Immunoglobulin G4-Related Disease Manifesting as Isolated, Typical, and Nontypical Gastroesophageal Lesion: A Research of Literature Review

Abstract

Background: Immunoglobulin G4-related disease (IgG4-RD) is an autoimmune inflammatory and fibrotic condition. The disease is characterized by tissue infiltration with dense lymphoplasmacytes and IgG4-positive plasma cells. Summary: The aim of this study was to provide gastroenterologists with novel insights into evaluating the gastroesophageal involvement with IgG4-RD or mimickers of this condition and to give special attention to clinicopathological features. A literature review was performed using the PubMed database. A total of 39 studies presenting cases in the form of isolated, typical, and nontypical gastroesophageal involvement with IgG4-RD published between 2010 and 2018 were included. These studies were thoroughly reviewed for symptoms, lesion location, lesion type, lesion size, immune-histopathology, associated diseases, treatment, and follow-up. Of the 39 studies reviewed, 9 were esophageal IgG4-RD lesions, isolated esophageal IgG4-RD 66.66% (6/9), a typical form of esophageal IgG4-RD 11.11% (1/9), and nontypical form esophageal IgG4-RD 22.22% (2/9). The 30 gastric IgG4-RD that include isolated gastric IgG4-RD 46.66% (14/30), typical gastric IgG4-RD 40% (12/30), and nontypical gastric IgG4-RD 13.33% (4/30). The majority of lesions were inflammatory tumors, ulceration, nodular lesions, chronic gastritis, and malignant lesions. Key Messages: IgG4-RD may be manifested by isolated, typical and nontypical forms of gastroesophageal lesions and should be taken into consideration in the differential diagnosis. Corticosteroids may be the sole diagnostic treatment for this condition.

An Update on Eosinophilic Esophagitis: Etiological Factors, Coexisting Diseases, and Complications

“…According to the study by Clayton et al [101], esophageal deposits of IgG4 in EoE arise from dense plasmacytic infiltrates in the lamina propria, which shows abundant IgG4-containing plasmacytes, and IgG4 serum levels reactive to particular foods, indicating that EoE in adults is IgG4-associated. Moreover, Zukerberg reported 58 and 40% of cases with EoE and GERD that were positive for mucosal IgG4 plasmacytes, indicating that the presence of intrasquamous IgG4 deposits is a useful adjunctive marker in differentiation between GERD and EoE patients [83, 104, 105].…”

“…These findings indicate the possible associations between EoE and IgG4-related diseases. However, fibrosis is a characteristic feature of both IgG4-related disease and EoE, although the quality and the pattern of fibrosis are generally different: IgG4-related disease is characterized by fibrosis of the storiform type, whereas EoE has a pattern-less fibrosis pattern [2, 104].…”

The eosinophilic esophagitis and IgG4-related disease involving the esophagus

“…IgG4-ассоциированное поражение пищевода. Гистологические признаки IgG4-связанных заболеваний (IgG4-СЗ)плотный лимфоплазмоцитарный инфильтрат; фиброз; более 10 IgG4+ плазматических клеток в поле зрения (¥400); IgG4+/IgG+ соотношение более 40% [12].…”

“…на цветной вклейке) [30][31][32][33][34]36]. Независимо от локализации очаги поражения при IgG4-ассоциированной болезни имеют сходную патоморфологическую картину: муароподобный фиброз, облитерирующий флебит и лимфоплазмоцитарный инфильтрат с высоким содержанием IgG4+ клеток и соотношением IgG4+/IgG+ клеток более 40% [12,35]. При этом до 40% пациентов с IgG4-ассоциированной болезнью могут иметь нормальный уровень IgG4 в сыворотке крови [31,35].…”

“…Пересмотр гистологического материала с иммуногистохимическим исследованием позволил провести дифференциальную диагностику выявленных патологических изменений. Весь клеточный массив представлен CD138+ клетками, экспрессирующими IgG4 и IgG4+/IgG+ соотношение более 40%, что позволило расценить патологический процесс как IgG4-СЗ [12]. В настоящее время в доступной литературе описаны единичные клинические случаи IgG4-связанного поражения пищевода.…”