A 48-year-old man was admitted for evaluation of abnormal shadows on chest radiograph. Chest computed tomography (CT) showed cysts, nodules, and cervical and axillary lymphadenopathies. Elevated serum levels of IgG4 and interleukin (IL)-6 suggested IgG4-related disease (IgG4-RD) or multicentric Castleman's disease (MCD). Histologic findings of the cervical lymph node and right lung S6 biopsies ...

A 68-year-old man was admitted because of a pulsatile mass and pain in the left temporal region, and computed tomography demonstrated the superficial temporal artery aneurysm. He underwent aneurysmectomy, and pathologic investigation revealed marked thickness of the adventitia with substantial plasmacyte infiltration. On immunoglobulin G4 (IgG4) immunohistochemistry, IgG4-positive lymphocytes w...

IgG4 aortitis is one of the entities seen in the spectrum of IgG4-related disease (IgG4-RD). It is characterized by serologic (elevated serum IgG4) and histologic features including a lymphoplasmacytic infiltrate with increased numbers of IgG4-positive plasma cells, storiform fibrosis and obliterative phlebitis. Some studies have described a correlation between infections and IgG4 aortitis. We ...

PURPOSE OF THE REVIEW Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibroinflammatory disorder affecting nearly all organs, including the kidney. Tubulointerstitial nephritis (IgG4-TIN) is the most common form of IgG4-related kidney disease (IgG4-RKD) and is the focus of this concise review. OBJECTIVE The study aims to describe when IgG4-TIN should be suspected and to summarize th...

OBJECTIVE The prevalence and clinical characteristics of IgG4-related hypophysitis remain unclear due to the limited number of case reports. Therefore, in this study, we screened consecutive outpatients with hypopituitarism and/or diabetes insipidus (DI) to estimate its prevalence. METHODS A total of 170 consecutive outpatients with hypopituitarism and/or central DI were screened at Kobe Univ...

Immunoglobulin G4-related disease (IgG4-RD) is a recently recognized new disease entity characterized by elevated serum IgG4 and infiltration of IgG4 plasma cells in affected tissues. Histological examination is essential for definitive diagnosis, as other pathological conditions can also present with serum IgG4 elevation. However, IgG4-RD frequently involves vital or internal organs that are d...

Dear Sir, We read with great interest the paper of Gupta et al. concerning a case of hypocomplementaemic tubulointerstitial nephritis (TIN) [1]. Hypocomplementaemic immune complex TIN in the absence of autoimmune disorders is a rare condition, and only a limited number of cases have been reported, of which most have been labelled idiopathic [2]. However, in recent years, several case reports an...

The IgG4-RD is characterized by pseudotumoral inflammatory lesions caused by lymphoplasmocytic infiltration of IgG4+ cells and elevated serum IgG4. For decades, Mikulicz’s disease and Sjögren’s syndrome were considered identical conditions.1–5 IgG1 immunoglobulin is most prevalent (>50%) and the IgG4 variety constitutes less than 5%. IgG4 has disulfide bonds linking the heavy chains unsteadily,...

IgG4-related disease (IgG4-RD) is a recently recognized group of conditions, characterized by tumor-like swelling of involved organs, lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, variable degrees of fibrosis, and elevated serum IgG4 concentrations. Currently IgG4-RD is recognized as a systemic condition that can affect several organs and tissues. Herein we report the case of...

Background: Increased risk of developing cancer in patients with Immunoglobulin G4-related disease (IgG4-RD) have been reported. Association malignancies and IgG4-related ophthalmic (IgG4-ROD), however, remains unclear. Methods: A retrospective review medical records, orbital images histopathology reports from 2005 to 2019 a territory-wide, biopsy-proven cohort, fulfilling the “probable” or ...