IgG4-related Disease (IgG4-RD):One Case Report And Review Of The Literature

BackgroundIgG4-related disease (IgG4-RD) is a recently recognized chronic immune-mediated systemic fibro-inflammatory disorder.It is characterized by elevated serum IgG4 and mass-like tissue infiltration by IgG4-positive plasm cells. Up-regulated responses of T helper 2 and T regulatory cells and their cytokines play a major role in the progression. Most of the IgG4-RD patients are middle-aged male people. Type 1 autoimmune pancreatitis (AIP) is the most frequent solitary manifestation. There are also patients with IgG4-RD presented with sialadenitis, tubulointerstitial nephritis, dacryoadenitis, periaortitis and so on. However,up to now there is no case presenting as a solitary brain mass.ObjectivesTo improve the knowledge of IgG4-RD in its clinical manifestations,imaging characteristics,diagnosis,differential diagnosis and treatment methods.Materials and MethodsOne case diagnosed as IgG4-RD in Shandong provincial hospital was reviewed and analyzed. After preparation of her clinical materials and analysis of large number of literature data,we summarize the epidemiology,pathogenisis,clinical characteristics, imaging features, diagnosis,differential diagnosis and treatment methods.ResultsInformation of the patient:female,45 year-old. She was admitted to the hospital because of trauma.The spiral CT and MRI images demonstrated a mass in her cavum veluminterpositum.There was no positive signs in her physical examination. Gamma(IgA+IgG+IgM+CRP)=22.3%(9.0-16.0%). Operative findings:the mass is in the cavum veluminterpositum.It is red-yellow,hard-texture,moderate-blood supply and 4.0 cm x 3.5 cm x 3.2 cm in size. Pathological examination:CD38+、 CD138+、IgG4+>101个/HPF、IgG4/IgG>40%、κ+、λ+、Ki-67<5%、EMA-、 CK-、PR-、S-100-、CD68 (+)、CD34-、CDla、CD21 FDC+、LCA+、CD20 B cell (+)、D3 T cell (+).Pathological conclusion:IgG4-RD in the cavum veluminterpositum.ConclusionsThe imaging findings of IgG4-RD is non-specific. Diagnosis of IgG4-RD is challenging as there is no laboratory, imaging, or histopathological finding that would be exclusive for IgG-RD. We must integrate all the information. Especially, the histopathological findings are considered highly significant.Nowadays glucocorticoids is considered the first-line therapy in IgG4-RD.Definite diagnosis can avoid unessential surgical treatments.