title: Characterization and Comparison of Patient Subgroups Suspicious for IgG4-Related Disease and Malignant Lymphoma in Patients Followed-up for Sjögren's Syndrome.
abstract: Differential diagnosis of patients with Sjögren's syndrome (SS), IgG4-related disease (IgG4-RD) and SS patients having high risk for lymphoma (LHR) can be challenging. Some patients with IgG4-RD might be misdiagnosed as having SS. There are special symptoms of SS that raise the possibility of IgG4-RD whereas other symptoms identify patients as having LHR. The purpose of this study was to characterize and compare patients with SS, possible IgG4-RD and SS patients with LHR. Sixty-five SS patients were divided into 4 subgroups according to having possible IgG4-RD (n = 15), LHR (n = 16), eligible for both aforementioned groups (n = 20) and not eligible for either group (n = 14), respectively. Four patients fulfilled the diagnostic criteria for IgG4-RD. The serum levels of IgG4 were significantly higher in patients suspicious for IgG4-RD compared to that of LHR patients (0.46 g/l vs. 0.12 g/l, p = 0.032). Shared features of the patient groups (salivary gland swelling (SGS) and lymphadenopathy), were separately analysed: SGS patients had higher IgG4/IgG ratio (p = 0.036), lymphadenopathic patients had higher IgG4 levels (p = 0.042). Some patients may be "hidden" under the diagnosis of SS. Although patients with LHR and patients with possible IgG4-RD share some symptoms, they differ significantly regarding IgG4 levels and IgG4/IgG ratio.
authors.affiliation: Division of Clinical Immunology, Medical Faculty, University of Debrecen, 22 Móricz Zs. Krt, Debrecen, H-4032, Hungary.
authors.forename: Antónia
authors.initials: A
authors.lastname: Szántó
authors.affiliation: Department of Pediatrics, Jósa András County Hospital, 68 Szent István Street, Nyíregyháza, H-4400, Hungary.
authors.forename: Katalin
authors.initials: K
authors.lastname: Szabó
authors.affiliation: Medical Faculty, Institute for Laboratory Medicine, University of Debrecen, 22 Móricz Zs. Krt, Debrecen, H-4032, Hungary.
authors.forename: Gábor
authors.initials: G
authors.lastname: Nagy
authors.affiliation: Medical Faculty, Institute of Pathology, University of Debrecen, 98 Nagyerdei Krt., Debrecen, H-4032, Hungary.
authors.forename: Csaba
authors.initials: C
authors.lastname: Molnár
authors.affiliation: Division of Clinical Immunology, Medical Faculty, University of Debrecen, 22 Móricz Zs. Krt, Debrecen, H-4032, Hungary. zeher@iiibel.dote.hu.
authors.forename: Margit
authors.initials: M
authors.lastname: Zeher
mesh_terms: D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000378:metabolism*; D008223:Lymphoma / Q000175:diagnosis* / Q000378:metabolism*; D008297:Male; D012859:Sjogren's Syndrome / Q000175:diagnosis* / Q000378:metabolism*
chemical_list: D007074:Immunoglobulin G
keywords: IgG4-related disease; Malignant lymphoma; Sjögren’s syndrome