine.medical_specialty, Adolescent, Immunology, Enzyme-Linked Immunosorbent Assay, Hemorrhage, Immunofluorescence, Antibodies, Antineutrophil Cytoplasmic, Rheumatology, medicine, Humans, Clinical significance, cardiovascular diseases, Glucocorticoids, Retrospective Studies, Anti-neutrophil cytoplasmic antibody, Aged, Peroxidase, Cell Nucleus, business.industry, biology.organism_classification, medicine.disease, Capillaritis, respiratory tract diseases, Cohort Studie, business
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 ∼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. © 2005, American College of Rheumatology

Published
2005

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