Intrathoracic Manifestations of IgG4-Related Disease

Abstract

Intrathoracic involvement with

Immunoglobulin G4-Related Disease Mimicking an Anterior Mediastinal Tumor

“…Among its intrathoracic manifestations, there have been several reports of intrapulmonary lesions of IgG4-RD, affecting the lung parenchyma, pleura, mediastinal/ hilar lymph nodes, pericardium, and vasculature, the most common of which are mediastinal and hilar lymphadenopathies [2]. In the previous report of Lin and colleagues [6] on 118 patients with IgG4-RD, 32 cases (27%) showed pulmonary involvement and 4 (3.4%) had mediastinal fibrosis.…”

“…IgG4-RD is a collection of fibroinflammatory and protean disorders with common histologic, serologic, and clinical characteristics. A tumor-like swelling in the affected organ and lymphoplasmacytic infiltration with IgG4positive plasma cells found by microscopy with a distinctive storiform pattern of fibrosis can lead to its diagnosis, and 60% to 70% of patients have also been found to show elevated concentrations of serum IgG4 [1][2][3]. The histopathologic features of this disease are strikingly similar in all of the affected organs, making it somewhat analogous to sarcoidosis, another systemic disease with a consistent histopathologic process among the various organs affected.…”