{"entity": "publication", "iuid": "29ae448c028f473a8898f7c6bb5d022b", "timestamp": "2026-09-23T18:24:34.220Z", "links": {"self": {"href": "https://publications-affiliated.scilifelab.se/publication/29ae448c028f473a8898f7c6bb5d022b.json"}, "display": {"href": "https://publications-affiliated.scilifelab.se/publication/29ae448c028f473a8898f7c6bb5d022b"}}, "title": "Sporadic occurrence of non-diagnosed IgG4-related disease in lymphoma patients with a previous Sj\u00f6gren's syndrome diagnosis.", "authors": [{"family": "Vasaitis", "given": "Lilian", "initials": "L"}, {"family": "Sundstr\u00f6m", "given": "Christer", "initials": "C"}, {"family": "Backlin", "given": "Carin", "initials": "C"}, {"family": "Nordmark", "given": "Gunnel", "initials": "G"}, {"family": "Baecklund", "given": "Eva", "initials": "E"}], "type": "journal article", "published": "2016-05-19", "journal": {"title": "Acta Oncol", "issn": "1651-226X", "volume": "55", "issue": "9-10", "pages": "1139-1144", "issn-l": "0284-186X"}, "abstract": "IgG4-related disease (IgG4-RD) is a recently recognized fibro-inflammatory disorder, which may affect many organs, and often comes to clinical attention due to tumor-like organ swelling or is identified incidentally by specific biopsy findings. Typical histopathology of IgG4-RD is lymphoplasmacytic infiltration rich in IgG4 + plasma cells (PCs), storiform fibrosis, and obliterative phlebitis. Patients with sicca symptoms can be misdiagnosed as primary Sj\u00f6gren's syndrome (pSS) instead of IgG4-RD because of clinical and histopathological similarities. Moreover, an association with lymphoma development is described in both diseases. This study investigated signs of IgG4-RD in a population-based cohort of patients diagnosed with pSS complicated by lymphoma.\n\nPatients with pSS and lymphoma diagnoses and available lymphoma specimens were identified by linkage with the Swedish Patient Register 1964-2007 and the Cancer Register 1990-2007 (n = 79). Clinical data and lymphomas were reviewed and the diagnoses evaluated. All lymphoma tissues and available minor salivary gland biopsies (n = 11) were immunostained for IgG4 + PCs and evaluated for other histopathological signs of IgG4-RD. In a case with specific findings of IgG4-RD, other available tissue specimens of the same patient were investigated for IgG4-RD.\n\nOnly one patient of 79 (1.3%) had >10 IgG4 + PCs/high power field (HPF) in the lymphoma tissue, an unspecified low-grade B-cell lymphoma localized in the submandibular gland. This patient also had other histopathological features of IgG4-RD in the lymphoma and a surgical lung biopsy taken five years before lymphoma diagnosis and, therefore, fulfilled the criteria for IgG4-RD. Occasional IgG4 + PCs (<10/HPF) without signs of IgG4-RD were observed in another six lymphomas. No IgG4 + PCs were identified in the minor salivary gland biopsies.\n\nHistopathological findings of IgG4-RD may co-exist with low malignant B-cell lymphoma in patients with initially suspected pSS and may be associated with an underlying IgG4-RD.", "doi": "10.1080/0284186X.2016.1182644", "pmid": "27196149", "labels": [], "xrefs": [], "notes": [], "created": "2026-09-23T12:56:48.803Z", "modified": "2026-09-23T14:55:01.589Z"}