{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,9]],"date-time":"2026-10-09T13:32:31Z","timestamp":1791552751992,"version":"4.3.4"},"reference-count":42,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2015,8,26]],"date-time":"2015-08-26T00:00:00Z","timestamp":1440547200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"NIH"},{"DOI":"10.13039\/100000060","name":"National Institute of Allergy and Infectious Diseases","doi-asserted-by":"crossref","id":[{"id":"10.13039\/100000060","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Autoimmune Disease Center of Excellence Program award for IgG4-related disease to Massachusetts General Hospital"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Arthritis &amp; Rheumatology"],"published-print":{"date-parts":[[2015,9]]},"abstract":"<jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>IgG4\u2010related disease (IgG4\u2010RD) is an immune\u2010mediated fibroinflammatory condition that can affect nearly any organ. Prior studies have focused on individual cases of IgG4\u2010RD or small case series. This study was undertaken to report detailed clinical and laboratory findings in a larger group of patients with IgG4\u2010RD whose diagnosis was established by strict clinicopathologic correlation.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>The baseline features of 125 patients with biopsy\u2010proven IgG4\u2010RD were reviewed. The diagnosis was confirmed by pathologists\u2019 review, based on consensus diagnostic criteria and correlation with clinicopathologic features. Disease activity and damage were assessed using the IgG4\u2010RD Responder Index (RI). Flow cytometry was used to assess levels of circulating plasmablasts.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Of the 125 patients, 107 had active disease and 86 were not receiving treatment for IgG4\u2010RD. Only 51% of the patients with active disease had elevated serum IgG4 concentrations. However, patients with active disease and elevated serum IgG4 concentrations were older, had a higher IgG4\u2010RD RI score, a greater number of organs involved, lower complement levels, higher absolute eosinophil counts, and higher IgE levels compared to those with active disease but normal serum IgG4 concentrations (\n                      <jats:italic>P<\/jats:italic>\n                      &lt; 0.01 for all comparisons). The correlation between IgG4+ plasmablast levels and the IgG4\u2010RD RI of disease activity (Spearman's \u03c1\u2009=\u20090.45,\n                      <jats:italic>P<\/jats:italic>\n                      \u2009=\u20090.003) was stronger than the correlation between total plasmablast levels and the IgG4\u2010RD RI. Seventy\u2010six (61%) of the patients were male, but no significant differences according to sex were observed with regard to disease severity, organ involvement, or serum IgG4 concentrations. Treatment with glucocorticoids failed to produce sustained remission in 77% of patients.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Nearly 50% of this patient cohort with biopsy\u2010proven, clinically active IgG4\u2010RD had normal serum IgG4 concentrations. Elevations in the serum IgG4 concentration appeared to identify a subset of patients with a more severe disease phenotype. In addition, the levels of IgG4+ plasmablasts correlated well with the extent of disease activity.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/art.39205","type":"journal-article","created":{"date-parts":[[2015,5,18]],"date-time":"2015-05-18T15:02:02Z","timestamp":1431961322000},"page":"2466-2475","source":"Crossref","is-referenced-by-count":576,"title":["IgG4\u2010Related Disease: Clinical and Laboratory Features in One Hundred Twenty\u2010Five Patients"],"prefix":"10.1002","volume":"67","author":[{"given":"Zachary S.","family":"Wallace","sequence":"first","affiliation":[{"name":"Massachusetts General Hospital Boston"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vikram","family":"Deshpande","sequence":"additional","affiliation":[{"name":"Massachusetts General Hospital and Harvard Medical School Boston"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hamid","family":"Mattoo","sequence":"additional","affiliation":[{"name":"Massachusetts 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