Igg4-Related Disease, a Systemic or Local Disease: This Is the Problem Publisher



Soleimani V¹ ; Miri R² ; Aminishakib P³ ; Hamedi R¹ Show Affiliations
Source: Archives of Breast Cancer Published:2023

Abstract

Background: Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory condition that forms tumefactive lesions in various organs, presenting with diverse clinical manifestations. Diagnosis of IgG4-RD could be challenging and relies on characteristic histopathological findings and elevated IgG4+ plasma cell counts. Case Presentation: The patient was a 48-year-old woman with diabetes mellitus initially presented with a painless breast mass. Imaging indicated irregular opacities and lymph nodes in the breast. Biopsy results revealed lymphoplasmacytic infiltration with IgG and IgG4 positivity, overall compatible with IG4-related disease. A mass resection was performed, and intraoperative assessment ruled out malignancy. Then, with a 5-month interval of no symptoms, the patient developed a cheek mass, initially misdiagnosed as low-grade spindle cell sarcoma. Subsequently, she underwent partial maxillectomy, further examination, and histology confirmed IgG4-RD, meeting all diagnostic criteria. Conclusion: This case underscores the diagnostic challenges posed by IgG4-RD, often mistaken for malignancies due to its invasive behavior and emphasizes the importance of considering IgG4-RD in differential diagnoses for tumor-like lesions, particularly when affecting multiple organs, to enable timely treatment decisions and prevent unnecessary interventions. © 2023 The Author(s).