Rathinam Arun, Gupta Sushan, Khan Mehwish
Internal Medicine, Carle Foundation Hospital, Urbana, USA.
Rheumatology, Carle Foundation Hospital, Urbana, USA.
Cureus. 2023 Jul 12;15(7):e41764. doi: 10.7759/cureus.41764. eCollection 2023 Jul.
Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory condition characterized by tissue infiltration with lymphocytes and IgG4-secreting plasma cells. The presentation of IgG4-RD is heterogenous, making it difficult to diagnose. IgG4-RD presenting as a parapharyngeal mass is extremely rare. This report discusses the case of a 69-year-old African American female presenting with intermittent bilateral frontal headaches. Initial imaging revealed an ill-defined parapharyngeal mass encasing the left internal carotid artery and left internal jugular vein. Subsequent biopsy and immunohistochemistry showed a high concentration of IgG4-positive plasma cells with storiform fibrosis, despite normal serum IgG4 levels. The patient opted for conservative management. The localized parapharyngeal mass has remained stable over two years on annual imaging. This case report highlights that IgG4-RD can have varied and nonspecific presentations requiring high clinical suspicion to diagnose. Histopathology and IgG4 staining are vital to confirm the diagnosis of IgG4-RD, particularly in atypical cases not meeting the standard inclusion criteria.
免疫球蛋白G4相关性疾病（IgG4-RD）是一种纤维炎症性疾病，其特征是淋巴细胞和分泌IgG4的浆细胞浸润组织。IgG4-RD的表现具有异质性，难以诊断。表现为咽旁肿块的IgG4-RD极为罕见。本报告讨论了一例69岁非裔美国女性，她出现间歇性双侧额部头痛。初始影像学检查发现一个边界不清的咽旁肿块，包绕左颈内动脉和左颈内静脉。随后的活检和免疫组织化学显示，尽管血清IgG4水平正常，但有高浓度的IgG4阳性浆细胞伴席纹状纤维化。患者选择保守治疗。在每年的影像学检查中，局部咽旁肿块在两年内一直保持稳定。本病例报告强调，IgG4-RD可能有多种非特异性表现，需要高度的临床怀疑才能诊断。组织病理学和IgG4染色对于确诊IgG4-RD至关重要，特别是在不符合标准纳入标准的非典型病例中。
