{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,26]],"date-time":"2026-05-26T06:04:00Z","timestamp":1779775440694,"version":"3.53.1"},"reference-count":21,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2026,5,25]],"date-time":"2026-05-25T00:00:00Z","timestamp":1779667200000},"content-version":"vor","delay-in-days":24,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T00:00:00Z","timestamp":1777593600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Int J of Rheum Dis"],"published-print":{"date-parts":[[2026,5]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>IgG4\u2010related disease (IgG4\u2010RD) is a rare fibroinflammatory disorder characterized by tumefactive lesions and IgG4+ plasma cell infiltration. While corticosteroids induce remission, high relapse rates necessitate steroid\u2010sparing agents. Although well\u2010studied in East Asia, comprehensive Indian data remain limited.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>This retrospective study (2018\u20132024) analyzed 60 biopsy\u2010evaluated patients diagnosed per the 2020 Revised Comprehensive Diagnostic Criteria. All received glucocorticoid induction; steroid\u2010sparing agents were initiated concurrently in 97%. Treatment response was assessed using the IgG4\u2010RD Responder Index (complete response [CR]: RI &lt;\u20093 with \u2265\u20092\u2010point reduction; partial response [PR]: \u2265\u20092\u2010point reduction with RI \u2265\u20093; relapse: \u2265\u20092\u2010point rise after response).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      The cohort (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200960) was 57% male, mean age of 46.4\u2009\u00b1\u200916.1\u2009years. Most had definite IgG4\u2010RD (63%), and 42% presented with multisystem involvement. Distinct from East Asian cohorts, retroperitoneal fibrosis (32%) and lung involvement (28%) predominated, with pancreatic (5% vs. 60% in Japan) and salivary gland disease (25% vs. 72%) were rare. Histopathology revealed universal lymphoplasmacytic infiltrates (100%) and fibrosis (93%), but infrequent storiform fibrosis (7%) and obliterative phlebitis (12%). Treatment yielded a 90% response rate at 6\u2009months (28% CR, 62% PR), improving to 52% CR at 2\u2009years. Despite excellent induction, cumulative relapse reached 66% at a median of 13\u2009months follow\u2010up, underscoring the disease's chronic nature. In a small subgroup receiving rituximab plus methotrexate (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20094), 75% achieved CR with no documented relapses at 2\u2009years; larger studies are needed to confirm this signal. One death occurred from urosepsis.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Indian IgG4\u2010RD patients exhibit younger onset, retroperitoneal\u2010lung predominance, and minimal pancreatic involvement. Early combination immunosuppression provides excellent initial responses but cannot prevent the disease's relapsing nature, underscoring the need for long\u2010term maintenance strategies and region\u2010specific management approaches.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/1756-185x.70710","type":"journal-article","created":{"date-parts":[[2026,5,25]],"date-time":"2026-05-25T07:06:19Z","timestamp":1779692779000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Distinct Clinical Phenotypes and Treatment Responses in\n                    <scp>IgG4<\/scp>\n                    \u2010Related Disease: Insights From a Tertiary Care Centre in Western India"],"prefix":"10.1111","volume":"29","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6460-1743","authenticated-orcid":false,"given":"Sandeep","family":"Yadav","sequence":"first","affiliation":[{"name":"Department of Rheumatology PD Hinduja National Hospital and Medical Research Centre  Mumbai Maharashtra India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1875-4068","authenticated-orcid":false,"given":"Girija","family":"Sachdev","sequence":"additional","affiliation":[{"name":"Department of Rheumatology PD Hinduja National Hospital and Medical Research Centre  Mumbai Maharashtra India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2390-5043","authenticated-orcid":false,"given":"C.","family":"Balakrishnan","sequence":"additional","affiliation":[{"name":"Department of Rheumatology PD Hinduja National Hospital and Medical Research Centre  Mumbai Maharashtra India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8773-9884","authenticated-orcid":false,"given":"Rohini","family":"Samant","sequence":"additional","affiliation":[{"name":"Department of Rheumatology PD Hinduja National Hospital and Medical Research Centre  Mumbai Maharashtra India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0007-3548-3013","authenticated-orcid":false,"given":"Gurmeet","family":"Mangat","sequence":"additional","affiliation":[{"name":"Department of Rheumatology PD Hinduja National Hospital and Medical Research Centre  Mumbai Maharashtra 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