IgG4‐related disease—experience of 100 consecutive cases from a specialist centre

摘要: Aims To describe the features of 100 consecutive cases referred to a single UK institution in which diagnosis IgG4-related disease (IgG4-RD) was under consideration. Methods and results The histological were reviewed by histopathologist, categorized according 2012 Boston criteria: Category 1—histologically highly suggestive IgG4-RD; 2—probable histopathological 3—insufficient evidence IgG4-RD. A ‘global assessment’ performed with available clinical information: Assessment group 1—’definite/very likely IgG4-RD’; 2—’possible 3—’not 4—insufficient information. The mean IgG4+ plasma cell count IgG4+/IgG+ ratio highest 1 [134/high-power field (HPF); 57%] (113/HPF; 52%), lowest 3 (11/HPF; 18%) (43/HPF; 31%) (Category comparison ratio, both P < 0.001; count, 0.0002; = 0.04). non-IgG4-RD rare (7%) but common 2 (60%) (47%). Stromal reactions neoplasia chronic oral ulceration simulants IgG4-RD. Conclusions The criteria are linked likelihood Other conditions may show some IgG4-RD is much greater when reach threshold for than they thresholds Categories 3. Despite utility criteria, this study highlights crucial importance careful clinicopathological correlation consideration.