Background Immunoglobulin (Ig) G4-related disease (IgG4-RD) is an increasingly recognized systemic autoimmune disease in the past decade, and encompasses a wide spectrum of disorders previously considered to be unrelated, but with common pathologic, serologic, and clinical features.There have been several hundred literatures on IgG4-RD published in the past few years, however, we still cannot well characterize this complicated disease, including clinical features, treatments as well as mechanisms, which promotesus to further describe this disease.
Objectives To describe the clinical presentations, laboratory features, imaging manifestations, treatments and evolutions in a cohort of 34 Chinese patients with immunoglobulin G-related diseases (IgG4-RD).
Methods A retrospective study was performed in Wuhan Tongji Hospital. Electronically stored records of clinical, laboratory, imaging, and histological features were reviewed. The diagnoses were made according to comprehensive diagnostic criteria for IgG4-RD. Disease activity was assessed by IgG4-RD Responder Index (RI).This study was approved by the Ethical Committee of TongjiHospital (TJ-C20150603). All patients were contacted, and consents were obtained from all the participants.
Results We found that among the whole 34 patients enrolled in this study (median age=51 years, male/female ratio=1.4/1), 65.2% tested patients had a declined HDL level, 82.3% tested patients had an elevated IgG4 level, and lymph node was the most frequently involved organ. 18F-FDG PET/CT had high sensitivity in providing information about the organ distribution of IgG4-RD. The IgG4 level and IgG4-RD RI in the multi-organs affected patients were significantly higher than that in the localized ones. Most patients were prescribed with glucocorticoids (GCs) alone or combined with disease modifying antirheumatic drugs (DMARDs), the majorities responded well, while some relapsed. Patients given rituximab (RTX) obtained good responses.
Conclusions It was concluded that 18F-FDG PET/CT can be an effective tool to detect involved lesions of IgG4-RD. The addition of DMARDs to the initial GCs therapy in the remission induction stage may not bring much benefit. The most appropriate dosage schedule of RTX needs to be further investigated. Patients with younger age, longer duration of disease, higher IgG4 level and IgG4/IgG ratio may more commonly experience recurrence.
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Disclosure of Interest None declared