Background IgG4-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.
Objectives This study is aimed to describe the clinical and laboratory characteristics of a series of patients diagnosed with IgG4-RD in Turkey.
Methods A 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.1
Results Totally 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%.
Conclusions The 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.
Guma M, Firestein GS. IgG4 relateddiseases. Best PractClinRheum 2012;26:425-438.
Disclosure of Interest None declared
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