THU0291 Not Only Organ Involvement, but Also Inflammation Level Is Heterogeneous in IGG4-Related Disease

• Published in: Annals of the rheumatic diseases Vol. 74; no. Suppl 2; p. 301
• Main Authors: Karadag, O., Kalyoncu, U., Ayhan, A.S., Donmez, I., Kilic, L., Akdogan, A., Akdogan, B., Hazirolan, T., Baydar, D.E., Ertenli, I., Kiraz, S.
• Format: Journal Article
• Language: English
• Published: London BMJ Publishing Group LTD 01.06.2015
• ISSN: 0003-4967; 1468-2060
• DOI: 10.1136/annrheumdis-2015-eular.5444

BackgroundIgG4-Related Disease (IgG4-RD) is a recently recognized condition with a wide range of organ involvements.Few data exist on the global perspective of IgG4-RD regarding prevalence and the frequencies of the different clinical presentations.ObjectivesThis study is aimed to describe the clinical and laboratory characteristics of a series of patients diagnosed with IgG4-RD in Turkey.MethodsA retrospective study was performed. Demographic, clinical characteristics and imaging studies including PET-CT, in addition to serum IgG4 levels were recorded. Totally 48 pathologic specimens of the patients were re-evaluated regarding IgG4-RD morphologic findings and IgG4/total Ig G ratio. Patients were diagnosed according to diagnostic criteria for IgG4-RD.1ResultsTotally 27 patients fulfilling the diagnostic criteria were included in the study, 18 of whom (66.7%) were male. Mean age at diagnosis was 55.9±11.8 years.Retroperitoneal fibrosis is the most common finding. Fourteen (51.8%) of the patients had more than one clinical involvement. Thirty percent of patients had lymphadenopathy. Nine (33.3%) of patients had any additional cardiovascular involvement (thoracic aortitis/inflammatory abdominal aneurisms/coronary periarteritis). The global distribution by specific organ involvement was shown in Table. Three of the patients had normal serum IgG4 levels. Six patients had PET-CT. Patients had a wide range of inflammation assessed by PET-CT (SUV max levels were between 2.2-11.4). Twenty of the patients were pathologically proven IgG4-RD -16 of them had morphologic features and all had a ratio of IgG4/IgG ≥40%.Table 1.The global distribution of organ involvement in pateints with IgG4-RDn (%)Lachrymal glands1 (3.7)Orbita and periorbital tissues5 (18.5)Ears, nose and sinuses2 (7.4)Salivary glands2 (7.4)Meninges1 (3.7)Lymphadenopathy8 (29.6)Thyroid1 (3.7)Lungs1 (3.7)Any additional cardiovascular involvement9 (33.3)Aorta7 (25.9)Inflammatory abdominal aneurism2 (7.4)Coronary periarteritis3 (11.1)Retroperitoneal Fibrosis19 (70.4)Pancreas5 (18.5)Biliary ducts2 (7.4)ConclusionsThe most usual presentation of IgG4 related disease in our study is middle aged males and as retroperitoneal fibrosis. Cardiovascular involvement, lymphadenopathy, and orbital/periorbital involvements were the other frequent findings. Not also the organ involvement but also inflammation level could be in a very wide range in IgG4-RD. More collaborative studies are required in order to assess the real prevalence of the disease and the frequencies of the different presentations.ReferencesGuma M, Firestein GS. IgG4 relateddiseases. Best PractClinRheum 2012;26:425-438.Disclosure of InterestNone declared