Ab1504 clinical Spectrum of Igg4-Related Disease. Single University Hospital Experience and Literature Review

Abstract

BackgroundIgG4-related disease (IgG4-RD) is an inflammatory and fibrosing entity with very heterogeneous clinical manifestations. Its pathogenesis remains unknown, clinical features are heterogeneous and unspecific, and recently released classification criteria are invaluable in early recognition of the disease. Therefore, regrettably IgG4 related disease continues underdiagnosed.Objectivesa) To evaluate the clinical characteristics of patients diagnosed with IgG4-RD in a single University hospital;b) To compare with other large series.MethodsStudy of patients from a referral hospital and literature review of cases diagnosed with IgG4-RD. Diagnosis was made accordingly to these criteria: a) Okazaki;b) Umehara;c) ACR/EULAR 2020; and/ord) clinical, laboratory and imaging suggestive findings(ref. 1-3). For the literature review, we searched PubMed and the Cochrane library from its inception until 31 December 2022, selecting the largest series.ResultsWe include 11 patients (7 females/4 males) (mean±SD age; 61.2±15.6 years) diagnosed with IgG4-RD. The organs affected at diagnosis were: aorta (n=5), pleura/lung (n=4), lymph nodes (n=3), salivary glands (n=2), retroperitoneum (n=2), lacrimal glands (n=1), bile duct (n=1), kidney (n=1), orbit (n=1), subglottis (n=1), pericardium (n=1), mesentery (n=1), maxillary sinuses (n=1). IgG4 values were increase in 2 (18%) patients (median [IQR]; 250.5 [201.0-300.1] mg/dL) (normal value <135 mg/dL). Blood plasmablasts were increased in 8 (73%) patients (median; 831 [785-1218] cells/mL) (normal values <653 cells/mL). In the literature review, 6 series of more than 100 patients each were selected. The main data from the different series are listed intable 1.Thefigureshows the most frequently affected organs in the different series. The pancreas was one of the most frequently involved. In contrast, in our series, aortic involvement followed by lung/pleura were the most frequent.ConclusionIgG4-RD is a very heterogeneous disease with involvement of virtually every organ of the anatomy, usually presenting with involvement of more than one organ. Despite the name of the entity, serum IgG4 is not always elevated.References[1]Okazaki K et al. Int J Rheumatol. 2012. PMID: 22690221[2]Umehara H, et al. Mod Rheumatol. 2012. PMID: 21881964[3]Wallace ZS et al. Arthritis Rheumatol. 2020. PMID: 31793250Table 1.Main features in series of more than 100 patients and in current seriesReferenceCasesSex Female (F)/ Male (M)Age, median [IQR] or mean±SDDiagnosis criteriaNumber of organs affectedLevel of serum IgG4 (mg/dL), median [IQR]Lanzillotta et al. Rheumatology 2020131F (n=36),M (n=95)62 [53-70]-Umehara: possible (46%), probable (2%), definitive (52%)-1 organ (26%)-More of 1 organ (74%)224 [115-382]An et al.Orphanet J Rare Dis 2022127F (n=35),M (n=92)63 [55-69]-Umehara: possible (82.6%), probable (2.3%), definitive (11.8%)-1 organ (20%)-2-4 organs (68%)-More of 5 organs (9%)980 [390-1520]Zongfei et al.Clin Rheumatol 2021102F (n=25),M (n=77)62 [54.1-65.8]-Okazaki (100%)Median [IQR]: 2 [1-3]399 [199-776]Wang et al.Arthritis Res Ther 2018215F (n=67),M (n=148)54 [46-62]-Umehara: possible (47.9%), probable (4.7%), definitive (47.4%)-1-2 organs (36%)-3-4 organs (47%)-5 or more organs (17%)896 [350-1860]Inoue et al.Medicine (Baltimore) 2015235F (n=189), M(n=46)67-Symptoms+laboratory +image/ Compatible histology (100%)-1 organ (41%)-2 or more organs (59%)470 [ND]Wallace et al.Arthritis Rheumatol 2015125F (n=49),M (n=76)50.3±14.9- Symptoms+laboratory +image/ Compatible histology (100%)-1 organ (38%)-2 organs (24%)-3 or more organs (38%)NDCurrent series11F (n=7),M (n=4)57 [54.5-73.5]-Okazaki: 45%-Umehara: possible (18%), probable (18%), definitive (9%)-ACR/EULAR 2020 (9%)-Symptoms+laboratory +image/ Compatible histology (54%)-1 organ (36%)-2 organs (27%)-3 or more organs (36%)66.3 [22.2-116.3]Figure 1.Involved organs in series of more than 100 patients and in current series (%)Acknowledgements:NIL.Disclosure of InterestsLara Sanchez-Bilbao: None declared, Javier Loricera Speakers bureau: Roche, Novartis, UCB Pharma, MSD, Celgene, and Grünenthal, Carmen Álvarez-Reguera: None declared, Alba Herrero-Morant: None declared, Ricardo Blanco Speakers bureau: AbbVie, Pfizer, Roche, Bristol-Myers, Lilly, Janssen, and MSD, Grant/research support from: AbbVie, MSD, and Roche.