{"data":{"id":"10.6084/m9.figshare.1568515","type":"dois","attributes":{"doi":"10.6084/m9.figshare.1568515","prefix":"10.6084","suffix":"m9.figshare.1568515","identifiers":[],"alternateIdentifiers":[],"creators":[{"name":"C Campochiaro","affiliation":[],"nameIdentifiers":[]},{"name":"GA Ramirez","affiliation":[],"nameIdentifiers":[]},{"name":"EP Bozzolo","affiliation":[],"nameIdentifiers":[]},{"name":"M Lanzillotta","affiliation":[],"nameIdentifiers":[]},{"name":"A Berti","affiliation":[],"nameIdentifiers":[]},{"name":"E Baldissera","affiliation":[],"nameIdentifiers":[]},{"name":"L Dagna","affiliation":[],"nameIdentifiers":[]},{"name":"L Praderio","affiliation":[],"nameIdentifiers":[]},{"name":"R Scotti","affiliation":[],"nameIdentifiers":[]},{"name":"M Tresoldi","affiliation":[],"nameIdentifiers":[]},{"name":"L Roveri","affiliation":[],"nameIdentifiers":[]},{"name":"A Mariani","affiliation":[],"nameIdentifiers":[]},{"name":"G Balzano","affiliation":[],"nameIdentifiers":[]},{"name":"R Castoldi","affiliation":[],"nameIdentifiers":[]},{"name":"C Doglioni","affiliation":[],"nameIdentifiers":[]},{"name":"MG Sabbadini","affiliation":[],"nameIdentifiers":[]},{"name":"E Della-Torre","affiliation":[],"nameIdentifiers":[]}],"titles":[{"title":"IgG4-related disease in Italy: clinical features and outcomes of a large cohort of patients"}],"publisher":"Taylor \u0026 Francis","container":{},"publicationYear":2016,"subjects":[{"subject":"Medicine"},{"subject":"Cell Biology"},{"subject":"Biotechnology"},{"subject":"Immunology"},{"subject":"FOS: Clinical medicine","schemeUri":"http://www.oecd.org/science/inno/38235147.pdf","subjectScheme":"Fields of Science and Technology (FOS)"},{"subject":"FOS: Clinical medicine","subjectScheme":"Fields of Science and Technology (FOS)"},{"subject":"69999 Biological Sciences not elsewhere classified","subjectScheme":"FOR"},{"subject":"FOS: Biological sciences","schemeUri":"http://www.oecd.org/science/inno/38235147.pdf","subjectScheme":"Fields of Science and Technology (FOS)"},{"subject":"FOS: Biological sciences","subjectScheme":"Fields of Science and Technology (FOS)"}],"contributors":[],"dates":[{"date":"2015-10-08","dateType":"Created"},{"date":"2016-02-29","dateType":"Updated"},{"date":"2016","dateType":"Issued"}],"language":null,"types":{"ris":"DATA","bibtex":"misc","citeproc":"dataset","schemaOrg":"Dataset","resourceType":"Dataset","resourceTypeGeneral":"Dataset"},"relatedIdentifiers":[{"relationType":"IsSupplementTo","relatedIdentifier":"10.3109/03009742.2015.1055796","relatedIdentifierType":"DOI"}],"relatedItems":[],"sizes":["431813 Bytes"],"formats":[],"version":null,"rightsList":[{"rights":"Creative Commons Attribution 4.0 International","rightsUri":"https://creativecommons.org/licenses/by/4.0/legalcode","schemeUri":"https://spdx.org/licenses/","rightsIdentifier":"cc-by-4.0","rightsIdentifierScheme":"SPDX"}],"descriptions":[{"description":"\u003cb\u003eObjectives\u003c/b\u003e: To describe the clinical features, treatment response, and follow-up of a large cohort of Italian patients with immunoglobulin (Ig)G4-related disease (IgG4-RD) referred to a single tertiary care centre. \u003cb\u003eMethod\u003c/b\u003e: Clinical, laboratory, histological, and imaging features were retrospectively reviewed. IgG4-RD was classified as ‘definite’ or ‘possible’ according to international consensus guidelines and comprehensive diagnostic criteria for IgG4-RD. Disease activity was assessed by means of the IgG4-RD Responder Index (IgG4-RD RI). \u003cb\u003eResults\u003c/b\u003e: Forty-one patients (15 females, 26 males) were included in this study: 26 with ‘definite’ IgG4-RD and 15 with ‘possible’ IgG4-RD. The median age at diagnosis was 62 years. The median follow-up was 36 months (IQR 24–51). A history of atopy was present in 30% of patients. The pancreas, retroperitoneum, and major salivary glands were the most frequently involved organs. Serum IgG4 levels were elevated in 68% of cases. Thirty-six patients were initially treated with glucocorticoids (GCs) to induce remission. IgG4-RD RI decreased from a median of 7.8 at baseline to 2.9 after 1 month of therapy. Relapse occurred in 19/41 patients (46%) and required additional immunosuppressive drugs to maintain long-term remission. Multiple flares occurred in a minority of patients. A single case of orbital pseudotumour did not respond to medical therapy and underwent surgical debulking. \u003cb\u003eConclusions\u003c/b\u003e: IgG4-RD is an elusive inflammatory disease to be considered in the differential diagnosis of isolated or multiple tumefactive lesions. Long-term disease control can be achieved with corticosteroids and immunosuppressive drugs in the majority of cases.","descriptionType":"Abstract"}],"geoLocations":[],"fundingReferences":[],"xml":"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