From Suspicion to Diagnosis: Analysis on the Clinical Characteristics of 37 Cases of IgG4-Related Disease (IgG4-RD) in Northeast China

INTRODUCTION: IgG4-related disease (IgG4-RD) mimics a variety of disorders, the final diagnosis is heavily dependent on the doctor’s familiarity with the disease, most patients are expected to get a good prognosis by early diagnosis and timely treatment. METHODS: Retrospective analysis was carried out on 147 patients tested for serum IgG4 because of suspected IgG4-RD. These cases were grouped as the IgG4-RD group and non-IgG4-RD group according to the diagnostic criteria proposed by the Japanese IgG4-RD research group and the American College of Rheumatology and the European Union of Rheumatology. Characteristics of these patients were investigated and analyzed. RESULTS: The onset age of IgG4-RD was 57.29 ± 14.03 years old, male to female ratio of IgG4-RD was 1.31:1. The most commonly affected organs were the pancreas (48.6%), lymph nodes (40.5%) and biliary tract (35.1%), the proportion of patients with simultaneous involvement of multiple organs is as high as 83.2%. A history of allergy is more common in IgG4-RD patients (32.4% vs 14.5%), the optimal critical value of serum IgG4/IgG ratio for diagnosis of IgG4-RD was 0.09 (sensitivity 94.7%, specificity 91.7%) and the optimal threshold for IgG diagnosis of IgG4-RD was 15.25g/L (sensitivity 73.7%, specificity 77.8%) in this study. CONCLUSION: IgG4-RD is often manifested as multiple organ involvement, and is most likely to involve the pancreas, biliary tract and lymph nodes. Most patients were diagnosed in other departments instead of rheumatology. Serum IgG4 level, especially IgG4/IgG ratio has a higher predictive value for IgG4-RD. Early diagnosis is the key point to improve the prognosis.