Significance of high serum IgG4 in complete or non-full-fledged IgG4-related disease—a retrospective investigation of 845 patients and its clinical relevance

Objective Immunoglobulin G4-related disease (IgG4-RD) is a recently recognized heterogeneous, subacute, and usually silent autoimmune disease involving many organs with protean manifestations. However, high IgG4 in serum is not necessarily indicating an IgG4-RD. The aims of this study were to invest...

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Veröffentlicht in:Clinical rheumatology 2022, Vol.41 (1), p.115-122
Hauptverfasser: Tsai, Hung-Cheng, Tung, Hsiang-Yun, Liu, Chih-Wei, Su, Chin-Fang, Sun, Yi-Syuan, Chen, Wei-Sheng, Chen, Ming-Han, Lai, Chien-Chih, Liao, Hsien-Tzung, Yang, Ying-Ying, Huang, Yi-Hsiang, Tsai, Chang-Youh
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Sprache:eng
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Zusammenfassung:Objective Immunoglobulin G4-related disease (IgG4-RD) is a recently recognized heterogeneous, subacute, and usually silent autoimmune disease involving many organs with protean manifestations. However, high IgG4 in serum is not necessarily indicating an IgG4-RD. The aims of this study were to investigate the clinical relevance of high serum IgG4 level in IgG4-RD or non IgG4-RD patients, and to see if IgG4-RD in Taiwan differs from that in other parts of the world. Methods Eight hundred forty-five patients with high IgG4 were retrospectively reviewed from January 2002 to May 2020 in Taipei Veteran General Hospital. Two hundred sixty-seven patients fulfilled IgG4-RD criteria and were categorized into pancreato-hepato-biliary disease, retroperitoneal fibrosis and/or aortitis, head/neck-limited disease, classic Mikulicz syndrome with systemic involvement, CNS-limited disease, sclerosing vasculitis, skin-limited disease, and sensorineural hearing disease. These manifestations were correlated to smoking, atopy, hyper-IgE/eosinophilia, aging, malignancies, and hypocomplementemia. Five hundred seventy-eight patients were not fulfilling the criteria but were also analyzed for the prevalence of allergy, malignancy, connective tissue diseases, lung diseases, and infections. Results In IgG4-RD patients, 124 (46.4%) smoked. Top 4 clinical subtypes included Mikulicz syndrome with systemic involvement (33.3%), pancreato-hepatobiliary disease (31.4%), head/neck disease (19.4%), and retroperitoneal fibrosis/aortitis (12.7%). Top 4 co-morbid conditions included high serum IgE/eosinophilia (46.2%), hypocomplementemia (34%), malignancies (13.4%), and allergy (13.4%). Pancreato-biliary disease was associated with high IgE/eosinophilia ( r 2  = 0.380, P  = 0.025) and malignancy ( r 2  = 0.211, P  = 0.027), Miculicz syndrome with allergy ( r 2  = 0.396, P  
ISSN:0770-3198
1434-9949
DOI:10.1007/s10067-021-05772-x