Original article IgG4-related inflammatory orbital pseudotumors – a retrospective case series

Abstract

Orbital diseases may be divided into congenital defects of the orbit, infectious and inflammatory diseases, orbital tumors (including malignant and benign tumors) and injuries. Idiopathic inflammatory syndromes are often encountered within the orbit and are usually classified as orbital pseudotumors. The etiology of pseudotumors of the vision organ is unknown. Infectious agents, autoimmune disorders and improper healing are taken into consideration in the pathogenesis of this disorder. Thanks to detailed studies conducted in recent years, a new disease syndrome was identified in 2001. It is known as IgG4-related disease, and its differentiation is based on the analysis of IgG4 levels in the affected tissues. Orbital locations of the disease were first reported in Japan as late as at the end of 2009. This finding triggered the European studies on this subject. To date, no such studies have been conducted in Poland. The starting study population consisted of 167 patients with isolated infiltrative tumor diseases within the orbital region treated at the

Canine idiopathic orbital inflammation: A case series of four affected dogs (five orbits)

“…This group of diseases is characterized by widespread tissue infiltration by IgG4‐positive plasma cells and high serum levels of IgG4. Several organs may be affected by a diffuse lymphoplasmacytic infiltrate and an abundance of IgG4 + plasma cells on a background of fibroblasts, including ocular adnexa and orbit 2940 . IgG4‐related diseases have recently raised interest in the veterinary community and have been reported in dogs 26,41 .…”

Relapsing IgG4-related orbital inflammatory pseudotumor after achieving success with rituximab

“…The modulation of B cells and plasma cells offers good therapeutic effects. Currently, a chemotherapeutic agent, like bortezomib, is also in use [24]; its administration concomitant with cyclophosphamide produced the success of efficient depletion of long-lived plasma cells [25].…”

Simultaneous occurrence of pancreatic mixed acinar-ductal adenocarcinoma and primary follicular lymphoma of the duodenum, accompanied by increased number of IgG4 plasma cells in tumor-free parenchyma as concomitant IgG4-related disease or reaction to tumor? A case report

“…The diagnosis of this fibroinflammatory disease with a heterogeneous clinical picture is based on the clinical presentation and typical histological changes, such as lymphoplasmacytic infiltrate, fibrosis (at least focally arranged in a storiform pattern), obliterative phlebitis and an elevated number of IgG4 plasma cells which is defined by the Consensus statement (30 cells per high power field for pancreas biopsy and 50 for resections; the proposed IgG4-to-IgG ratio is more than 40%) [14,15,16,17,18]. IgG4 related disease can affect different organs and usually manifests as a tumorous mass in the affected organ [19]. However, many inflammatory conditions (gastrointestinal, skin) and malignant neoplasms may be accompanied by an increased number of IgG4 positive plasma cells.…”