Introduction/ObjectivesImmunoglobulin G4–related disease (IgG4-RD) is a heterogeneous fibroinflammatory condition. The 2019 American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) Classification Criteria for IgG4-RD were published to provide unified classification criteria in clinical research. The purpose of this study was to characterize demographics, disease manifestations, and treatments of patients with IgG4-RD and assess performance of the Classification Criteria in a heterogeneous cohort with a large population of Black patients.MethodsThis was a medical records review of all patients referred to a specialized IgG4-RD clinic. Demographics, serology, histopathology, disease manifestations, and treatment information were collected and analyzed. An ACR/EULAR Classification Criteria score for IgG4-RD was calculated to compare performance in definite diagnosis, probable diagnosis, and mimicker groups.ResultsA total of 198 patients were evaluated. Eighty-five (43%) were mimickers. Of the remaining 114, 58 (51%) were classified as definite, and 56 (49%) as probable cases by treating clinicians. Pancreatitis was the most common presentation (37%) among 28 different organ manifestations of IgG4-RD. In patients with definite clinical diagnosis of IgG4-RD, 84% met the IgG4-RD Classification Criteria (i.e., score ≥20) with mean score of 29. Only 9% of the probable cases met this threshold with an average score of 8. None of the mimickers met the Classification Criteria.ConclusionsThis study highlights the broad spectrum of IgG4-RD and validates the use of the ACR/EULAR Classification Criteria for IgG4-RD, including a large proportion of Black patients. As shown in this study, IgG4-RD is a heterogeneous disease and continues to represent a diagnostic challenge to clinicians.

In this single-center medical record review, almost half of the patients referred for diagnosis of IgG4-RD were found to have a mimic of the disease.• Based on specific cases and findings in our cohort, it is important to note that elevated serum IgG4 levels in isolation are not diagnostic for the disease. • A repeat tissue biopsy should be done in the majority of cases with atypical clinical presentation and those that do not respond to glucocorticoids.